Miami Children's Hospital: Annual Pediatric Postgraduate Course "Perspectives in Pediatrics"

MIAMI – Recent discoveries in epigenetics could soon make a clinical difference for children with attention-deficit/hyperactivity disorder, autism spectrum disorder, and other conditions, according to an expert.

The implications go beyond targeting treatment for an individual child in your practice based on a specific genetic mutation, Dr. Hakon Hakonarson, director of the center for applied genomics at the Children’s Hospital of Philadelphia, said at a pediatric update sponsored by Miami Children’s Hospital. Recent advances point to gene alterations that modify shared pathways, so the potential for wider application beyond ADHD and ASD -- such as unexpected discoveries related to pediatric neuroblastoma -- is a real possibility.

Dr. Hakonarson said that he had no relevant financial disclosures.

MIAMI – Recent discoveries in epigenetics could soon make a clinical difference for children with attention-deficit/hyperactivity disorder, autism spectrum disorder, and other conditions, according to an expert.

The implications go beyond targeting treatment for an individual child in your practice based on a specific genetic mutation, Dr. Hakon Hakonarson, director of the center for applied genomics at the Children’s Hospital of Philadelphia, said at a pediatric update sponsored by Miami Children’s Hospital. Recent advances point to gene alterations that modify shared pathways, so the potential for wider application beyond ADHD and ASD -- such as unexpected discoveries related to pediatric neuroblastoma -- is a real possibility.

Dr. Hakonarson said that he had no relevant financial disclosures.

MIAMI – Recent discoveries in epigenetics could soon make a clinical difference for children with attention-deficit/hyperactivity disorder, autism spectrum disorder, and other conditions, according to an expert.

The implications go beyond targeting treatment for an individual child in your practice based on a specific genetic mutation, Dr. Hakon Hakonarson, director of the center for applied genomics at the Children’s Hospital of Philadelphia, said at a pediatric update sponsored by Miami Children’s Hospital. Recent advances point to gene alterations that modify shared pathways, so the potential for wider application beyond ADHD and ASD -- such as unexpected discoveries related to pediatric neuroblastoma -- is a real possibility.

Dr. Hakonarson said that he had no relevant financial disclosures.

MIAMI – A pediatric nephrologist shared her perspective, including positive aspects and potential pitfalls, in the months since the American Academy of Pediatrics released updated clinical practice guidelines for diagnosis and management of an initial urinary tract infection in febrile children aged 2-24 months.

"These are guidelines, not protocols. These are here to guide us. They are not intended to replace your clinical judgment, please remember this," Dr. Ana L. Paredes said at a pediatric update sponsored by Miami Children’s Hospital.

Dr. Paredes reviewed the guideline and its seven action statements, most of which address routine, non-complicated cases, she said (Pediatrics 2011;128:595-610). Diagnosis of urinary tract infection (UTI) based on both pyuria and at least 50,000 colonies of a single urinary pathogen in a specimen collected by catheterization or suprapubic aspiration is a new recommendation. "There is a consideration that if your lab only reports from 10,000 to 100,000 colonies, then 10,000 would make the diagnosis," Dr. Paredes said.

Also, culture of a urine sample collected via one of these two methods is required prior to antibiotic treatment if a bagged urine sample tests positive for leukocytes or nitrates, according to the guidelines.

If a clinician judges that antimicrobial treatment is warranted for an infant with no apparent source of infection, a urine specimen for urinalysis and culture is always required before starting treatment, the guidelines state. Clinical status of the patient guides the route of antimicrobial administration.

Close follow-up and monitoring for recurrent infection is warranted after completion of the recommended 7-14 days of antimicrobial therapy. Instruct parents to seek prompt medical attention at the onset of any new fever to facilitate quick diagnosis and treatment of any recurrence.

"These are guidelines, not protocols. These are here to guide us."

The AAP initially released the guidelines in 1999. The update is based on a meta-analysis of six randomized controlled trials. The guideline writers asked each of these study authors to provide raw data from their trials and then extracted information for infants aged 2-24 months ("AAP Updates Policy on First UTI in Febrile Infant," Pediatric News).

Although the meta-analysis included 1,091 infants, there were none with vesicoureter reflux grades IV and only 5 infants with grade V, Dr. Paredes said. A lack of more severe reflux in the meta-analysis is a potential limitation of the guidelines, she said.

The seven physician authors, all members of the AAP Subcommittee on Urinary Tract Infection, determined that antimicrobial prophylaxis is not warranted to prevent febrile recurrent UTI for infants who have no or grade I to IV vesicoureteral reflux.

"As per the AAP, a voiding cystourethrography [VCUG] is not routinely indicated after the first febrile UTI," said Dr. Paredes, pediatric nephrologist and director of renal research at Miami Children’s Hospital. She was not an author of the guidelines.

"A take-home message is perform a renal and bladder ultrasound" for febrile UTI in infants 2-24 months of age, Dr. Paredes said. "This is recommended during the first 2 days of treatment when clinical presentation is severe," such as during the first 48 hours of hospital admission.

If the renal and bladder ultrasound reveals hydronephrosis, scarring, or other evidence of high grade vesicoureteral reflux or obstructive uropathy, VCUG is indicated. "Also, VCUG is always indicated after a second febrile UTI," Dr. Paredes said.

A useful table in the guidelines lists risk factors for UTI by gender. For girls, white race; age less than 12 months; a fever lasting 2 days or longer; temperature of 39° C or greater; and absence of another source of infection increase the probability of a UTI. For boys, non–black race; temperature of 39° C or greater; fever lasting more than 24 hours; and, again, absence of another source of infection were risk factors identified.

Recommended oral antimicrobial agents include cephalosporins (cefixime, cefdinir, cefuroxime, and cephalexin), sulfonamides, or amoxicillin-clavulanate. Preferred parenteral antimicrobials include ceftriaxone, cefotaxime, ceftazidime, gentamicin, and tobramycin.

Dr. Paredes reported that she had no relevant financial disclosures.

MIAMI – A pediatric nephrologist shared her perspective, including positive aspects and potential pitfalls, in the months since the American Academy of Pediatrics released updated clinical practice guidelines for diagnosis and management of an initial urinary tract infection in febrile children aged 2-24 months.

"These are guidelines, not protocols. These are here to guide us. They are not intended to replace your clinical judgment, please remember this," Dr. Ana L. Paredes said at a pediatric update sponsored by Miami Children’s Hospital.

Dr. Paredes reviewed the guideline and its seven action statements, most of which address routine, non-complicated cases, she said (Pediatrics 2011;128:595-610). Diagnosis of urinary tract infection (UTI) based on both pyuria and at least 50,000 colonies of a single urinary pathogen in a specimen collected by catheterization or suprapubic aspiration is a new recommendation. "There is a consideration that if your lab only reports from 10,000 to 100,000 colonies, then 10,000 would make the diagnosis," Dr. Paredes said.

Also, culture of a urine sample collected via one of these two methods is required prior to antibiotic treatment if a bagged urine sample tests positive for leukocytes or nitrates, according to the guidelines.

