At birth, this child had a lesion on his shoulder that now—a year later—has doubled in size. His parents report no systemic symptoms or medication use for their son. They say that the child exhibits no distress; he does not attempt to scratch at the affected patch of skin. However, they observe that if the lesion is touched, it swells and then (within minutes) returns to normal.

There is no family history of similar problems. However, both the patient and his mother are highly atopic.

EXAMINATION
The lesion—a low, orange, oval plaque—measures about 3.5 x 2 cm. Barely palpable, it urticates when stroked with a fingernail edge but does not appear to cause any discomfort.

No other lesions of note are found. The child appears quite healthy and is in no distress.

What is the diagnosis?

When stroked, mast cell lesions degranulate portions of the cell, releasing histamine precursors and leukotrienes (eg, IL 1 and IL 31). In most cases, stroking merely leads to short-lived urtication. But if the problem is more widespread (eg, urticaria pigmentosa) and the lesions are sufficiently traumatized, the release of these substances can lead to problems such as hypotension, malaise, fever, and abdominal pain.

Fortunately, this is rare, as is systemic mastocytosis—a condition in which mast cells infiltrate internal organs and bone marrow, interrupting normal function and, in the extreme, leading to mast cell leukemia. Our patient is not at risk for these complications; his lesion should resolve completely by age 3.

The differential for this patient’s lesion includes congenital nevus, lichen aureus, and café au lait spot.

TAKE-HOME LEARNING POINTS

• Cutaneous mastocytosis manifests as a reddish orange maculopapular patch, which urticates upon forceful stroking.
• Stroking the lesion degranulates the mast cells comprising it, leading to the release of histamine precursors.
• Mast cells can infiltrate internal organs and bone marrow, leading, in the extreme, to mast cell leukemia.
• Urticaria pigmentosa is a variation of mastocytosis in which hundreds of such lesions develop all over the body.

At birth, this child had a lesion on his shoulder that now—a year later—has doubled in size. His parents report no systemic symptoms or medication use for their son. They say that the child exhibits no distress; he does not attempt to scratch at the affected patch of skin. However, they observe that if the lesion is touched, it swells and then (within minutes) returns to normal.

There is no family history of similar problems. However, both the patient and his mother are highly atopic.

EXAMINATION
The lesion—a low, orange, oval plaque—measures about 3.5 x 2 cm. Barely palpable, it urticates when stroked with a fingernail edge but does not appear to cause any discomfort.

No other lesions of note are found. The child appears quite healthy and is in no distress.

What is the diagnosis?

When stroked, mast cell lesions degranulate portions of the cell, releasing histamine precursors and leukotrienes (eg, IL 1 and IL 31). In most cases, stroking merely leads to short-lived urtication. But if the problem is more widespread (eg, urticaria pigmentosa) and the lesions are sufficiently traumatized, the release of these substances can lead to problems such as hypotension, malaise, fever, and abdominal pain.

Fortunately, this is rare, as is systemic mastocytosis—a condition in which mast cells infiltrate internal organs and bone marrow, interrupting normal function and, in the extreme, leading to mast cell leukemia. Our patient is not at risk for these complications; his lesion should resolve completely by age 3.

The differential for this patient’s lesion includes congenital nevus, lichen aureus, and café au lait spot.

TAKE-HOME LEARNING POINTS

• Cutaneous mastocytosis manifests as a reddish orange maculopapular patch, which urticates upon forceful stroking.
• Stroking the lesion degranulates the mast cells comprising it, leading to the release of histamine precursors.
• Mast cells can infiltrate internal organs and bone marrow, leading, in the extreme, to mast cell leukemia.
• Urticaria pigmentosa is a variation of mastocytosis in which hundreds of such lesions develop all over the body.

At birth, this child had a lesion on his shoulder that now—a year later—has doubled in size. His parents report no systemic symptoms or medication use for their son. They say that the child exhibits no distress; he does not attempt to scratch at the affected patch of skin. However, they observe that if the lesion is touched, it swells and then (within minutes) returns to normal.

There is no family history of similar problems. However, both the patient and his mother are highly atopic.

EXAMINATION
The lesion—a low, orange, oval plaque—measures about 3.5 x 2 cm. Barely palpable, it urticates when stroked with a fingernail edge but does not appear to cause any discomfort.

No other lesions of note are found. The child appears quite healthy and is in no distress.

What is the diagnosis?

When stroked, mast cell lesions degranulate portions of the cell, releasing histamine precursors and leukotrienes (eg, IL 1 and IL 31). In most cases, stroking merely leads to short-lived urtication. But if the problem is more widespread (eg, urticaria pigmentosa) and the lesions are sufficiently traumatized, the release of these substances can lead to problems such as hypotension, malaise, fever, and abdominal pain.

Fortunately, this is rare, as is systemic mastocytosis—a condition in which mast cells infiltrate internal organs and bone marrow, interrupting normal function and, in the extreme, leading to mast cell leukemia. Our patient is not at risk for these complications; his lesion should resolve completely by age 3.

The differential for this patient’s lesion includes congenital nevus, lichen aureus, and café au lait spot.

TAKE-HOME LEARNING POINTS

• Cutaneous mastocytosis manifests as a reddish orange maculopapular patch, which urticates upon forceful stroking.
• Stroking the lesion degranulates the mast cells comprising it, leading to the release of histamine precursors.
• Mast cells can infiltrate internal organs and bone marrow, leading, in the extreme, to mast cell leukemia.
• Urticaria pigmentosa is a variation of mastocytosis in which hundreds of such lesions develop all over the body.

Medicare beneficiaries with myeloma who have prescription drug coverage have shown both decreased used of classic cytotoxic chemotherapy and better survival, according to new research.

vitanovski/Thinkstock.com

The findings suggested that prescription drug coverage brings better access to all existing treatment options.

“In this analysis of Medicare beneficiaries with myeloma, the receipt of therapy and survival differed according to prescription drug coverage status,” Adam Olszewski, MD, of the Lifespan Cancer Institute at Rhode Island Hospital in Providence, R.I., and his colleagues noted in the study. “Patients with PDP [prescription drug plan coverage through Medicare Part D] or OCC [other credible prescription drug coverage] more often received active myeloma care, compared to those without coverage,” they wrote in Journal of Clinical Oncology.

The researchers looked at 9,755 patients diagnosed with myeloma during 2006-2011 and examined what was used to treat the myeloma as a first line treatment. The cohort included 1,460 patients with no prescription drug coverage, 3,283 with PDP coverage, 3,607 with OCC, and 1,405 dual eligibility for Medicare and Medicaid coverage.

The study found that, compared with beneficiaries with no coverage, Medicare beneficiaries with PDP coverage “were 14% less likely to be treated with parenteral chemotherapy and 38% less likely to receive classic cytotoxic agents.” Additionally, among the cohort of beneficiaries that were without drug coverage prior to the diagnosis of myeloma, 41% actively obtained coverage, but even then, their survival was “significantly worse, compared with the beneficiaries who had coverage at diagnosis.”

Beneficiaries classified as having other credible coverage were 3% more likely to receive active myeloma care than were those without coverage, but the use of parenteral regimens did not differ between those groups.

Researchers noted that overall survival was 10% higher at 1 year and 6% higher at 3 years for beneficiaries with PDP coverage or OCC than it was for those without coverage, but they added that the analysis required cautious interpretation “as it is confounded by multiple baseline factors and mediated by the quality of cancer treatment. ... We could not discern whether worse survival in the group without coverage was a result of not receiving therapy at all, an inability to access IMiDs [immunomodulatory drugs], or poor control of other medical issues.”

However, a comparison with the control group “strongly suggest[s] that patients with myeloma without prescription drug coverage may not have received the most effective first-line therapy,” Dr. Olszewski and his colleagues added. “Survival for PDP and OCC groups remained identical, which supports the notion that having any prescription drug coverage contributed to optimal treatment and outcomes.”

The study was limited by the fact that unobserved clinical differences between beneficiaries with or without prescription drug coverage could have accounted for differences in mortality and that the comparison of treatments was restricted to parenteral regimens because IMiDs were observed to have been administered only for PDP enrollees.

Dr. Olszewski and study coauthor Amy Davidoff, PhD, of Yale University, New Haven, Conn., disclosed acting in consulting or advisory roles and receiving research funding from several pharmaceutical companies that develop cancer treatments.

