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A 50-year-old White male presented with a 4- to 5-year history of progressively growing violaceous lesions on his left lower extremity
with scarce T-cells, classically presenting as rapidly progressive, plum-colored lesions on the lower extremities.1,2 CBCLs, with PCDLBCL-LT accounting for 4%, make up the minority of cutaneous lymphomas in the Western world.1-3 The leg type variant, typically demonstrating a female predominance and median age of onset in the 70s, is clinically aggressive and associated with a poorer prognosis, increased recurrence rate, and 40%-60% 5-year survival rate.1-5
Histologically, this variant demonstrates a diffuse sheet-like growth of enlarged atypical B-cells distinctively separated from the epidermis by a prominent grenz zone. Classic PCDLBCL-LT immunophenotype includes B-cell markers CD20 and IgM; triple expressor phenotype indicating c-MYC, BCL-2, and BCL-6 positivity; as well as CD10 negativity, lack of BCL-2 rearrangement, and presence of a positive MYD-88 molecular result.
Other characteristic histopathological findings include positivity for post-germinal markers IRF4/MUM-1 and FOXP-1, positivity for additional B-cell markers, including CD79 and PAX5, and negativity of t(14;18) (q32;21).1,3-5
This case is of significant interest as it falls within the approximately 10% of PCDLBCL-LT cases demonstrating weak to negative MUM-1 staining, in addition to its presentation in a younger male individual.
While MUM-1 positivity is common in this subtype, its presence, or lack thereof, should not be looked at in isolation when evaluating diagnostic criteria, nor has it been shown to have a statistically significant effect on survival rate – in contrast to factors like lesion location on the leg versus non-leg lesions, multiple lesions at diagnosis, and dissemination to other sites.2,6
PCDLBCL-LT can uncommonly present in non-leg locations and only 10% depict associated B-symptoms, such as fatigue, night sweats, weight loss, or lymphadenopathy.2,6 First-line treatment is with the R-CHOP chemotherapy regimen – consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone – although radiotherapy is sometimes considered in patients with a single small lesion.1,2
Because of possible cutaneous involvement beyond the legs, common lack of systemic symptoms, and variable immunophenotypes, this case of MUM-1 negative PCDLBCL-LT highlights the importance of a clinicopathological approach to differentiate the subtypes of CBCLs, allowing for proper and individualized stratification of risk, prognosis, and treatment.
This case was submitted and written by Marlee Hill, BS, Michael Franzetti, MD, Jeffrey McBride, MD, and Allison Hood, MD, of the University of Oklahoma, Oklahoma City. They also provided the photos. Donna Bilu Martin, MD, edited the column.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].
References
1. Willemze R et al. Blood. 2019;133(16):1703-14.
2. Willemze R et al. Blood. 2005;105(10):3768-85.
3. Sukswai N et al. Pathology. 2020;52(1):53-67.
4. Hristov AC. Arch Pathol Lab Med. 2012;136(8):876-81.
5. Sokol L et al. Cancer Control. 2012;19(3):236-44.
6. Grange F et al. Arch Dermatol. 2007;143(9):1144-50.
with scarce T-cells, classically presenting as rapidly progressive, plum-colored lesions on the lower extremities.1,2 CBCLs, with PCDLBCL-LT accounting for 4%, make up the minority of cutaneous lymphomas in the Western world.1-3 The leg type variant, typically demonstrating a female predominance and median age of onset in the 70s, is clinically aggressive and associated with a poorer prognosis, increased recurrence rate, and 40%-60% 5-year survival rate.1-5
Histologically, this variant demonstrates a diffuse sheet-like growth of enlarged atypical B-cells distinctively separated from the epidermis by a prominent grenz zone. Classic PCDLBCL-LT immunophenotype includes B-cell markers CD20 and IgM; triple expressor phenotype indicating c-MYC, BCL-2, and BCL-6 positivity; as well as CD10 negativity, lack of BCL-2 rearrangement, and presence of a positive MYD-88 molecular result.
Other characteristic histopathological findings include positivity for post-germinal markers IRF4/MUM-1 and FOXP-1, positivity for additional B-cell markers, including CD79 and PAX5, and negativity of t(14;18) (q32;21).1,3-5
This case is of significant interest as it falls within the approximately 10% of PCDLBCL-LT cases demonstrating weak to negative MUM-1 staining, in addition to its presentation in a younger male individual.
While MUM-1 positivity is common in this subtype, its presence, or lack thereof, should not be looked at in isolation when evaluating diagnostic criteria, nor has it been shown to have a statistically significant effect on survival rate – in contrast to factors like lesion location on the leg versus non-leg lesions, multiple lesions at diagnosis, and dissemination to other sites.2,6
PCDLBCL-LT can uncommonly present in non-leg locations and only 10% depict associated B-symptoms, such as fatigue, night sweats, weight loss, or lymphadenopathy.2,6 First-line treatment is with the R-CHOP chemotherapy regimen – consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone – although radiotherapy is sometimes considered in patients with a single small lesion.1,2
Because of possible cutaneous involvement beyond the legs, common lack of systemic symptoms, and variable immunophenotypes, this case of MUM-1 negative PCDLBCL-LT highlights the importance of a clinicopathological approach to differentiate the subtypes of CBCLs, allowing for proper and individualized stratification of risk, prognosis, and treatment.
This case was submitted and written by Marlee Hill, BS, Michael Franzetti, MD, Jeffrey McBride, MD, and Allison Hood, MD, of the University of Oklahoma, Oklahoma City. They also provided the photos. Donna Bilu Martin, MD, edited the column.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].
References
1. Willemze R et al. Blood. 2019;133(16):1703-14.
2. Willemze R et al. Blood. 2005;105(10):3768-85.
3. Sukswai N et al. Pathology. 2020;52(1):53-67.
4. Hristov AC. Arch Pathol Lab Med. 2012;136(8):876-81.
5. Sokol L et al. Cancer Control. 2012;19(3):236-44.
6. Grange F et al. Arch Dermatol. 2007;143(9):1144-50.
with scarce T-cells, classically presenting as rapidly progressive, plum-colored lesions on the lower extremities.1,2 CBCLs, with PCDLBCL-LT accounting for 4%, make up the minority of cutaneous lymphomas in the Western world.1-3 The leg type variant, typically demonstrating a female predominance and median age of onset in the 70s, is clinically aggressive and associated with a poorer prognosis, increased recurrence rate, and 40%-60% 5-year survival rate.1-5
Histologically, this variant demonstrates a diffuse sheet-like growth of enlarged atypical B-cells distinctively separated from the epidermis by a prominent grenz zone. Classic PCDLBCL-LT immunophenotype includes B-cell markers CD20 and IgM; triple expressor phenotype indicating c-MYC, BCL-2, and BCL-6 positivity; as well as CD10 negativity, lack of BCL-2 rearrangement, and presence of a positive MYD-88 molecular result.
Other characteristic histopathological findings include positivity for post-germinal markers IRF4/MUM-1 and FOXP-1, positivity for additional B-cell markers, including CD79 and PAX5, and negativity of t(14;18) (q32;21).1,3-5
This case is of significant interest as it falls within the approximately 10% of PCDLBCL-LT cases demonstrating weak to negative MUM-1 staining, in addition to its presentation in a younger male individual.
While MUM-1 positivity is common in this subtype, its presence, or lack thereof, should not be looked at in isolation when evaluating diagnostic criteria, nor has it been shown to have a statistically significant effect on survival rate – in contrast to factors like lesion location on the leg versus non-leg lesions, multiple lesions at diagnosis, and dissemination to other sites.2,6
PCDLBCL-LT can uncommonly present in non-leg locations and only 10% depict associated B-symptoms, such as fatigue, night sweats, weight loss, or lymphadenopathy.2,6 First-line treatment is with the R-CHOP chemotherapy regimen – consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone – although radiotherapy is sometimes considered in patients with a single small lesion.1,2
Because of possible cutaneous involvement beyond the legs, common lack of systemic symptoms, and variable immunophenotypes, this case of MUM-1 negative PCDLBCL-LT highlights the importance of a clinicopathological approach to differentiate the subtypes of CBCLs, allowing for proper and individualized stratification of risk, prognosis, and treatment.
This case was submitted and written by Marlee Hill, BS, Michael Franzetti, MD, Jeffrey McBride, MD, and Allison Hood, MD, of the University of Oklahoma, Oklahoma City. They also provided the photos. Donna Bilu Martin, MD, edited the column.
Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].
References
1. Willemze R et al. Blood. 2019;133(16):1703-14.
2. Willemze R et al. Blood. 2005;105(10):3768-85.
3. Sukswai N et al. Pathology. 2020;52(1):53-67.
4. Hristov AC. Arch Pathol Lab Med. 2012;136(8):876-81.
5. Sokol L et al. Cancer Control. 2012;19(3):236-44.
6. Grange F et al. Arch Dermatol. 2007;143(9):1144-50.
There was no cervical, axillary, or inguinal lymphadenopathy.
New 46-week PsA data released for IL-17A inhibitor izokibep
out to 46 weeks, according to an announcement reporting some of the long-term data by the drug’s developer, Acelyrin.
Izokibep is an antibody mimetic designed to inhibit IL-17A that the company says has “high potency and the potential for robust tissue penetration due to its small molecular size, about one-tenth the size of a monoclonal antibody.”
“Patients want both rapid and meaningful improvement of their symptoms, as well as lasting – and ideally improving – resolution of disease over time. Building on the 16-week data for izokibep reported at EULAR and ACR [American College of Rheumatology] last year, the 46-week data now show not only continued but marked improvements over time in key areas of psoriatic arthritis including joint pain, skin psoriasis, and enthesitis,” Philip J. Mease, MD, director of rheumatology research at the Swedish Medical Center and clinical professor at the University of Washington, both in Seattle, and an investigator in the izokibep PsA program, said in the announcement.
The phase 2 trial tested two doses of izokibep – 40 mg and 80 mg – given by subcutaneous injection every 2 weeks – against placebo in 135 adult patients with active PsA. For inclusion in the trial, patients had to have at least three swollen and at least three tender joints and an inadequate response to prior therapy including NSAIDs, conventional synthetic disease-modifying antirheumatic drugs, or tumor necrosis factor inhibitors. At week 16, the placebo group transitioned to 80 mg izokibep every 2 weeks and the trial treatment period continued for up to 46 weeks.
The trial’s primary endpoint of a 50% or higher level of improvement in ACR response criteria (ACR 50) was achieved by 48% of those on the 40 mg dose at week 16 and by 50% at week 46. For the 80-mg group, this rate rose from 52% to 79%. In the group that went from placebo to 80 mg, the ACR 50 rose from 13% with placebo to 73% with izokibep at week 46.
Resolution of enthesitis, measured by the Leeds Enthesitis Index, among those on the 40 mg dose, was achieved by 63% at week 16 and 83% at week 46, and among those on the 80 mg dose, 88% at week 16 and 89% at week 46. Those on placebo who switched to 80 mg of izokibep at week 16 had an 80% rate of enthesitis resolution at week 46.
Total resolution of skin involvement – 100% clearance of psoriasis based on the Psoriasis Area Severity Index (PASI) – was observed at 46 weeks in 50% of those on 40 mg, 71% of those on 80 mg, and 67% of those on 80 mg after week 16.
In its announcement, Acelyrin did not report withdrawal rates from the study after 16 weeks and through 46 weeks, although the statement said that izokibep “was generally well tolerated through 46 weeks, which is in line with previous trials of izokibep.” The most common adverse event was localized injection site reactions, with the majority graded mild to moderate in severity. They were generally the size of a quarter to half-dollar, and typically presented within the first few injections, after which they declined in incidence. In the trial, a case of vulvar cancer was determined to be potentially drug related, the company said.
Acelyrin is currently conducting a phase 2b/3 trial in PsA evaluating a range of doses, including significantly higher doses than in the phase 2 trial, that the company said “could potentially result in better ACR, PASI, and enthesitis resolution responses.”
The drug has been tested at doses up to 160 mg, in some cases for up to 3 years, in more than 400 patients with psoriasis, spondyloarthritis, noninfective uveitis, and hidradenitis suppurativa.
The full 46-week data from this trial will be presented at a future scientific meeting, according to the company.
out to 46 weeks, according to an announcement reporting some of the long-term data by the drug’s developer, Acelyrin.
Izokibep is an antibody mimetic designed to inhibit IL-17A that the company says has “high potency and the potential for robust tissue penetration due to its small molecular size, about one-tenth the size of a monoclonal antibody.”
“Patients want both rapid and meaningful improvement of their symptoms, as well as lasting – and ideally improving – resolution of disease over time. Building on the 16-week data for izokibep reported at EULAR and ACR [American College of Rheumatology] last year, the 46-week data now show not only continued but marked improvements over time in key areas of psoriatic arthritis including joint pain, skin psoriasis, and enthesitis,” Philip J. Mease, MD, director of rheumatology research at the Swedish Medical Center and clinical professor at the University of Washington, both in Seattle, and an investigator in the izokibep PsA program, said in the announcement.
The phase 2 trial tested two doses of izokibep – 40 mg and 80 mg – given by subcutaneous injection every 2 weeks – against placebo in 135 adult patients with active PsA. For inclusion in the trial, patients had to have at least three swollen and at least three tender joints and an inadequate response to prior therapy including NSAIDs, conventional synthetic disease-modifying antirheumatic drugs, or tumor necrosis factor inhibitors. At week 16, the placebo group transitioned to 80 mg izokibep every 2 weeks and the trial treatment period continued for up to 46 weeks.
The trial’s primary endpoint of a 50% or higher level of improvement in ACR response criteria (ACR 50) was achieved by 48% of those on the 40 mg dose at week 16 and by 50% at week 46. For the 80-mg group, this rate rose from 52% to 79%. In the group that went from placebo to 80 mg, the ACR 50 rose from 13% with placebo to 73% with izokibep at week 46.
Resolution of enthesitis, measured by the Leeds Enthesitis Index, among those on the 40 mg dose, was achieved by 63% at week 16 and 83% at week 46, and among those on the 80 mg dose, 88% at week 16 and 89% at week 46. Those on placebo who switched to 80 mg of izokibep at week 16 had an 80% rate of enthesitis resolution at week 46.
Total resolution of skin involvement – 100% clearance of psoriasis based on the Psoriasis Area Severity Index (PASI) – was observed at 46 weeks in 50% of those on 40 mg, 71% of those on 80 mg, and 67% of those on 80 mg after week 16.
In its announcement, Acelyrin did not report withdrawal rates from the study after 16 weeks and through 46 weeks, although the statement said that izokibep “was generally well tolerated through 46 weeks, which is in line with previous trials of izokibep.” The most common adverse event was localized injection site reactions, with the majority graded mild to moderate in severity. They were generally the size of a quarter to half-dollar, and typically presented within the first few injections, after which they declined in incidence. In the trial, a case of vulvar cancer was determined to be potentially drug related, the company said.
Acelyrin is currently conducting a phase 2b/3 trial in PsA evaluating a range of doses, including significantly higher doses than in the phase 2 trial, that the company said “could potentially result in better ACR, PASI, and enthesitis resolution responses.”
The drug has been tested at doses up to 160 mg, in some cases for up to 3 years, in more than 400 patients with psoriasis, spondyloarthritis, noninfective uveitis, and hidradenitis suppurativa.
The full 46-week data from this trial will be presented at a future scientific meeting, according to the company.
out to 46 weeks, according to an announcement reporting some of the long-term data by the drug’s developer, Acelyrin.
Izokibep is an antibody mimetic designed to inhibit IL-17A that the company says has “high potency and the potential for robust tissue penetration due to its small molecular size, about one-tenth the size of a monoclonal antibody.”
“Patients want both rapid and meaningful improvement of their symptoms, as well as lasting – and ideally improving – resolution of disease over time. Building on the 16-week data for izokibep reported at EULAR and ACR [American College of Rheumatology] last year, the 46-week data now show not only continued but marked improvements over time in key areas of psoriatic arthritis including joint pain, skin psoriasis, and enthesitis,” Philip J. Mease, MD, director of rheumatology research at the Swedish Medical Center and clinical professor at the University of Washington, both in Seattle, and an investigator in the izokibep PsA program, said in the announcement.
The phase 2 trial tested two doses of izokibep – 40 mg and 80 mg – given by subcutaneous injection every 2 weeks – against placebo in 135 adult patients with active PsA. For inclusion in the trial, patients had to have at least three swollen and at least three tender joints and an inadequate response to prior therapy including NSAIDs, conventional synthetic disease-modifying antirheumatic drugs, or tumor necrosis factor inhibitors. At week 16, the placebo group transitioned to 80 mg izokibep every 2 weeks and the trial treatment period continued for up to 46 weeks.
The trial’s primary endpoint of a 50% or higher level of improvement in ACR response criteria (ACR 50) was achieved by 48% of those on the 40 mg dose at week 16 and by 50% at week 46. For the 80-mg group, this rate rose from 52% to 79%. In the group that went from placebo to 80 mg, the ACR 50 rose from 13% with placebo to 73% with izokibep at week 46.
Resolution of enthesitis, measured by the Leeds Enthesitis Index, among those on the 40 mg dose, was achieved by 63% at week 16 and 83% at week 46, and among those on the 80 mg dose, 88% at week 16 and 89% at week 46. Those on placebo who switched to 80 mg of izokibep at week 16 had an 80% rate of enthesitis resolution at week 46.
Total resolution of skin involvement – 100% clearance of psoriasis based on the Psoriasis Area Severity Index (PASI) – was observed at 46 weeks in 50% of those on 40 mg, 71% of those on 80 mg, and 67% of those on 80 mg after week 16.
In its announcement, Acelyrin did not report withdrawal rates from the study after 16 weeks and through 46 weeks, although the statement said that izokibep “was generally well tolerated through 46 weeks, which is in line with previous trials of izokibep.” The most common adverse event was localized injection site reactions, with the majority graded mild to moderate in severity. They were generally the size of a quarter to half-dollar, and typically presented within the first few injections, after which they declined in incidence. In the trial, a case of vulvar cancer was determined to be potentially drug related, the company said.
Acelyrin is currently conducting a phase 2b/3 trial in PsA evaluating a range of doses, including significantly higher doses than in the phase 2 trial, that the company said “could potentially result in better ACR, PASI, and enthesitis resolution responses.”
The drug has been tested at doses up to 160 mg, in some cases for up to 3 years, in more than 400 patients with psoriasis, spondyloarthritis, noninfective uveitis, and hidradenitis suppurativa.
The full 46-week data from this trial will be presented at a future scientific meeting, according to the company.
AHA statement targets nuance in CVD risk assessment of women
In a new scientific statement, the American Heart Association highlighted the importance of incorporating nonbiological risk factors and social determinants of health in cardiovascular disease (CVD) risk assessment for women, particularly women from different racial and ethnic backgrounds.
CVD risk assessment in women is multifaceted and goes well beyond traditional risk factors to include sex-specific biological risk factors, as well as social, behavioral, and environmental factors, the writing group noted.
They said a greater focus on addressing all CVD risk factors among women from underrepresented races and ethnicities is warranted to avert future CVD.
The scientific statement was published online in Circulation.
Look beyond traditional risk factors
“Risk assessment is the first step in preventing heart disease, yet there are many limitations to traditional risk factors and their ability to comprehensively estimate a woman’s risk for cardiovascular disease,” Jennifer H. Mieres, MD, vice chair of the writing group and professor of cardiology at Hofstra University, Hempstead, N.Y., said in a news release.
“The delivery of equitable cardiovascular health care for women depends on improving the knowledge and awareness of all members of the healthcare team about the full spectrum of cardiovascular risk factors for women, including female-specific and female-predominant risk factors,” Dr. Mieres added.
Female-specific factors that should be included in CVD risk assessment include pregnancy-related conditions such as preeclampsia, preterm delivery, and gestational diabetes, the writing group said.
Other factors include menstrual cycle history; types of birth control and/or hormone replacement therapy used; polycystic ovarian syndrome (PCOS), which affects 10% of women of reproductive age and is associated with increased CVD risk; and autoimmune disorders, depression, and PTSD, all of which are more common in women and are also associated with higher risk for CVD.
The statement also highlights the key role that social determinants of health (SDOH) play in the development of CVD in women, particularly women from diverse racial and ethnic backgrounds. SDOH include education level, economic stability, neighborhood safety, working conditions, environmental hazards, and access to quality health care.
“It is critical that risk assessment be expanded to include [SDOH] as risk factors if we are to improve health outcomes in all women,” Laxmi Mehta, MD, chair of the writing group and director of preventative cardiology and women’s cardiovascular health at Ohio State University Wexner Medical Center, Columbus, said in the news release.
“It is also important for the health care team to consider [SDOH] when working with women on shared decisions about cardiovascular disease prevention and treatment,” Dr. Mehta noted.
No one-size-fits-all approach
The statement highlighted significant differences in CVD risk among women of different racial and ethnic backgrounds and provides detailed CV risk factor profiles for non-Hispanic Black, Hispanic/Latinx, Asian and American Indian/Alaska Native women.
It noted that language barriers, discrimination, acculturation, and health care access disproportionately affect women of underrepresented racial and ethnic groups. These factors result in a higher prevalence of CVD and significant challenges in CVD diagnosis and treatment.