If a clinician judges that antimicrobial treatment is warranted for an infant with no apparent source of infection, a urine specimen for urinalysis and culture is always required before starting treatment, the guidelines state. Clinical status of the patient guides the route of antimicrobial administration.

Close follow-up and monitoring for recurrent infection is warranted after completion of the recommended 7-14 days of antimicrobial therapy. Instruct parents to seek prompt medical attention at the onset of any new fever to facilitate quick diagnosis and treatment of any recurrence.

"These are guidelines, not protocols. These are here to guide us."

The AAP initially released the guidelines in 1999. The update is based on a meta-analysis of six randomized controlled trials. The guideline writers asked each of these study authors to provide raw data from their trials and then extracted information for infants aged 2-24 months ("AAP Updates Policy on First UTI in Febrile Infant," Pediatric News).

Although the meta-analysis included 1,091 infants, there were none with vesicoureter reflux grades IV and only 5 infants with grade V, Dr. Paredes said. A lack of more severe reflux in the meta-analysis is a potential limitation of the guidelines, she said.

The seven physician authors, all members of the AAP Subcommittee on Urinary Tract Infection, determined that antimicrobial prophylaxis is not warranted to prevent febrile recurrent UTI for infants who have no or grade I to IV vesicoureteral reflux.

"As per the AAP, a voiding cystourethrography [VCUG] is not routinely indicated after the first febrile UTI," said Dr. Paredes, pediatric nephrologist and director of renal research at Miami Children’s Hospital. She was not an author of the guidelines.

"A take-home message is perform a renal and bladder ultrasound" for febrile UTI in infants 2-24 months of age, Dr. Paredes said. "This is recommended during the first 2 days of treatment when clinical presentation is severe," such as during the first 48 hours of hospital admission.

If the renal and bladder ultrasound reveals hydronephrosis, scarring, or other evidence of high grade vesicoureteral reflux or obstructive uropathy, VCUG is indicated. "Also, VCUG is always indicated after a second febrile UTI," Dr. Paredes said.

A useful table in the guidelines lists risk factors for UTI by gender. For girls, white race; age less than 12 months; a fever lasting 2 days or longer; temperature of 39° C or greater; and absence of another source of infection increase the probability of a UTI. For boys, non–black race; temperature of 39° C or greater; fever lasting more than 24 hours; and, again, absence of another source of infection were risk factors identified.

Recommended oral antimicrobial agents include cephalosporins (cefixime, cefdinir, cefuroxime, and cephalexin), sulfonamides, or amoxicillin-clavulanate. Preferred parenteral antimicrobials include ceftriaxone, cefotaxime, ceftazidime, gentamicin, and tobramycin.

Dr. Paredes reported that she had no relevant financial disclosures.

MIAMI – A pediatric nephrologist shared her perspective, including positive aspects and potential pitfalls, in the months since the American Academy of Pediatrics released updated clinical practice guidelines for diagnosis and management of an initial urinary tract infection in febrile children aged 2-24 months.

"These are guidelines, not protocols. These are here to guide us. They are not intended to replace your clinical judgment, please remember this," Dr. Ana L. Paredes said at a pediatric update sponsored by Miami Children’s Hospital.

Dr. Paredes reviewed the guideline and its seven action statements, most of which address routine, non-complicated cases, she said (Pediatrics 2011;128:595-610). Diagnosis of urinary tract infection (UTI) based on both pyuria and at least 50,000 colonies of a single urinary pathogen in a specimen collected by catheterization or suprapubic aspiration is a new recommendation. "There is a consideration that if your lab only reports from 10,000 to 100,000 colonies, then 10,000 would make the diagnosis," Dr. Paredes said.

Also, culture of a urine sample collected via one of these two methods is required prior to antibiotic treatment if a bagged urine sample tests positive for leukocytes or nitrates, according to the guidelines.

If a clinician judges that antimicrobial treatment is warranted for an infant with no apparent source of infection, a urine specimen for urinalysis and culture is always required before starting treatment, the guidelines state. Clinical status of the patient guides the route of antimicrobial administration.

Close follow-up and monitoring for recurrent infection is warranted after completion of the recommended 7-14 days of antimicrobial therapy. Instruct parents to seek prompt medical attention at the onset of any new fever to facilitate quick diagnosis and treatment of any recurrence.

"These are guidelines, not protocols. These are here to guide us."

The AAP initially released the guidelines in 1999. The update is based on a meta-analysis of six randomized controlled trials. The guideline writers asked each of these study authors to provide raw data from their trials and then extracted information for infants aged 2-24 months ("AAP Updates Policy on First UTI in Febrile Infant," Pediatric News).

Although the meta-analysis included 1,091 infants, there were none with vesicoureter reflux grades IV and only 5 infants with grade V, Dr. Paredes said. A lack of more severe reflux in the meta-analysis is a potential limitation of the guidelines, she said.

The seven physician authors, all members of the AAP Subcommittee on Urinary Tract Infection, determined that antimicrobial prophylaxis is not warranted to prevent febrile recurrent UTI for infants who have no or grade I to IV vesicoureteral reflux.

"As per the AAP, a voiding cystourethrography [VCUG] is not routinely indicated after the first febrile UTI," said Dr. Paredes, pediatric nephrologist and director of renal research at Miami Children’s Hospital. She was not an author of the guidelines.

"A take-home message is perform a renal and bladder ultrasound" for febrile UTI in infants 2-24 months of age, Dr. Paredes said. "This is recommended during the first 2 days of treatment when clinical presentation is severe," such as during the first 48 hours of hospital admission.

If the renal and bladder ultrasound reveals hydronephrosis, scarring, or other evidence of high grade vesicoureteral reflux or obstructive uropathy, VCUG is indicated. "Also, VCUG is always indicated after a second febrile UTI," Dr. Paredes said.

A useful table in the guidelines lists risk factors for UTI by gender. For girls, white race; age less than 12 months; a fever lasting 2 days or longer; temperature of 39° C or greater; and absence of another source of infection increase the probability of a UTI. For boys, non–black race; temperature of 39° C or greater; fever lasting more than 24 hours; and, again, absence of another source of infection were risk factors identified.

Recommended oral antimicrobial agents include cephalosporins (cefixime, cefdinir, cefuroxime, and cephalexin), sulfonamides, or amoxicillin-clavulanate. Preferred parenteral antimicrobials include ceftriaxone, cefotaxime, ceftazidime, gentamicin, and tobramycin.

Dr. Paredes reported that she had no relevant financial disclosures.

MIAMI – Pediatricians play an essential role in optimizing the diagnosis, care, and outcomes of children with inflammatory bowel disease, according to a pediatric gastroenterologist with expertise in this area.

"What would we like you to do as a pediatrician, perhaps, before they come to our office? There are definitely things you can start as far as the workup is concerned," Dr. Alisa J. Muñiz Crim said at a pediatric update sponsored by Miami Children’s Hospital. The pediatrician can rule out infectious diarrhea; evaluate the patient for rashes and other external manifestations; and order specific laboratory and biomarker tests, for example.