[email protected]

SOURCE: Olszewski A et al. J Clin Oncol. 2018 Aug 16. doi: 10.1200/JCO.2018.77.8894.

Medicare beneficiaries with myeloma who have prescription drug coverage have shown both decreased used of classic cytotoxic chemotherapy and better survival, according to new research.

vitanovski/Thinkstock.com

The findings suggested that prescription drug coverage brings better access to all existing treatment options.

“In this analysis of Medicare beneficiaries with myeloma, the receipt of therapy and survival differed according to prescription drug coverage status,” Adam Olszewski, MD, of the Lifespan Cancer Institute at Rhode Island Hospital in Providence, R.I., and his colleagues noted in the study. “Patients with PDP [prescription drug plan coverage through Medicare Part D] or OCC [other credible prescription drug coverage] more often received active myeloma care, compared to those without coverage,” they wrote in Journal of Clinical Oncology.

The researchers looked at 9,755 patients diagnosed with myeloma during 2006-2011 and examined what was used to treat the myeloma as a first line treatment. The cohort included 1,460 patients with no prescription drug coverage, 3,283 with PDP coverage, 3,607 with OCC, and 1,405 dual eligibility for Medicare and Medicaid coverage.

The study found that, compared with beneficiaries with no coverage, Medicare beneficiaries with PDP coverage “were 14% less likely to be treated with parenteral chemotherapy and 38% less likely to receive classic cytotoxic agents.” Additionally, among the cohort of beneficiaries that were without drug coverage prior to the diagnosis of myeloma, 41% actively obtained coverage, but even then, their survival was “significantly worse, compared with the beneficiaries who had coverage at diagnosis.”

Beneficiaries classified as having other credible coverage were 3% more likely to receive active myeloma care than were those without coverage, but the use of parenteral regimens did not differ between those groups.

Researchers noted that overall survival was 10% higher at 1 year and 6% higher at 3 years for beneficiaries with PDP coverage or OCC than it was for those without coverage, but they added that the analysis required cautious interpretation “as it is confounded by multiple baseline factors and mediated by the quality of cancer treatment. ... We could not discern whether worse survival in the group without coverage was a result of not receiving therapy at all, an inability to access IMiDs [immunomodulatory drugs], or poor control of other medical issues.”

However, a comparison with the control group “strongly suggest[s] that patients with myeloma without prescription drug coverage may not have received the most effective first-line therapy,” Dr. Olszewski and his colleagues added. “Survival for PDP and OCC groups remained identical, which supports the notion that having any prescription drug coverage contributed to optimal treatment and outcomes.”

The study was limited by the fact that unobserved clinical differences between beneficiaries with or without prescription drug coverage could have accounted for differences in mortality and that the comparison of treatments was restricted to parenteral regimens because IMiDs were observed to have been administered only for PDP enrollees.

Dr. Olszewski and study coauthor Amy Davidoff, PhD, of Yale University, New Haven, Conn., disclosed acting in consulting or advisory roles and receiving research funding from several pharmaceutical companies that develop cancer treatments.

[email protected]

SOURCE: Olszewski A et al. J Clin Oncol. 2018 Aug 16. doi: 10.1200/JCO.2018.77.8894.

Medicare beneficiaries with myeloma who have prescription drug coverage have shown both decreased used of classic cytotoxic chemotherapy and better survival, according to new research.

vitanovski/Thinkstock.com

The findings suggested that prescription drug coverage brings better access to all existing treatment options.

“In this analysis of Medicare beneficiaries with myeloma, the receipt of therapy and survival differed according to prescription drug coverage status,” Adam Olszewski, MD, of the Lifespan Cancer Institute at Rhode Island Hospital in Providence, R.I., and his colleagues noted in the study. “Patients with PDP [prescription drug plan coverage through Medicare Part D] or OCC [other credible prescription drug coverage] more often received active myeloma care, compared to those without coverage,” they wrote in Journal of Clinical Oncology.

The researchers looked at 9,755 patients diagnosed with myeloma during 2006-2011 and examined what was used to treat the myeloma as a first line treatment. The cohort included 1,460 patients with no prescription drug coverage, 3,283 with PDP coverage, 3,607 with OCC, and 1,405 dual eligibility for Medicare and Medicaid coverage.

The study found that, compared with beneficiaries with no coverage, Medicare beneficiaries with PDP coverage “were 14% less likely to be treated with parenteral chemotherapy and 38% less likely to receive classic cytotoxic agents.” Additionally, among the cohort of beneficiaries that were without drug coverage prior to the diagnosis of myeloma, 41% actively obtained coverage, but even then, their survival was “significantly worse, compared with the beneficiaries who had coverage at diagnosis.”

Beneficiaries classified as having other credible coverage were 3% more likely to receive active myeloma care than were those without coverage, but the use of parenteral regimens did not differ between those groups.

Researchers noted that overall survival was 10% higher at 1 year and 6% higher at 3 years for beneficiaries with PDP coverage or OCC than it was for those without coverage, but they added that the analysis required cautious interpretation “as it is confounded by multiple baseline factors and mediated by the quality of cancer treatment. ... We could not discern whether worse survival in the group without coverage was a result of not receiving therapy at all, an inability to access IMiDs [immunomodulatory drugs], or poor control of other medical issues.”

However, a comparison with the control group “strongly suggest[s] that patients with myeloma without prescription drug coverage may not have received the most effective first-line therapy,” Dr. Olszewski and his colleagues added. “Survival for PDP and OCC groups remained identical, which supports the notion that having any prescription drug coverage contributed to optimal treatment and outcomes.”

The study was limited by the fact that unobserved clinical differences between beneficiaries with or without prescription drug coverage could have accounted for differences in mortality and that the comparison of treatments was restricted to parenteral regimens because IMiDs were observed to have been administered only for PDP enrollees.

Dr. Olszewski and study coauthor Amy Davidoff, PhD, of Yale University, New Haven, Conn., disclosed acting in consulting or advisory roles and receiving research funding from several pharmaceutical companies that develop cancer treatments.

[email protected]

SOURCE: Olszewski A et al. J Clin Oncol. 2018 Aug 16. doi: 10.1200/JCO.2018.77.8894.

A first-in-human trial of a live, attenuated Zika virus vaccine has begun, according to an announcement by the National Institutes of Health.

Courtesy National Institute of Allergy and Infectious Diseases

[email protected]

SOURCE: NIH, August 16, 2018. News Release.

A first-in-human trial of a live, attenuated Zika virus vaccine has begun, according to an announcement by the National Institutes of Health.

Courtesy National Institute of Allergy and Infectious Diseases

[email protected]

SOURCE: NIH, August 16, 2018. News Release.

A first-in-human trial of a live, attenuated Zika virus vaccine has begun, according to an announcement by the National Institutes of Health.

Courtesy National Institute of Allergy and Infectious Diseases

[email protected]

SOURCE: NIH, August 16, 2018. News Release.

Prominent in the news has been the plight of thousands of immigrant families who are separated from one another at the Mexico-United States border. Now, as reunification is occurring for some, what is the aftermath of the forced separations?

A recent report by the PBS News Hour focused on 3-year-old “Sofi” (last name withheld). She and her grandmother and guardian, Angelina, were among those separated when they made their way legally from Mexico to a U.S. immigration checkpoint in Texas, seeking to escape reprisals from a Mexican drug cartel. For 47 days, infant Sofi was on her own without her family, first in a migrant shelter in El Paso, Texas, and then in a facility in Pennsylvania. For Ana, whose mother was in California already, the time apart from Sofi was one of mind-numbing worry over the fate of her daughter and mother.

Now, life is better. Through an interpreter, Ana said: “I feel good, very good to see her with my mom, and to know that she will now be with us, and she won’t be apart from me.”

The separation was hard on Sofi.

“She cried all the time, told me she didn’t want to be there,” said Ana of Sofi’s ordeal. “One time, she didn’t sound OK. She couldn’t speak clearly, and they were giving her a bad look. They would scold her. And she wanted to tell me something, but couldn’t, because they would scold her. So that had me very worried.”

The early days of reunification have gone fairly well. But unease remains. Sofi mentioned medicine she was given and of being punished for crying or refusing to eat. Whether those incidents are real or embellished, and whether emotional scars remain, is the stuff of the unknown.