“When customizing CVD prevention and treatment strategies to improve cardiovascular health for women, a one-size-fits-all approach is unlikely to be successful,” Dr. Mieres said.
“We must be cognizant of the complex interplay of sex, race and ethnicity, as well as social determinants of health, and how they impact the risk of cardiovascular disease and adverse outcomes in order to avert future CVD morbidity and mortality,” Dr. Mieres added.
Looking ahead, the writing group said future CVD prevention guidelines could be strengthened by including culturally-specific lifestyle recommendations.
They also said community-based approaches, faith-based community partnerships, and peer support to encourage a healthy lifestyle could play a key role in preventing CVD among all women.
This scientific statement was prepared by the volunteer writing group on behalf of the AHA’s Cardiovascular Disease and Stroke in Women and Underrepresented Populations Committee of the Council on Clinical Cardiology, the Council on Cardiovascular and Stroke Nursing, the Council on Hypertension, the Council on Lifelong Congenital Heart Disease and Heart Health in the Young, the Council on Lifestyle and Cardiometabolic Health, the Council on Peripheral Vascular Disease, and the Stroke Council.
A version of this article first appeared on Medscape.com.
In a new scientific statement, the American Heart Association highlighted the importance of incorporating nonbiological risk factors and social determinants of health in cardiovascular disease (CVD) risk assessment for women, particularly women from different racial and ethnic backgrounds.
CVD risk assessment in women is multifaceted and goes well beyond traditional risk factors to include sex-specific biological risk factors, as well as social, behavioral, and environmental factors, the writing group noted.
They said a greater focus on addressing all CVD risk factors among women from underrepresented races and ethnicities is warranted to avert future CVD.
The scientific statement was published online in Circulation.
Look beyond traditional risk factors
“Risk assessment is the first step in preventing heart disease, yet there are many limitations to traditional risk factors and their ability to comprehensively estimate a woman’s risk for cardiovascular disease,” Jennifer H. Mieres, MD, vice chair of the writing group and professor of cardiology at Hofstra University, Hempstead, N.Y., said in a news release.
“The delivery of equitable cardiovascular health care for women depends on improving the knowledge and awareness of all members of the healthcare team about the full spectrum of cardiovascular risk factors for women, including female-specific and female-predominant risk factors,” Dr. Mieres added.
Female-specific factors that should be included in CVD risk assessment include pregnancy-related conditions such as preeclampsia, preterm delivery, and gestational diabetes, the writing group said.
Other factors include menstrual cycle history; types of birth control and/or hormone replacement therapy used; polycystic ovarian syndrome (PCOS), which affects 10% of women of reproductive age and is associated with increased CVD risk; and autoimmune disorders, depression, and PTSD, all of which are more common in women and are also associated with higher risk for CVD.
The statement also highlights the key role that social determinants of health (SDOH) play in the development of CVD in women, particularly women from diverse racial and ethnic backgrounds. SDOH include education level, economic stability, neighborhood safety, working conditions, environmental hazards, and access to quality health care.
“It is critical that risk assessment be expanded to include [SDOH] as risk factors if we are to improve health outcomes in all women,” Laxmi Mehta, MD, chair of the writing group and director of preventative cardiology and women’s cardiovascular health at Ohio State University Wexner Medical Center, Columbus, said in the news release.
“It is also important for the health care team to consider [SDOH] when working with women on shared decisions about cardiovascular disease prevention and treatment,” Dr. Mehta noted.
No one-size-fits-all approach
The statement highlighted significant differences in CVD risk among women of different racial and ethnic backgrounds and provides detailed CV risk factor profiles for non-Hispanic Black, Hispanic/Latinx, Asian and American Indian/Alaska Native women.
It noted that language barriers, discrimination, acculturation, and health care access disproportionately affect women of underrepresented racial and ethnic groups. These factors result in a higher prevalence of CVD and significant challenges in CVD diagnosis and treatment.
“When customizing CVD prevention and treatment strategies to improve cardiovascular health for women, a one-size-fits-all approach is unlikely to be successful,” Dr. Mieres said.
“We must be cognizant of the complex interplay of sex, race and ethnicity, as well as social determinants of health, and how they impact the risk of cardiovascular disease and adverse outcomes in order to avert future CVD morbidity and mortality,” Dr. Mieres added.
Looking ahead, the writing group said future CVD prevention guidelines could be strengthened by including culturally-specific lifestyle recommendations.
They also said community-based approaches, faith-based community partnerships, and peer support to encourage a healthy lifestyle could play a key role in preventing CVD among all women.
This scientific statement was prepared by the volunteer writing group on behalf of the AHA’s Cardiovascular Disease and Stroke in Women and Underrepresented Populations Committee of the Council on Clinical Cardiology, the Council on Cardiovascular and Stroke Nursing, the Council on Hypertension, the Council on Lifelong Congenital Heart Disease and Heart Health in the Young, the Council on Lifestyle and Cardiometabolic Health, the Council on Peripheral Vascular Disease, and the Stroke Council.
A version of this article first appeared on Medscape.com.
In a new scientific statement, the American Heart Association highlighted the importance of incorporating nonbiological risk factors and social determinants of health in cardiovascular disease (CVD) risk assessment for women, particularly women from different racial and ethnic backgrounds.
CVD risk assessment in women is multifaceted and goes well beyond traditional risk factors to include sex-specific biological risk factors, as well as social, behavioral, and environmental factors, the writing group noted.
They said a greater focus on addressing all CVD risk factors among women from underrepresented races and ethnicities is warranted to avert future CVD.
The scientific statement was published online in Circulation.
Look beyond traditional risk factors
“Risk assessment is the first step in preventing heart disease, yet there are many limitations to traditional risk factors and their ability to comprehensively estimate a woman’s risk for cardiovascular disease,” Jennifer H. Mieres, MD, vice chair of the writing group and professor of cardiology at Hofstra University, Hempstead, N.Y., said in a news release.
“The delivery of equitable cardiovascular health care for women depends on improving the knowledge and awareness of all members of the healthcare team about the full spectrum of cardiovascular risk factors for women, including female-specific and female-predominant risk factors,” Dr. Mieres added.
Female-specific factors that should be included in CVD risk assessment include pregnancy-related conditions such as preeclampsia, preterm delivery, and gestational diabetes, the writing group said.
Other factors include menstrual cycle history; types of birth control and/or hormone replacement therapy used; polycystic ovarian syndrome (PCOS), which affects 10% of women of reproductive age and is associated with increased CVD risk; and autoimmune disorders, depression, and PTSD, all of which are more common in women and are also associated with higher risk for CVD.
The statement also highlights the key role that social determinants of health (SDOH) play in the development of CVD in women, particularly women from diverse racial and ethnic backgrounds. SDOH include education level, economic stability, neighborhood safety, working conditions, environmental hazards, and access to quality health care.
“It is critical that risk assessment be expanded to include [SDOH] as risk factors if we are to improve health outcomes in all women,” Laxmi Mehta, MD, chair of the writing group and director of preventative cardiology and women’s cardiovascular health at Ohio State University Wexner Medical Center, Columbus, said in the news release.
“It is also important for the health care team to consider [SDOH] when working with women on shared decisions about cardiovascular disease prevention and treatment,” Dr. Mehta noted.
No one-size-fits-all approach
The statement highlighted significant differences in CVD risk among women of different racial and ethnic backgrounds and provides detailed CV risk factor profiles for non-Hispanic Black, Hispanic/Latinx, Asian and American Indian/Alaska Native women.
It noted that language barriers, discrimination, acculturation, and health care access disproportionately affect women of underrepresented racial and ethnic groups. These factors result in a higher prevalence of CVD and significant challenges in CVD diagnosis and treatment.
“When customizing CVD prevention and treatment strategies to improve cardiovascular health for women, a one-size-fits-all approach is unlikely to be successful,” Dr. Mieres said.
“We must be cognizant of the complex interplay of sex, race and ethnicity, as well as social determinants of health, and how they impact the risk of cardiovascular disease and adverse outcomes in order to avert future CVD morbidity and mortality,” Dr. Mieres added.
Looking ahead, the writing group said future CVD prevention guidelines could be strengthened by including culturally-specific lifestyle recommendations.
They also said community-based approaches, faith-based community partnerships, and peer support to encourage a healthy lifestyle could play a key role in preventing CVD among all women.
This scientific statement was prepared by the volunteer writing group on behalf of the AHA’s Cardiovascular Disease and Stroke in Women and Underrepresented Populations Committee of the Council on Clinical Cardiology, the Council on Cardiovascular and Stroke Nursing, the Council on Hypertension, the Council on Lifelong Congenital Heart Disease and Heart Health in the Young, the Council on Lifestyle and Cardiometabolic Health, the Council on Peripheral Vascular Disease, and the Stroke Council.
A version of this article first appeared on Medscape.com.
FROM CIRCULATION
New ASE guideline on interventional echocardiography training
The American Society of Echocardiography (ASE) has issued guidance on all critical aspects of training for cardiology and anesthesiology trainees and postgraduate echocardiographers who plan to specialize in interventional echocardiography (IE).
The guideline outlines requirements of the training institution, the duration and core competencies of training, minimal procedural volume for competency in IE, and knowledge of specific structural health disease (SHD) procedures.
The 16-page guideline was published online in the Journal of the American Society of Echocardiography.
Specific skill set
IE is the primary imaging modality used to support and guide SHD interventions, such as heart valve replacements and other cardiac catheterization procedures, the writing group notes.
They say the “emerging specialty” of IE requires a specific set of skills to support an array of transcatheter therapies, with successful outcomes highly dependent on the skill of the echocardiography team.
“IE techniques are unique since imaging is performed in real-time, it is highly dependent on 3D and non-standard views, and it has immediate and profound implications for patient management,” Stephen H. Little, MD, ASE president and co-chair of the guideline writing group, says in a news release.
“Additionally, IE requires candid, accurate, and timely communication with other members of the multidisciplinary SHD team,” Dr. Little adds.
The new ASE guideline expands on the 2019 statement on echocardiography training put forward by the American College of Cardiology, American Heart Association, and ASE, by focusing specifically on interventional echocardiographers.
It outlines core competencies common to all transcatheter therapies, as well as specific transcatheter procedures. It provides consensus recommendations for specific knowledge, experience, and skills to be learned and demonstrated within an IE training program or during postgraduate training.
A “core principle” in the guideline states that the length of IE training or achieved number of procedures performed are less important than the demonstration of procedure-specific competencies within the milestone domains of knowledge, skill, and communication.
“Transcatheter therapies for SHD continue to grow at a rapid pace, which means that the demand for skilled interventional echocardiographers has steadily increased,” Vera H. Rigolin, MD, co-chair of the guideline writing, says in the release.
“Training standards are needed to ensure that interventional echocardiographers have the necessary expertise to provide fast, accurate, and high-quality image acquisition and interpretation in real-time,” Dr. Rigolin adds.
In addition, the guidelines states that use of simulation training has a role in IE training.
Virtual and simulation training could shorten the learning curve for trainees and, when combined with remote learning, could permit societies to standardize a teaching curriculum and allow the trainee to complete training in a reasonable timeframe. Simulator training may also improve access to training and thus promote diversity and inclusivity, the writing group says.
The guideline has been endorsed by 21 ASE international partners.
Writing group co-chairs Little and Rigolin have declared no conflicts of interest. A complete list of disclosures for the writing group is available with the original article.
A version of this article first appeared on Medscape.com.
The American Society of Echocardiography (ASE) has issued guidance on all critical aspects of training for cardiology and anesthesiology trainees and postgraduate echocardiographers who plan to specialize in interventional echocardiography (IE).
The guideline outlines requirements of the training institution, the duration and core competencies of training, minimal procedural volume for competency in IE, and knowledge of specific structural health disease (SHD) procedures.
The 16-page guideline was published online in the Journal of the American Society of Echocardiography.
Specific skill set
IE is the primary imaging modality used to support and guide SHD interventions, such as heart valve replacements and other cardiac catheterization procedures, the writing group notes.
They say the “emerging specialty” of IE requires a specific set of skills to support an array of transcatheter therapies, with successful outcomes highly dependent on the skill of the echocardiography team.
“IE techniques are unique since imaging is performed in real-time, it is highly dependent on 3D and non-standard views, and it has immediate and profound implications for patient management,” Stephen H. Little, MD, ASE president and co-chair of the guideline writing group, says in a news release.
“Additionally, IE requires candid, accurate, and timely communication with other members of the multidisciplinary SHD team,” Dr. Little adds.
The new ASE guideline expands on the 2019 statement on echocardiography training put forward by the American College of Cardiology, American Heart Association, and ASE, by focusing specifically on interventional echocardiographers.
It outlines core competencies common to all transcatheter therapies, as well as specific transcatheter procedures. It provides consensus recommendations for specific knowledge, experience, and skills to be learned and demonstrated within an IE training program or during postgraduate training.
A “core principle” in the guideline states that the length of IE training or achieved number of procedures performed are less important than the demonstration of procedure-specific competencies within the milestone domains of knowledge, skill, and communication.
“Transcatheter therapies for SHD continue to grow at a rapid pace, which means that the demand for skilled interventional echocardiographers has steadily increased,” Vera H. Rigolin, MD, co-chair of the guideline writing, says in the release.
“Training standards are needed to ensure that interventional echocardiographers have the necessary expertise to provide fast, accurate, and high-quality image acquisition and interpretation in real-time,” Dr. Rigolin adds.
In addition, the guidelines states that use of simulation training has a role in IE training.
Virtual and simulation training could shorten the learning curve for trainees and, when combined with remote learning, could permit societies to standardize a teaching curriculum and allow the trainee to complete training in a reasonable timeframe. Simulator training may also improve access to training and thus promote diversity and inclusivity, the writing group says.
The guideline has been endorsed by 21 ASE international partners.
Writing group co-chairs Little and Rigolin have declared no conflicts of interest. A complete list of disclosures for the writing group is available with the original article.
A version of this article first appeared on Medscape.com.
The American Society of Echocardiography (ASE) has issued guidance on all critical aspects of training for cardiology and anesthesiology trainees and postgraduate echocardiographers who plan to specialize in interventional echocardiography (IE).
The guideline outlines requirements of the training institution, the duration and core competencies of training, minimal procedural volume for competency in IE, and knowledge of specific structural health disease (SHD) procedures.
The 16-page guideline was published online in the Journal of the American Society of Echocardiography.
Specific skill set
IE is the primary imaging modality used to support and guide SHD interventions, such as heart valve replacements and other cardiac catheterization procedures, the writing group notes.
They say the “emerging specialty” of IE requires a specific set of skills to support an array of transcatheter therapies, with successful outcomes highly dependent on the skill of the echocardiography team.
“IE techniques are unique since imaging is performed in real-time, it is highly dependent on 3D and non-standard views, and it has immediate and profound implications for patient management,” Stephen H. Little, MD, ASE president and co-chair of the guideline writing group, says in a news release.
“Additionally, IE requires candid, accurate, and timely communication with other members of the multidisciplinary SHD team,” Dr. Little adds.
The new ASE guideline expands on the 2019 statement on echocardiography training put forward by the American College of Cardiology, American Heart Association, and ASE, by focusing specifically on interventional echocardiographers.
It outlines core competencies common to all transcatheter therapies, as well as specific transcatheter procedures. It provides consensus recommendations for specific knowledge, experience, and skills to be learned and demonstrated within an IE training program or during postgraduate training.
A “core principle” in the guideline states that the length of IE training or achieved number of procedures performed are less important than the demonstration of procedure-specific competencies within the milestone domains of knowledge, skill, and communication.
“Transcatheter therapies for SHD continue to grow at a rapid pace, which means that the demand for skilled interventional echocardiographers has steadily increased,” Vera H. Rigolin, MD, co-chair of the guideline writing, says in the release.
“Training standards are needed to ensure that interventional echocardiographers have the necessary expertise to provide fast, accurate, and high-quality image acquisition and interpretation in real-time,” Dr. Rigolin adds.
In addition, the guidelines states that use of simulation training has a role in IE training.
Virtual and simulation training could shorten the learning curve for trainees and, when combined with remote learning, could permit societies to standardize a teaching curriculum and allow the trainee to complete training in a reasonable timeframe. Simulator training may also improve access to training and thus promote diversity and inclusivity, the writing group says.
The guideline has been endorsed by 21 ASE international partners.
Writing group co-chairs Little and Rigolin have declared no conflicts of interest. A complete list of disclosures for the writing group is available with the original article.
A version of this article first appeared on Medscape.com.
Urban green and blue spaces linked to less psychological distress
The findings of the study, which was released ahead of its scheduled presentation at the annual meeting of the American Academy of Neurology, build on a growing understanding of the relationship between types and qualities of urban environments and dementia risk.
Adithya Vegaraju, a student at Washington State University, Spokane, led the study, which looked at data from the Washington State Behavioral Risk Factor Surveillance System to assess prevalence of serious psychological distress among 42,980 Washington state residents aged 65 and over.
The data, collected between 2011 and 2019, used a self-reported questionnaire to determine serious psychological distress, which is defined as a level of mental distress considered debilitating enough to warrant treatment.
Mr. Vegaraju and his coauthor Solmaz Amiri, DDes, also of Washington State University, used ZIP codes, along with U.S. census data, to approximate the urban adults’ proximity to green and blue spaces.
After controlling for potential confounders of age, sex, ethnicity, education, and marital status, the investigators found that people living within half a mile of green or blue spaces had a 17% lower risk of experiencing serious psychological distress, compared with people living farther from these spaces, the investigators said in a news release.
Implications for cognitive decline and dementia?
Psychological distress in adults has been linked in population-based longitudinal studies to later cognitive decline and dementia. One study in older adults found the risk of dementia to be more than 50% higher among adults aged 50-70 with persistent depression. Blue and green spaces have also been investigated in relation to neurodegenerative disease among older adults; a 2022 study looking at data from some 62 million Medicare beneficiaries found those living in areas with more vegetation saw lower risk of hospitalizations for Alzheimer’s disease and related dementias.
“Since we lack effective prevention methods or treatments for mild cognitive impairment and dementia, we need to get creative in how we look at these issues,” Dr. Amiri commented in a press statement about her and Mr. Vegaraju’s findings. “Our hope is that this study showing better mental health among people living close to parks and water will trigger other studies about how these benefits work and whether this proximity can help prevent or delay mild cognitive impairment and dementia.”
The investigators acknowledged that their findings were limited by reliance on a self-reported measure of psychological distress.
A bidirectional connection with depression and dementia
In a comment, Anjum Hajat, PhD, an epidemiologist at University of Washington School of Public Health in Seattle who has also studied the relationship between green space and dementia risk in older adults, noted some further apparent limitations of the new study, for which only an abstract was available at publication.
“It has been shown that people with depression are at higher risk for dementia, but the opposite is also true,” Dr. Hajat commented. “Those with dementia are more likely to develop depression. This bidirectionality makes this study abstract difficult to interpret since the study is based on cross-sectional data: Individuals are not followed over time to see which develops first, dementia or depression.”
Additionally, Dr. Hajat noted, the data used to determine proximity to green and blue spaces did not allow for the calculation of precise distances between subjects’ homes and these spaces.
Mr. Vegaraju and Dr. Amiri’s study had no outside support, and the investigators declared no conflicts of interest. Dr. Hajat declared no conflicts of interest.
The findings of the study, which was released ahead of its scheduled presentation at the annual meeting of the American Academy of Neurology, build on a growing understanding of the relationship between types and qualities of urban environments and dementia risk.
Adithya Vegaraju, a student at Washington State University, Spokane, led the study, which looked at data from the Washington State Behavioral Risk Factor Surveillance System to assess prevalence of serious psychological distress among 42,980 Washington state residents aged 65 and over.
The data, collected between 2011 and 2019, used a self-reported questionnaire to determine serious psychological distress, which is defined as a level of mental distress considered debilitating enough to warrant treatment.
Mr. Vegaraju and his coauthor Solmaz Amiri, DDes, also of Washington State University, used ZIP codes, along with U.S. census data, to approximate the urban adults’ proximity to green and blue spaces.
After controlling for potential confounders of age, sex, ethnicity, education, and marital status, the investigators found that people living within half a mile of green or blue spaces had a 17% lower risk of experiencing serious psychological distress, compared with people living farther from these spaces, the investigators said in a news release.
Implications for cognitive decline and dementia?
Psychological distress in adults has been linked in population-based longitudinal studies to later cognitive decline and dementia. One study in older adults found the risk of dementia to be more than 50% higher among adults aged 50-70 with persistent depression. Blue and green spaces have also been investigated in relation to neurodegenerative disease among older adults; a 2022 study looking at data from some 62 million Medicare beneficiaries found those living in areas with more vegetation saw lower risk of hospitalizations for Alzheimer’s disease and related dementias.
“Since we lack effective prevention methods or treatments for mild cognitive impairment and dementia, we need to get creative in how we look at these issues,” Dr. Amiri commented in a press statement about her and Mr. Vegaraju’s findings. “Our hope is that this study showing better mental health among people living close to parks and water will trigger other studies about how these benefits work and whether this proximity can help prevent or delay mild cognitive impairment and dementia.”