If further evaluation with endoscopy, colonoscopy, or imaging is warranted, consult a pediatric gastroenterology specialist, said Dr. Muñiz Crim, a pediatric gastroenterologist at Miami Children’s Hospital.

Suppression of inflammation, alteration of the disease course, and improvement in quality of life are among the shared goals of IBD treatment, Dr. Muñiz Crim said. Prevention of complications and disease relapse are additional aims.

Of the estimated 1 million to 2 million affected Americans, approximately 100,000 are younger than 18 years. These figures include about a 50% increase in children who have been diagnosed with IBD in the past decade. "We believe this is a true increase in the incidence of inflammatory bowel disease, not just an increase in the diagnostic capabilities," Dr. Muñiz Crim said.

Although IBD (which includes both ulcerative colitis and Crohn’s disease) affects both adults and children, there are special considerations for the pediatric population. Abdominal pain, diarrhea, rectal bleeding, anemia, and perianal disease are classic IBD symptoms. However, "presentations can be more severe in children," Dr. Muñiz Crim said.

As an example, children with ulcerative colitis experience a higher incidence (up to 80%) of a more severe form called pancolitis. Pancolitis is the single biggest risk factor for colorectal cancer in patients with ulcerative colitis.

The risk for colorectal cancer in the setting of ulcerative colitis is estimated at 2% at 10 years after diagnosis, 8% by 20 years, and 18% by 30 years. "Those numbers are very high and ... alarming to families," Dr. Muñiz Crim said. "We don’t deal with it very much as pediatric physicians, but it’s something [patients] need to know early on." Emphasize that adolescents who transition to adult provider still need to be closely monitored.

A greater risk of progression to surgery for some children with Crohn’s disease, as well as greater risk of psychosocial impact from their disease, also characterize pediatric IBD, Dr. Muñiz Crim said.

Pediatricians can help address another high risk concern in these patients: growth and pubertal delay. "Growth failure is very common, more in Crohn’s disease than in ulcerative colitis," Dr. Muñiz Crim said. Approximately 36%-39% of children with IBD will demonstrate decreased height percentiles.

Diagnosis and intervention typically lead to gradual improvements, "but their height and weight don’t make it back" to where those measures would be without the disease, Dr. Muñiz Crim said. "Adult height is often compromised. It is a big issue for our male adolescent patients in particular."

Multiple factors in IBD can impede growth, including inadequate dietary intake, malabsorption, and disease location. Children typically experience more growth failure when IBD affects the small intestine, Dr. Muñiz Crim said.

Early diagnosis and intervention improve the likelihood of minimizing adverse growth effects. Optimize nutrition through careful evaluation and calorie supplementation in concert with a nutritionist, Dr. Muñiz Crim suggested. Use routine laboratory testing to monitor disease progression.

Stay away from prolonged corticosteroid treatment, said Dr. Muñiz Crim. Although corticosteroids are appropriately prescribed to induce disease remission initially, "prolonged use can have debilitating effects" on growth, bone metabolism, and other effects. You might need to carefully counsel patients and families about the dangers of long-term steroid use, she added, because it seems counterintuitive to stop therapy when it makes patients feel better. Biologics and immunomodulators are the preferred maintenance medications to help patients achieve their growth potential, Dr. Muñiz Crim said.

The treatment of pediatric IBD patients relies on clinical judgment because most evidence is extrapolated from adult studies. Some pediatric studies with smaller numbers of participants show, for example, that therapy can induce disease remission to an extent similar to that in adults. A remaining issue, Dr. Muñiz Crim said, is that "we don’t know the lifetime toxicity of some of these medications because they haven’t been around long enough."

Continue routine immunizations in most children with IBD, Dr. Muñiz Crim said. Importantly, catch up with any vaccinations they might be missing upon diagnosis, she added, and avoid live vaccines, as always, in immunocompromised patients.

Useful serum assays include C-reactive protein, erythrocyte sedimentation rate, albumin, and hemoglobin and hematocrit. Appropriate antibody assays include P-ANCA (perinuclear antineutrophil cytoplasmic antibodies) and ASCA (anti–Saccharomyces cerevisiae antibodies). Extraintestinal manifestations of IBD that you might see include aphthous ulcerations, erythema nodosum, pyoderma gangrenosum, and perianal disease.

Dr. Muñiz Crim said that she had no relevant financial disclosures.

MIAMI – Pediatricians play an essential role in optimizing the diagnosis, care, and outcomes of children with inflammatory bowel disease, according to a pediatric gastroenterologist with expertise in this area.

"What would we like you to do as a pediatrician, perhaps, before they come to our office? There are definitely things you can start as far as the workup is concerned," Dr. Alisa J. Muñiz Crim said at a pediatric update sponsored by Miami Children’s Hospital. The pediatrician can rule out infectious diarrhea; evaluate the patient for rashes and other external manifestations; and order specific laboratory and biomarker tests, for example.

If further evaluation with endoscopy, colonoscopy, or imaging is warranted, consult a pediatric gastroenterology specialist, said Dr. Muñiz Crim, a pediatric gastroenterologist at Miami Children’s Hospital.

Suppression of inflammation, alteration of the disease course, and improvement in quality of life are among the shared goals of IBD treatment, Dr. Muñiz Crim said. Prevention of complications and disease relapse are additional aims.

Of the estimated 1 million to 2 million affected Americans, approximately 100,000 are younger than 18 years. These figures include about a 50% increase in children who have been diagnosed with IBD in the past decade. "We believe this is a true increase in the incidence of inflammatory bowel disease, not just an increase in the diagnostic capabilities," Dr. Muñiz Crim said.

Although IBD (which includes both ulcerative colitis and Crohn’s disease) affects both adults and children, there are special considerations for the pediatric population. Abdominal pain, diarrhea, rectal bleeding, anemia, and perianal disease are classic IBD symptoms. However, "presentations can be more severe in children," Dr. Muñiz Crim said.

As an example, children with ulcerative colitis experience a higher incidence (up to 80%) of a more severe form called pancolitis. Pancolitis is the single biggest risk factor for colorectal cancer in patients with ulcerative colitis.

The risk for colorectal cancer in the setting of ulcerative colitis is estimated at 2% at 10 years after diagnosis, 8% by 20 years, and 18% by 30 years. "Those numbers are very high and ... alarming to families," Dr. Muñiz Crim said. "We don’t deal with it very much as pediatric physicians, but it’s something [patients] need to know early on." Emphasize that adolescents who transition to adult provider still need to be closely monitored.

A greater risk of progression to surgery for some children with Crohn’s disease, as well as greater risk of psychosocial impact from their disease, also characterize pediatric IBD, Dr. Muñiz Crim said.

Pediatricians can help address another high risk concern in these patients: growth and pubertal delay. "Growth failure is very common, more in Crohn’s disease than in ulcerative colitis," Dr. Muñiz Crim said. Approximately 36%-39% of children with IBD will demonstrate decreased height percentiles.

Diagnosis and intervention typically lead to gradual improvements, "but their height and weight don’t make it back" to where those measures would be without the disease, Dr. Muñiz Crim said. "Adult height is often compromised. It is a big issue for our male adolescent patients in particular."