“We haven’t asked her many questions because she says that it was a bad place. We want to eventually ask her little by little how she was treated. I think that, with time, we need to let her know that they separated her from us for some time,” Angelina said. “We want to take her to see a therapist, for her to be examined to see how she is, how her health is, because she looks good now – but who knows how she will react later on.” Sofi is home. But hundreds of other children reportedly remain separated from their families.

Click here to watch the News Hour report.

Prominent in the news has been the plight of thousands of immigrant families who are separated from one another at the Mexico-United States border. Now, as reunification is occurring for some, what is the aftermath of the forced separations?

A recent report by the PBS News Hour focused on 3-year-old “Sofi” (last name withheld). She and her grandmother and guardian, Angelina, were among those separated when they made their way legally from Mexico to a U.S. immigration checkpoint in Texas, seeking to escape reprisals from a Mexican drug cartel. For 47 days, infant Sofi was on her own without her family, first in a migrant shelter in El Paso, Texas, and then in a facility in Pennsylvania. For Ana, whose mother was in California already, the time apart from Sofi was one of mind-numbing worry over the fate of her daughter and mother.

Now, life is better. Through an interpreter, Ana said: “I feel good, very good to see her with my mom, and to know that she will now be with us, and she won’t be apart from me.”

The separation was hard on Sofi.

“She cried all the time, told me she didn’t want to be there,” said Ana of Sofi’s ordeal. “One time, she didn’t sound OK. She couldn’t speak clearly, and they were giving her a bad look. They would scold her. And she wanted to tell me something, but couldn’t, because they would scold her. So that had me very worried.”

The early days of reunification have gone fairly well. But unease remains. Sofi mentioned medicine she was given and of being punished for crying or refusing to eat. Whether those incidents are real or embellished, and whether emotional scars remain, is the stuff of the unknown.

“We haven’t asked her many questions because she says that it was a bad place. We want to eventually ask her little by little how she was treated. I think that, with time, we need to let her know that they separated her from us for some time,” Angelina said. “We want to take her to see a therapist, for her to be examined to see how she is, how her health is, because she looks good now – but who knows how she will react later on.” Sofi is home. But hundreds of other children reportedly remain separated from their families.

Click here to watch the News Hour report.

Prominent in the news has been the plight of thousands of immigrant families who are separated from one another at the Mexico-United States border. Now, as reunification is occurring for some, what is the aftermath of the forced separations?

A recent report by the PBS News Hour focused on 3-year-old “Sofi” (last name withheld). She and her grandmother and guardian, Angelina, were among those separated when they made their way legally from Mexico to a U.S. immigration checkpoint in Texas, seeking to escape reprisals from a Mexican drug cartel. For 47 days, infant Sofi was on her own without her family, first in a migrant shelter in El Paso, Texas, and then in a facility in Pennsylvania. For Ana, whose mother was in California already, the time apart from Sofi was one of mind-numbing worry over the fate of her daughter and mother.

Now, life is better. Through an interpreter, Ana said: “I feel good, very good to see her with my mom, and to know that she will now be with us, and she won’t be apart from me.”

The separation was hard on Sofi.

“She cried all the time, told me she didn’t want to be there,” said Ana of Sofi’s ordeal. “One time, she didn’t sound OK. She couldn’t speak clearly, and they were giving her a bad look. They would scold her. And she wanted to tell me something, but couldn’t, because they would scold her. So that had me very worried.”

The early days of reunification have gone fairly well. But unease remains. Sofi mentioned medicine she was given and of being punished for crying or refusing to eat. Whether those incidents are real or embellished, and whether emotional scars remain, is the stuff of the unknown.

“We haven’t asked her many questions because she says that it was a bad place. We want to eventually ask her little by little how she was treated. I think that, with time, we need to let her know that they separated her from us for some time,” Angelina said. “We want to take her to see a therapist, for her to be examined to see how she is, how her health is, because she looks good now – but who knows how she will react later on.” Sofi is home. But hundreds of other children reportedly remain separated from their families.

Click here to watch the News Hour report.

Many men (yours truly included) are not the smoothest creatures on the dance floor. But what is embarrassing dorky in public works like magic at home, with the clunky moves drawing howls of laughter from family.

But there’s the rub ... behavior that is fun in private can become something to be avoided in public. The drumbeat of generations past about men who are strong and capable can surface, curbing those tapping feet and sending dads to the edge of the dance floor.

That’s why the latest Internet rage is welcome. Videos of dads putting on their best moves are providing an example of how love can overcome less-than-stellar footwork. Check out https://www.youtube.com/watch?v=SGSSHSJPhv4 for an example.

According to Sarah L. Kaufman, dance critic at the Washington Post, “therein lies the coolness of the real-life dancing dad. He’s the opposite of embarrassing! He’s uplifting by way of a profound, unspoken understanding of the situation and of his child.”

Motivated by love and unafraid to show the world their moves, the dorky dancing dad can become heroic dancing dad, especially to a son or daughter who is on shaky ground and in need of support. The behavior of these dads also runs counter to the toxic masculinity that seems to pervade much of American culture.

“Within his body, stereotypes are vanquished. The distant dad is overshadowed by the physically present dad. The clueless dad yields to the one who knows exactly what his kid needs,” Ms. Kaufman wrote.

So go, dancing dad, go!

To read Ms. Kaufman’s article, click here.

Many men (yours truly included) are not the smoothest creatures on the dance floor. But what is embarrassing dorky in public works like magic at home, with the clunky moves drawing howls of laughter from family.

But there’s the rub ... behavior that is fun in private can become something to be avoided in public. The drumbeat of generations past about men who are strong and capable can surface, curbing those tapping feet and sending dads to the edge of the dance floor.

That’s why the latest Internet rage is welcome. Videos of dads putting on their best moves are providing an example of how love can overcome less-than-stellar footwork. Check out https://www.youtube.com/watch?v=SGSSHSJPhv4 for an example.

According to Sarah L. Kaufman, dance critic at the Washington Post, “therein lies the coolness of the real-life dancing dad. He’s the opposite of embarrassing! He’s uplifting by way of a profound, unspoken understanding of the situation and of his child.”

Motivated by love and unafraid to show the world their moves, the dorky dancing dad can become heroic dancing dad, especially to a son or daughter who is on shaky ground and in need of support. The behavior of these dads also runs counter to the toxic masculinity that seems to pervade much of American culture.

“Within his body, stereotypes are vanquished. The distant dad is overshadowed by the physically present dad. The clueless dad yields to the one who knows exactly what his kid needs,” Ms. Kaufman wrote.

So go, dancing dad, go!

To read Ms. Kaufman’s article, click here.

Many men (yours truly included) are not the smoothest creatures on the dance floor. But what is embarrassing dorky in public works like magic at home, with the clunky moves drawing howls of laughter from family.

But there’s the rub ... behavior that is fun in private can become something to be avoided in public. The drumbeat of generations past about men who are strong and capable can surface, curbing those tapping feet and sending dads to the edge of the dance floor.

That’s why the latest Internet rage is welcome. Videos of dads putting on their best moves are providing an example of how love can overcome less-than-stellar footwork. Check out https://www.youtube.com/watch?v=SGSSHSJPhv4 for an example.

According to Sarah L. Kaufman, dance critic at the Washington Post, “therein lies the coolness of the real-life dancing dad. He’s the opposite of embarrassing! He’s uplifting by way of a profound, unspoken understanding of the situation and of his child.”

Motivated by love and unafraid to show the world their moves, the dorky dancing dad can become heroic dancing dad, especially to a son or daughter who is on shaky ground and in need of support. The behavior of these dads also runs counter to the toxic masculinity that seems to pervade much of American culture.

“Within his body, stereotypes are vanquished. The distant dad is overshadowed by the physically present dad. The clueless dad yields to the one who knows exactly what his kid needs,” Ms. Kaufman wrote.

So go, dancing dad, go!

To read Ms. Kaufman’s article, click here.

Among psoriasis patients, treatment with infliximab was associated with an increased risk of serious infections that led to hospitalization, the use of intravenous antimicrobial therapy, or death, according to a prospective cohort study of cases in the United Kingdom and the Republic of Ireland.

The new data suggest a risk associated with infliximab treatment that previous clinical trials and observational studies were insufficiently powered to detect, according to the investigators, led by Zenas Yiu, of the University of Manchester (England). They found no associations between infection risk and treatment with etanercept, adalimumab, or ustekinumab, and they noted that there are no such data yet on more recently approved biologic therapies for psoriasis, such as secukinumab or ixekizumab.