The investigators acknowledged that their findings were limited by reliance on a self-reported measure of psychological distress.
A bidirectional connection with depression and dementia
In a comment, Anjum Hajat, PhD, an epidemiologist at University of Washington School of Public Health in Seattle who has also studied the relationship between green space and dementia risk in older adults, noted some further apparent limitations of the new study, for which only an abstract was available at publication.
“It has been shown that people with depression are at higher risk for dementia, but the opposite is also true,” Dr. Hajat commented. “Those with dementia are more likely to develop depression. This bidirectionality makes this study abstract difficult to interpret since the study is based on cross-sectional data: Individuals are not followed over time to see which develops first, dementia or depression.”
Additionally, Dr. Hajat noted, the data used to determine proximity to green and blue spaces did not allow for the calculation of precise distances between subjects’ homes and these spaces.
Mr. Vegaraju and Dr. Amiri’s study had no outside support, and the investigators declared no conflicts of interest. Dr. Hajat declared no conflicts of interest.
The findings of the study, which was released ahead of its scheduled presentation at the annual meeting of the American Academy of Neurology, build on a growing understanding of the relationship between types and qualities of urban environments and dementia risk.
Adithya Vegaraju, a student at Washington State University, Spokane, led the study, which looked at data from the Washington State Behavioral Risk Factor Surveillance System to assess prevalence of serious psychological distress among 42,980 Washington state residents aged 65 and over.
The data, collected between 2011 and 2019, used a self-reported questionnaire to determine serious psychological distress, which is defined as a level of mental distress considered debilitating enough to warrant treatment.
Mr. Vegaraju and his coauthor Solmaz Amiri, DDes, also of Washington State University, used ZIP codes, along with U.S. census data, to approximate the urban adults’ proximity to green and blue spaces.
After controlling for potential confounders of age, sex, ethnicity, education, and marital status, the investigators found that people living within half a mile of green or blue spaces had a 17% lower risk of experiencing serious psychological distress, compared with people living farther from these spaces, the investigators said in a news release.
Implications for cognitive decline and dementia?
Psychological distress in adults has been linked in population-based longitudinal studies to later cognitive decline and dementia. One study in older adults found the risk of dementia to be more than 50% higher among adults aged 50-70 with persistent depression. Blue and green spaces have also been investigated in relation to neurodegenerative disease among older adults; a 2022 study looking at data from some 62 million Medicare beneficiaries found those living in areas with more vegetation saw lower risk of hospitalizations for Alzheimer’s disease and related dementias.
“Since we lack effective prevention methods or treatments for mild cognitive impairment and dementia, we need to get creative in how we look at these issues,” Dr. Amiri commented in a press statement about her and Mr. Vegaraju’s findings. “Our hope is that this study showing better mental health among people living close to parks and water will trigger other studies about how these benefits work and whether this proximity can help prevent or delay mild cognitive impairment and dementia.”
The investigators acknowledged that their findings were limited by reliance on a self-reported measure of psychological distress.
A bidirectional connection with depression and dementia
In a comment, Anjum Hajat, PhD, an epidemiologist at University of Washington School of Public Health in Seattle who has also studied the relationship between green space and dementia risk in older adults, noted some further apparent limitations of the new study, for which only an abstract was available at publication.
“It has been shown that people with depression are at higher risk for dementia, but the opposite is also true,” Dr. Hajat commented. “Those with dementia are more likely to develop depression. This bidirectionality makes this study abstract difficult to interpret since the study is based on cross-sectional data: Individuals are not followed over time to see which develops first, dementia or depression.”
Additionally, Dr. Hajat noted, the data used to determine proximity to green and blue spaces did not allow for the calculation of precise distances between subjects’ homes and these spaces.
Mr. Vegaraju and Dr. Amiri’s study had no outside support, and the investigators declared no conflicts of interest. Dr. Hajat declared no conflicts of interest.
FROM AAN 2023
Bad sleep cuts years off life, but exercise can save us
Experts recommend that most adults get 7-9 hours of sleep a night.
Plenty of research points to sleep and physical activity as crucial factors affecting life expectancy. Regular exercise can lengthen life, while too little or too much sleep may cut it short.
But evidence is growing that exercise may counteract the negative effects of poor sleep. A 2022 study found that being physically active for at least 25 minutes a day can erase the risk of early death associated with too much sleep or trouble falling asleep. And a 2021 study found that lower levels of physical activity may exacerbate the impact of poor sleep on early death, heart disease, and cancer.
The latest such study, published in the European Journal of Preventive Cardiology, suggests that higher volumes of exercise can virtually eliminate the risk of early death associated with sleeping too little or too long.
This study is unique, the researchers say, because it used accelerometers (motion-tracking sensors) to quantify sleep and physical activity. Other studies asked participants to report their own data, opening the door to false reports and mistakes.
Some 92,000 participants in the United Kingdom (mean age, 62 years; 56% women) wore the activity trackers for a week to measure how much they moved and slept. In the following 7 years, 3,080 participants died, mostly from cardiovascular disease or cancer.
As one might expect, the participants who were least likely to die also exercised the most and slept the “normal” amount (6-8 hours a night, as defined by the study).
Compared with that group, those who exercised the least and slept less than 6 hours were 2.5 times more likely to die during those 7 years (P < .001). Less active persons who got the recommended sleep were 79% more likely to die (P < .001). The risk was slightly higher than that for those who logged more than 8 hours a night.
But those risks disappeared for short- or long-sleeping participants who logged at least 150 minutes a week of moderate to vigorous activity.
“Exercise fights inflammatory and metabolic dysregulations and abnormal sympathetic nervous system activity,” said study author Jihui Zhang, PhD, of the Affiliated Brain Hospital of Guangzhou (China). Those problems are associated with cardiovascular diseases and other potentially fatal conditions.
More objective data – with tech
A study’s findings are only as good as the data it relies on. That’s why obtaining objective data not influenced by individual perception is key.
“Self-report questionnaires are prone to misperception, or recall or response bias,” Dr. Zhang explains.
Take sleep, for example. Research reveals that several factors can affect how we judge our sleep. When people have to sleep at irregular times, they often underestimate how many hours they sleep but overestimate how long they nap, found a study in the Journal of Clinical Sleep Medicine.
Another study showed that when people are under a lot of stress, they’ll report more sleep problems than they actually have, as revealed by an Actiheart monitor.
With exercise, participants often report doing more exercise, and doing it at a higher intensity, than objective measurements show they did. At the same time, self-reports typically don’t account for much of the unplanned, low-effort movement people do throughout the day.
Staying active when you’re tired
The study raises a practical question: If you don’t get the proper amount of sleep, how are you supposed to find the time, energy, and motivation to exercise?
The solution is to use one to fix the other.
Exercise and sleep have “a robust directional relationship,” Dr. Zhang said. Exercise improves sleep, while better sleep makes it easier to stick with an exercise program.
Ideally, that program will include a mix of cardio and resistance exercise, said Mitch Duncan, PhD, a professor of public health at the University of Newcastle, Australia.
As Dr. Duncan and his co-authors showed in a recent study, “the largest benefits to health occur when people do a combination of both aerobic and muscle-strengthening activity,” Dr. Duncan said.
“In terms of benefits to sleep, there doesn’t seem to be consistent evidence that favors either as being most effective.”
The timing or intensity of exercise doesn’t seem to matter much, either.
“But there is evidence that a greater duration contributes to larger improvements in sleep,” Dr. Duncan said.
In other words, longer workouts are generally better, but they don’t necessarily have to be super-intense.
The strongest evidence of all, however, shows that recent and regular exercise offer the biggest benefits at bedtime.
Today’s workout will improve tonight’s sleep. And the better you sleep tonight, the more likely you are to stick with the program.
A version of this article first appeared on WebMD.com.
Experts recommend that most adults get 7-9 hours of sleep a night.
Plenty of research points to sleep and physical activity as crucial factors affecting life expectancy. Regular exercise can lengthen life, while too little or too much sleep may cut it short.
But evidence is growing that exercise may counteract the negative effects of poor sleep. A 2022 study found that being physically active for at least 25 minutes a day can erase the risk of early death associated with too much sleep or trouble falling asleep. And a 2021 study found that lower levels of physical activity may exacerbate the impact of poor sleep on early death, heart disease, and cancer.
The latest such study, published in the European Journal of Preventive Cardiology, suggests that higher volumes of exercise can virtually eliminate the risk of early death associated with sleeping too little or too long.
This study is unique, the researchers say, because it used accelerometers (motion-tracking sensors) to quantify sleep and physical activity. Other studies asked participants to report their own data, opening the door to false reports and mistakes.
Some 92,000 participants in the United Kingdom (mean age, 62 years; 56% women) wore the activity trackers for a week to measure how much they moved and slept. In the following 7 years, 3,080 participants died, mostly from cardiovascular disease or cancer.
As one might expect, the participants who were least likely to die also exercised the most and slept the “normal” amount (6-8 hours a night, as defined by the study).
Compared with that group, those who exercised the least and slept less than 6 hours were 2.5 times more likely to die during those 7 years (P < .001). Less active persons who got the recommended sleep were 79% more likely to die (P < .001). The risk was slightly higher than that for those who logged more than 8 hours a night.
But those risks disappeared for short- or long-sleeping participants who logged at least 150 minutes a week of moderate to vigorous activity.
“Exercise fights inflammatory and metabolic dysregulations and abnormal sympathetic nervous system activity,” said study author Jihui Zhang, PhD, of the Affiliated Brain Hospital of Guangzhou (China). Those problems are associated with cardiovascular diseases and other potentially fatal conditions.
More objective data – with tech
A study’s findings are only as good as the data it relies on. That’s why obtaining objective data not influenced by individual perception is key.
“Self-report questionnaires are prone to misperception, or recall or response bias,” Dr. Zhang explains.
Take sleep, for example. Research reveals that several factors can affect how we judge our sleep. When people have to sleep at irregular times, they often underestimate how many hours they sleep but overestimate how long they nap, found a study in the Journal of Clinical Sleep Medicine.
Another study showed that when people are under a lot of stress, they’ll report more sleep problems than they actually have, as revealed by an Actiheart monitor.
With exercise, participants often report doing more exercise, and doing it at a higher intensity, than objective measurements show they did. At the same time, self-reports typically don’t account for much of the unplanned, low-effort movement people do throughout the day.
Staying active when you’re tired
The study raises a practical question: If you don’t get the proper amount of sleep, how are you supposed to find the time, energy, and motivation to exercise?
The solution is to use one to fix the other.
Exercise and sleep have “a robust directional relationship,” Dr. Zhang said. Exercise improves sleep, while better sleep makes it easier to stick with an exercise program.
Ideally, that program will include a mix of cardio and resistance exercise, said Mitch Duncan, PhD, a professor of public health at the University of Newcastle, Australia.
As Dr. Duncan and his co-authors showed in a recent study, “the largest benefits to health occur when people do a combination of both aerobic and muscle-strengthening activity,” Dr. Duncan said.
“In terms of benefits to sleep, there doesn’t seem to be consistent evidence that favors either as being most effective.”
The timing or intensity of exercise doesn’t seem to matter much, either.
“But there is evidence that a greater duration contributes to larger improvements in sleep,” Dr. Duncan said.
In other words, longer workouts are generally better, but they don’t necessarily have to be super-intense.
The strongest evidence of all, however, shows that recent and regular exercise offer the biggest benefits at bedtime.
Today’s workout will improve tonight’s sleep. And the better you sleep tonight, the more likely you are to stick with the program.
A version of this article first appeared on WebMD.com.
Experts recommend that most adults get 7-9 hours of sleep a night.
Plenty of research points to sleep and physical activity as crucial factors affecting life expectancy. Regular exercise can lengthen life, while too little or too much sleep may cut it short.
But evidence is growing that exercise may counteract the negative effects of poor sleep. A 2022 study found that being physically active for at least 25 minutes a day can erase the risk of early death associated with too much sleep or trouble falling asleep. And a 2021 study found that lower levels of physical activity may exacerbate the impact of poor sleep on early death, heart disease, and cancer.
The latest such study, published in the European Journal of Preventive Cardiology, suggests that higher volumes of exercise can virtually eliminate the risk of early death associated with sleeping too little or too long.
This study is unique, the researchers say, because it used accelerometers (motion-tracking sensors) to quantify sleep and physical activity. Other studies asked participants to report their own data, opening the door to false reports and mistakes.
Some 92,000 participants in the United Kingdom (mean age, 62 years; 56% women) wore the activity trackers for a week to measure how much they moved and slept. In the following 7 years, 3,080 participants died, mostly from cardiovascular disease or cancer.
As one might expect, the participants who were least likely to die also exercised the most and slept the “normal” amount (6-8 hours a night, as defined by the study).
Compared with that group, those who exercised the least and slept less than 6 hours were 2.5 times more likely to die during those 7 years (P < .001). Less active persons who got the recommended sleep were 79% more likely to die (P < .001). The risk was slightly higher than that for those who logged more than 8 hours a night.
But those risks disappeared for short- or long-sleeping participants who logged at least 150 minutes a week of moderate to vigorous activity.
“Exercise fights inflammatory and metabolic dysregulations and abnormal sympathetic nervous system activity,” said study author Jihui Zhang, PhD, of the Affiliated Brain Hospital of Guangzhou (China). Those problems are associated with cardiovascular diseases and other potentially fatal conditions.
More objective data – with tech
A study’s findings are only as good as the data it relies on. That’s why obtaining objective data not influenced by individual perception is key.
“Self-report questionnaires are prone to misperception, or recall or response bias,” Dr. Zhang explains.
Take sleep, for example. Research reveals that several factors can affect how we judge our sleep. When people have to sleep at irregular times, they often underestimate how many hours they sleep but overestimate how long they nap, found a study in the Journal of Clinical Sleep Medicine.
Another study showed that when people are under a lot of stress, they’ll report more sleep problems than they actually have, as revealed by an Actiheart monitor.
With exercise, participants often report doing more exercise, and doing it at a higher intensity, than objective measurements show they did. At the same time, self-reports typically don’t account for much of the unplanned, low-effort movement people do throughout the day.
Staying active when you’re tired
The study raises a practical question: If you don’t get the proper amount of sleep, how are you supposed to find the time, energy, and motivation to exercise?
The solution is to use one to fix the other.
Exercise and sleep have “a robust directional relationship,” Dr. Zhang said. Exercise improves sleep, while better sleep makes it easier to stick with an exercise program.
Ideally, that program will include a mix of cardio and resistance exercise, said Mitch Duncan, PhD, a professor of public health at the University of Newcastle, Australia.
As Dr. Duncan and his co-authors showed in a recent study, “the largest benefits to health occur when people do a combination of both aerobic and muscle-strengthening activity,” Dr. Duncan said.
“In terms of benefits to sleep, there doesn’t seem to be consistent evidence that favors either as being most effective.”
The timing or intensity of exercise doesn’t seem to matter much, either.
“But there is evidence that a greater duration contributes to larger improvements in sleep,” Dr. Duncan said.
In other words, longer workouts are generally better, but they don’t necessarily have to be super-intense.
The strongest evidence of all, however, shows that recent and regular exercise offer the biggest benefits at bedtime.
Today’s workout will improve tonight’s sleep. And the better you sleep tonight, the more likely you are to stick with the program.
A version of this article first appeared on WebMD.com.
FROM EUROPEAN JOURNAL OF PREVENTIVE CARDIOLOGY
NSAID use in diabetes may worsen risk for first HF hospitalization
suggests a prospective, controlled study.
Certain subgroups may account for much of the excess risk, the results suggest, including the very elderly, patients with uncontrolled diabetes, those prescribed an NSAID for the first time, and patients already taking both a renin-angiotensin system inhibitor (RASi) and a diuretic.
Such patients with a firm indication for NSAIDs potentially could “be the ones benefiting most from closer follow-up, reduced dosage, or other mitigation strategies,” Anders Holt, MD, said in an interview.
Dr. Holt, of Copenhagen University Hospital and Herlev-Gentofte Hospital in Hellerup, Denmark, is lead author on the analysis of Danish registry data published in the Journal of the American College of Cardiology. He presented essentially the same results in preliminary form at the 2022 annual congress of the European Society of Cardiology.
HF hospitalizations linked to NSAIDs, the published report notes, are often attributed to symptoms from temporary fluid overload, often without worsening cardiac function, that stem from the drugs’ renal effects.
“One could speculate,” Dr. Holt said, that such HF events might be less severe and even associated with better outcomes, compared with other forms of heart failure.
But the current analysis provides a hint to the contrary, he observed. The 5-year mortality was similar for patients with HF linked to NSAIDs and those with other forms of HF, “which could suggest that NSAID-associated heart failure is more than transient fluid overload.”
The drugs may promote HF through direct effects on the heart by any of several proposed mechanisms, including “induction of arrhythmias and heart fibrosis, vasoconstriction, subclinical inflammation, and blood pressure elevation,” Dr. Holt said.
The current study doesn’t determine whether NSAID-associated HF stems from transient fluid overload or direct cardiac effects, but it’s “most likely both.”
In other limitations, the analysis is unable to “reliably explore” whether promotion of HF is an NSAID class effect, a “clinically relevant” point given the drugs’ varying effects on cardiovascular risk, states an accompanying editorial. Nor was it able to determine whether the drugs exert a dose-response effect on HF risk, noted Hassan Khan, MD, PhD, Norton Healthcare, Louisville, Ky., and Setor K. Kunutsor, MD, PhD, University of Leicester (England).
Still, “given the well-established relationship between the use of NSAIDs and increased HF, these findings are not unexpected because type 2 diabetes is also a major risk factor for HF.”
But it may be “premature to issue guideline recommendations based on a single observational study,” the editorialists wrote. “Further robust clinical trial evidence is needed to replicate these results and investigate the relationship of the type and dose of NSAIDs with HF risk. However, it should be realized that short-term or long-term use of NSAIDs may be detrimental to cardiovascular health.”
The analysis covered 23,308 patients from throughout Denmark with a type 2 diabetes diagnosis and no HF history who experienced a first HF hospitalization; their age averaged 76 years and 39% were women.
They served as their own controls; their NSAID exposures at two 28-day periods preceding the HF event, the one immediately before and the other preceding it by 56 days, were compared as the index and control periods, respectively.
Exposure to NSAIDs was defined as obtaining a prescription for celecoxib, diclofenac, ibuprofen, or naproxen, “as these are NSAIDs used primarily in Denmark,” the report states.
The odds ratios for HF hospitalization associated with NSAID exposure within 28 days preceding the event were 1.43 (95% confidence interval, 1.27-1.63) overall, 1.41 (95% CI, 1.16-1.71) for an NSAID given on top of both RASi and diuretics, 1.68 (95% CI, 1.00-2.88) for patients with elevated hemoglobin A1c, 1.78 (95% CI, 1.39-2.28) for those 80 or older, and 2.71 (95% CI, 1.78-4.23) for those with prior NSAID use.
That NSAID use and diabetes are each associated with increased risk for HF is well established, Dr. Holt observed. Yet the drugs had been prescribed to 16% of patients in the study.
“One of the more surprising findings, to me, was the quite substantial use of prescribed NSAIDs in a population of patients with diabetes, a patient group with a well-established cardiovascular risk,” he said.
“This patient group is only growing, so emphasis on the possible associations between even short-term NSAID use and incident heart failure is probably timely and perhaps needed.”
Dr. Holt and the study were supported by grants from Ib Mogens Kristiansens Almene Fond, Helsefonden, Snedkermester Sophus Jacobsen og hustru Astrid Jacobsen Fond, Marie og M.B. Richters Fond, and the Dagmar Marshalls Fond. Dr. Khan and Dr. Kunutsor reported no relevant relationships.
A version of this article first appeared on Medscape.com.
suggests a prospective, controlled study.
Certain subgroups may account for much of the excess risk, the results suggest, including the very elderly, patients with uncontrolled diabetes, those prescribed an NSAID for the first time, and patients already taking both a renin-angiotensin system inhibitor (RASi) and a diuretic.
Such patients with a firm indication for NSAIDs potentially could “be the ones benefiting most from closer follow-up, reduced dosage, or other mitigation strategies,” Anders Holt, MD, said in an interview.
Dr. Holt, of Copenhagen University Hospital and Herlev-Gentofte Hospital in Hellerup, Denmark, is lead author on the analysis of Danish registry data published in the Journal of the American College of Cardiology. He presented essentially the same results in preliminary form at the 2022 annual congress of the European Society of Cardiology.
HF hospitalizations linked to NSAIDs, the published report notes, are often attributed to symptoms from temporary fluid overload, often without worsening cardiac function, that stem from the drugs’ renal effects.
“One could speculate,” Dr. Holt said, that such HF events might be less severe and even associated with better outcomes, compared with other forms of heart failure.
But the current analysis provides a hint to the contrary, he observed. The 5-year mortality was similar for patients with HF linked to NSAIDs and those with other forms of HF, “which could suggest that NSAID-associated heart failure is more than transient fluid overload.”
The drugs may promote HF through direct effects on the heart by any of several proposed mechanisms, including “induction of arrhythmias and heart fibrosis, vasoconstriction, subclinical inflammation, and blood pressure elevation,” Dr. Holt said.