Multiple factors in IBD can impede growth, including inadequate dietary intake, malabsorption, and disease location. Children typically experience more growth failure when IBD affects the small intestine, Dr. Muñiz Crim said.

Early diagnosis and intervention improve the likelihood of minimizing adverse growth effects. Optimize nutrition through careful evaluation and calorie supplementation in concert with a nutritionist, Dr. Muñiz Crim suggested. Use routine laboratory testing to monitor disease progression.

Stay away from prolonged corticosteroid treatment, said Dr. Muñiz Crim. Although corticosteroids are appropriately prescribed to induce disease remission initially, "prolonged use can have debilitating effects" on growth, bone metabolism, and other effects. You might need to carefully counsel patients and families about the dangers of long-term steroid use, she added, because it seems counterintuitive to stop therapy when it makes patients feel better. Biologics and immunomodulators are the preferred maintenance medications to help patients achieve their growth potential, Dr. Muñiz Crim said.

The treatment of pediatric IBD patients relies on clinical judgment because most evidence is extrapolated from adult studies. Some pediatric studies with smaller numbers of participants show, for example, that therapy can induce disease remission to an extent similar to that in adults. A remaining issue, Dr. Muñiz Crim said, is that "we don’t know the lifetime toxicity of some of these medications because they haven’t been around long enough."

Continue routine immunizations in most children with IBD, Dr. Muñiz Crim said. Importantly, catch up with any vaccinations they might be missing upon diagnosis, she added, and avoid live vaccines, as always, in immunocompromised patients.

Useful serum assays include C-reactive protein, erythrocyte sedimentation rate, albumin, and hemoglobin and hematocrit. Appropriate antibody assays include P-ANCA (perinuclear antineutrophil cytoplasmic antibodies) and ASCA (anti–Saccharomyces cerevisiae antibodies). Extraintestinal manifestations of IBD that you might see include aphthous ulcerations, erythema nodosum, pyoderma gangrenosum, and perianal disease.

Dr. Muñiz Crim said that she had no relevant financial disclosures.

MIAMI – Pediatricians play an essential role in optimizing the diagnosis, care, and outcomes of children with inflammatory bowel disease, according to a pediatric gastroenterologist with expertise in this area.

"What would we like you to do as a pediatrician, perhaps, before they come to our office? There are definitely things you can start as far as the workup is concerned," Dr. Alisa J. Muñiz Crim said at a pediatric update sponsored by Miami Children’s Hospital. The pediatrician can rule out infectious diarrhea; evaluate the patient for rashes and other external manifestations; and order specific laboratory and biomarker tests, for example.

If further evaluation with endoscopy, colonoscopy, or imaging is warranted, consult a pediatric gastroenterology specialist, said Dr. Muñiz Crim, a pediatric gastroenterologist at Miami Children’s Hospital.

Suppression of inflammation, alteration of the disease course, and improvement in quality of life are among the shared goals of IBD treatment, Dr. Muñiz Crim said. Prevention of complications and disease relapse are additional aims.

Of the estimated 1 million to 2 million affected Americans, approximately 100,000 are younger than 18 years. These figures include about a 50% increase in children who have been diagnosed with IBD in the past decade. "We believe this is a true increase in the incidence of inflammatory bowel disease, not just an increase in the diagnostic capabilities," Dr. Muñiz Crim said.

Although IBD (which includes both ulcerative colitis and Crohn’s disease) affects both adults and children, there are special considerations for the pediatric population. Abdominal pain, diarrhea, rectal bleeding, anemia, and perianal disease are classic IBD symptoms. However, "presentations can be more severe in children," Dr. Muñiz Crim said.

As an example, children with ulcerative colitis experience a higher incidence (up to 80%) of a more severe form called pancolitis. Pancolitis is the single biggest risk factor for colorectal cancer in patients with ulcerative colitis.

The risk for colorectal cancer in the setting of ulcerative colitis is estimated at 2% at 10 years after diagnosis, 8% by 20 years, and 18% by 30 years. "Those numbers are very high and ... alarming to families," Dr. Muñiz Crim said. "We don’t deal with it very much as pediatric physicians, but it’s something [patients] need to know early on." Emphasize that adolescents who transition to adult provider still need to be closely monitored.

A greater risk of progression to surgery for some children with Crohn’s disease, as well as greater risk of psychosocial impact from their disease, also characterize pediatric IBD, Dr. Muñiz Crim said.

Pediatricians can help address another high risk concern in these patients: growth and pubertal delay. "Growth failure is very common, more in Crohn’s disease than in ulcerative colitis," Dr. Muñiz Crim said. Approximately 36%-39% of children with IBD will demonstrate decreased height percentiles.

Diagnosis and intervention typically lead to gradual improvements, "but their height and weight don’t make it back" to where those measures would be without the disease, Dr. Muñiz Crim said. "Adult height is often compromised. It is a big issue for our male adolescent patients in particular."

Multiple factors in IBD can impede growth, including inadequate dietary intake, malabsorption, and disease location. Children typically experience more growth failure when IBD affects the small intestine, Dr. Muñiz Crim said.

Early diagnosis and intervention improve the likelihood of minimizing adverse growth effects. Optimize nutrition through careful evaluation and calorie supplementation in concert with a nutritionist, Dr. Muñiz Crim suggested. Use routine laboratory testing to monitor disease progression.

Stay away from prolonged corticosteroid treatment, said Dr. Muñiz Crim. Although corticosteroids are appropriately prescribed to induce disease remission initially, "prolonged use can have debilitating effects" on growth, bone metabolism, and other effects. You might need to carefully counsel patients and families about the dangers of long-term steroid use, she added, because it seems counterintuitive to stop therapy when it makes patients feel better. Biologics and immunomodulators are the preferred maintenance medications to help patients achieve their growth potential, Dr. Muñiz Crim said.

The treatment of pediatric IBD patients relies on clinical judgment because most evidence is extrapolated from adult studies. Some pediatric studies with smaller numbers of participants show, for example, that therapy can induce disease remission to an extent similar to that in adults. A remaining issue, Dr. Muñiz Crim said, is that "we don’t know the lifetime toxicity of some of these medications because they haven’t been around long enough."

Continue routine immunizations in most children with IBD, Dr. Muñiz Crim said. Importantly, catch up with any vaccinations they might be missing upon diagnosis, she added, and avoid live vaccines, as always, in immunocompromised patients.

Useful serum assays include C-reactive protein, erythrocyte sedimentation rate, albumin, and hemoglobin and hematocrit. Appropriate antibody assays include P-ANCA (perinuclear antineutrophil cytoplasmic antibodies) and ASCA (anti–Saccharomyces cerevisiae antibodies). Extraintestinal manifestations of IBD that you might see include aphthous ulcerations, erythema nodosum, pyoderma gangrenosum, and perianal disease.

Dr. Muñiz Crim said that she had no relevant financial disclosures.

MIAMI – Genetic microarrays are driving advances in detection of some important pediatric neurodevelopmental disorders, including fragile X syndrome, microdeletion syndrome, and Klinefelter syndrome.