The British Association of Dermatologists (BAD) recommends infliximab, a tumor necrosis factor (TNF)–blocker, only for severe cases of psoriasis (Psoriasis Area and Severity Index greater than or equal to 20 and a Dermatology Life Quality Index greater than 18), or when other biologics fail or cannot be used.

To address the insufficient power of earlier studies, the researchers used data from the BAD Biologic Interventions Register (BADBIR), a large, prospective psoriasis registry in the United Kingdom and Ireland established in 2007. The analysis included 3,421 subjects in the nonbiologic systemic therapy cohort, and 422 subjects in the all-lines infliximab cohort. The median follow-up period was 1.49 person-years (interquartile range, 2.50 person-years) for the all-lines (not just first-line) infliximab group, and 1.51 person-years (1.84 person-years) for the nonbiologics group.*

Treatment with infliximab was associated with a statistically significant increased risk of serious infection (defined as an infection associated with prolonged hospitalization or use of IV antimicrobial therapy; or an infection that resulted in death), with an adjusted hazard ratio of 1.95 (95% confidence interval, 1.01-3.75), compared with nonbiologic systemic treatments. The risk was higher in the first 6 months (adjusted HR, 3.49; 95% CI, 1.14-10.70), and from 6 months to 1 year (aHR, 2.99; 95% CI, 1.10-8.14,) but did not reach statistical significance at 1 year to 2 years (aHR, 2.03; 95% CI, 0.61-6.79).

There was also an increased risk of serious infection with infliximab compared with methotrexate (aHR, 2.96; 95% CI, 1.58-5.57).

“Given our findings of a higher risk of serious infection associated with infliximab, we provide real-world evidence to reinforce the position of infliximab in the psoriasis treatment hierarchy,” the authors wrote, adding that “patients with severe psoriasis who fulfill the criteria for the prescription of infliximab should be counseled” about the risk of serious infection.

Dr. Yiu disclosed having received nonfinancial support form Novartis, two authors had no disclosures, and the remainder had various disclosures related to pharmaceutical companies. BADBIR is funded by BAD, which receives funding from Pfizer, Janssen Cilag, AbbVie, Novartis, Samsung Bioepis and Eli Lilly for providing pharmacovigilance services.

SOURCE: Yiu ZZN et al. Br J Dermatol. 2018 Aug 2. doi: 10.1111/bjd.17036.

*This article was updated to correctly indicate that the median follow-up period was 1.49 person-years (interquartile range, 2.50 person-years) for the all-lines (not just first-line) infliximab group, and 1.51 person-years (1.84 person-years) for the nonbiologics group.

Among psoriasis patients, treatment with infliximab was associated with an increased risk of serious infections that led to hospitalization, the use of intravenous antimicrobial therapy, or death, according to a prospective cohort study of cases in the United Kingdom and the Republic of Ireland.

The new data suggest a risk associated with infliximab treatment that previous clinical trials and observational studies were insufficiently powered to detect, according to the investigators, led by Zenas Yiu, of the University of Manchester (England). They found no associations between infection risk and treatment with etanercept, adalimumab, or ustekinumab, and they noted that there are no such data yet on more recently approved biologic therapies for psoriasis, such as secukinumab or ixekizumab.

The British Association of Dermatologists (BAD) recommends infliximab, a tumor necrosis factor (TNF)–blocker, only for severe cases of psoriasis (Psoriasis Area and Severity Index greater than or equal to 20 and a Dermatology Life Quality Index greater than 18), or when other biologics fail or cannot be used.

To address the insufficient power of earlier studies, the researchers used data from the BAD Biologic Interventions Register (BADBIR), a large, prospective psoriasis registry in the United Kingdom and Ireland established in 2007. The analysis included 3,421 subjects in the nonbiologic systemic therapy cohort, and 422 subjects in the all-lines infliximab cohort. The median follow-up period was 1.49 person-years (interquartile range, 2.50 person-years) for the all-lines (not just first-line) infliximab group, and 1.51 person-years (1.84 person-years) for the nonbiologics group.*

Treatment with infliximab was associated with a statistically significant increased risk of serious infection (defined as an infection associated with prolonged hospitalization or use of IV antimicrobial therapy; or an infection that resulted in death), with an adjusted hazard ratio of 1.95 (95% confidence interval, 1.01-3.75), compared with nonbiologic systemic treatments. The risk was higher in the first 6 months (adjusted HR, 3.49; 95% CI, 1.14-10.70), and from 6 months to 1 year (aHR, 2.99; 95% CI, 1.10-8.14,) but did not reach statistical significance at 1 year to 2 years (aHR, 2.03; 95% CI, 0.61-6.79).

There was also an increased risk of serious infection with infliximab compared with methotrexate (aHR, 2.96; 95% CI, 1.58-5.57).

“Given our findings of a higher risk of serious infection associated with infliximab, we provide real-world evidence to reinforce the position of infliximab in the psoriasis treatment hierarchy,” the authors wrote, adding that “patients with severe psoriasis who fulfill the criteria for the prescription of infliximab should be counseled” about the risk of serious infection.

Dr. Yiu disclosed having received nonfinancial support form Novartis, two authors had no disclosures, and the remainder had various disclosures related to pharmaceutical companies. BADBIR is funded by BAD, which receives funding from Pfizer, Janssen Cilag, AbbVie, Novartis, Samsung Bioepis and Eli Lilly for providing pharmacovigilance services.

SOURCE: Yiu ZZN et al. Br J Dermatol. 2018 Aug 2. doi: 10.1111/bjd.17036.

*This article was updated to correctly indicate that the median follow-up period was 1.49 person-years (interquartile range, 2.50 person-years) for the all-lines (not just first-line) infliximab group, and 1.51 person-years (1.84 person-years) for the nonbiologics group.

Among psoriasis patients, treatment with infliximab was associated with an increased risk of serious infections that led to hospitalization, the use of intravenous antimicrobial therapy, or death, according to a prospective cohort study of cases in the United Kingdom and the Republic of Ireland.

The new data suggest a risk associated with infliximab treatment that previous clinical trials and observational studies were insufficiently powered to detect, according to the investigators, led by Zenas Yiu, of the University of Manchester (England). They found no associations between infection risk and treatment with etanercept, adalimumab, or ustekinumab, and they noted that there are no such data yet on more recently approved biologic therapies for psoriasis, such as secukinumab or ixekizumab.

The British Association of Dermatologists (BAD) recommends infliximab, a tumor necrosis factor (TNF)–blocker, only for severe cases of psoriasis (Psoriasis Area and Severity Index greater than or equal to 20 and a Dermatology Life Quality Index greater than 18), or when other biologics fail or cannot be used.

To address the insufficient power of earlier studies, the researchers used data from the BAD Biologic Interventions Register (BADBIR), a large, prospective psoriasis registry in the United Kingdom and Ireland established in 2007. The analysis included 3,421 subjects in the nonbiologic systemic therapy cohort, and 422 subjects in the all-lines infliximab cohort. The median follow-up period was 1.49 person-years (interquartile range, 2.50 person-years) for the all-lines (not just first-line) infliximab group, and 1.51 person-years (1.84 person-years) for the nonbiologics group.*

Treatment with infliximab was associated with a statistically significant increased risk of serious infection (defined as an infection associated with prolonged hospitalization or use of IV antimicrobial therapy; or an infection that resulted in death), with an adjusted hazard ratio of 1.95 (95% confidence interval, 1.01-3.75), compared with nonbiologic systemic treatments. The risk was higher in the first 6 months (adjusted HR, 3.49; 95% CI, 1.14-10.70), and from 6 months to 1 year (aHR, 2.99; 95% CI, 1.10-8.14,) but did not reach statistical significance at 1 year to 2 years (aHR, 2.03; 95% CI, 0.61-6.79).

There was also an increased risk of serious infection with infliximab compared with methotrexate (aHR, 2.96; 95% CI, 1.58-5.57).

“Given our findings of a higher risk of serious infection associated with infliximab, we provide real-world evidence to reinforce the position of infliximab in the psoriasis treatment hierarchy,” the authors wrote, adding that “patients with severe psoriasis who fulfill the criteria for the prescription of infliximab should be counseled” about the risk of serious infection.

Dr. Yiu disclosed having received nonfinancial support form Novartis, two authors had no disclosures, and the remainder had various disclosures related to pharmaceutical companies. BADBIR is funded by BAD, which receives funding from Pfizer, Janssen Cilag, AbbVie, Novartis, Samsung Bioepis and Eli Lilly for providing pharmacovigilance services.