The current study doesn’t determine whether NSAID-associated HF stems from transient fluid overload or direct cardiac effects, but it’s “most likely both.”
In other limitations, the analysis is unable to “reliably explore” whether promotion of HF is an NSAID class effect, a “clinically relevant” point given the drugs’ varying effects on cardiovascular risk, states an accompanying editorial. Nor was it able to determine whether the drugs exert a dose-response effect on HF risk, noted Hassan Khan, MD, PhD, Norton Healthcare, Louisville, Ky., and Setor K. Kunutsor, MD, PhD, University of Leicester (England).
Still, “given the well-established relationship between the use of NSAIDs and increased HF, these findings are not unexpected because type 2 diabetes is also a major risk factor for HF.”
But it may be “premature to issue guideline recommendations based on a single observational study,” the editorialists wrote. “Further robust clinical trial evidence is needed to replicate these results and investigate the relationship of the type and dose of NSAIDs with HF risk. However, it should be realized that short-term or long-term use of NSAIDs may be detrimental to cardiovascular health.”
The analysis covered 23,308 patients from throughout Denmark with a type 2 diabetes diagnosis and no HF history who experienced a first HF hospitalization; their age averaged 76 years and 39% were women.
They served as their own controls; their NSAID exposures at two 28-day periods preceding the HF event, the one immediately before and the other preceding it by 56 days, were compared as the index and control periods, respectively.
Exposure to NSAIDs was defined as obtaining a prescription for celecoxib, diclofenac, ibuprofen, or naproxen, “as these are NSAIDs used primarily in Denmark,” the report states.
The odds ratios for HF hospitalization associated with NSAID exposure within 28 days preceding the event were 1.43 (95% confidence interval, 1.27-1.63) overall, 1.41 (95% CI, 1.16-1.71) for an NSAID given on top of both RASi and diuretics, 1.68 (95% CI, 1.00-2.88) for patients with elevated hemoglobin A1c, 1.78 (95% CI, 1.39-2.28) for those 80 or older, and 2.71 (95% CI, 1.78-4.23) for those with prior NSAID use.
That NSAID use and diabetes are each associated with increased risk for HF is well established, Dr. Holt observed. Yet the drugs had been prescribed to 16% of patients in the study.
“One of the more surprising findings, to me, was the quite substantial use of prescribed NSAIDs in a population of patients with diabetes, a patient group with a well-established cardiovascular risk,” he said.
“This patient group is only growing, so emphasis on the possible associations between even short-term NSAID use and incident heart failure is probably timely and perhaps needed.”
Dr. Holt and the study were supported by grants from Ib Mogens Kristiansens Almene Fond, Helsefonden, Snedkermester Sophus Jacobsen og hustru Astrid Jacobsen Fond, Marie og M.B. Richters Fond, and the Dagmar Marshalls Fond. Dr. Khan and Dr. Kunutsor reported no relevant relationships.
A version of this article first appeared on Medscape.com.
suggests a prospective, controlled study.
Certain subgroups may account for much of the excess risk, the results suggest, including the very elderly, patients with uncontrolled diabetes, those prescribed an NSAID for the first time, and patients already taking both a renin-angiotensin system inhibitor (RASi) and a diuretic.
Such patients with a firm indication for NSAIDs potentially could “be the ones benefiting most from closer follow-up, reduced dosage, or other mitigation strategies,” Anders Holt, MD, said in an interview.
Dr. Holt, of Copenhagen University Hospital and Herlev-Gentofte Hospital in Hellerup, Denmark, is lead author on the analysis of Danish registry data published in the Journal of the American College of Cardiology. He presented essentially the same results in preliminary form at the 2022 annual congress of the European Society of Cardiology.
HF hospitalizations linked to NSAIDs, the published report notes, are often attributed to symptoms from temporary fluid overload, often without worsening cardiac function, that stem from the drugs’ renal effects.
“One could speculate,” Dr. Holt said, that such HF events might be less severe and even associated with better outcomes, compared with other forms of heart failure.
But the current analysis provides a hint to the contrary, he observed. The 5-year mortality was similar for patients with HF linked to NSAIDs and those with other forms of HF, “which could suggest that NSAID-associated heart failure is more than transient fluid overload.”
The drugs may promote HF through direct effects on the heart by any of several proposed mechanisms, including “induction of arrhythmias and heart fibrosis, vasoconstriction, subclinical inflammation, and blood pressure elevation,” Dr. Holt said.
The current study doesn’t determine whether NSAID-associated HF stems from transient fluid overload or direct cardiac effects, but it’s “most likely both.”
In other limitations, the analysis is unable to “reliably explore” whether promotion of HF is an NSAID class effect, a “clinically relevant” point given the drugs’ varying effects on cardiovascular risk, states an accompanying editorial. Nor was it able to determine whether the drugs exert a dose-response effect on HF risk, noted Hassan Khan, MD, PhD, Norton Healthcare, Louisville, Ky., and Setor K. Kunutsor, MD, PhD, University of Leicester (England).
Still, “given the well-established relationship between the use of NSAIDs and increased HF, these findings are not unexpected because type 2 diabetes is also a major risk factor for HF.”
But it may be “premature to issue guideline recommendations based on a single observational study,” the editorialists wrote. “Further robust clinical trial evidence is needed to replicate these results and investigate the relationship of the type and dose of NSAIDs with HF risk. However, it should be realized that short-term or long-term use of NSAIDs may be detrimental to cardiovascular health.”
The analysis covered 23,308 patients from throughout Denmark with a type 2 diabetes diagnosis and no HF history who experienced a first HF hospitalization; their age averaged 76 years and 39% were women.
They served as their own controls; their NSAID exposures at two 28-day periods preceding the HF event, the one immediately before and the other preceding it by 56 days, were compared as the index and control periods, respectively.
Exposure to NSAIDs was defined as obtaining a prescription for celecoxib, diclofenac, ibuprofen, or naproxen, “as these are NSAIDs used primarily in Denmark,” the report states.
The odds ratios for HF hospitalization associated with NSAID exposure within 28 days preceding the event were 1.43 (95% confidence interval, 1.27-1.63) overall, 1.41 (95% CI, 1.16-1.71) for an NSAID given on top of both RASi and diuretics, 1.68 (95% CI, 1.00-2.88) for patients with elevated hemoglobin A1c, 1.78 (95% CI, 1.39-2.28) for those 80 or older, and 2.71 (95% CI, 1.78-4.23) for those with prior NSAID use.
That NSAID use and diabetes are each associated with increased risk for HF is well established, Dr. Holt observed. Yet the drugs had been prescribed to 16% of patients in the study.
“One of the more surprising findings, to me, was the quite substantial use of prescribed NSAIDs in a population of patients with diabetes, a patient group with a well-established cardiovascular risk,” he said.
“This patient group is only growing, so emphasis on the possible associations between even short-term NSAID use and incident heart failure is probably timely and perhaps needed.”
Dr. Holt and the study were supported by grants from Ib Mogens Kristiansens Almene Fond, Helsefonden, Snedkermester Sophus Jacobsen og hustru Astrid Jacobsen Fond, Marie og M.B. Richters Fond, and the Dagmar Marshalls Fond. Dr. Khan and Dr. Kunutsor reported no relevant relationships.
A version of this article first appeared on Medscape.com.
FROM THE JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
75-year-old man • recent history of hand-foot-mouth disease • discolored fingernails and toenails lifting from the proximal end • Dx?
THE CASE
A 75-year-old man sought care from his primary care physician because his “fingernails and toenails [were] all falling off.” He did not feel ill and had no other complaints. His vital signs were unremarkable. He had no history of malignancies, chronic skin conditions, or systemic diseases. His fingernails and toenails were discolored and lifting from the proximal end of his nail beds (FIGURE). One of his great toenails had already fallen off, 1 thumb nail was minimally attached with the cuticle, and the rest of his nails were loose and in the process of separating from their nail beds. There was no nail pitting, rash, or joint swelling and tenderness.
The patient reported that while on vacation in Hawaii 3 weeks earlier, he had sought care at an urgent care clinic for a painless rash on his hands and the soles of his feet. At that time, he did not feel ill or have mouth ulcers, penile discharge, or arthralgia. There had been no recent changes to his prescription medications, which included finasteride, terazosin, omeprazole, and an albuterol inhaler. He denied taking over-the-counter medications or supplements.
The physical exam at the urgent care had revealed multiple blotchy, dark, 0.5- to 1-cm nonpruritic lesions that were desquamating. No oral lesions were seen. He had been given a diagnosis of hand-foot-mouth disease (HFMD) and reassured that it would resolve on its own in about 10 days.
THE DIAGNOSIS
Several possible diagnoses for nail disorders came to mind with this patient, including onychomycosis, onychoschizia, onycholysis, and onychomadesis.
Onychomycosis is a chronic fungal infection of the nail that affects toenails more often than fingernails.1 The most common form is distal subungual onychomycosis, which begins distally and slowly migrates proximally through the nail matrix.1 Often onychomycosis affects only a few nails unless the patient is elderly or has comorbid conditions, and the nails rarely separate from the nail bed.
Onychoschizia involves lamellar splitting and peeling of the dorsal surface of the nail plate.2 Usually white discolorations appear on the distal edges of the nail.3 It is more common in women than in men and is often caused by nail dehydration from repeated excessive immersion in water with detergents or recurrent application of nail polish.2 However, the nails do not separate from the nail bed, and usually only the fingernails are involved.
Onycholysis is a nail attachment disorder in which the nail plate distally separates from the nail bed. Areas of separation will appear white or yellow. There are many etiologies for onycholysis, including trauma, psoriasis, fungal infection, and contact irritant reactions.3 It also can be caused by medications and thyroid disease.3,4
Continue to: Onychomadesis
Onychomadesis, sometimes considered a severe form of Beau’s line,5,6 is defined by the spontaneous separation of the nail plate from the nail matrix. Although the nail will initially remain attached, proximal shedding will eventually occur.7 When several nails are involved, a systemic source—such as an acute infection, autoimmune disease, medication, malignancy (eg, cutaneous T-cell lymphoma), Kawasaki disease, skin disorders (eg, pemphigus vulgaris or keratosis punctata et planters), or chemotherapy—may be the cause.6-8 If only a few nails are involved, it may be associated with trauma, and in rare cases, onychomadesis can be idiopathic.5,7
In this case, all signs pointed to onychomadesis. All of the patient’s nails were affected (discolored and lifting), his nail loss involved spontaneous proximal separation of the nail plate from the nail matrix, and he had a recent previous infection: HFMD.
DISCUSSION
Onychomadesis is a rare nail-shedding disorder thought to be caused by the temporary arrest of the nail matrix.8 It is a potential late complication of infection, such as HFMD,9 and was first reported in children in Chicago in 2000.10 Since then, onychomadesis has been noted in children in many countries.8 Reports of onychomadesis following HFMD in adults are rare, but it may be underreported because HFMD is more common in children and symptoms are usually minor in adults.11
Molecular studies have associated onychomadesis with coxsackievirus (CV)A6 and CVA10.4 Other serotypes associated with onychomadesis include CVB1, CVB2, CVA5, CVA16, and enteroviruses 71 and 9.4 Most known outbreaks seem to be caused by CVA6.4
No treatment is needed for onychomadesis; physicians can reassure patients that normal nail growth will begin within 1 to 4 months. Because onychomadesis is rare, it does not have its own billing code, so one can use code L60.8 for “Other nail disorders.”12
Our patient was seen in the primary care clinic 3 months after his initial visit. At that time, his nails were no longer discolored and no other abnormalities were present. All of the nails on his fingers and toes were firmly attached and growing normally.
THE TAKEAWAY
The sudden asymptomatic loss of multiple fingernails and toenails—especially with proximal nail shedding—is a rare disorder known as onychomadesis. It can be caused by various etiologies and can be a late complication of HFMD or other viral infections. Onychomadesis should be considered when evaluating older patients, particularly when all of their nails are involved after a viral infection.
CORRESPONDENCE
Jon F. Peters, MD, MS, FAAFP, 14486 SE Lyon Court, Happy Valley, OR 97086; [email protected]
1. Rodgers P, Bassler M. Treating onychomycosis. Am Fam Physician. 2001;63:663-672, 677-678.
2. Sparavigna A, Tenconi B, La Penna L. Efficacy and tolerability of a biomineral formulation for treatment of onychoschizia: a randomized trial. Clin Cosmet Investig Dermatol. 2019:12:355-362. doi: 10.2147/CCID.S187305
3. Singal A, Arora R. Nail as a window of systemic diseases. Indian Dermatol Online J. 2015;6:67-74. doi: 10.4103/2229-5178.153002
4. Cleveland Clinic. Onycholysis. Accessed March 1, 2023. https://my.clevelandclinic.org/health/diseases/22903-onycholysis
5. Chiu H-H, Liu M-T, Chung W-H, et al. The mechanism of onychomadesis (nail shedding) and Beau’s lines following hand-foot-mouth disease. Viruses. 2019;11:522. doi: 10.3390/v11060522
6. Suchonwanit P, Nitayavardhana S. Idiopathic sporadic onychomadesis of toenails. Case Rep Dermatol Med. 2016;2016:6451327. doi: 10.1155/2016/6451327
7. Hardin J, Haber RM. Onychomadesis: literature review. Br J Dermatol. 2015;172:592-596. doi: 10.1111/bjd.13339
8. Li D, Yang W, Xing X, et al. Onychomadesis and potential association with HFMD outbreak in a kindergarten in Hubei providence, China, 2017. BMC Infect Dis. 2019:19:995. doi: 10.1186/s12879-019-4560-8
9. Chiu HH, Wu CS, Lan CE. Onychomadesis: a late complication of hand, foot, and mouth disease. J Emerg Med. 2017;52:243-245. doi: 10.1016/j.jemermed.2016.01.034
10. Clementz GC, Mancini AJ. Nail matrix arrest following hand-foot-mouth disease: a report of five children. Pediatr Dermatol. 2000;17:7-11. doi: 10.1046/j.1525-1470.2000.01702.x
11. Scarfi F, Arunachalam M, Galeone M, et al. An uncommon onychomadesis in adults. Int J Derm. 2014;53:1392-1394. doi: 10.1111/j.1365-4632.2012.05774.x
12. ICD10Data.com. 2023 ICD-10-CM codes. Accessed February 15, 2023. www.icd10data.com/ICD10CM/codes
THE CASE
A 75-year-old man sought care from his primary care physician because his “fingernails and toenails [were] all falling off.” He did not feel ill and had no other complaints. His vital signs were unremarkable. He had no history of malignancies, chronic skin conditions, or systemic diseases. His fingernails and toenails were discolored and lifting from the proximal end of his nail beds (FIGURE). One of his great toenails had already fallen off, 1 thumb nail was minimally attached with the cuticle, and the rest of his nails were loose and in the process of separating from their nail beds. There was no nail pitting, rash, or joint swelling and tenderness.
The patient reported that while on vacation in Hawaii 3 weeks earlier, he had sought care at an urgent care clinic for a painless rash on his hands and the soles of his feet. At that time, he did not feel ill or have mouth ulcers, penile discharge, or arthralgia. There had been no recent changes to his prescription medications, which included finasteride, terazosin, omeprazole, and an albuterol inhaler. He denied taking over-the-counter medications or supplements.
The physical exam at the urgent care had revealed multiple blotchy, dark, 0.5- to 1-cm nonpruritic lesions that were desquamating. No oral lesions were seen. He had been given a diagnosis of hand-foot-mouth disease (HFMD) and reassured that it would resolve on its own in about 10 days.
THE DIAGNOSIS
Several possible diagnoses for nail disorders came to mind with this patient, including onychomycosis, onychoschizia, onycholysis, and onychomadesis.
Onychomycosis is a chronic fungal infection of the nail that affects toenails more often than fingernails.1 The most common form is distal subungual onychomycosis, which begins distally and slowly migrates proximally through the nail matrix.1 Often onychomycosis affects only a few nails unless the patient is elderly or has comorbid conditions, and the nails rarely separate from the nail bed.
Onychoschizia involves lamellar splitting and peeling of the dorsal surface of the nail plate.2 Usually white discolorations appear on the distal edges of the nail.3 It is more common in women than in men and is often caused by nail dehydration from repeated excessive immersion in water with detergents or recurrent application of nail polish.2 However, the nails do not separate from the nail bed, and usually only the fingernails are involved.
Onycholysis is a nail attachment disorder in which the nail plate distally separates from the nail bed. Areas of separation will appear white or yellow. There are many etiologies for onycholysis, including trauma, psoriasis, fungal infection, and contact irritant reactions.3 It also can be caused by medications and thyroid disease.3,4
Continue to: Onychomadesis
Onychomadesis, sometimes considered a severe form of Beau’s line,5,6 is defined by the spontaneous separation of the nail plate from the nail matrix. Although the nail will initially remain attached, proximal shedding will eventually occur.7 When several nails are involved, a systemic source—such as an acute infection, autoimmune disease, medication, malignancy (eg, cutaneous T-cell lymphoma), Kawasaki disease, skin disorders (eg, pemphigus vulgaris or keratosis punctata et planters), or chemotherapy—may be the cause.6-8 If only a few nails are involved, it may be associated with trauma, and in rare cases, onychomadesis can be idiopathic.5,7
In this case, all signs pointed to onychomadesis. All of the patient’s nails were affected (discolored and lifting), his nail loss involved spontaneous proximal separation of the nail plate from the nail matrix, and he had a recent previous infection: HFMD.
DISCUSSION
Onychomadesis is a rare nail-shedding disorder thought to be caused by the temporary arrest of the nail matrix.8 It is a potential late complication of infection, such as HFMD,9 and was first reported in children in Chicago in 2000.10 Since then, onychomadesis has been noted in children in many countries.8 Reports of onychomadesis following HFMD in adults are rare, but it may be underreported because HFMD is more common in children and symptoms are usually minor in adults.11
Molecular studies have associated onychomadesis with coxsackievirus (CV)A6 and CVA10.4 Other serotypes associated with onychomadesis include CVB1, CVB2, CVA5, CVA16, and enteroviruses 71 and 9.4 Most known outbreaks seem to be caused by CVA6.4
No treatment is needed for onychomadesis; physicians can reassure patients that normal nail growth will begin within 1 to 4 months. Because onychomadesis is rare, it does not have its own billing code, so one can use code L60.8 for “Other nail disorders.”12
Our patient was seen in the primary care clinic 3 months after his initial visit. At that time, his nails were no longer discolored and no other abnormalities were present. All of the nails on his fingers and toes were firmly attached and growing normally.
THE TAKEAWAY
The sudden asymptomatic loss of multiple fingernails and toenails—especially with proximal nail shedding—is a rare disorder known as onychomadesis. It can be caused by various etiologies and can be a late complication of HFMD or other viral infections. Onychomadesis should be considered when evaluating older patients, particularly when all of their nails are involved after a viral infection.
CORRESPONDENCE
Jon F. Peters, MD, MS, FAAFP, 14486 SE Lyon Court, Happy Valley, OR 97086; [email protected]
THE CASE
A 75-year-old man sought care from his primary care physician because his “fingernails and toenails [were] all falling off.” He did not feel ill and had no other complaints. His vital signs were unremarkable. He had no history of malignancies, chronic skin conditions, or systemic diseases. His fingernails and toenails were discolored and lifting from the proximal end of his nail beds (FIGURE). One of his great toenails had already fallen off, 1 thumb nail was minimally attached with the cuticle, and the rest of his nails were loose and in the process of separating from their nail beds. There was no nail pitting, rash, or joint swelling and tenderness.
The patient reported that while on vacation in Hawaii 3 weeks earlier, he had sought care at an urgent care clinic for a painless rash on his hands and the soles of his feet. At that time, he did not feel ill or have mouth ulcers, penile discharge, or arthralgia. There had been no recent changes to his prescription medications, which included finasteride, terazosin, omeprazole, and an albuterol inhaler. He denied taking over-the-counter medications or supplements.
The physical exam at the urgent care had revealed multiple blotchy, dark, 0.5- to 1-cm nonpruritic lesions that were desquamating. No oral lesions were seen. He had been given a diagnosis of hand-foot-mouth disease (HFMD) and reassured that it would resolve on its own in about 10 days.
THE DIAGNOSIS
Several possible diagnoses for nail disorders came to mind with this patient, including onychomycosis, onychoschizia, onycholysis, and onychomadesis.
Onychomycosis is a chronic fungal infection of the nail that affects toenails more often than fingernails.1 The most common form is distal subungual onychomycosis, which begins distally and slowly migrates proximally through the nail matrix.1 Often onychomycosis affects only a few nails unless the patient is elderly or has comorbid conditions, and the nails rarely separate from the nail bed.
Onychoschizia involves lamellar splitting and peeling of the dorsal surface of the nail plate.2 Usually white discolorations appear on the distal edges of the nail.3 It is more common in women than in men and is often caused by nail dehydration from repeated excessive immersion in water with detergents or recurrent application of nail polish.2 However, the nails do not separate from the nail bed, and usually only the fingernails are involved.