The discoveries afforded by these microarrays are coming so rapidly that adequate guidance on their use in practice is available largely from experts and specialized sources, said Dr. Nicole R. Tartaglia, a developmental and behavioral pediatrician at Children’s Hospital Colorado, Denver.

In a 2011 review, the American Academy of Neurology evaluated available neurodevelopmental testing and said that microarrays can identify a genetic etiology for 8% of children with global developmental delays or up to 11% if syndromic features are present (Neurology. 2011;77:1629-35). The American Academy of Pediatrics guidelines on Clinical Genetic Evaluation of the Child with Mental Retardation or Developmental Delays and Identification and Evaluation of Children with Autism Spectrum Disorder are several years old and precede the widespread use of microarray analysis.

Microarrays are steering researchers to develop syndrome-specific screening instruments and are likely to lead to the development of targeted therapies for these disorders, further validating the use of genetic testing for developmental delays, Dr. Tartaglia said at a pediatric update sponsored by Miami Children’s Hospital.

Microarray analysis and comprehensive genetic testing often require comprehensive patient and family counseling. Include the uncertainty and scope of genetic testing during your informed consent process, Dr. Tartaglia recommended. "The clinical implications may not be known." In addition, she said, "The lab may request testing follow-up for the parents to identify the implications of the finding if we do detect an abnormality."

Dr. Tartaglia highlighted three specific pediatric neurodevelopmental syndromes where genetic-based screening and potential targeted treatments are particularly promising:

– Fragile X syndrome. Patients with fragile X syndrome can present with an elongated face, prominent ears and among postpubertal males, bigger testicles. "Genetic testing is still important (for diagnosis) because not all will have the facial features," Dr. Tartaglia said.

A differential diagnosis is important because behavioral features overlap with autism spectrum disorder. Hand biting, sensory sensitivities, anxiety, and intellectual disability are examples. The syndrome weakens neural connections and the dendritic spines can become smaller or narrower.

A higher-than-normal repetition of the genetic CGC sequence causes fragile X syndrome, which is the most common inherited disorder of developmental disability, affecting an estimated 1 in 4,000 boys and 1 in 8,000 girls. Normally, there are fewer than 55of these CGC repeats. Emotional problems often arise in patients with 55-200 repeats, Dr. Tartaglia said.

– Microdeletion syndrome. This common genetic disorder is also known as 22q11.2 deletion syndrome and affects approximately 1 in 4,000 children, with only about 15% of cases inherited.

Early speech and motor delays, hypernasal speech caused by palate abnormalities, and intellectual disabilities are characteristic. Approximately 30%-50% of these children will be diagnosed with attention-deficit/hyperactivity disorder. At last count, there are more than 180 clinical features of this syndrome, Dr. Tartaglia said.

Counseling and follow-up are important, as these children often develop multiple medical comorbidities. In addition, they are at increased risk for schizophrenia and other psychotic disorders. "Up to 25% can develop [psychotic disorders] in late adolescence or adulthood."

– Klinefelter syndrome. This disorder affects approximately 1 in 650 males and is the most common sex-chromosome–linked form of aneuploidy. Neurodevelopmental symptoms vary widely, Dr. Tartaglia said. About three-fourths of affected children will have speech and motor delays and about 80% will require special education assistance in school. These boys are often shy and have social difficulties. About one-third are diagnosed with ADHD, mostly the inattentive subtype. "It is important for families to understand the delays." Early recognition of the syndrome can lead to timely initiation of testosterone therapy.

Genetic testing will reveal the boy is born with 47 chromosomes (instead of XY, they feature XXY sex chromosomes). "Even though it’s one of the most common genetic abnormalities, it is underdiagnosed," Dr. Tartaglia said. About 75% remain undiagnosed during their lifetime, perhaps due to the lack of early physical abnormalities that would prompt screening.

"It is important to screen because we know the difficulties later," Dr. Tartaglia said. Testicular dysgenesis during adolescence, mood dysregulation, and osteopenia or osteoporosis can emerge later, for example. Adult men with Klinefelter syndrome also can experience testosterone deficiency, microorchidism, and infertility.

Dr. Tartaglia said she receives research funding from Seaside Therapeutics.

MIAMI – Genetic microarrays are driving advances in detection of some important pediatric neurodevelopmental disorders, including fragile X syndrome, microdeletion syndrome, and Klinefelter syndrome.

The discoveries afforded by these microarrays are coming so rapidly that adequate guidance on their use in practice is available largely from experts and specialized sources, said Dr. Nicole R. Tartaglia, a developmental and behavioral pediatrician at Children’s Hospital Colorado, Denver.

In a 2011 review, the American Academy of Neurology evaluated available neurodevelopmental testing and said that microarrays can identify a genetic etiology for 8% of children with global developmental delays or up to 11% if syndromic features are present (Neurology. 2011;77:1629-35). The American Academy of Pediatrics guidelines on Clinical Genetic Evaluation of the Child with Mental Retardation or Developmental Delays and Identification and Evaluation of Children with Autism Spectrum Disorder are several years old and precede the widespread use of microarray analysis.

Microarrays are steering researchers to develop syndrome-specific screening instruments and are likely to lead to the development of targeted therapies for these disorders, further validating the use of genetic testing for developmental delays, Dr. Tartaglia said at a pediatric update sponsored by Miami Children’s Hospital.

Microarray analysis and comprehensive genetic testing often require comprehensive patient and family counseling. Include the uncertainty and scope of genetic testing during your informed consent process, Dr. Tartaglia recommended. "The clinical implications may not be known." In addition, she said, "The lab may request testing follow-up for the parents to identify the implications of the finding if we do detect an abnormality."

Dr. Tartaglia highlighted three specific pediatric neurodevelopmental syndromes where genetic-based screening and potential targeted treatments are particularly promising:

– Fragile X syndrome. Patients with fragile X syndrome can present with an elongated face, prominent ears and among postpubertal males, bigger testicles. "Genetic testing is still important (for diagnosis) because not all will have the facial features," Dr. Tartaglia said.

A differential diagnosis is important because behavioral features overlap with autism spectrum disorder. Hand biting, sensory sensitivities, anxiety, and intellectual disability are examples. The syndrome weakens neural connections and the dendritic spines can become smaller or narrower.

A higher-than-normal repetition of the genetic CGC sequence causes fragile X syndrome, which is the most common inherited disorder of developmental disability, affecting an estimated 1 in 4,000 boys and 1 in 8,000 girls. Normally, there are fewer than 55of these CGC repeats. Emotional problems often arise in patients with 55-200 repeats, Dr. Tartaglia said.

– Microdeletion syndrome. This common genetic disorder is also known as 22q11.2 deletion syndrome and affects approximately 1 in 4,000 children, with only about 15% of cases inherited.

Early speech and motor delays, hypernasal speech caused by palate abnormalities, and intellectual disabilities are characteristic. Approximately 30%-50% of these children will be diagnosed with attention-deficit/hyperactivity disorder. At last count, there are more than 180 clinical features of this syndrome, Dr. Tartaglia said.