SOURCE: Yiu ZZN et al. Br J Dermatol. 2018 Aug 2. doi: 10.1111/bjd.17036.

*This article was updated to correctly indicate that the median follow-up period was 1.49 person-years (interquartile range, 2.50 person-years) for the all-lines (not just first-line) infliximab group, and 1.51 person-years (1.84 person-years) for the nonbiologics group.

Black women who smoke cigarettes are at increased risk of systemic lupus erythematosus (SLE), while those who drink alcohol in moderation have a decreased risk, data from the prospective Black Women’s Health Study suggests.

The current findings among black women, who are the demographic group at the highest risk of SLE in the U.S. population, are consistent with the previously reported positive association of cigarette smoking with risk of SLE and inverse association of alcohol consumption with SLE,” lead researchers Yvette Cozier, DSc, and Medha Barbhaiya, MD, and their colleagues wrote in a study published in Arthritis Care & Research.

“The role of environmental factors in the pathogenesis of SLE is of great interest, as genetic factors do not explain a major portion of the incidence. Cigarette smoking has been associated with SLE risk in several, but not all, past studies,” wrote Dr. Cozier of the Slone Epidemiology Center at Boston University and Dr. Barbhaiya of the Hospital for Special Surgery, New York, and their coauthors.

Their analysis of data from the Black Women’s Health Study, a prospective study following 59,000 black women in the United States since 1995, identified 127 new cases of SLE between 1995 and 2015 that were confirmed by medical records.

At baseline, 8,851 women (16%) were current smokers, and 10,447 (18%) were past smokers. A total of 14,001 (25%) were current drinkers, and 10,255 (18%) were past drinkers.

The researchers reported that, compared with never smokers, women who had ever smoked had a 45% higher risk for SLE than did never smokers (hazard ratio, 1.45; 95% confidence interval, 0.97-2.18), but the finding was not statistically significant.

The risk of SLE was greater among current smokers (52% higher risk) than among past smokers (41% higher risk), and greater for 20 or more pack-years of smoking (60% higher risk) than for less than 20 pack-years of smoking (37% higher risk), although the authors noted that none of these differences were statistically significant.

Current and past alcohol consumption were associated with nonsignificant reductions in risk for SLE, 29% and 21%, respectively. However, “moderate” current alcohol consumption, measured as four or more drinks per week, was associated with a 57% reduction in risk for SLE (HR, 0.43; 95% CI, 0.19-0.96).

The researchers said that the associations between cigarette smoking and alcohol intake with SLE risk were biologically plausible and may lead to insights into SLE pathogenesis. For example, exposure to toxic components from cigarette smoke was associated with increased oxidative stress and stimulation of autoantibody production, and such exposure can directly damage endogenous proteins and DNA. Alcohol suppresses the synthesis of proinflammatory cytokines and may inhibit DNA synthesis.

The small number of lupus cases in the Black Women’s Health Study cohort limited the statistical power of the analysis. Smoking and alcohol use were also relatively uncommon among women in the Black Women’s Health Study cohort, the investigators said.

“Future studies are needed to confirm these findings and establish the biologic mechanisms by which cigarette smoking and alcohol consumption influence the risk of SLE in this population and others,“ they wrote.

The study was supported by individual investigators’ grant awards from the National Cancer Institute, the National Institute of Arthritis and Musculoskeletal and Skin Diseases, the Rheumatology Research Foundation, and the Lupus Foundation of America. The authors declared no relevant conflicts of interest.

SOURCE: Cozier Y et al. Arthritis Care Res. 2018 Aug 9. doi: 10.1002/acr.23703.

Black women who smoke cigarettes are at increased risk of systemic lupus erythematosus (SLE), while those who drink alcohol in moderation have a decreased risk, data from the prospective Black Women’s Health Study suggests.

The current findings among black women, who are the demographic group at the highest risk of SLE in the U.S. population, are consistent with the previously reported positive association of cigarette smoking with risk of SLE and inverse association of alcohol consumption with SLE,” lead researchers Yvette Cozier, DSc, and Medha Barbhaiya, MD, and their colleagues wrote in a study published in Arthritis Care & Research.

“The role of environmental factors in the pathogenesis of SLE is of great interest, as genetic factors do not explain a major portion of the incidence. Cigarette smoking has been associated with SLE risk in several, but not all, past studies,” wrote Dr. Cozier of the Slone Epidemiology Center at Boston University and Dr. Barbhaiya of the Hospital for Special Surgery, New York, and their coauthors.

Their analysis of data from the Black Women’s Health Study, a prospective study following 59,000 black women in the United States since 1995, identified 127 new cases of SLE between 1995 and 2015 that were confirmed by medical records.

At baseline, 8,851 women (16%) were current smokers, and 10,447 (18%) were past smokers. A total of 14,001 (25%) were current drinkers, and 10,255 (18%) were past drinkers.

The researchers reported that, compared with never smokers, women who had ever smoked had a 45% higher risk for SLE than did never smokers (hazard ratio, 1.45; 95% confidence interval, 0.97-2.18), but the finding was not statistically significant.

The risk of SLE was greater among current smokers (52% higher risk) than among past smokers (41% higher risk), and greater for 20 or more pack-years of smoking (60% higher risk) than for less than 20 pack-years of smoking (37% higher risk), although the authors noted that none of these differences were statistically significant.

Current and past alcohol consumption were associated with nonsignificant reductions in risk for SLE, 29% and 21%, respectively. However, “moderate” current alcohol consumption, measured as four or more drinks per week, was associated with a 57% reduction in risk for SLE (HR, 0.43; 95% CI, 0.19-0.96).

The researchers said that the associations between cigarette smoking and alcohol intake with SLE risk were biologically plausible and may lead to insights into SLE pathogenesis. For example, exposure to toxic components from cigarette smoke was associated with increased oxidative stress and stimulation of autoantibody production, and such exposure can directly damage endogenous proteins and DNA. Alcohol suppresses the synthesis of proinflammatory cytokines and may inhibit DNA synthesis.

The small number of lupus cases in the Black Women’s Health Study cohort limited the statistical power of the analysis. Smoking and alcohol use were also relatively uncommon among women in the Black Women’s Health Study cohort, the investigators said.

“Future studies are needed to confirm these findings and establish the biologic mechanisms by which cigarette smoking and alcohol consumption influence the risk of SLE in this population and others,“ they wrote.

The study was supported by individual investigators’ grant awards from the National Cancer Institute, the National Institute of Arthritis and Musculoskeletal and Skin Diseases, the Rheumatology Research Foundation, and the Lupus Foundation of America. The authors declared no relevant conflicts of interest.

SOURCE: Cozier Y et al. Arthritis Care Res. 2018 Aug 9. doi: 10.1002/acr.23703.

Black women who smoke cigarettes are at increased risk of systemic lupus erythematosus (SLE), while those who drink alcohol in moderation have a decreased risk, data from the prospective Black Women’s Health Study suggests.

The current findings among black women, who are the demographic group at the highest risk of SLE in the U.S. population, are consistent with the previously reported positive association of cigarette smoking with risk of SLE and inverse association of alcohol consumption with SLE,” lead researchers Yvette Cozier, DSc, and Medha Barbhaiya, MD, and their colleagues wrote in a study published in Arthritis Care & Research.

“The role of environmental factors in the pathogenesis of SLE is of great interest, as genetic factors do not explain a major portion of the incidence. Cigarette smoking has been associated with SLE risk in several, but not all, past studies,” wrote Dr. Cozier of the Slone Epidemiology Center at Boston University and Dr. Barbhaiya of the Hospital for Special Surgery, New York, and their coauthors.

Their analysis of data from the Black Women’s Health Study, a prospective study following 59,000 black women in the United States since 1995, identified 127 new cases of SLE between 1995 and 2015 that were confirmed by medical records.

At baseline, 8,851 women (16%) were current smokers, and 10,447 (18%) were past smokers. A total of 14,001 (25%) were current drinkers, and 10,255 (18%) were past drinkers.

The researchers reported that, compared with never smokers, women who had ever smoked had a 45% higher risk for SLE than did never smokers (hazard ratio, 1.45; 95% confidence interval, 0.97-2.18), but the finding was not statistically significant.