Onycholysis is a nail attachment disorder in which the nail plate distally separates from the nail bed. Areas of separation will appear white or yellow. There are many etiologies for onycholysis, including trauma, psoriasis, fungal infection, and contact irritant reactions.3 It also can be caused by medications and thyroid disease.3,4
Continue to: Onychomadesis
Onychomadesis, sometimes considered a severe form of Beau’s line,5,6 is defined by the spontaneous separation of the nail plate from the nail matrix. Although the nail will initially remain attached, proximal shedding will eventually occur.7 When several nails are involved, a systemic source—such as an acute infection, autoimmune disease, medication, malignancy (eg, cutaneous T-cell lymphoma), Kawasaki disease, skin disorders (eg, pemphigus vulgaris or keratosis punctata et planters), or chemotherapy—may be the cause.6-8 If only a few nails are involved, it may be associated with trauma, and in rare cases, onychomadesis can be idiopathic.5,7
In this case, all signs pointed to onychomadesis. All of the patient’s nails were affected (discolored and lifting), his nail loss involved spontaneous proximal separation of the nail plate from the nail matrix, and he had a recent previous infection: HFMD.
DISCUSSION
Onychomadesis is a rare nail-shedding disorder thought to be caused by the temporary arrest of the nail matrix.8 It is a potential late complication of infection, such as HFMD,9 and was first reported in children in Chicago in 2000.10 Since then, onychomadesis has been noted in children in many countries.8 Reports of onychomadesis following HFMD in adults are rare, but it may be underreported because HFMD is more common in children and symptoms are usually minor in adults.11
Molecular studies have associated onychomadesis with coxsackievirus (CV)A6 and CVA10.4 Other serotypes associated with onychomadesis include CVB1, CVB2, CVA5, CVA16, and enteroviruses 71 and 9.4 Most known outbreaks seem to be caused by CVA6.4
No treatment is needed for onychomadesis; physicians can reassure patients that normal nail growth will begin within 1 to 4 months. Because onychomadesis is rare, it does not have its own billing code, so one can use code L60.8 for “Other nail disorders.”12
Our patient was seen in the primary care clinic 3 months after his initial visit. At that time, his nails were no longer discolored and no other abnormalities were present. All of the nails on his fingers and toes were firmly attached and growing normally.
THE TAKEAWAY
The sudden asymptomatic loss of multiple fingernails and toenails—especially with proximal nail shedding—is a rare disorder known as onychomadesis. It can be caused by various etiologies and can be a late complication of HFMD or other viral infections. Onychomadesis should be considered when evaluating older patients, particularly when all of their nails are involved after a viral infection.
CORRESPONDENCE
Jon F. Peters, MD, MS, FAAFP, 14486 SE Lyon Court, Happy Valley, OR 97086; [email protected]
1. Rodgers P, Bassler M. Treating onychomycosis. Am Fam Physician. 2001;63:663-672, 677-678.
2. Sparavigna A, Tenconi B, La Penna L. Efficacy and tolerability of a biomineral formulation for treatment of onychoschizia: a randomized trial. Clin Cosmet Investig Dermatol. 2019:12:355-362. doi: 10.2147/CCID.S187305
3. Singal A, Arora R. Nail as a window of systemic diseases. Indian Dermatol Online J. 2015;6:67-74. doi: 10.4103/2229-5178.153002
4. Cleveland Clinic. Onycholysis. Accessed March 1, 2023. https://my.clevelandclinic.org/health/diseases/22903-onycholysis
5. Chiu H-H, Liu M-T, Chung W-H, et al. The mechanism of onychomadesis (nail shedding) and Beau’s lines following hand-foot-mouth disease. Viruses. 2019;11:522. doi: 10.3390/v11060522
6. Suchonwanit P, Nitayavardhana S. Idiopathic sporadic onychomadesis of toenails. Case Rep Dermatol Med. 2016;2016:6451327. doi: 10.1155/2016/6451327
7. Hardin J, Haber RM. Onychomadesis: literature review. Br J Dermatol. 2015;172:592-596. doi: 10.1111/bjd.13339
8. Li D, Yang W, Xing X, et al. Onychomadesis and potential association with HFMD outbreak in a kindergarten in Hubei providence, China, 2017. BMC Infect Dis. 2019:19:995. doi: 10.1186/s12879-019-4560-8
9. Chiu HH, Wu CS, Lan CE. Onychomadesis: a late complication of hand, foot, and mouth disease. J Emerg Med. 2017;52:243-245. doi: 10.1016/j.jemermed.2016.01.034
10. Clementz GC, Mancini AJ. Nail matrix arrest following hand-foot-mouth disease: a report of five children. Pediatr Dermatol. 2000;17:7-11. doi: 10.1046/j.1525-1470.2000.01702.x
11. Scarfi F, Arunachalam M, Galeone M, et al. An uncommon onychomadesis in adults. Int J Derm. 2014;53:1392-1394. doi: 10.1111/j.1365-4632.2012.05774.x
12. ICD10Data.com. 2023 ICD-10-CM codes. Accessed February 15, 2023. www.icd10data.com/ICD10CM/codes
1. Rodgers P, Bassler M. Treating onychomycosis. Am Fam Physician. 2001;63:663-672, 677-678.
2. Sparavigna A, Tenconi B, La Penna L. Efficacy and tolerability of a biomineral formulation for treatment of onychoschizia: a randomized trial. Clin Cosmet Investig Dermatol. 2019:12:355-362. doi: 10.2147/CCID.S187305
3. Singal A, Arora R. Nail as a window of systemic diseases. Indian Dermatol Online J. 2015;6:67-74. doi: 10.4103/2229-5178.153002
4. Cleveland Clinic. Onycholysis. Accessed March 1, 2023. https://my.clevelandclinic.org/health/diseases/22903-onycholysis
5. Chiu H-H, Liu M-T, Chung W-H, et al. The mechanism of onychomadesis (nail shedding) and Beau’s lines following hand-foot-mouth disease. Viruses. 2019;11:522. doi: 10.3390/v11060522
6. Suchonwanit P, Nitayavardhana S. Idiopathic sporadic onychomadesis of toenails. Case Rep Dermatol Med. 2016;2016:6451327. doi: 10.1155/2016/6451327
7. Hardin J, Haber RM. Onychomadesis: literature review. Br J Dermatol. 2015;172:592-596. doi: 10.1111/bjd.13339
8. Li D, Yang W, Xing X, et al. Onychomadesis and potential association with HFMD outbreak in a kindergarten in Hubei providence, China, 2017. BMC Infect Dis. 2019:19:995. doi: 10.1186/s12879-019-4560-8
9. Chiu HH, Wu CS, Lan CE. Onychomadesis: a late complication of hand, foot, and mouth disease. J Emerg Med. 2017;52:243-245. doi: 10.1016/j.jemermed.2016.01.034
10. Clementz GC, Mancini AJ. Nail matrix arrest following hand-foot-mouth disease: a report of five children. Pediatr Dermatol. 2000;17:7-11. doi: 10.1046/j.1525-1470.2000.01702.x
11. Scarfi F, Arunachalam M, Galeone M, et al. An uncommon onychomadesis in adults. Int J Derm. 2014;53:1392-1394. doi: 10.1111/j.1365-4632.2012.05774.x
12. ICD10Data.com. 2023 ICD-10-CM codes. Accessed February 15, 2023. www.icd10data.com/ICD10CM/codes
► Recent history of hand-foot-mouth disease
► Discolored fingernails and toenails lifting from the proximal end
Should RAAS blockade therapy be continued in patients with advanced renal disease?
Evidence summary
Mixed results, Yes, but no evidence of harm in continuing RAAS therapy
A 2014 cohort study assessed the effect of treatment with angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (ACEIs/ARBs) on all-cause mortality in US veterans (N = 141,413) with non-dialysis chronic kidney disease (CKD)—defined as either a stable estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m2 or a stable eGFR ≥ 60 mL/min/1.73 m2 and an elevated urine microalbumin measurement.1 In an intention-to-treat analysis, ACEI/ARB treatment was associated with a significantly decreased risk for all-cause mortality (hazard ratio [HR] = 0.81; 95% CI, 0.78-0.84).
A 2018 meta-analysis analyzed data from 9 RCTs comparing RAAS blockade therapy to placebo or alternative antihypertensive agents in patients with non-dialysis CKD stages 3 to 5.2 Although the meta-analysis authors focused on patients with comorbid diabetes and non-dialysis CKD (N = 9797), some included studies had a mixed population (ie, only a subset of patients had diabetes). This, among other variances in characteristics, participants, interventions, and endpoints, resulted in different numbers of participants included in the data extraction and analysis of outcomes. Overall, there was no difference between the RAAS group and the control group in terms of all-cause mortality (N = 5309; risk ratio [RR] = 0.97; 95% CI, 0.85-1.10), cardiovascular mortality (N = 3748; RR = 1.03; 95% CI, 0.75-1.41), or adverse events (N = 1822; RR = 1.05; 95% CI, 0.89-1.25). Compared to the control group, the RAAS group was less likely to experience a nonfatal cardiovascular event (N = 6138; RR = 0.90; 95% CI, 0.81-1.00). For the composite endpoint of need for renal replacement therapy/doubling of serum creatinine, RAAS therapy was associated with reduced risk in both the overall population (N = 5202; RR = 0.81; 95% CI, 0.70-0.92) and in patients with comorbid diabetes (N = 3314; RR = 0.78; 95% CI, 0.67-0.90).
A 2022 open-label trial (STOP ACEi) randomly assigned 411 patients with stage 4 or 5 CKD to either continue (N = 205) or discontinue (N = 206) RAAS inhibitor therapy.3 The primary outcome measure was eGFR at 3 years. The difference in the rate of decline in eGFR between groups was –0.7% (95% CI, –2.5 to 1.0; P = .42), favoring the group that continued therapy.
Recommendations from others
After reviewing data from multiple clinical trials, the authors of the 2018 report from the National Kidney Foundation–Kidney Disease Outcomes Quality Initiative (NKF–KDOQI) concluded that the decision to continue or stop RAAS therapy in patients with advanced CKD should be individualized.4 Criteria that should be considered in the decision-making process include the presence or absence of large acute declines in eGFR (> 20% in the absence of a significant decrease in proteinuria), hypotension, or acute kidney injury with significant risk for worsening.
In 2021, the Renal Association and the Association of British Clinical Diabetologists published updated clinical practice guidelines for the management of hypertension and RAAS blockade in adults with diabetic kidney disease.5 Collective data indicated that, although outcomes varied based on type of diabetes (1 vs 2) and degree of proteinuria, blockade therapy overall led to improved outcomes; this was hypothesized to be due to the effects of reduced blood pressure. However, discontinuation of RAAS blockade therapy may be warranted when the patient (1) has a potassium level > 5 mmol/L pretreatment or ≥ 6 mmol/L with treatment, (2) demonstrates a decrease in eGFR > 25% or an increase in serum creatinine > 30% upon initiation of blockade, without another cause of renal deterioration, (3) is pregnant, or (4) has an acute illness with fluid depletion (in which case, RAAS therapy can be restarted 24 to 48 hours after recovery).
Editor’s takeaway
Evidence supports continuation of RAAS blockade, particularly in patients with significant comorbidities (diabetes and cardiovascular disease). Study data indicate continuation is either beneficial or neutral to further morbidity. The only caveat is that these patients should have their renal function and potassium level continuously monitored. The evidence should provide reassurance to patients and physicians that continuation is the correct course of action.
1. Molnar MZ, Kalantar-Zadeh K, Lott EH, et al. Angiotensin-converting enzyme inhibitor, angiotensin receptor blocker use, and mortality in patients with chronic kidney disease. J Am Coll Cardiol. 2014;63:650-658. doi: 10.1016/j.jacc.2013.10.050
2. Nistor I, De Sutter J, Drechsler C, et al. Effect of renin-angiotensin-aldosterone system blockade in adults with diabetes mellitus and advanced chronic kidney disease not on dialysis: a systematic review and meta-analysis. Nephrol Dial Transplant. 2018;33:12-22. doi: 10.1093/ndt/gfx072
3. Bhandari S, Mehta S, Khwaja A, et al. Renin-angiotensin system inhibition in advanced chronic kidney disease. N Engl J Med. 2022;387:2021-2032. doi: 10.1056/NEJMoa2210639
4. Weir MR, Lakkis JI, Jaar B, et al. Use of renin-angiotensin system blockade in advanced CKD: an NKF–KDOQI controversies report. Am J Kidney Dis. 2018;72:873-884. doi: 10.1053/j.ajkd.2018.06.010
5. Banerjee D, Winocour P, Chowdhury TA, et al. Management of hypertension and renin-angiotensin-aldosterone system blockade in adults with diabetic kidney disease: Association of British Clinical Diabetologists and the Renal Association UK guideline update 2021. BMC Nephrol. 2022;23:9. doi: 10.1186/s12882-021-02587-5
Evidence summary
Mixed results, Yes, but no evidence of harm in continuing RAAS therapy
A 2014 cohort study assessed the effect of treatment with angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (ACEIs/ARBs) on all-cause mortality in US veterans (N = 141,413) with non-dialysis chronic kidney disease (CKD)—defined as either a stable estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m2 or a stable eGFR ≥ 60 mL/min/1.73 m2 and an elevated urine microalbumin measurement.1 In an intention-to-treat analysis, ACEI/ARB treatment was associated with a significantly decreased risk for all-cause mortality (hazard ratio [HR] = 0.81; 95% CI, 0.78-0.84).
A 2018 meta-analysis analyzed data from 9 RCTs comparing RAAS blockade therapy to placebo or alternative antihypertensive agents in patients with non-dialysis CKD stages 3 to 5.2 Although the meta-analysis authors focused on patients with comorbid diabetes and non-dialysis CKD (N = 9797), some included studies had a mixed population (ie, only a subset of patients had diabetes). This, among other variances in characteristics, participants, interventions, and endpoints, resulted in different numbers of participants included in the data extraction and analysis of outcomes. Overall, there was no difference between the RAAS group and the control group in terms of all-cause mortality (N = 5309; risk ratio [RR] = 0.97; 95% CI, 0.85-1.10), cardiovascular mortality (N = 3748; RR = 1.03; 95% CI, 0.75-1.41), or adverse events (N = 1822; RR = 1.05; 95% CI, 0.89-1.25). Compared to the control group, the RAAS group was less likely to experience a nonfatal cardiovascular event (N = 6138; RR = 0.90; 95% CI, 0.81-1.00). For the composite endpoint of need for renal replacement therapy/doubling of serum creatinine, RAAS therapy was associated with reduced risk in both the overall population (N = 5202; RR = 0.81; 95% CI, 0.70-0.92) and in patients with comorbid diabetes (N = 3314; RR = 0.78; 95% CI, 0.67-0.90).
A 2022 open-label trial (STOP ACEi) randomly assigned 411 patients with stage 4 or 5 CKD to either continue (N = 205) or discontinue (N = 206) RAAS inhibitor therapy.3 The primary outcome measure was eGFR at 3 years. The difference in the rate of decline in eGFR between groups was –0.7% (95% CI, –2.5 to 1.0; P = .42), favoring the group that continued therapy.
Recommendations from others
After reviewing data from multiple clinical trials, the authors of the 2018 report from the National Kidney Foundation–Kidney Disease Outcomes Quality Initiative (NKF–KDOQI) concluded that the decision to continue or stop RAAS therapy in patients with advanced CKD should be individualized.4 Criteria that should be considered in the decision-making process include the presence or absence of large acute declines in eGFR (> 20% in the absence of a significant decrease in proteinuria), hypotension, or acute kidney injury with significant risk for worsening.
In 2021, the Renal Association and the Association of British Clinical Diabetologists published updated clinical practice guidelines for the management of hypertension and RAAS blockade in adults with diabetic kidney disease.5 Collective data indicated that, although outcomes varied based on type of diabetes (1 vs 2) and degree of proteinuria, blockade therapy overall led to improved outcomes; this was hypothesized to be due to the effects of reduced blood pressure. However, discontinuation of RAAS blockade therapy may be warranted when the patient (1) has a potassium level > 5 mmol/L pretreatment or ≥ 6 mmol/L with treatment, (2) demonstrates a decrease in eGFR > 25% or an increase in serum creatinine > 30% upon initiation of blockade, without another cause of renal deterioration, (3) is pregnant, or (4) has an acute illness with fluid depletion (in which case, RAAS therapy can be restarted 24 to 48 hours after recovery).
Editor’s takeaway
Evidence supports continuation of RAAS blockade, particularly in patients with significant comorbidities (diabetes and cardiovascular disease). Study data indicate continuation is either beneficial or neutral to further morbidity. The only caveat is that these patients should have their renal function and potassium level continuously monitored. The evidence should provide reassurance to patients and physicians that continuation is the correct course of action.
Evidence summary
Mixed results, Yes, but no evidence of harm in continuing RAAS therapy
A 2014 cohort study assessed the effect of treatment with angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (ACEIs/ARBs) on all-cause mortality in US veterans (N = 141,413) with non-dialysis chronic kidney disease (CKD)—defined as either a stable estimated glomerular filtration rate (eGFR) < 60 mL/min/1.73 m2 or a stable eGFR ≥ 60 mL/min/1.73 m2 and an elevated urine microalbumin measurement.1 In an intention-to-treat analysis, ACEI/ARB treatment was associated with a significantly decreased risk for all-cause mortality (hazard ratio [HR] = 0.81; 95% CI, 0.78-0.84).
A 2018 meta-analysis analyzed data from 9 RCTs comparing RAAS blockade therapy to placebo or alternative antihypertensive agents in patients with non-dialysis CKD stages 3 to 5.2 Although the meta-analysis authors focused on patients with comorbid diabetes and non-dialysis CKD (N = 9797), some included studies had a mixed population (ie, only a subset of patients had diabetes). This, among other variances in characteristics, participants, interventions, and endpoints, resulted in different numbers of participants included in the data extraction and analysis of outcomes. Overall, there was no difference between the RAAS group and the control group in terms of all-cause mortality (N = 5309; risk ratio [RR] = 0.97; 95% CI, 0.85-1.10), cardiovascular mortality (N = 3748; RR = 1.03; 95% CI, 0.75-1.41), or adverse events (N = 1822; RR = 1.05; 95% CI, 0.89-1.25). Compared to the control group, the RAAS group was less likely to experience a nonfatal cardiovascular event (N = 6138; RR = 0.90; 95% CI, 0.81-1.00). For the composite endpoint of need for renal replacement therapy/doubling of serum creatinine, RAAS therapy was associated with reduced risk in both the overall population (N = 5202; RR = 0.81; 95% CI, 0.70-0.92) and in patients with comorbid diabetes (N = 3314; RR = 0.78; 95% CI, 0.67-0.90).
A 2022 open-label trial (STOP ACEi) randomly assigned 411 patients with stage 4 or 5 CKD to either continue (N = 205) or discontinue (N = 206) RAAS inhibitor therapy.3 The primary outcome measure was eGFR at 3 years. The difference in the rate of decline in eGFR between groups was –0.7% (95% CI, –2.5 to 1.0; P = .42), favoring the group that continued therapy.
Recommendations from others
After reviewing data from multiple clinical trials, the authors of the 2018 report from the National Kidney Foundation–Kidney Disease Outcomes Quality Initiative (NKF–KDOQI) concluded that the decision to continue or stop RAAS therapy in patients with advanced CKD should be individualized.4 Criteria that should be considered in the decision-making process include the presence or absence of large acute declines in eGFR (> 20% in the absence of a significant decrease in proteinuria), hypotension, or acute kidney injury with significant risk for worsening.
In 2021, the Renal Association and the Association of British Clinical Diabetologists published updated clinical practice guidelines for the management of hypertension and RAAS blockade in adults with diabetic kidney disease.5 Collective data indicated that, although outcomes varied based on type of diabetes (1 vs 2) and degree of proteinuria, blockade therapy overall led to improved outcomes; this was hypothesized to be due to the effects of reduced blood pressure. However, discontinuation of RAAS blockade therapy may be warranted when the patient (1) has a potassium level > 5 mmol/L pretreatment or ≥ 6 mmol/L with treatment, (2) demonstrates a decrease in eGFR > 25% or an increase in serum creatinine > 30% upon initiation of blockade, without another cause of renal deterioration, (3) is pregnant, or (4) has an acute illness with fluid depletion (in which case, RAAS therapy can be restarted 24 to 48 hours after recovery).
Editor’s takeaway
Evidence supports continuation of RAAS blockade, particularly in patients with significant comorbidities (diabetes and cardiovascular disease). Study data indicate continuation is either beneficial or neutral to further morbidity. The only caveat is that these patients should have their renal function and potassium level continuously monitored. The evidence should provide reassurance to patients and physicians that continuation is the correct course of action.