Counseling and follow-up are important, as these children often develop multiple medical comorbidities. In addition, they are at increased risk for schizophrenia and other psychotic disorders. "Up to 25% can develop [psychotic disorders] in late adolescence or adulthood."

– Klinefelter syndrome. This disorder affects approximately 1 in 650 males and is the most common sex-chromosome–linked form of aneuploidy. Neurodevelopmental symptoms vary widely, Dr. Tartaglia said. About three-fourths of affected children will have speech and motor delays and about 80% will require special education assistance in school. These boys are often shy and have social difficulties. About one-third are diagnosed with ADHD, mostly the inattentive subtype. "It is important for families to understand the delays." Early recognition of the syndrome can lead to timely initiation of testosterone therapy.

Genetic testing will reveal the boy is born with 47 chromosomes (instead of XY, they feature XXY sex chromosomes). "Even though it’s one of the most common genetic abnormalities, it is underdiagnosed," Dr. Tartaglia said. About 75% remain undiagnosed during their lifetime, perhaps due to the lack of early physical abnormalities that would prompt screening.

"It is important to screen because we know the difficulties later," Dr. Tartaglia said. Testicular dysgenesis during adolescence, mood dysregulation, and osteopenia or osteoporosis can emerge later, for example. Adult men with Klinefelter syndrome also can experience testosterone deficiency, microorchidism, and infertility.

Dr. Tartaglia said she receives research funding from Seaside Therapeutics.

MIAMI – Genetic microarrays are driving advances in detection of some important pediatric neurodevelopmental disorders, including fragile X syndrome, microdeletion syndrome, and Klinefelter syndrome.

The discoveries afforded by these microarrays are coming so rapidly that adequate guidance on their use in practice is available largely from experts and specialized sources, said Dr. Nicole R. Tartaglia, a developmental and behavioral pediatrician at Children’s Hospital Colorado, Denver.

In a 2011 review, the American Academy of Neurology evaluated available neurodevelopmental testing and said that microarrays can identify a genetic etiology for 8% of children with global developmental delays or up to 11% if syndromic features are present (Neurology. 2011;77:1629-35). The American Academy of Pediatrics guidelines on Clinical Genetic Evaluation of the Child with Mental Retardation or Developmental Delays and Identification and Evaluation of Children with Autism Spectrum Disorder are several years old and precede the widespread use of microarray analysis.

Microarrays are steering researchers to develop syndrome-specific screening instruments and are likely to lead to the development of targeted therapies for these disorders, further validating the use of genetic testing for developmental delays, Dr. Tartaglia said at a pediatric update sponsored by Miami Children’s Hospital.

Microarray analysis and comprehensive genetic testing often require comprehensive patient and family counseling. Include the uncertainty and scope of genetic testing during your informed consent process, Dr. Tartaglia recommended. "The clinical implications may not be known." In addition, she said, "The lab may request testing follow-up for the parents to identify the implications of the finding if we do detect an abnormality."

Dr. Tartaglia highlighted three specific pediatric neurodevelopmental syndromes where genetic-based screening and potential targeted treatments are particularly promising:

– Fragile X syndrome. Patients with fragile X syndrome can present with an elongated face, prominent ears and among postpubertal males, bigger testicles. "Genetic testing is still important (for diagnosis) because not all will have the facial features," Dr. Tartaglia said.

A differential diagnosis is important because behavioral features overlap with autism spectrum disorder. Hand biting, sensory sensitivities, anxiety, and intellectual disability are examples. The syndrome weakens neural connections and the dendritic spines can become smaller or narrower.

A higher-than-normal repetition of the genetic CGC sequence causes fragile X syndrome, which is the most common inherited disorder of developmental disability, affecting an estimated 1 in 4,000 boys and 1 in 8,000 girls. Normally, there are fewer than 55of these CGC repeats. Emotional problems often arise in patients with 55-200 repeats, Dr. Tartaglia said.

– Microdeletion syndrome. This common genetic disorder is also known as 22q11.2 deletion syndrome and affects approximately 1 in 4,000 children, with only about 15% of cases inherited.

Early speech and motor delays, hypernasal speech caused by palate abnormalities, and intellectual disabilities are characteristic. Approximately 30%-50% of these children will be diagnosed with attention-deficit/hyperactivity disorder. At last count, there are more than 180 clinical features of this syndrome, Dr. Tartaglia said.

Counseling and follow-up are important, as these children often develop multiple medical comorbidities. In addition, they are at increased risk for schizophrenia and other psychotic disorders. "Up to 25% can develop [psychotic disorders] in late adolescence or adulthood."

– Klinefelter syndrome. This disorder affects approximately 1 in 650 males and is the most common sex-chromosome–linked form of aneuploidy. Neurodevelopmental symptoms vary widely, Dr. Tartaglia said. About three-fourths of affected children will have speech and motor delays and about 80% will require special education assistance in school. These boys are often shy and have social difficulties. About one-third are diagnosed with ADHD, mostly the inattentive subtype. "It is important for families to understand the delays." Early recognition of the syndrome can lead to timely initiation of testosterone therapy.

Genetic testing will reveal the boy is born with 47 chromosomes (instead of XY, they feature XXY sex chromosomes). "Even though it’s one of the most common genetic abnormalities, it is underdiagnosed," Dr. Tartaglia said. About 75% remain undiagnosed during their lifetime, perhaps due to the lack of early physical abnormalities that would prompt screening.

"It is important to screen because we know the difficulties later," Dr. Tartaglia said. Testicular dysgenesis during adolescence, mood dysregulation, and osteopenia or osteoporosis can emerge later, for example. Adult men with Klinefelter syndrome also can experience testosterone deficiency, microorchidism, and infertility.

Dr. Tartaglia said she receives research funding from Seaside Therapeutics.

MIAMI – Physicians can optimize quality of life and health for children and adolescents with cystic fibrosis by helping them overcome some considerable nutritional challenges, according to a pediatric gastroenterologist.

Following a diagnosis of cystic fibrosis (which can come as early as the newborn period, with a failure to thrive), "nutritional issues are very common. They are the cornerstone of the deficiencies," Dr. William I. Muinos said.

Parents and patients will need your help to comply with a high-fat diet, for example, or to dose the right amount of pancreatic enzyme therapy as the infant grows through childhood and beyond.

Supporting an appropriate diet is the simplest and most obvious intervention, Dr. Muinos said. However, compliance with a diet in which 35%-40% of total calories come from dietary fat can be challenging. "It sounds easy: Eat more fat! But try to get a picky toddler to comply. He may not want to eat in the hypermetabolic state."

Even with dietary supplements and calorie-dense foods, "it is very difficult, especially in growing children, to consume this amount of dietary fat," Dr. Muinos said at a pediatric update sponsored by Miami Children’s Hospital.

A hypermetabolic state adds to the difficulty. "Think of a long-distance runner trying to eat a Whopper and french fries and drink a milk shake while he’s running a 5-minute mile for 26 miles, and trying to do the work to absorb this food while he’s running. It’s very difficult to do that," he said.