The risk of SLE was greater among current smokers (52% higher risk) than among past smokers (41% higher risk), and greater for 20 or more pack-years of smoking (60% higher risk) than for less than 20 pack-years of smoking (37% higher risk), although the authors noted that none of these differences were statistically significant.

Current and past alcohol consumption were associated with nonsignificant reductions in risk for SLE, 29% and 21%, respectively. However, “moderate” current alcohol consumption, measured as four or more drinks per week, was associated with a 57% reduction in risk for SLE (HR, 0.43; 95% CI, 0.19-0.96).

The researchers said that the associations between cigarette smoking and alcohol intake with SLE risk were biologically plausible and may lead to insights into SLE pathogenesis. For example, exposure to toxic components from cigarette smoke was associated with increased oxidative stress and stimulation of autoantibody production, and such exposure can directly damage endogenous proteins and DNA. Alcohol suppresses the synthesis of proinflammatory cytokines and may inhibit DNA synthesis.

The small number of lupus cases in the Black Women’s Health Study cohort limited the statistical power of the analysis. Smoking and alcohol use were also relatively uncommon among women in the Black Women’s Health Study cohort, the investigators said.

“Future studies are needed to confirm these findings and establish the biologic mechanisms by which cigarette smoking and alcohol consumption influence the risk of SLE in this population and others,“ they wrote.

The study was supported by individual investigators’ grant awards from the National Cancer Institute, the National Institute of Arthritis and Musculoskeletal and Skin Diseases, the Rheumatology Research Foundation, and the Lupus Foundation of America. The authors declared no relevant conflicts of interest.

SOURCE: Cozier Y et al. Arthritis Care Res. 2018 Aug 9. doi: 10.1002/acr.23703.

An update to the first-ever guideline on adult congenital heart disease, released today, provides new recommendations and a more nuanced classification system based on data and expertise accrued in the field over the past decade.

Better road map

The document is intended to provide a “better road map” for all providers who will see such patients in their practice, said Dr. Daniels, director of the adult congenital heart disease and pulmonary hypertension program at Ohio State University Heart Center and Nationwide Children’s Hospital, Columbus.

“There are not enough adult congenital heart disease cardiologists and programs in the country to care for the almost 1.5 million adults with congenital heart disease in the United States, so we know these patients are cared for by general cardiologists,” Dr. Daniels said in an interview. “Having some guidelines about when to refer to those patients was a huge part of the purpose of these updated guidelines.”

The revamped classification system underlying the new guidelines seeks to better characterize disease severity based on the complexity of its anatomy and physiology, according to Dr. Daniels.

Previous documents, including the original 2008 AHA/ACC guideline (Circulation. 2008 Nov 7;118:e714-833), focused mainly on anatomic classifications to rank severity, he said.

“Traditionally, we’ve based the severity of congenital heart disease based on the complexity of anatomy they were born with, but that goes only so far,” Dr. Daniels said in an interview.

More than just anatomy

In the new system, anatomy is classified as simple (I, e.g., isolated small atrial septal defect), moderately complex (II, e.g., coarctation of the aorta), or greatly complex (III, e.g., cyanotic congenital heart defect), while physiologic stages range from A to D, increasing along with the severity of physiologic variables such as New York Heart Association functional class; exercise capacity; aortic enlargement; arrhythmias; renal, hepatic, or pulmonary function; and venal or arterial stenosis.

A normotensive patient with repaired coarctation of the aorta would be classified as IIA if she had normal end-organ function and exercise capacity, whereas a similar patient with an ascending aortic diameter of 4.0 cm would be classified as IIB, and with the addition of moderate aortic stenosis, would be classified as IIIC, according to an example provided in the guidelines.

Congenital heart disease specialist Robert “Jake” Jaquiss, MD, said in an interview that consideration of physiology alongside anatomy is one of the most important features of the new guidelines.

“This is a way of thinking about patients that involves not just their anatomy, but also considering a variety of domains in which there may be physiologic dysfunction that can modify the underlying anatomy,” said Dr. Jaquiss, chief of pediatric and congenital heart surgery at Children’s Medical Center/University of Texas Southwestern Medical Center, Dallas. “I think that is a more clinically relevant way to think about patients and patient evaluation management, and I commend the authors for that focus.”

For example, one patient who has undergone a Fontan procedure may be fully functional, whereas another with nearly identical anatomy may be burdened by arrhythmias, fluid retention, and impaired exercise function. “If we don’t grasp the physiologic difference, we treat the two patients the same way, which is obviously inappropriate,” Dr. Jaquiss said.

Research directions

Much more research needs to be done, according to guideline authors, who list 60 unresolved research questions that represent evidence gaps and future directions for study.

For example, outstanding questions related to tetralogy of Fallot focus on the optimal timing for pulmonary valve replacement, the role of pulmonary valve replacement and ventricular tachycardia ablation in decreasing sudden cardiac death risk, and whether implantable cardioverter-defibrillators reduce mortality.

“These are the kinds of evolutionary sort of understandings and alterations in the intervention, both at the original operation and for assessment of care, and what we think about it and how we think about it in 2018 is quite different than how we thought in 2008, and I dare say it’ll be even more different in 2028,” Dr. Jaquiss said.

The AHA/ACC guideline was developed in association with the American Association for Thoracic Surgery, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons.

Dr. Daniels reported a relevant relationship with Actelion. Guideline coauthors reported relevant relationships in that same category with Actelion, Boehringer Ingelheim, Cormatrix, Edward Lifesciences, Gilead, Medtronic, Novartis, Sorin (LivaNova), St. Jude Medical, and United Therapeutics. No other disclosures were reported.

SOURCE: Stout KK et al. J Am Coll Cardiol. 2018 Aug 16. Copublished in Circulation.

An update to the first-ever guideline on adult congenital heart disease, released today, provides new recommendations and a more nuanced classification system based on data and expertise accrued in the field over the past decade.

Better road map

The document is intended to provide a “better road map” for all providers who will see such patients in their practice, said Dr. Daniels, director of the adult congenital heart disease and pulmonary hypertension program at Ohio State University Heart Center and Nationwide Children’s Hospital, Columbus.

“There are not enough adult congenital heart disease cardiologists and programs in the country to care for the almost 1.5 million adults with congenital heart disease in the United States, so we know these patients are cared for by general cardiologists,” Dr. Daniels said in an interview. “Having some guidelines about when to refer to those patients was a huge part of the purpose of these updated guidelines.”

The revamped classification system underlying the new guidelines seeks to better characterize disease severity based on the complexity of its anatomy and physiology, according to Dr. Daniels.

Previous documents, including the original 2008 AHA/ACC guideline (Circulation. 2008 Nov 7;118:e714-833), focused mainly on anatomic classifications to rank severity, he said.

“Traditionally, we’ve based the severity of congenital heart disease based on the complexity of anatomy they were born with, but that goes only so far,” Dr. Daniels said in an interview.

More than just anatomy

In the new system, anatomy is classified as simple (I, e.g., isolated small atrial septal defect), moderately complex (II, e.g., coarctation of the aorta), or greatly complex (III, e.g., cyanotic congenital heart defect), while physiologic stages range from A to D, increasing along with the severity of physiologic variables such as New York Heart Association functional class; exercise capacity; aortic enlargement; arrhythmias; renal, hepatic, or pulmonary function; and venal or arterial stenosis.

A normotensive patient with repaired coarctation of the aorta would be classified as IIA if she had normal end-organ function and exercise capacity, whereas a similar patient with an ascending aortic diameter of 4.0 cm would be classified as IIB, and with the addition of moderate aortic stenosis, would be classified as IIIC, according to an example provided in the guidelines.

Congenital heart disease specialist Robert “Jake” Jaquiss, MD, said in an interview that consideration of physiology alongside anatomy is one of the most important features of the new guidelines.

“This is a way of thinking about patients that involves not just their anatomy, but also considering a variety of domains in which there may be physiologic dysfunction that can modify the underlying anatomy,” said Dr. Jaquiss, chief of pediatric and congenital heart surgery at Children’s Medical Center/University of Texas Southwestern Medical Center, Dallas. “I think that is a more clinically relevant way to think about patients and patient evaluation management, and I commend the authors for that focus.”

For example, one patient who has undergone a Fontan procedure may be fully functional, whereas another with nearly identical anatomy may be burdened by arrhythmias, fluid retention, and impaired exercise function. “If we don’t grasp the physiologic difference, we treat the two patients the same way, which is obviously inappropriate,” Dr. Jaquiss said.