1. Molnar MZ, Kalantar-Zadeh K, Lott EH, et al. Angiotensin-converting enzyme inhibitor, angiotensin receptor blocker use, and mortality in patients with chronic kidney disease. J Am Coll Cardiol. 2014;63:650-658. doi: 10.1016/j.jacc.2013.10.050
2. Nistor I, De Sutter J, Drechsler C, et al. Effect of renin-angiotensin-aldosterone system blockade in adults with diabetes mellitus and advanced chronic kidney disease not on dialysis: a systematic review and meta-analysis. Nephrol Dial Transplant. 2018;33:12-22. doi: 10.1093/ndt/gfx072
3. Bhandari S, Mehta S, Khwaja A, et al. Renin-angiotensin system inhibition in advanced chronic kidney disease. N Engl J Med. 2022;387:2021-2032. doi: 10.1056/NEJMoa2210639
4. Weir MR, Lakkis JI, Jaar B, et al. Use of renin-angiotensin system blockade in advanced CKD: an NKF–KDOQI controversies report. Am J Kidney Dis. 2018;72:873-884. doi: 10.1053/j.ajkd.2018.06.010
5. Banerjee D, Winocour P, Chowdhury TA, et al. Management of hypertension and renin-angiotensin-aldosterone system blockade in adults with diabetic kidney disease: Association of British Clinical Diabetologists and the Renal Association UK guideline update 2021. BMC Nephrol. 2022;23:9. doi: 10.1186/s12882-021-02587-5
1. Molnar MZ, Kalantar-Zadeh K, Lott EH, et al. Angiotensin-converting enzyme inhibitor, angiotensin receptor blocker use, and mortality in patients with chronic kidney disease. J Am Coll Cardiol. 2014;63:650-658. doi: 10.1016/j.jacc.2013.10.050
2. Nistor I, De Sutter J, Drechsler C, et al. Effect of renin-angiotensin-aldosterone system blockade in adults with diabetes mellitus and advanced chronic kidney disease not on dialysis: a systematic review and meta-analysis. Nephrol Dial Transplant. 2018;33:12-22. doi: 10.1093/ndt/gfx072
3. Bhandari S, Mehta S, Khwaja A, et al. Renin-angiotensin system inhibition in advanced chronic kidney disease. N Engl J Med. 2022;387:2021-2032. doi: 10.1056/NEJMoa2210639
4. Weir MR, Lakkis JI, Jaar B, et al. Use of renin-angiotensin system blockade in advanced CKD: an NKF–KDOQI controversies report. Am J Kidney Dis. 2018;72:873-884. doi: 10.1053/j.ajkd.2018.06.010
5. Banerjee D, Winocour P, Chowdhury TA, et al. Management of hypertension and renin-angiotensin-aldosterone system blockade in adults with diabetic kidney disease: Association of British Clinical Diabetologists and the Renal Association UK guideline update 2021. BMC Nephrol. 2022;23:9. doi: 10.1186/s12882-021-02587-5
EVIDENCE-BASED REVIEW:
PROBABLY. Renin-angiotensin- aldosterone system (RAAS) blockade therapy should be continued in most patients with advanced renal disease and comorbid conditions; however, individualized treatment is warranted as data on the benefits and harms in all-cause mortality, cardiovascular mortality, and risk for renal replacement therapy are inconclusive (strength of recommendation [SOR]: B, based on observational studies, systematic reviews, and meta-analyses of randomized controlled trials [RCTs]). Certain patient populations, such as patients with diabetes or those with cardiovascular risk or history, may benefit most from continued RAAS blockade therapy (SOR: A, based on systematic reviews and meta-analyses of RCTs).
Sports: An underutilized tool for patients with disabilities
Approximately 6.5 million people in the United States have an intellectual disability, the most common type of developmental disability.1 People with disabilities are 3 times more likely to have heart disease, stroke, or diabetes than adults without disabilities.2
Sports as a treatment modality are not used to full advantage to combat these conditions in people with intellectual/developmental disabilities (IDDs). Participation in sport activities can lead to weight loss, reduce risk for cardiovascular disease, and optimize physical health. Sports also can help enhance social and communication skills and improve quality of life for this patient population (TABLE).3-6
However, a 2014 report found that while inactive adults with disabilities (hearing, vision, cognition, mobility) were 50% more likely to report 1 or more chronic diseases than those who were physically active, only 44% of adults with disabilities who visited a health professional in the previous 12 months received a physical activity recommendation.7 In addition, more than 50% of adults with disabilities are not meeting US recommended exercise guidelines.7-9
Family physicians may not feel they have adequate training to counsel patients with IDDs. Additional limiting factors include dependence on caregivers for exercise participation, expense, transportation difficulties, a lack of choice in sporting activities, and the patient’s level of motivation.10The guidance reviewed here details how to modify the pre-participation sports physical exam specifically for patients with IDDs. It also provides sport and exercise recommendations for patients with 3 disabilities: Down syndrome, cerebral palsy, and autism spectrum disorder.
Worth noting: As is true for adults without disabilities, those with IDDs should participate in at least 150 minutes of moderate-intensity, or 75 minutes of vigorous intensity, aerobic physical activity each week.9 Recommend muscle-strengthening activities be performed at least 2 days each week.9
Exercise recommendations for patients with Down syndrome
One in every 700 babies receives a diagnosis of Down syndrome.11 Among its many possible manifestations—which include intellectual disability, heart disease, and diabetes—Down syndrome is associated with an increased risk for obesity, which makes exercise an extremely important lifestyle modification for these patients. Obesity can lead to obstructive sleep apnea causing cor pulmonale and even premature death. Continuous positive airway pressure intervention can be difficult in terms of patient compliance. However, weight loss through exercise and sports is an effective intervention to mitigate these obesity-related health comorbidities.
Pre-participation exam. A focused history and physical exam are often conducted before a patient engages in organized competitive or recreational sports. The pre-participation sports physical exam typically focuses on cardiac, neurologic, hereditary, and musculoskeletal disorders. While we recommend including these baseline elements as part of the exam for patients with disabilities, we also recommend modifying the exam to include disability-specific screening for associated comorbidities.
Continue to: For patients with Down syndrome...
For patients with Down syndrome, a complete pre-participation sports physical exam is warranted. Inquire specifically about neck pain or dislocations, heart murmur, cardiac surgery, seizures, sleep issues, history of congenital abdominal defect, hematologic malignancy, and bone pain as part of the focused physical exam.
Look for evidence of patellofemoral instability, pes planus, scoliosis, hallux deformities, decreased muscle tone, and muscular weakness. Check for cataracts and perform a thorough cardiovascular exam to assess for murmur or signs of chronic hypoxia, such as cyanosis. If a heart murmur is detected, refer the patient to a cardiologist.
Patients with Down syndrome are also at increased risk for atlantoaxial instability. A thorough neurologic evaluation to screen for this condition is indicated; however, routine radiologic screening is not needed.12
An annual complete blood cell count and thyroid-stimulating hormone test are recommended for all children with Down syndrome.13 For patients with Down syndrome who are 13 to 21 years of age, an echocardiogram also is recommended for concerning symptoms.13 Ferritin levels also should be assessed annually for patients who are younger than 13 years of age to check for iron-deficiency anemia.13Consider high-risk screening strategies for patients with diabetes and metabolic syndrome.
Special considerations. Patients with Down syndrome were found to be injured more frequently than individuals with other disabilities during the Special Olympics.14 These patients may be hypersensitive to pain with prolonged pain responses, or unable to verbally communicate their pain or injury.15
Continue to: The complexity of pain assessment...
The complexity of pain assessment in patients with Down syndrome may increase the difficulty of accurately diagnosing an injury, leading to underdiagnosis or overdiagnosis. To increase accuracy of pain assessment in this setting, we recommend using the Wong-Baker FACES Pain Rating Scale or a numeric pain rating scale in verbal patients.15 In nonverbal patients, facial expressions are reliable indicators of pain.
Which exercise? Healthy patients with Down syndrome can participate in any sport. Aerobic exercise can help lower body fat, reduce oxidative stress, and improve blood flow.6 Muscle-strengthening exercises can lead to improved daily functioning and balance. Strength training and aerobic exercise benefit aging patients with Down syndrome who are struggling with obesity. Such exercise also helps increase bone mineral density and improve cardiovascular fitness, especially when initiated at a young age. Consistent exercise promotes positive health outcomes throughout the lifespan.16
Exercise recommendations for patients with cerebral palsy
Cerebral palsy, the most common motor disability in children, is associated with intellectual disability, seizures, respiratory insufficiency, scoliosis, osteoporosis, mood disorders, dysphagia, and speech and hearing impairment.17 The increasing survival of premature babies born with cerebral palsy and the growing prevalence of adults with the condition point to the importance of expanding one’s knowledge of how best to care for this population.18
Pre-participation exam. In addition to a complete sport physical exam, it’s important to further evaluate patients with cerebral palsy for epilepsy, joint contractures, muscle weakness, spinal deformities, and respiratory insufficiency. The Gross Motor Function Classification system, commonly used for patients with cerebral palsy, scores functional ability in 5 levels.18 Patients at Level I are the most mobile; patients at Level V need wheelchair transport in all settings.
Further evaluation of spinal deformities can be initiated with x-ray screening. Consider ordering dual x-ray absorptiometry scans to evaluate bone mass.17
Continue to: Special considerations
Special considerations. Patients with cerebral palsy have a heightened risk for depression and anxiety.19 Mental health can be assessed via the General Anxiety Disorder-7, the Patient Health Questionnaire-9, and the Ask Suicide-Screening Questions tools, among others. Mental health screening may need to be adjusted depending on the patient’s level of cognition and ability to communicate. The patient’s caregiver also can provide supplemental information.
Consider screening vitamin D levels in patients with cerebral palsy. Approximately 50% of adults with cerebral palsy are vitamin D–deficient secondary to sedentary behavior and lack of sun exposure.20-22
Optimal medical management has been shown to decrease muscle spasticity and may be beneficial before initiating an exercise program. For patients with moderate-to-severe symptoms, referral for physical therapy to further improve gross motor function and spasticity may be required before initiating an exercise program.
Which exercise? Individuals with cerebral palsy spend 76% to 99% of their waking hours being sedentary.5 Consequently, they typically have decreased cardiorespiratory endurance and decreased muscle strength. Strength training may improve muscle spasticity, gross motor function, joint health, and respiratory insufficiency.5 Even in those who function at Level IV-V of the Gross Motor Function Classification system, exercise reduces vertebral fractures and improves time spent standing.23 By improving endurance, spasticity, and strength with exercise, deconditioning can be mitigated.
Involvement in sports promotes peer interactions, personal interests, and positive self-identity. It can give a newfound passion for life. Additionally, families of children with disabilities who engage in leisure activities together have less caregiver burden.24,25 Sporting activities offer a way to optimize psychosocial well-being for the patient and the entire family.
Continue to: Dance promotes functionality...
Dance promotes functionality and psychosocial adjustment.26 Hippotherapy, defined as therapy and rehabilitation during which the patient interacts with horses, can diminish muscle spasticity.27 Aquatic therapy also may increase muscle strength.28
Sports for patients with autism spectrum disorder
Autism spectrum disorder is defined as persistent deficits in social communication and social interaction that are usually evident in the first 3 years of life.29 Autism can manifest with or without intellectual or language impairment. Patients with autism commonly have difficulty processing sensory stimuli and can experience “sensory overload.” More than half have a coexisting mental health disorder, such as attention-deficit/hyperactivity disorder, anxiety, depression, schizophrenia, or bipolar disorder.30
Aversions to foods and food selectivity, as well as adverse effects from medical treatment of autism-related agitation, result in a higher incidence of obesity in patients with autism.31,32
Pre-participation exam. In addition to a comprehensive pre-participation exam, the Autism Spectrum Syndrome Questionnaire (ASSQ) and Modified Checklist for Autism in Toddlers are tools to screen school-age children with normal cognition to mild intellectual disability.33 These questionnaires have limitations, however. For example, ASSQ has limited ability to identify the female autistic phenotype.34 As such, these are solely screening tools. Final diagnosis is based on clinical judgment.
Special considerations. Include screening for constipation or diarrhea, fiber intake, food aversions, and common mental health comorbidities using Diagnostic and Statistical Manual of Mental Disorders-Fifth Edition criteria.29 Psychiatric referral may be necessary if certain previously undiagnosed condition(s) become apparent. The patient’s caregiver can provide supplemental information
Continue to: During the physical exam...
During the physical exam, limit sensory stimuli as much as possible, including lights and sounds. Verbalize components of the exam before touching a patient with autism who is sensitive to physical touch.
Which exercise? Participation in sports is an effective therapy for autism and can help patients develop communication skills and promote socialization. Vigorous exercise is associated with a reduction in stereotypic behaviors, hyperactivity, aggression, and self-injury.3 Sports also can offer an alternative channel for social interaction. Children with autism may have impaired or delayed motor skills, and exercise can improve motor skill proficiency.4
The prevalence of feeding problems in children with autism spectrum disorder is estimated to be as high as 90%, and close to 70% are selective eaters.31,35,36 For those with gastrointestinal disorders, exercise can exert positive effects on the microbiome-gut-brain axis.37 Additionally, patients with autism are much more likely to be overweight or obese.32 Physical activity offers those with autism health benefits similar to those for the general population.32
Children with autism spectrum disorder have similar odds of injury, including serious injury, relative to population controls.38 Karate and swimming are among the most researched sports therapy options for patients with autism.38-40 Both are shown to improve motor ability and reduce communication deficits.
Summing up
The literature, although limited, demonstrates that exercise and sports improve the health and well-being of people with IDDs throughout the lifespan, especially if childhood exercise/sports involvement is maintained.
Encourage your patients to participate in sports, but be aware of factors that can limit (or facilitate) participation.41 Exercise participation increases based on, among other things, the individual’s desire to be fit and active, skills practice, peer involvement, family support, accessible facilities, and skilled staff.10
Additional resources that can help people with IDDs access sports and recreational activities include the Special Olympics; Paralympics; YMCA; after-school programs; The American College of Sports Medicine; The National Center on Health, Physical Activity, and Disability; and disability-certified inclusive fitness trainers.
CORRESPONDENCE
Kristina Jones, BS, 12901 Bruce B. Downs Boulevard, Tampa, FL 33612; [email protected]
1. CDC. Addressing gaps in healthcare for individuals with intellectual disabilities. Updated October 15, 2019. Accessed January 21, 2023. www.cdc.gov/grand-rounds/pp/2019/20191015-intellectual-disabilities.html
2. CDC. Vital signs: adults with disabilities. Physical activity is for everybody. Updated November 16, 2018. Accessed January 21, 2023. www.cdc.gov/vitalsigns/disabilities/index.html
3. Di Palma D, Molisso V. Sport for autism. J Humanities Soc Pol. 2017;3:42-49.
4. Pan CY, Chu CH, Tsai CL, et al. The impacts of physical activity intervention on physical and cognitive outcomes in children with autism spectrum disorder. Autism. 2017;21:190-202. doi: 10.1177/1362361316633562
5. Verschuren O, Peterson MD, Balemans AC, et al. Exercise and physical activity recommendations for people with cerebral palsy. Dev Med Child Neurol. 2016;58:798-808. doi: 10.1111/dmcn.13053
6. Paul Y, Ellapen TJ, Barnard M, et al. The health benefits of exercise therapy for patients with Down syndrome: a systematic review. Afr J Disabil. 2019;8:576. doi: 10.4102/ajod.v8i0.576
7. Carroll DD, Courtney-Long EA, Stevens AC, et al. Vital signs: disability and physical activity—United States, 2009-2012. MMWR Morb Mortal Wkly Rep. 2014;63:407-413.
8. Rimmer JH. Physical activity for people with disabilities: how do we reach those with the greatest need? NAM Perspectives. Published April 6, 2015. Accessed March 23, 2023. https://nam.edu/perspectives-2015-physical-activity-for-people-with-disabilities-how-do-we-reach-those-with-the-greatest-need/
9. Department of Health and Human Services. Physical Activity Guidelines For Americans. 2nd edition. Published 2018. Accessed March 23, 2023. https://health.gov/sites/default/files/2019-09/Physical_Activity_Guidelines_2nd_edition.pdf
10. Darcy S, Dowse L. In search of a level playing field—the constraints and benefits of sport participation for people with intellectual disability. Disabil Soc. 2013;28:393-407. doi: 10.1080/ 09687599.2012.714258
11. Mai CT, Isenburg JL, Canfield MA, et al. National population‐based estimates for major birth defects, 2010-2014. Birth Defects Res. 2019;111:1420-1435. doi: 10.1002/bdr2.1589
12. MyŚliwiec A, Posłuszny A, Saulicz E, et al. Atlanto-axial instability in people with Down’s syndrome and its impact on the ability to perform sports activities—a review. J Hum Kinet. 2015;48:17-24. doi: 10.1515/hukin-2015-0087
13. Bunt CW, Bunt SK. Role of the family physician in the care of children with Down syndrome. Am Fam Physician. 2014;90:851-858.
14. McCormick DP, Niebuhr VN, Risser WL. Injury and illness surveillance at local Special Olympic Games. Br J Sports Med. 1990; 24:221-224. doi: 10.1136/bjsm.24.4.221
15. McGuire BE, Defrin R. Pain perception in people with Down syndrome: a synthesis of clinical and experimental research. Front Behav Neurosci. 2015;9. doi: 10.3389/fnbeh.2015.00194
16. Barnhart RC, Connolly B. Aging and Down syndrome: implications for physical therapy. Phys Ther. 2007;87:1399-1406. doi: 10.2522/ptj.20060334
17. Vitrikas K, Dalton H, Breish D. Cerebral palsy: an overview. Am Fam Physician. 2020;101:213-220.
18. Maenner MJ, Blumberg SJ, Kogan MD, et al. Prevalence of cerebral palsy and intellectual disability among children identified in two US national surveys, 2011-2013. Ann Epidemiol. 2016;26:222-226. doi: 10.1016/j.annepidem.2016.01.001
19. Smith KJ, Peterson MD, O’Connell NE, et al. Risk of depression and anxiety in adults with cerebral palsy. JAMA Neurol. 2019;76;294-300. doi: 10.1001/jamaneurol.2018.4147
20. Peterson MD, Haapala HJ, Chaddha A, et al. Abdominal obesity is an independent predictor of serum 25-hydroxyvitamin D deficiency in adults with cerebral palsy. Nutr Metab (Lond). 2014;11:22. doi: 10.1186/1743-7075-11-22
21. Yi YG, Jung SH, Bang MS. Emerging issues in cerebral palsy associated with aging: a physiatrist perspective. Ann Rehabil Med. 2019;43:241-249. doi: 10.5535/arm.2019.43.3.241
22. Sarathy K, Doshi C, Aroojis A. Clinical examination of children with cerebral palsy. Indian J Orthop. 2019;53:35-44. doi: 10.4103/ortho.IJOrtho_409_17
23. Caulton JM, Ward KA, Alsop CW, et al. A randomised controlled trial of standing programme on bone mineral density in non-ambulant children with cerebral palsy. Arch Dis Child. 2004;89:131-135. doi: 10.1136/adc.2002.009316
24. Clutterbuck G, Auld M, Johnston L. Active exercise interventions improve gross motor function of ambulant/semi-ambulant children with cerebral palsy: a systematic review. Disabil Rehabil. 2019;41:1131-1151. doi: 10.1080/09638288.2017.1422035
25. Shikako-Thomas K, Majnemer A, Law M, et al. Determinants of participation in leisure activities in children and youth with cerebral palsy: systematic review. Phys Occup Ther Pedi. 2008;28:155-169. doi: 10.1080/01942630802031834
26. Teixeira-Machado L, Azevedo-Santos I, DeSantana JM. Dance improves functionality and psychosocial adjustment in cerebral palsy: a randomized controlled clinical trial. Am J Phys Med Rehabil. 2017;96:424-429. doi: 10.1097/PHM.0000000000000646
27. Lucena-Antón D, Rosety-Rodríguez I, Moral-Munoz JA. Effects of a hippotherapy intervention on muscle spasticity in children with cerebral palsy: a randomized controlled trial. Complement Ther Clin Pract. 2018;31:188-192. doi: 10.1016/j.ctcp.2018.02.013
28. Roostaei M, Baharlouei H, Azadi H, et al. Effects of aquatic intervention on gross motor skills in children with cerebral palsy: a systematic review. Phys Occup Ther Pediatr. 2017;37:496-515. doi: 10.1080/01942638.2016.1247938
29. American Psychiatric Association. Autism spectrum disorder, section II. Diagnostic and Statistical Manual of Mental Disorders. 5th ed. 2013:50-56.
30. Romero M, Aguilar JM, Del-Rey-Mejías Á, et al. Psychiatric comorbidities in autism spectrum disorder: a comparative study between DSM-IV-TR and DSM-5 diagnosis. Int J Clin Health Psychol. 2016;16:266-275. doi: 10.1016/j.ijchp.2016.03.001
31. Volkert VM, Vaz PC. Recent studies on feeding problems in children with autism. J Appl Behav Anal. 2015;43:155-159. doi: 10.1901/jaba.2010.43-155
32. Broder-Fingert S, Brazauskas K, Lindgren K, et al. Prevalence of overweight and obesity in a large clinical sample of children with autism. Acad Pediatr. 2014;14:408-414. doi: 10.1016/j.acap.2014.04.004. PMID: 24976353
33. Adachi M, Takahashi M, Takayanagi N, et al. Adaptation of the Autism Spectrum Screening Questionnaire (ASSQ) to preschool children. PLoS One. 2018;10;13:e0199590. doi: 10.1371/journal.pone.0199590
34. Kopp S. Gillberg C. The Autism Spectrum Screening Questionnaire (ASSQ)-Revised Extended Version (ASSQ-REV): an instrument for better capturing the autism phenotype in girls? A preliminary study involving 191 clinical cases and community controls. Res Develop Disabil. 2011:32: 2875-2888.