Ideally, nutritional deficiencies should be addressed before they contribute to significant morbidity. Cystic fibrosis adversely affects multiple systems, including many gastrointestinal organs. The high-fat dietary requirements stem in part from deficiencies in pancreatic enzymes that break down fat in the intestine, combined with malabsorption along many portions of the GI tract.

"Manifestations in the gastrointestinal tract are what I deal with on a day-to-day basis," said Dr. Muinos, a pediatric gastroenterologist at Miami Children’s Hospital.

Pancreatic enzyme therapy, therefore, is a cornerstone of cystic fibrosis treatment. Primary care physicians can help families because it can be very difficult to calculate the amount a growing infant, child, or teenager should receive. In general, patients will need 500-4,000 lipase units per gram of fat ingested per day, Dr. Muinos said.

"Children and parents are asked to figure out how many enzymes to give, based on how much fat they are ingesting." Both weight-based and age-based dosing regimens are challenging, he added.

Another issue is that most parents do not know all the fat content in the food," Dr. Muinos said. "How many of you know how much fat was in the doughnut you ate this morning?"

Enzyme therapy also presents some practical hurdles that vary by age. "Infants might spit them up. Toddlers are toddlers and might refuse to take them. Older kids don’t want to be different; they don’t want to take the enzymes in front of their friends."

Watch for adverse events. Colonic strictures, for example, have been reported with high-dose enzyme therapy. Refer the patient to a gastroenterology specialist if adverse effects or symptoms of nutritional malabsorption persist, Dr. Muinos said.

Because of the fat metabolism and absorption challenges, clinical deficiencies in vitamins A, D, E, and K are common. "The high incidence of fat-soluble vitamin deficiencies contribute to the significant morbidity and mortality for our patients," Dr. Muinos said. For example, vitamin A deficiencies can lead to blindness; vitamin D deficiency can cause immune system dysfunction; vitamin E deficiency can cause truncal ataxia; and vitamin K deficiency can significantly affect coagulation factors.

This means the child will be taking more pills, Dr. Muinos said. In addition to the enzyme pills, they have to take fat-soluble vitamins on a daily basis.

"If things fail, we always have tubes in gastroenterology." Tube feeding can address high energy requirements, poor absorption, and/or poor intake. Tube feeding is generally indicated for children with cystic fibrosis whose height is below the 85th percentile and who demonstrates no weight gain for 2-3 months (if aged younger than 5 years) or no weight gain for 6 months or weight loss for 2-3 months (if aged 5 years or older).

"The challenges in treating these patients are incredible," Dr. Muinos said. He added that the only way to truly improve quality of life and outcomes for these patients is to find a cure.

Dr. Muinos reported that he had no relevant financial disclosures.

MIAMI – Physicians can optimize quality of life and health for children and adolescents with cystic fibrosis by helping them overcome some considerable nutritional challenges, according to a pediatric gastroenterologist.

Following a diagnosis of cystic fibrosis (which can come as early as the newborn period, with a failure to thrive), "nutritional issues are very common. They are the cornerstone of the deficiencies," Dr. William I. Muinos said.

Parents and patients will need your help to comply with a high-fat diet, for example, or to dose the right amount of pancreatic enzyme therapy as the infant grows through childhood and beyond.

Supporting an appropriate diet is the simplest and most obvious intervention, Dr. Muinos said. However, compliance with a diet in which 35%-40% of total calories come from dietary fat can be challenging. "It sounds easy: Eat more fat! But try to get a picky toddler to comply. He may not want to eat in the hypermetabolic state."

Even with dietary supplements and calorie-dense foods, "it is very difficult, especially in growing children, to consume this amount of dietary fat," Dr. Muinos said at a pediatric update sponsored by Miami Children’s Hospital.

A hypermetabolic state adds to the difficulty. "Think of a long-distance runner trying to eat a Whopper and french fries and drink a milk shake while he’s running a 5-minute mile for 26 miles, and trying to do the work to absorb this food while he’s running. It’s very difficult to do that," he said.

Ideally, nutritional deficiencies should be addressed before they contribute to significant morbidity. Cystic fibrosis adversely affects multiple systems, including many gastrointestinal organs. The high-fat dietary requirements stem in part from deficiencies in pancreatic enzymes that break down fat in the intestine, combined with malabsorption along many portions of the GI tract.

"Manifestations in the gastrointestinal tract are what I deal with on a day-to-day basis," said Dr. Muinos, a pediatric gastroenterologist at Miami Children’s Hospital.

Pancreatic enzyme therapy, therefore, is a cornerstone of cystic fibrosis treatment. Primary care physicians can help families because it can be very difficult to calculate the amount a growing infant, child, or teenager should receive. In general, patients will need 500-4,000 lipase units per gram of fat ingested per day, Dr. Muinos said.

"Children and parents are asked to figure out how many enzymes to give, based on how much fat they are ingesting." Both weight-based and age-based dosing regimens are challenging, he added.

Another issue is that most parents do not know all the fat content in the food," Dr. Muinos said. "How many of you know how much fat was in the doughnut you ate this morning?"

Enzyme therapy also presents some practical hurdles that vary by age. "Infants might spit them up. Toddlers are toddlers and might refuse to take them. Older kids don’t want to be different; they don’t want to take the enzymes in front of their friends."

Watch for adverse events. Colonic strictures, for example, have been reported with high-dose enzyme therapy. Refer the patient to a gastroenterology specialist if adverse effects or symptoms of nutritional malabsorption persist, Dr. Muinos said.

Because of the fat metabolism and absorption challenges, clinical deficiencies in vitamins A, D, E, and K are common. "The high incidence of fat-soluble vitamin deficiencies contribute to the significant morbidity and mortality for our patients," Dr. Muinos said. For example, vitamin A deficiencies can lead to blindness; vitamin D deficiency can cause immune system dysfunction; vitamin E deficiency can cause truncal ataxia; and vitamin K deficiency can significantly affect coagulation factors.

This means the child will be taking more pills, Dr. Muinos said. In addition to the enzyme pills, they have to take fat-soluble vitamins on a daily basis.

"If things fail, we always have tubes in gastroenterology." Tube feeding can address high energy requirements, poor absorption, and/or poor intake. Tube feeding is generally indicated for children with cystic fibrosis whose height is below the 85th percentile and who demonstrates no weight gain for 2-3 months (if aged younger than 5 years) or no weight gain for 6 months or weight loss for 2-3 months (if aged 5 years or older).

"The challenges in treating these patients are incredible," Dr. Muinos said. He added that the only way to truly improve quality of life and outcomes for these patients is to find a cure.

Dr. Muinos reported that he had no relevant financial disclosures.

MIAMI – Physicians can optimize quality of life and health for children and adolescents with cystic fibrosis by helping them overcome some considerable nutritional challenges, according to a pediatric gastroenterologist.

Following a diagnosis of cystic fibrosis (which can come as early as the newborn period, with a failure to thrive), "nutritional issues are very common. They are the cornerstone of the deficiencies," Dr. William I. Muinos said.

Parents and patients will need your help to comply with a high-fat diet, for example, or to dose the right amount of pancreatic enzyme therapy as the infant grows through childhood and beyond.