Research directions

Much more research needs to be done, according to guideline authors, who list 60 unresolved research questions that represent evidence gaps and future directions for study.

For example, outstanding questions related to tetralogy of Fallot focus on the optimal timing for pulmonary valve replacement, the role of pulmonary valve replacement and ventricular tachycardia ablation in decreasing sudden cardiac death risk, and whether implantable cardioverter-defibrillators reduce mortality.

“These are the kinds of evolutionary sort of understandings and alterations in the intervention, both at the original operation and for assessment of care, and what we think about it and how we think about it in 2018 is quite different than how we thought in 2008, and I dare say it’ll be even more different in 2028,” Dr. Jaquiss said.

The AHA/ACC guideline was developed in association with the American Association for Thoracic Surgery, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons.

Dr. Daniels reported a relevant relationship with Actelion. Guideline coauthors reported relevant relationships in that same category with Actelion, Boehringer Ingelheim, Cormatrix, Edward Lifesciences, Gilead, Medtronic, Novartis, Sorin (LivaNova), St. Jude Medical, and United Therapeutics. No other disclosures were reported.

SOURCE: Stout KK et al. J Am Coll Cardiol. 2018 Aug 16. Copublished in Circulation.

An update to the first-ever guideline on adult congenital heart disease, released today, provides new recommendations and a more nuanced classification system based on data and expertise accrued in the field over the past decade.

Better road map

The document is intended to provide a “better road map” for all providers who will see such patients in their practice, said Dr. Daniels, director of the adult congenital heart disease and pulmonary hypertension program at Ohio State University Heart Center and Nationwide Children’s Hospital, Columbus.

“There are not enough adult congenital heart disease cardiologists and programs in the country to care for the almost 1.5 million adults with congenital heart disease in the United States, so we know these patients are cared for by general cardiologists,” Dr. Daniels said in an interview. “Having some guidelines about when to refer to those patients was a huge part of the purpose of these updated guidelines.”

The revamped classification system underlying the new guidelines seeks to better characterize disease severity based on the complexity of its anatomy and physiology, according to Dr. Daniels.

Previous documents, including the original 2008 AHA/ACC guideline (Circulation. 2008 Nov 7;118:e714-833), focused mainly on anatomic classifications to rank severity, he said.

“Traditionally, we’ve based the severity of congenital heart disease based on the complexity of anatomy they were born with, but that goes only so far,” Dr. Daniels said in an interview.

More than just anatomy

In the new system, anatomy is classified as simple (I, e.g., isolated small atrial septal defect), moderately complex (II, e.g., coarctation of the aorta), or greatly complex (III, e.g., cyanotic congenital heart defect), while physiologic stages range from A to D, increasing along with the severity of physiologic variables such as New York Heart Association functional class; exercise capacity; aortic enlargement; arrhythmias; renal, hepatic, or pulmonary function; and venal or arterial stenosis.

A normotensive patient with repaired coarctation of the aorta would be classified as IIA if she had normal end-organ function and exercise capacity, whereas a similar patient with an ascending aortic diameter of 4.0 cm would be classified as IIB, and with the addition of moderate aortic stenosis, would be classified as IIIC, according to an example provided in the guidelines.

Congenital heart disease specialist Robert “Jake” Jaquiss, MD, said in an interview that consideration of physiology alongside anatomy is one of the most important features of the new guidelines.

“This is a way of thinking about patients that involves not just their anatomy, but also considering a variety of domains in which there may be physiologic dysfunction that can modify the underlying anatomy,” said Dr. Jaquiss, chief of pediatric and congenital heart surgery at Children’s Medical Center/University of Texas Southwestern Medical Center, Dallas. “I think that is a more clinically relevant way to think about patients and patient evaluation management, and I commend the authors for that focus.”

For example, one patient who has undergone a Fontan procedure may be fully functional, whereas another with nearly identical anatomy may be burdened by arrhythmias, fluid retention, and impaired exercise function. “If we don’t grasp the physiologic difference, we treat the two patients the same way, which is obviously inappropriate,” Dr. Jaquiss said.

Research directions

Much more research needs to be done, according to guideline authors, who list 60 unresolved research questions that represent evidence gaps and future directions for study.

For example, outstanding questions related to tetralogy of Fallot focus on the optimal timing for pulmonary valve replacement, the role of pulmonary valve replacement and ventricular tachycardia ablation in decreasing sudden cardiac death risk, and whether implantable cardioverter-defibrillators reduce mortality.

“These are the kinds of evolutionary sort of understandings and alterations in the intervention, both at the original operation and for assessment of care, and what we think about it and how we think about it in 2018 is quite different than how we thought in 2008, and I dare say it’ll be even more different in 2028,” Dr. Jaquiss said.

The AHA/ACC guideline was developed in association with the American Association for Thoracic Surgery, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons.

Dr. Daniels reported a relevant relationship with Actelion. Guideline coauthors reported relevant relationships in that same category with Actelion, Boehringer Ingelheim, Cormatrix, Edward Lifesciences, Gilead, Medtronic, Novartis, Sorin (LivaNova), St. Jude Medical, and United Therapeutics. No other disclosures were reported.

SOURCE: Stout KK et al. J Am Coll Cardiol. 2018 Aug 16. Copublished in Circulation.

Even as summer continues on, the long, hot, sunny days and backyard barbecues can be tinged with the realization that the new school year looms ahead. For those in traditional school systems, September is the return to the classroom. This has long been a source of angst for many children who may face a new school or a return to the challenging, even overwhelming, business of learning.

“The end of summer and the beginning of a new school year can be a stressful time for parents and children,” says psychologist Lynn Bufka, PhD, assistant execute director for practice, research, and policy for the American Psychological Association. “While trying to manage work and the household, parents can sometimes overlook their children’s feelings of nervousness or anxiety as school begins. Working with your children to build resilience and manage their emotions can be beneficial for the psychological health of the whole family.”

According to the association, parents can help ease their child’s worries by offering support and encouragement and by listening to their child’s concerns, which can help foster their resilience. A dry run about a week before the big day can help reset the summer sleep schedule. Getting school supplies together and ready for action is another bit of preparation that helps get the mind ready for the reality of school. Visiting a new school, if permitted, can remove some sense of the unknown and lay the path for that first day through the front door. Empathy can be a powerful aid; understanding that a child might be apprehensive can prevent the potentially misguided advice to just tough it out.

But children aren’t the only ones with back-to-school anxiety. Increasingly, this time of year is generating stress for parents. When budgets are stretched tight, the additional school expenses can be a strain. The horrors of school shooting incidents can be on parents’ minds, especially when their child is enrolled in a new and unfamiliar school. An era of heightened competitiveness for postsecondary education and scholarship money comes with the baggage of worry that a son or daughter is lagging and already might be behind the eight ball in the game of life.

Parental stress can be tough to deal with. Budget planning, participating in safe-school activities, fostering relationships with teachers and staff, having a heightened awareness of signs of school-related stress in their children, and maintaining faith in their children’s ability to learn and succeed can go a long way toward easing the parental burden.

Click here to read the APA’s back-to-school tips.

Even as summer continues on, the long, hot, sunny days and backyard barbecues can be tinged with the realization that the new school year looms ahead. For those in traditional school systems, September is the return to the classroom. This has long been a source of angst for many children who may face a new school or a return to the challenging, even overwhelming, business of learning.

“The end of summer and the beginning of a new school year can be a stressful time for parents and children,” says psychologist Lynn Bufka, PhD, assistant execute director for practice, research, and policy for the American Psychological Association. “While trying to manage work and the household, parents can sometimes overlook their children’s feelings of nervousness or anxiety as school begins. Working with your children to build resilience and manage their emotions can be beneficial for the psychological health of the whole family.”

According to the association, parents can help ease their child’s worries by offering support and encouragement and by listening to their child’s concerns, which can help foster their resilience. A dry run about a week before the big day can help reset the summer sleep schedule. Getting school supplies together and ready for action is another bit of preparation that helps get the mind ready for the reality of school. Visiting a new school, if permitted, can remove some sense of the unknown and lay the path for that first day through the front door. Empathy can be a powerful aid; understanding that a child might be apprehensive can prevent the potentially misguided advice to just tough it out.