35. Kotak T. Piazza CC. Assessment and behavioral treatment of feeding and sleeping disorders in children with autism spectrum disorders. Child Adol Psych Clin North Am. 2008;17:887-905. doi: 10.1016/j.chc.2008.06.005
36. Twachtman-Reilly J, Amaral SC, Zebrowski PP. Addressing feeding behaviors in children on the autism spectrum in school-based settings: physiological and behavioral issues. Lang Speech Hear Serv Sch. 2008:39:261-272. doi: 10.1044/0161-1461(2008/025)
37. Dalton A, Mermier C, Zuhl M. Exercise influence on the microbiome-gut-brain axis. Gut Microbes. 2019;10:555-568. doi: 10.1080/19490976.2018.1562268
38. Iliadis I, Apteslis N. The role of physical education and exercise for children with autism spectrum disorder and the effects on socialization, communication, behavior, fitness, and quality of life. Dial Clin Neurosc Mental Health. 2020;3:71-78. doi: 10.26386/obrela.v3i1.178
39. Phung JN, Goldberg WA. Promoting executive functioning in children with autism spectrum disorder through mixed martial arts training. J Autism Dev Dis. 2019;49:3660-3684. doi: 10.1007/s10803-019-04072-3
40. Bahrami F, Movahedi A, Marandi SM, et al. The effect of karate techniques training on communication deficit of children with autism spectrum disorder. J Autism Dev Disord. 2016;46: 978-986. doi: 10.1007/s10803-015-2643-y
41. Shields N, Synnot A. Perceived barriers and facilitators to participation in physical activity for children with disability: a qualitative study. BMC Pediatr. 2016;16:9. doi: 10.1186/s12887-016-0544-7
Approximately 6.5 million people in the United States have an intellectual disability, the most common type of developmental disability.1 People with disabilities are 3 times more likely to have heart disease, stroke, or diabetes than adults without disabilities.2
Sports as a treatment modality are not used to full advantage to combat these conditions in people with intellectual/developmental disabilities (IDDs). Participation in sport activities can lead to weight loss, reduce risk for cardiovascular disease, and optimize physical health. Sports also can help enhance social and communication skills and improve quality of life for this patient population (TABLE).3-6
However, a 2014 report found that while inactive adults with disabilities (hearing, vision, cognition, mobility) were 50% more likely to report 1 or more chronic diseases than those who were physically active, only 44% of adults with disabilities who visited a health professional in the previous 12 months received a physical activity recommendation.7 In addition, more than 50% of adults with disabilities are not meeting US recommended exercise guidelines.7-9
Family physicians may not feel they have adequate training to counsel patients with IDDs. Additional limiting factors include dependence on caregivers for exercise participation, expense, transportation difficulties, a lack of choice in sporting activities, and the patient’s level of motivation.10The guidance reviewed here details how to modify the pre-participation sports physical exam specifically for patients with IDDs. It also provides sport and exercise recommendations for patients with 3 disabilities: Down syndrome, cerebral palsy, and autism spectrum disorder.
Worth noting: As is true for adults without disabilities, those with IDDs should participate in at least 150 minutes of moderate-intensity, or 75 minutes of vigorous intensity, aerobic physical activity each week.9 Recommend muscle-strengthening activities be performed at least 2 days each week.9
Exercise recommendations for patients with Down syndrome
One in every 700 babies receives a diagnosis of Down syndrome.11 Among its many possible manifestations—which include intellectual disability, heart disease, and diabetes—Down syndrome is associated with an increased risk for obesity, which makes exercise an extremely important lifestyle modification for these patients. Obesity can lead to obstructive sleep apnea causing cor pulmonale and even premature death. Continuous positive airway pressure intervention can be difficult in terms of patient compliance. However, weight loss through exercise and sports is an effective intervention to mitigate these obesity-related health comorbidities.
Pre-participation exam. A focused history and physical exam are often conducted before a patient engages in organized competitive or recreational sports. The pre-participation sports physical exam typically focuses on cardiac, neurologic, hereditary, and musculoskeletal disorders. While we recommend including these baseline elements as part of the exam for patients with disabilities, we also recommend modifying the exam to include disability-specific screening for associated comorbidities.
Continue to: For patients with Down syndrome...
For patients with Down syndrome, a complete pre-participation sports physical exam is warranted. Inquire specifically about neck pain or dislocations, heart murmur, cardiac surgery, seizures, sleep issues, history of congenital abdominal defect, hematologic malignancy, and bone pain as part of the focused physical exam.
Look for evidence of patellofemoral instability, pes planus, scoliosis, hallux deformities, decreased muscle tone, and muscular weakness. Check for cataracts and perform a thorough cardiovascular exam to assess for murmur or signs of chronic hypoxia, such as cyanosis. If a heart murmur is detected, refer the patient to a cardiologist.
Patients with Down syndrome are also at increased risk for atlantoaxial instability. A thorough neurologic evaluation to screen for this condition is indicated; however, routine radiologic screening is not needed.12
An annual complete blood cell count and thyroid-stimulating hormone test are recommended for all children with Down syndrome.13 For patients with Down syndrome who are 13 to 21 years of age, an echocardiogram also is recommended for concerning symptoms.13 Ferritin levels also should be assessed annually for patients who are younger than 13 years of age to check for iron-deficiency anemia.13Consider high-risk screening strategies for patients with diabetes and metabolic syndrome.
Special considerations. Patients with Down syndrome were found to be injured more frequently than individuals with other disabilities during the Special Olympics.14 These patients may be hypersensitive to pain with prolonged pain responses, or unable to verbally communicate their pain or injury.15
Continue to: The complexity of pain assessment...
The complexity of pain assessment in patients with Down syndrome may increase the difficulty of accurately diagnosing an injury, leading to underdiagnosis or overdiagnosis. To increase accuracy of pain assessment in this setting, we recommend using the Wong-Baker FACES Pain Rating Scale or a numeric pain rating scale in verbal patients.15 In nonverbal patients, facial expressions are reliable indicators of pain.
Which exercise? Healthy patients with Down syndrome can participate in any sport. Aerobic exercise can help lower body fat, reduce oxidative stress, and improve blood flow.6 Muscle-strengthening exercises can lead to improved daily functioning and balance. Strength training and aerobic exercise benefit aging patients with Down syndrome who are struggling with obesity. Such exercise also helps increase bone mineral density and improve cardiovascular fitness, especially when initiated at a young age. Consistent exercise promotes positive health outcomes throughout the lifespan.16
Exercise recommendations for patients with cerebral palsy
Cerebral palsy, the most common motor disability in children, is associated with intellectual disability, seizures, respiratory insufficiency, scoliosis, osteoporosis, mood disorders, dysphagia, and speech and hearing impairment.17 The increasing survival of premature babies born with cerebral palsy and the growing prevalence of adults with the condition point to the importance of expanding one’s knowledge of how best to care for this population.18
Pre-participation exam. In addition to a complete sport physical exam, it’s important to further evaluate patients with cerebral palsy for epilepsy, joint contractures, muscle weakness, spinal deformities, and respiratory insufficiency. The Gross Motor Function Classification system, commonly used for patients with cerebral palsy, scores functional ability in 5 levels.18 Patients at Level I are the most mobile; patients at Level V need wheelchair transport in all settings.
Further evaluation of spinal deformities can be initiated with x-ray screening. Consider ordering dual x-ray absorptiometry scans to evaluate bone mass.17
Continue to: Special considerations
Special considerations. Patients with cerebral palsy have a heightened risk for depression and anxiety.19 Mental health can be assessed via the General Anxiety Disorder-7, the Patient Health Questionnaire-9, and the Ask Suicide-Screening Questions tools, among others. Mental health screening may need to be adjusted depending on the patient’s level of cognition and ability to communicate. The patient’s caregiver also can provide supplemental information.
Consider screening vitamin D levels in patients with cerebral palsy. Approximately 50% of adults with cerebral palsy are vitamin D–deficient secondary to sedentary behavior and lack of sun exposure.20-22
Optimal medical management has been shown to decrease muscle spasticity and may be beneficial before initiating an exercise program. For patients with moderate-to-severe symptoms, referral for physical therapy to further improve gross motor function and spasticity may be required before initiating an exercise program.
Which exercise? Individuals with cerebral palsy spend 76% to 99% of their waking hours being sedentary.5 Consequently, they typically have decreased cardiorespiratory endurance and decreased muscle strength. Strength training may improve muscle spasticity, gross motor function, joint health, and respiratory insufficiency.5 Even in those who function at Level IV-V of the Gross Motor Function Classification system, exercise reduces vertebral fractures and improves time spent standing.23 By improving endurance, spasticity, and strength with exercise, deconditioning can be mitigated.
Involvement in sports promotes peer interactions, personal interests, and positive self-identity. It can give a newfound passion for life. Additionally, families of children with disabilities who engage in leisure activities together have less caregiver burden.24,25 Sporting activities offer a way to optimize psychosocial well-being for the patient and the entire family.
Continue to: Dance promotes functionality...
Dance promotes functionality and psychosocial adjustment.26 Hippotherapy, defined as therapy and rehabilitation during which the patient interacts with horses, can diminish muscle spasticity.27 Aquatic therapy also may increase muscle strength.28
Sports for patients with autism spectrum disorder
Autism spectrum disorder is defined as persistent deficits in social communication and social interaction that are usually evident in the first 3 years of life.29 Autism can manifest with or without intellectual or language impairment. Patients with autism commonly have difficulty processing sensory stimuli and can experience “sensory overload.” More than half have a coexisting mental health disorder, such as attention-deficit/hyperactivity disorder, anxiety, depression, schizophrenia, or bipolar disorder.30
Aversions to foods and food selectivity, as well as adverse effects from medical treatment of autism-related agitation, result in a higher incidence of obesity in patients with autism.31,32
Pre-participation exam. In addition to a comprehensive pre-participation exam, the Autism Spectrum Syndrome Questionnaire (ASSQ) and Modified Checklist for Autism in Toddlers are tools to screen school-age children with normal cognition to mild intellectual disability.33 These questionnaires have limitations, however. For example, ASSQ has limited ability to identify the female autistic phenotype.34 As such, these are solely screening tools. Final diagnosis is based on clinical judgment.
Special considerations. Include screening for constipation or diarrhea, fiber intake, food aversions, and common mental health comorbidities using Diagnostic and Statistical Manual of Mental Disorders-Fifth Edition criteria.29 Psychiatric referral may be necessary if certain previously undiagnosed condition(s) become apparent. The patient’s caregiver can provide supplemental information
Continue to: During the physical exam...
During the physical exam, limit sensory stimuli as much as possible, including lights and sounds. Verbalize components of the exam before touching a patient with autism who is sensitive to physical touch.
Which exercise? Participation in sports is an effective therapy for autism and can help patients develop communication skills and promote socialization. Vigorous exercise is associated with a reduction in stereotypic behaviors, hyperactivity, aggression, and self-injury.3 Sports also can offer an alternative channel for social interaction. Children with autism may have impaired or delayed motor skills, and exercise can improve motor skill proficiency.4
The prevalence of feeding problems in children with autism spectrum disorder is estimated to be as high as 90%, and close to 70% are selective eaters.31,35,36 For those with gastrointestinal disorders, exercise can exert positive effects on the microbiome-gut-brain axis.37 Additionally, patients with autism are much more likely to be overweight or obese.32 Physical activity offers those with autism health benefits similar to those for the general population.32
Children with autism spectrum disorder have similar odds of injury, including serious injury, relative to population controls.38 Karate and swimming are among the most researched sports therapy options for patients with autism.38-40 Both are shown to improve motor ability and reduce communication deficits.
Summing up
The literature, although limited, demonstrates that exercise and sports improve the health and well-being of people with IDDs throughout the lifespan, especially if childhood exercise/sports involvement is maintained.
Encourage your patients to participate in sports, but be aware of factors that can limit (or facilitate) participation.41 Exercise participation increases based on, among other things, the individual’s desire to be fit and active, skills practice, peer involvement, family support, accessible facilities, and skilled staff.10
Additional resources that can help people with IDDs access sports and recreational activities include the Special Olympics; Paralympics; YMCA; after-school programs; The American College of Sports Medicine; The National Center on Health, Physical Activity, and Disability; and disability-certified inclusive fitness trainers.
CORRESPONDENCE
Kristina Jones, BS, 12901 Bruce B. Downs Boulevard, Tampa, FL 33612; [email protected]
Approximately 6.5 million people in the United States have an intellectual disability, the most common type of developmental disability.1 People with disabilities are 3 times more likely to have heart disease, stroke, or diabetes than adults without disabilities.2
Sports as a treatment modality are not used to full advantage to combat these conditions in people with intellectual/developmental disabilities (IDDs). Participation in sport activities can lead to weight loss, reduce risk for cardiovascular disease, and optimize physical health. Sports also can help enhance social and communication skills and improve quality of life for this patient population (TABLE).3-6
However, a 2014 report found that while inactive adults with disabilities (hearing, vision, cognition, mobility) were 50% more likely to report 1 or more chronic diseases than those who were physically active, only 44% of adults with disabilities who visited a health professional in the previous 12 months received a physical activity recommendation.7 In addition, more than 50% of adults with disabilities are not meeting US recommended exercise guidelines.7-9
Family physicians may not feel they have adequate training to counsel patients with IDDs. Additional limiting factors include dependence on caregivers for exercise participation, expense, transportation difficulties, a lack of choice in sporting activities, and the patient’s level of motivation.10The guidance reviewed here details how to modify the pre-participation sports physical exam specifically for patients with IDDs. It also provides sport and exercise recommendations for patients with 3 disabilities: Down syndrome, cerebral palsy, and autism spectrum disorder.
Worth noting: As is true for adults without disabilities, those with IDDs should participate in at least 150 minutes of moderate-intensity, or 75 minutes of vigorous intensity, aerobic physical activity each week.9 Recommend muscle-strengthening activities be performed at least 2 days each week.9
Exercise recommendations for patients with Down syndrome
One in every 700 babies receives a diagnosis of Down syndrome.11 Among its many possible manifestations—which include intellectual disability, heart disease, and diabetes—Down syndrome is associated with an increased risk for obesity, which makes exercise an extremely important lifestyle modification for these patients. Obesity can lead to obstructive sleep apnea causing cor pulmonale and even premature death. Continuous positive airway pressure intervention can be difficult in terms of patient compliance. However, weight loss through exercise and sports is an effective intervention to mitigate these obesity-related health comorbidities.
Pre-participation exam. A focused history and physical exam are often conducted before a patient engages in organized competitive or recreational sports. The pre-participation sports physical exam typically focuses on cardiac, neurologic, hereditary, and musculoskeletal disorders. While we recommend including these baseline elements as part of the exam for patients with disabilities, we also recommend modifying the exam to include disability-specific screening for associated comorbidities.
Continue to: For patients with Down syndrome...
For patients with Down syndrome, a complete pre-participation sports physical exam is warranted. Inquire specifically about neck pain or dislocations, heart murmur, cardiac surgery, seizures, sleep issues, history of congenital abdominal defect, hematologic malignancy, and bone pain as part of the focused physical exam.
Look for evidence of patellofemoral instability, pes planus, scoliosis, hallux deformities, decreased muscle tone, and muscular weakness. Check for cataracts and perform a thorough cardiovascular exam to assess for murmur or signs of chronic hypoxia, such as cyanosis. If a heart murmur is detected, refer the patient to a cardiologist.
Patients with Down syndrome are also at increased risk for atlantoaxial instability. A thorough neurologic evaluation to screen for this condition is indicated; however, routine radiologic screening is not needed.12
An annual complete blood cell count and thyroid-stimulating hormone test are recommended for all children with Down syndrome.13 For patients with Down syndrome who are 13 to 21 years of age, an echocardiogram also is recommended for concerning symptoms.13 Ferritin levels also should be assessed annually for patients who are younger than 13 years of age to check for iron-deficiency anemia.13Consider high-risk screening strategies for patients with diabetes and metabolic syndrome.
Special considerations. Patients with Down syndrome were found to be injured more frequently than individuals with other disabilities during the Special Olympics.14 These patients may be hypersensitive to pain with prolonged pain responses, or unable to verbally communicate their pain or injury.15
Continue to: The complexity of pain assessment...
The complexity of pain assessment in patients with Down syndrome may increase the difficulty of accurately diagnosing an injury, leading to underdiagnosis or overdiagnosis. To increase accuracy of pain assessment in this setting, we recommend using the Wong-Baker FACES Pain Rating Scale or a numeric pain rating scale in verbal patients.15 In nonverbal patients, facial expressions are reliable indicators of pain.
Which exercise? Healthy patients with Down syndrome can participate in any sport. Aerobic exercise can help lower body fat, reduce oxidative stress, and improve blood flow.6 Muscle-strengthening exercises can lead to improved daily functioning and balance. Strength training and aerobic exercise benefit aging patients with Down syndrome who are struggling with obesity. Such exercise also helps increase bone mineral density and improve cardiovascular fitness, especially when initiated at a young age. Consistent exercise promotes positive health outcomes throughout the lifespan.16
Exercise recommendations for patients with cerebral palsy
Cerebral palsy, the most common motor disability in children, is associated with intellectual disability, seizures, respiratory insufficiency, scoliosis, osteoporosis, mood disorders, dysphagia, and speech and hearing impairment.17 The increasing survival of premature babies born with cerebral palsy and the growing prevalence of adults with the condition point to the importance of expanding one’s knowledge of how best to care for this population.18
Pre-participation exam. In addition to a complete sport physical exam, it’s important to further evaluate patients with cerebral palsy for epilepsy, joint contractures, muscle weakness, spinal deformities, and respiratory insufficiency. The Gross Motor Function Classification system, commonly used for patients with cerebral palsy, scores functional ability in 5 levels.18 Patients at Level I are the most mobile; patients at Level V need wheelchair transport in all settings.
Further evaluation of spinal deformities can be initiated with x-ray screening. Consider ordering dual x-ray absorptiometry scans to evaluate bone mass.17
Continue to: Special considerations
Special considerations. Patients with cerebral palsy have a heightened risk for depression and anxiety.19 Mental health can be assessed via the General Anxiety Disorder-7, the Patient Health Questionnaire-9, and the Ask Suicide-Screening Questions tools, among others. Mental health screening may need to be adjusted depending on the patient’s level of cognition and ability to communicate. The patient’s caregiver also can provide supplemental information.
Consider screening vitamin D levels in patients with cerebral palsy. Approximately 50% of adults with cerebral palsy are vitamin D–deficient secondary to sedentary behavior and lack of sun exposure.20-22
Optimal medical management has been shown to decrease muscle spasticity and may be beneficial before initiating an exercise program. For patients with moderate-to-severe symptoms, referral for physical therapy to further improve gross motor function and spasticity may be required before initiating an exercise program.
Which exercise? Individuals with cerebral palsy spend 76% to 99% of their waking hours being sedentary.5 Consequently, they typically have decreased cardiorespiratory endurance and decreased muscle strength. Strength training may improve muscle spasticity, gross motor function, joint health, and respiratory insufficiency.5 Even in those who function at Level IV-V of the Gross Motor Function Classification system, exercise reduces vertebral fractures and improves time spent standing.23 By improving endurance, spasticity, and strength with exercise, deconditioning can be mitigated.
Involvement in sports promotes peer interactions, personal interests, and positive self-identity. It can give a newfound passion for life. Additionally, families of children with disabilities who engage in leisure activities together have less caregiver burden.24,25 Sporting activities offer a way to optimize psychosocial well-being for the patient and the entire family.
Continue to: Dance promotes functionality...
Dance promotes functionality and psychosocial adjustment.26 Hippotherapy, defined as therapy and rehabilitation during which the patient interacts with horses, can diminish muscle spasticity.27 Aquatic therapy also may increase muscle strength.28
Sports for patients with autism spectrum disorder
Autism spectrum disorder is defined as persistent deficits in social communication and social interaction that are usually evident in the first 3 years of life.29 Autism can manifest with or without intellectual or language impairment. Patients with autism commonly have difficulty processing sensory stimuli and can experience “sensory overload.” More than half have a coexisting mental health disorder, such as attention-deficit/hyperactivity disorder, anxiety, depression, schizophrenia, or bipolar disorder.30
Aversions to foods and food selectivity, as well as adverse effects from medical treatment of autism-related agitation, result in a higher incidence of obesity in patients with autism.31,32
Pre-participation exam. In addition to a comprehensive pre-participation exam, the Autism Spectrum Syndrome Questionnaire (ASSQ) and Modified Checklist for Autism in Toddlers are tools to screen school-age children with normal cognition to mild intellectual disability.33 These questionnaires have limitations, however. For example, ASSQ has limited ability to identify the female autistic phenotype.34 As such, these are solely screening tools. Final diagnosis is based on clinical judgment.