But children aren’t the only ones with back-to-school anxiety. Increasingly, this time of year is generating stress for parents. When budgets are stretched tight, the additional school expenses can be a strain. The horrors of school shooting incidents can be on parents’ minds, especially when their child is enrolled in a new and unfamiliar school. An era of heightened competitiveness for postsecondary education and scholarship money comes with the baggage of worry that a son or daughter is lagging and already might be behind the eight ball in the game of life.

Parental stress can be tough to deal with. Budget planning, participating in safe-school activities, fostering relationships with teachers and staff, having a heightened awareness of signs of school-related stress in their children, and maintaining faith in their children’s ability to learn and succeed can go a long way toward easing the parental burden.

Click here to read the APA’s back-to-school tips.

Even as summer continues on, the long, hot, sunny days and backyard barbecues can be tinged with the realization that the new school year looms ahead. For those in traditional school systems, September is the return to the classroom. This has long been a source of angst for many children who may face a new school or a return to the challenging, even overwhelming, business of learning.

“The end of summer and the beginning of a new school year can be a stressful time for parents and children,” says psychologist Lynn Bufka, PhD, assistant execute director for practice, research, and policy for the American Psychological Association. “While trying to manage work and the household, parents can sometimes overlook their children’s feelings of nervousness or anxiety as school begins. Working with your children to build resilience and manage their emotions can be beneficial for the psychological health of the whole family.”

According to the association, parents can help ease their child’s worries by offering support and encouragement and by listening to their child’s concerns, which can help foster their resilience. A dry run about a week before the big day can help reset the summer sleep schedule. Getting school supplies together and ready for action is another bit of preparation that helps get the mind ready for the reality of school. Visiting a new school, if permitted, can remove some sense of the unknown and lay the path for that first day through the front door. Empathy can be a powerful aid; understanding that a child might be apprehensive can prevent the potentially misguided advice to just tough it out.

But children aren’t the only ones with back-to-school anxiety. Increasingly, this time of year is generating stress for parents. When budgets are stretched tight, the additional school expenses can be a strain. The horrors of school shooting incidents can be on parents’ minds, especially when their child is enrolled in a new and unfamiliar school. An era of heightened competitiveness for postsecondary education and scholarship money comes with the baggage of worry that a son or daughter is lagging and already might be behind the eight ball in the game of life.

Parental stress can be tough to deal with. Budget planning, participating in safe-school activities, fostering relationships with teachers and staff, having a heightened awareness of signs of school-related stress in their children, and maintaining faith in their children’s ability to learn and succeed can go a long way toward easing the parental burden.

Click here to read the APA’s back-to-school tips.

Physicians take the “first do no harm” aphorism seriously. Still, as humans, their biases and opinions can – and do – cause harm. An example, according to an opinion piece written in the Globe and Mail, is the way physicians sometimes treat women who are overweight.

The article cites an Alabama woman named Kayla Rahm, whose complaints of weight gain, abdominal swelling, and shortness of breath fell on the deaf ears of four doctors who, instead of getting to the bottom of her problem, pointed to her weight as the culprit. Ultimately, she had a 50-pound ovarian cyst removed (thankfully, benign.)

“She was seeking help from multiple physicians, and we had missed it – as a medical community, we had missed it,” said Gregory Jones, DO, the obstetrician-gynecologist who performed the eventual surgery, in an interview with the Washington Post.

The weight-related rebuff is all too common for women. The consequences can be tragic. Consider Ellen Maud Bennett, a Canadian woman whose complaints of ill health stretching back years had been met with suggestions to lose weight. All the while, cancer was developing, and when the truth was finally recognized, it was too late.

Her obituary offered this poignant message: “A final message Ellen wanted to share was about the fat shaming she endured from the medical profession. Over the past few years of feeling unwell, she sought out medical intervention and no one offered any support or suggestions beyond weight loss. Ellen’s dying wish was that women of size make her death matter by advocating strongly for their health and not accepting that fat is the only relevant health issue.”

Overweight women are reportedly less likely to be referred for cervical and breast-cancer screenings. Fat shaming can dissuade women from seeking medical help. Instead, they can buy into the view that their health problems are self-imposed.

A recent study conducted in Canada involving more than 54,000 clinically obese people, some with metabolic risk factors and others were who just obese, found that those with no metabolic risk factors were no more likely to die than people of lower weight. “This means that hundreds of thousands of people in North America alone with metabolically healthy obesity will be told to lose weight when it’s questionable how much benefit they’ll actually receive ,” head researcher Jennifer L. Kuk, PhD, said in an interview with Science Daily.

Click here to read about Dr. Kuk’s study.
 

Physicians take the “first do no harm” aphorism seriously. Still, as humans, their biases and opinions can – and do – cause harm. An example, according to an opinion piece written in the Globe and Mail, is the way physicians sometimes treat women who are overweight.

The article cites an Alabama woman named Kayla Rahm, whose complaints of weight gain, abdominal swelling, and shortness of breath fell on the deaf ears of four doctors who, instead of getting to the bottom of her problem, pointed to her weight as the culprit. Ultimately, she had a 50-pound ovarian cyst removed (thankfully, benign.)

“She was seeking help from multiple physicians, and we had missed it – as a medical community, we had missed it,” said Gregory Jones, DO, the obstetrician-gynecologist who performed the eventual surgery, in an interview with the Washington Post.

The weight-related rebuff is all too common for women. The consequences can be tragic. Consider Ellen Maud Bennett, a Canadian woman whose complaints of ill health stretching back years had been met with suggestions to lose weight. All the while, cancer was developing, and when the truth was finally recognized, it was too late.

Her obituary offered this poignant message: “A final message Ellen wanted to share was about the fat shaming she endured from the medical profession. Over the past few years of feeling unwell, she sought out medical intervention and no one offered any support or suggestions beyond weight loss. Ellen’s dying wish was that women of size make her death matter by advocating strongly for their health and not accepting that fat is the only relevant health issue.”

Overweight women are reportedly less likely to be referred for cervical and breast-cancer screenings. Fat shaming can dissuade women from seeking medical help. Instead, they can buy into the view that their health problems are self-imposed.

A recent study conducted in Canada involving more than 54,000 clinically obese people, some with metabolic risk factors and others were who just obese, found that those with no metabolic risk factors were no more likely to die than people of lower weight. “This means that hundreds of thousands of people in North America alone with metabolically healthy obesity will be told to lose weight when it’s questionable how much benefit they’ll actually receive ,” head researcher Jennifer L. Kuk, PhD, said in an interview with Science Daily.

Click here to read about Dr. Kuk’s study.
 

Physicians take the “first do no harm” aphorism seriously. Still, as humans, their biases and opinions can – and do – cause harm. An example, according to an opinion piece written in the Globe and Mail, is the way physicians sometimes treat women who are overweight.

The article cites an Alabama woman named Kayla Rahm, whose complaints of weight gain, abdominal swelling, and shortness of breath fell on the deaf ears of four doctors who, instead of getting to the bottom of her problem, pointed to her weight as the culprit. Ultimately, she had a 50-pound ovarian cyst removed (thankfully, benign.)

“She was seeking help from multiple physicians, and we had missed it – as a medical community, we had missed it,” said Gregory Jones, DO, the obstetrician-gynecologist who performed the eventual surgery, in an interview with the Washington Post.

The weight-related rebuff is all too common for women. The consequences can be tragic. Consider Ellen Maud Bennett, a Canadian woman whose complaints of ill health stretching back years had been met with suggestions to lose weight. All the while, cancer was developing, and when the truth was finally recognized, it was too late.

Her obituary offered this poignant message: “A final message Ellen wanted to share was about the fat shaming she endured from the medical profession. Over the past few years of feeling unwell, she sought out medical intervention and no one offered any support or suggestions beyond weight loss. Ellen’s dying wish was that women of size make her death matter by advocating strongly for their health and not accepting that fat is the only relevant health issue.”

Overweight women are reportedly less likely to be referred for cervical and breast-cancer screenings. Fat shaming can dissuade women from seeking medical help. Instead, they can buy into the view that their health problems are self-imposed.

A recent study conducted in Canada involving more than 54,000 clinically obese people, some with metabolic risk factors and others were who just obese, found that those with no metabolic risk factors were no more likely to die than people of lower weight. “This means that hundreds of thousands of people in North America alone with metabolically healthy obesity will be told to lose weight when it’s questionable how much benefit they’ll actually receive ,” head researcher Jennifer L. Kuk, PhD, said in an interview with Science Daily.

Click here to read about Dr. Kuk’s study.