Special considerations. Include screening for constipation or diarrhea, fiber intake, food aversions, and common mental health comorbidities using Diagnostic and Statistical Manual of Mental Disorders-Fifth Edition criteria.29 Psychiatric referral may be necessary if certain previously undiagnosed condition(s) become apparent. The patient’s caregiver can provide supplemental information
Continue to: During the physical exam...
During the physical exam, limit sensory stimuli as much as possible, including lights and sounds. Verbalize components of the exam before touching a patient with autism who is sensitive to physical touch.
Which exercise? Participation in sports is an effective therapy for autism and can help patients develop communication skills and promote socialization. Vigorous exercise is associated with a reduction in stereotypic behaviors, hyperactivity, aggression, and self-injury.3 Sports also can offer an alternative channel for social interaction. Children with autism may have impaired or delayed motor skills, and exercise can improve motor skill proficiency.4
The prevalence of feeding problems in children with autism spectrum disorder is estimated to be as high as 90%, and close to 70% are selective eaters.31,35,36 For those with gastrointestinal disorders, exercise can exert positive effects on the microbiome-gut-brain axis.37 Additionally, patients with autism are much more likely to be overweight or obese.32 Physical activity offers those with autism health benefits similar to those for the general population.32
Children with autism spectrum disorder have similar odds of injury, including serious injury, relative to population controls.38 Karate and swimming are among the most researched sports therapy options for patients with autism.38-40 Both are shown to improve motor ability and reduce communication deficits.
Summing up
The literature, although limited, demonstrates that exercise and sports improve the health and well-being of people with IDDs throughout the lifespan, especially if childhood exercise/sports involvement is maintained.
Encourage your patients to participate in sports, but be aware of factors that can limit (or facilitate) participation.41 Exercise participation increases based on, among other things, the individual’s desire to be fit and active, skills practice, peer involvement, family support, accessible facilities, and skilled staff.10
Additional resources that can help people with IDDs access sports and recreational activities include the Special Olympics; Paralympics; YMCA; after-school programs; The American College of Sports Medicine; The National Center on Health, Physical Activity, and Disability; and disability-certified inclusive fitness trainers.
CORRESPONDENCE
Kristina Jones, BS, 12901 Bruce B. Downs Boulevard, Tampa, FL 33612; [email protected]
1. CDC. Addressing gaps in healthcare for individuals with intellectual disabilities. Updated October 15, 2019. Accessed January 21, 2023. www.cdc.gov/grand-rounds/pp/2019/20191015-intellectual-disabilities.html
2. CDC. Vital signs: adults with disabilities. Physical activity is for everybody. Updated November 16, 2018. Accessed January 21, 2023. www.cdc.gov/vitalsigns/disabilities/index.html
3. Di Palma D, Molisso V. Sport for autism. J Humanities Soc Pol. 2017;3:42-49.
4. Pan CY, Chu CH, Tsai CL, et al. The impacts of physical activity intervention on physical and cognitive outcomes in children with autism spectrum disorder. Autism. 2017;21:190-202. doi: 10.1177/1362361316633562
5. Verschuren O, Peterson MD, Balemans AC, et al. Exercise and physical activity recommendations for people with cerebral palsy. Dev Med Child Neurol. 2016;58:798-808. doi: 10.1111/dmcn.13053
6. Paul Y, Ellapen TJ, Barnard M, et al. The health benefits of exercise therapy for patients with Down syndrome: a systematic review. Afr J Disabil. 2019;8:576. doi: 10.4102/ajod.v8i0.576
7. Carroll DD, Courtney-Long EA, Stevens AC, et al. Vital signs: disability and physical activity—United States, 2009-2012. MMWR Morb Mortal Wkly Rep. 2014;63:407-413.
8. Rimmer JH. Physical activity for people with disabilities: how do we reach those with the greatest need? NAM Perspectives. Published April 6, 2015. Accessed March 23, 2023. https://nam.edu/perspectives-2015-physical-activity-for-people-with-disabilities-how-do-we-reach-those-with-the-greatest-need/
9. Department of Health and Human Services. Physical Activity Guidelines For Americans. 2nd edition. Published 2018. Accessed March 23, 2023. https://health.gov/sites/default/files/2019-09/Physical_Activity_Guidelines_2nd_edition.pdf
10. Darcy S, Dowse L. In search of a level playing field—the constraints and benefits of sport participation for people with intellectual disability. Disabil Soc. 2013;28:393-407. doi: 10.1080/ 09687599.2012.714258
11. Mai CT, Isenburg JL, Canfield MA, et al. National population‐based estimates for major birth defects, 2010-2014. Birth Defects Res. 2019;111:1420-1435. doi: 10.1002/bdr2.1589
12. MyŚliwiec A, Posłuszny A, Saulicz E, et al. Atlanto-axial instability in people with Down’s syndrome and its impact on the ability to perform sports activities—a review. J Hum Kinet. 2015;48:17-24. doi: 10.1515/hukin-2015-0087
13. Bunt CW, Bunt SK. Role of the family physician in the care of children with Down syndrome. Am Fam Physician. 2014;90:851-858.
14. McCormick DP, Niebuhr VN, Risser WL. Injury and illness surveillance at local Special Olympic Games. Br J Sports Med. 1990; 24:221-224. doi: 10.1136/bjsm.24.4.221
15. McGuire BE, Defrin R. Pain perception in people with Down syndrome: a synthesis of clinical and experimental research. Front Behav Neurosci. 2015;9. doi: 10.3389/fnbeh.2015.00194
16. Barnhart RC, Connolly B. Aging and Down syndrome: implications for physical therapy. Phys Ther. 2007;87:1399-1406. doi: 10.2522/ptj.20060334
17. Vitrikas K, Dalton H, Breish D. Cerebral palsy: an overview. Am Fam Physician. 2020;101:213-220.
18. Maenner MJ, Blumberg SJ, Kogan MD, et al. Prevalence of cerebral palsy and intellectual disability among children identified in two US national surveys, 2011-2013. Ann Epidemiol. 2016;26:222-226. doi: 10.1016/j.annepidem.2016.01.001
19. Smith KJ, Peterson MD, O’Connell NE, et al. Risk of depression and anxiety in adults with cerebral palsy. JAMA Neurol. 2019;76;294-300. doi: 10.1001/jamaneurol.2018.4147
20. Peterson MD, Haapala HJ, Chaddha A, et al. Abdominal obesity is an independent predictor of serum 25-hydroxyvitamin D deficiency in adults with cerebral palsy. Nutr Metab (Lond). 2014;11:22. doi: 10.1186/1743-7075-11-22
21. Yi YG, Jung SH, Bang MS. Emerging issues in cerebral palsy associated with aging: a physiatrist perspective. Ann Rehabil Med. 2019;43:241-249. doi: 10.5535/arm.2019.43.3.241
22. Sarathy K, Doshi C, Aroojis A. Clinical examination of children with cerebral palsy. Indian J Orthop. 2019;53:35-44. doi: 10.4103/ortho.IJOrtho_409_17
23. Caulton JM, Ward KA, Alsop CW, et al. A randomised controlled trial of standing programme on bone mineral density in non-ambulant children with cerebral palsy. Arch Dis Child. 2004;89:131-135. doi: 10.1136/adc.2002.009316
24. Clutterbuck G, Auld M, Johnston L. Active exercise interventions improve gross motor function of ambulant/semi-ambulant children with cerebral palsy: a systematic review. Disabil Rehabil. 2019;41:1131-1151. doi: 10.1080/09638288.2017.1422035
25. Shikako-Thomas K, Majnemer A, Law M, et al. Determinants of participation in leisure activities in children and youth with cerebral palsy: systematic review. Phys Occup Ther Pedi. 2008;28:155-169. doi: 10.1080/01942630802031834
26. Teixeira-Machado L, Azevedo-Santos I, DeSantana JM. Dance improves functionality and psychosocial adjustment in cerebral palsy: a randomized controlled clinical trial. Am J Phys Med Rehabil. 2017;96:424-429. doi: 10.1097/PHM.0000000000000646
27. Lucena-Antón D, Rosety-Rodríguez I, Moral-Munoz JA. Effects of a hippotherapy intervention on muscle spasticity in children with cerebral palsy: a randomized controlled trial. Complement Ther Clin Pract. 2018;31:188-192. doi: 10.1016/j.ctcp.2018.02.013
28. Roostaei M, Baharlouei H, Azadi H, et al. Effects of aquatic intervention on gross motor skills in children with cerebral palsy: a systematic review. Phys Occup Ther Pediatr. 2017;37:496-515. doi: 10.1080/01942638.2016.1247938
29. American Psychiatric Association. Autism spectrum disorder, section II. Diagnostic and Statistical Manual of Mental Disorders. 5th ed. 2013:50-56.
30. Romero M, Aguilar JM, Del-Rey-Mejías Á, et al. Psychiatric comorbidities in autism spectrum disorder: a comparative study between DSM-IV-TR and DSM-5 diagnosis. Int J Clin Health Psychol. 2016;16:266-275. doi: 10.1016/j.ijchp.2016.03.001
31. Volkert VM, Vaz PC. Recent studies on feeding problems in children with autism. J Appl Behav Anal. 2015;43:155-159. doi: 10.1901/jaba.2010.43-155
32. Broder-Fingert S, Brazauskas K, Lindgren K, et al. Prevalence of overweight and obesity in a large clinical sample of children with autism. Acad Pediatr. 2014;14:408-414. doi: 10.1016/j.acap.2014.04.004. PMID: 24976353
33. Adachi M, Takahashi M, Takayanagi N, et al. Adaptation of the Autism Spectrum Screening Questionnaire (ASSQ) to preschool children. PLoS One. 2018;10;13:e0199590. doi: 10.1371/journal.pone.0199590
34. Kopp S. Gillberg C. The Autism Spectrum Screening Questionnaire (ASSQ)-Revised Extended Version (ASSQ-REV): an instrument for better capturing the autism phenotype in girls? A preliminary study involving 191 clinical cases and community controls. Res Develop Disabil. 2011:32: 2875-2888.
35. Kotak T. Piazza CC. Assessment and behavioral treatment of feeding and sleeping disorders in children with autism spectrum disorders. Child Adol Psych Clin North Am. 2008;17:887-905. doi: 10.1016/j.chc.2008.06.005
36. Twachtman-Reilly J, Amaral SC, Zebrowski PP. Addressing feeding behaviors in children on the autism spectrum in school-based settings: physiological and behavioral issues. Lang Speech Hear Serv Sch. 2008:39:261-272. doi: 10.1044/0161-1461(2008/025)
37. Dalton A, Mermier C, Zuhl M. Exercise influence on the microbiome-gut-brain axis. Gut Microbes. 2019;10:555-568. doi: 10.1080/19490976.2018.1562268
38. Iliadis I, Apteslis N. The role of physical education and exercise for children with autism spectrum disorder and the effects on socialization, communication, behavior, fitness, and quality of life. Dial Clin Neurosc Mental Health. 2020;3:71-78. doi: 10.26386/obrela.v3i1.178
39. Phung JN, Goldberg WA. Promoting executive functioning in children with autism spectrum disorder through mixed martial arts training. J Autism Dev Dis. 2019;49:3660-3684. doi: 10.1007/s10803-019-04072-3
40. Bahrami F, Movahedi A, Marandi SM, et al. The effect of karate techniques training on communication deficit of children with autism spectrum disorder. J Autism Dev Disord. 2016;46: 978-986. doi: 10.1007/s10803-015-2643-y
41. Shields N, Synnot A. Perceived barriers and facilitators to participation in physical activity for children with disability: a qualitative study. BMC Pediatr. 2016;16:9. doi: 10.1186/s12887-016-0544-7
1. CDC. Addressing gaps in healthcare for individuals with intellectual disabilities. Updated October 15, 2019. Accessed January 21, 2023. www.cdc.gov/grand-rounds/pp/2019/20191015-intellectual-disabilities.html
2. CDC. Vital signs: adults with disabilities. Physical activity is for everybody. Updated November 16, 2018. Accessed January 21, 2023. www.cdc.gov/vitalsigns/disabilities/index.html
3. Di Palma D, Molisso V. Sport for autism. J Humanities Soc Pol. 2017;3:42-49.
4. Pan CY, Chu CH, Tsai CL, et al. The impacts of physical activity intervention on physical and cognitive outcomes in children with autism spectrum disorder. Autism. 2017;21:190-202. doi: 10.1177/1362361316633562
5. Verschuren O, Peterson MD, Balemans AC, et al. Exercise and physical activity recommendations for people with cerebral palsy. Dev Med Child Neurol. 2016;58:798-808. doi: 10.1111/dmcn.13053
6. Paul Y, Ellapen TJ, Barnard M, et al. The health benefits of exercise therapy for patients with Down syndrome: a systematic review. Afr J Disabil. 2019;8:576. doi: 10.4102/ajod.v8i0.576
7. Carroll DD, Courtney-Long EA, Stevens AC, et al. Vital signs: disability and physical activity—United States, 2009-2012. MMWR Morb Mortal Wkly Rep. 2014;63:407-413.
8. Rimmer JH. Physical activity for people with disabilities: how do we reach those with the greatest need? NAM Perspectives. Published April 6, 2015. Accessed March 23, 2023. https://nam.edu/perspectives-2015-physical-activity-for-people-with-disabilities-how-do-we-reach-those-with-the-greatest-need/
9. Department of Health and Human Services. Physical Activity Guidelines For Americans. 2nd edition. Published 2018. Accessed March 23, 2023. https://health.gov/sites/default/files/2019-09/Physical_Activity_Guidelines_2nd_edition.pdf
10. Darcy S, Dowse L. In search of a level playing field—the constraints and benefits of sport participation for people with intellectual disability. Disabil Soc. 2013;28:393-407. doi: 10.1080/ 09687599.2012.714258
11. Mai CT, Isenburg JL, Canfield MA, et al. National population‐based estimates for major birth defects, 2010-2014. Birth Defects Res. 2019;111:1420-1435. doi: 10.1002/bdr2.1589
12. MyŚliwiec A, Posłuszny A, Saulicz E, et al. Atlanto-axial instability in people with Down’s syndrome and its impact on the ability to perform sports activities—a review. J Hum Kinet. 2015;48:17-24. doi: 10.1515/hukin-2015-0087
13. Bunt CW, Bunt SK. Role of the family physician in the care of children with Down syndrome. Am Fam Physician. 2014;90:851-858.
14. McCormick DP, Niebuhr VN, Risser WL. Injury and illness surveillance at local Special Olympic Games. Br J Sports Med. 1990; 24:221-224. doi: 10.1136/bjsm.24.4.221
15. McGuire BE, Defrin R. Pain perception in people with Down syndrome: a synthesis of clinical and experimental research. Front Behav Neurosci. 2015;9. doi: 10.3389/fnbeh.2015.00194
16. Barnhart RC, Connolly B. Aging and Down syndrome: implications for physical therapy. Phys Ther. 2007;87:1399-1406. doi: 10.2522/ptj.20060334
17. Vitrikas K, Dalton H, Breish D. Cerebral palsy: an overview. Am Fam Physician. 2020;101:213-220.
18. Maenner MJ, Blumberg SJ, Kogan MD, et al. Prevalence of cerebral palsy and intellectual disability among children identified in two US national surveys, 2011-2013. Ann Epidemiol. 2016;26:222-226. doi: 10.1016/j.annepidem.2016.01.001
19. Smith KJ, Peterson MD, O’Connell NE, et al. Risk of depression and anxiety in adults with cerebral palsy. JAMA Neurol. 2019;76;294-300. doi: 10.1001/jamaneurol.2018.4147
20. Peterson MD, Haapala HJ, Chaddha A, et al. Abdominal obesity is an independent predictor of serum 25-hydroxyvitamin D deficiency in adults with cerebral palsy. Nutr Metab (Lond). 2014;11:22. doi: 10.1186/1743-7075-11-22
21. Yi YG, Jung SH, Bang MS. Emerging issues in cerebral palsy associated with aging: a physiatrist perspective. Ann Rehabil Med. 2019;43:241-249. doi: 10.5535/arm.2019.43.3.241
22. Sarathy K, Doshi C, Aroojis A. Clinical examination of children with cerebral palsy. Indian J Orthop. 2019;53:35-44. doi: 10.4103/ortho.IJOrtho_409_17
23. Caulton JM, Ward KA, Alsop CW, et al. A randomised controlled trial of standing programme on bone mineral density in non-ambulant children with cerebral palsy. Arch Dis Child. 2004;89:131-135. doi: 10.1136/adc.2002.009316
24. Clutterbuck G, Auld M, Johnston L. Active exercise interventions improve gross motor function of ambulant/semi-ambulant children with cerebral palsy: a systematic review. Disabil Rehabil. 2019;41:1131-1151. doi: 10.1080/09638288.2017.1422035
25. Shikako-Thomas K, Majnemer A, Law M, et al. Determinants of participation in leisure activities in children and youth with cerebral palsy: systematic review. Phys Occup Ther Pedi. 2008;28:155-169. doi: 10.1080/01942630802031834
26. Teixeira-Machado L, Azevedo-Santos I, DeSantana JM. Dance improves functionality and psychosocial adjustment in cerebral palsy: a randomized controlled clinical trial. Am J Phys Med Rehabil. 2017;96:424-429. doi: 10.1097/PHM.0000000000000646
27. Lucena-Antón D, Rosety-Rodríguez I, Moral-Munoz JA. Effects of a hippotherapy intervention on muscle spasticity in children with cerebral palsy: a randomized controlled trial. Complement Ther Clin Pract. 2018;31:188-192. doi: 10.1016/j.ctcp.2018.02.013
28. Roostaei M, Baharlouei H, Azadi H, et al. Effects of aquatic intervention on gross motor skills in children with cerebral palsy: a systematic review. Phys Occup Ther Pediatr. 2017;37:496-515. doi: 10.1080/01942638.2016.1247938
29. American Psychiatric Association. Autism spectrum disorder, section II. Diagnostic and Statistical Manual of Mental Disorders. 5th ed. 2013:50-56.
30. Romero M, Aguilar JM, Del-Rey-Mejías Á, et al. Psychiatric comorbidities in autism spectrum disorder: a comparative study between DSM-IV-TR and DSM-5 diagnosis. Int J Clin Health Psychol. 2016;16:266-275. doi: 10.1016/j.ijchp.2016.03.001
31. Volkert VM, Vaz PC. Recent studies on feeding problems in children with autism. J Appl Behav Anal. 2015;43:155-159. doi: 10.1901/jaba.2010.43-155
32. Broder-Fingert S, Brazauskas K, Lindgren K, et al. Prevalence of overweight and obesity in a large clinical sample of children with autism. Acad Pediatr. 2014;14:408-414. doi: 10.1016/j.acap.2014.04.004. PMID: 24976353
33. Adachi M, Takahashi M, Takayanagi N, et al. Adaptation of the Autism Spectrum Screening Questionnaire (ASSQ) to preschool children. PLoS One. 2018;10;13:e0199590. doi: 10.1371/journal.pone.0199590
34. Kopp S. Gillberg C. The Autism Spectrum Screening Questionnaire (ASSQ)-Revised Extended Version (ASSQ-REV): an instrument for better capturing the autism phenotype in girls? A preliminary study involving 191 clinical cases and community controls. Res Develop Disabil. 2011:32: 2875-2888.
35. Kotak T. Piazza CC. Assessment and behavioral treatment of feeding and sleeping disorders in children with autism spectrum disorders. Child Adol Psych Clin North Am. 2008;17:887-905. doi: 10.1016/j.chc.2008.06.005
36. Twachtman-Reilly J, Amaral SC, Zebrowski PP. Addressing feeding behaviors in children on the autism spectrum in school-based settings: physiological and behavioral issues. Lang Speech Hear Serv Sch. 2008:39:261-272. doi: 10.1044/0161-1461(2008/025)
37. Dalton A, Mermier C, Zuhl M. Exercise influence on the microbiome-gut-brain axis. Gut Microbes. 2019;10:555-568. doi: 10.1080/19490976.2018.1562268
38. Iliadis I, Apteslis N. The role of physical education and exercise for children with autism spectrum disorder and the effects on socialization, communication, behavior, fitness, and quality of life. Dial Clin Neurosc Mental Health. 2020;3:71-78. doi: 10.26386/obrela.v3i1.178
39. Phung JN, Goldberg WA. Promoting executive functioning in children with autism spectrum disorder through mixed martial arts training. J Autism Dev Dis. 2019;49:3660-3684. doi: 10.1007/s10803-019-04072-3
40. Bahrami F, Movahedi A, Marandi SM, et al. The effect of karate techniques training on communication deficit of children with autism spectrum disorder. J Autism Dev Disord. 2016;46: 978-986. doi: 10.1007/s10803-015-2643-y
41. Shields N, Synnot A. Perceived barriers and facilitators to participation in physical activity for children with disability: a qualitative study. BMC Pediatr. 2016;16:9. doi: 10.1186/s12887-016-0544-7
PRACTICE RECOMMENDATIONS
› Recommend physical activity as an adjunct to traditional medical management to maximize physical and psychosocial benefits in patients with intellectual/developmental disabilities. B
Strength of recommendation (SOR)
A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series