Supporting an appropriate diet is the simplest and most obvious intervention, Dr. Muinos said. However, compliance with a diet in which 35%-40% of total calories come from dietary fat can be challenging. "It sounds easy: Eat more fat! But try to get a picky toddler to comply. He may not want to eat in the hypermetabolic state."

Even with dietary supplements and calorie-dense foods, "it is very difficult, especially in growing children, to consume this amount of dietary fat," Dr. Muinos said at a pediatric update sponsored by Miami Children’s Hospital.

A hypermetabolic state adds to the difficulty. "Think of a long-distance runner trying to eat a Whopper and french fries and drink a milk shake while he’s running a 5-minute mile for 26 miles, and trying to do the work to absorb this food while he’s running. It’s very difficult to do that," he said.

Ideally, nutritional deficiencies should be addressed before they contribute to significant morbidity. Cystic fibrosis adversely affects multiple systems, including many gastrointestinal organs. The high-fat dietary requirements stem in part from deficiencies in pancreatic enzymes that break down fat in the intestine, combined with malabsorption along many portions of the GI tract.

"Manifestations in the gastrointestinal tract are what I deal with on a day-to-day basis," said Dr. Muinos, a pediatric gastroenterologist at Miami Children’s Hospital.

Pancreatic enzyme therapy, therefore, is a cornerstone of cystic fibrosis treatment. Primary care physicians can help families because it can be very difficult to calculate the amount a growing infant, child, or teenager should receive. In general, patients will need 500-4,000 lipase units per gram of fat ingested per day, Dr. Muinos said.

"Children and parents are asked to figure out how many enzymes to give, based on how much fat they are ingesting." Both weight-based and age-based dosing regimens are challenging, he added.

Another issue is that most parents do not know all the fat content in the food," Dr. Muinos said. "How many of you know how much fat was in the doughnut you ate this morning?"

Enzyme therapy also presents some practical hurdles that vary by age. "Infants might spit them up. Toddlers are toddlers and might refuse to take them. Older kids don’t want to be different; they don’t want to take the enzymes in front of their friends."

Watch for adverse events. Colonic strictures, for example, have been reported with high-dose enzyme therapy. Refer the patient to a gastroenterology specialist if adverse effects or symptoms of nutritional malabsorption persist, Dr. Muinos said.

Because of the fat metabolism and absorption challenges, clinical deficiencies in vitamins A, D, E, and K are common. "The high incidence of fat-soluble vitamin deficiencies contribute to the significant morbidity and mortality for our patients," Dr. Muinos said. For example, vitamin A deficiencies can lead to blindness; vitamin D deficiency can cause immune system dysfunction; vitamin E deficiency can cause truncal ataxia; and vitamin K deficiency can significantly affect coagulation factors.

This means the child will be taking more pills, Dr. Muinos said. In addition to the enzyme pills, they have to take fat-soluble vitamins on a daily basis.

"If things fail, we always have tubes in gastroenterology." Tube feeding can address high energy requirements, poor absorption, and/or poor intake. Tube feeding is generally indicated for children with cystic fibrosis whose height is below the 85th percentile and who demonstrates no weight gain for 2-3 months (if aged younger than 5 years) or no weight gain for 6 months or weight loss for 2-3 months (if aged 5 years or older).

"The challenges in treating these patients are incredible," Dr. Muinos said. He added that the only way to truly improve quality of life and outcomes for these patients is to find a cure.

Dr. Muinos reported that he had no relevant financial disclosures.

MIAMI – Make sure patient and family expectations are realistic when you assess a child with a drooling disorder, Dr. Yamilet Tirado said.

"Success almost never means complete resolution of drooling," Dr. Tirado said at a pediatric update sponsored by Miami Children’s Hospital.

The use of fewer bibs, a decrease in soiled clothing, and improved socialization are examples of personal objectives that can dictate the success of conservative, medical, and/or surgical interventions to address sialorrhea, or excessive drooling. "In that context, about two-thirds of patients have some improvement," Dr. Tirado said.

Even without complete resolution of symptoms, intervention is important because sialorrhea is associated with significant quality of life impairment. "Patients and caregivers are equally affected by this problem," she said.

Patients can experience physical discomfort, skin infection, dehydration, and recurrent pneumonia that lead to stigmatization, social rejection, and isolation. Frequent patient hospitalizations, clothing changes, and laundry are among the caregiver burdens. Parents also experience anxiety, worry, and social isolation, added Dr. Tirado, a pediatric otolaryngologist at Miami Children’s Hospital.

Sialorrhea is a pathologic loss of saliva from the oral cavity. Interestingly, the majority of cases stem from an inability to swallow saliva, not from saliva overproduction, Dr. Tirado said. The etiology is often neurologic. Cerebral palsy, stroke, pseudobulbar palsy, juvenile Parkinson’s disease, and mental retardation can cause sialorrhea. Other culprits include peripheral neurologic disorders such as facial nerve palsy and glossopharyngeal nerve palsy. "Almost all affected patients have poor oral motor control and inefficient swallowing. They can also have poor head control, mouth breathing, and poor lip and tongue control," Dr. Tirado said.

A multidisciplinary approach can optimize outcomes. "Assessment and management of chronic drooling in children is best coordinated by a specialist medical team," Dr. Tirado said. The "drooling team" can consist of a pediatrician, an occupational therapist, a physical therapist, a dentist, an otolaryngologist, a pulmonologist, a neurologist, and a social worker.

The Drooling Rating Scale (Dysphagia 1988;3:73-8) is widely used to determine the frequency and severity of drooling, Dr. Tirado said. Parent questionnaires and visual analog rating scales are additional clinical assessment tools.

Consider both expectations and clinical presentation when devising your management strategy. "There are a considerable number of options for treatment depending on the age of the child and severity of the problem. The overall goal is to optimize quality of life without compromising oral or general health," she said.

Conservative approaches include wearing a bib, specific oral-motor exercises, and prescribed intraoral devices. Medical treatment usually precedes surgical intervention, but can be used as an adjunctive approach after surgery as well, Dr. Tirado said. She suggested that behavioral therapy be employed "whenever possible to reinforce techniques to improve swallowing."

Glycopyrrolate, scopolamine patches, benztropine, and ipratropium bromide are some of the medication options, "but there can be side effects," she said. Dry mouth, thick secretions, and urinary retention are examples.

Botulinum toxin type A "is now the preferred treatment when the medical treatment fails." The toxin is injected directly into the salivary gland under ultrasound guidance, usually under general anesthesia. The toxin blocks the release of acetylcholine, and the response lasts 3-6 months in the majority of patients, Dr. Tirado said. The maximum benefit occurs 4-6 weeks after injection.

About 60% of patients experience an excellent response; 20%, a good response; and the remaining 20%, a poor response – including 10% who do not respond even to high doses.

Botulinum toxin A therapy is safe and effective, Dr. Tirado said, and does not affect the adrenergic portion of salivary gland innervation. Adverse effects can include dysphagia, weak mastication, and gland infection.

Surgery is an option after medical therapy fails if a child is at least 4 years old, Dr. Tirado said. A patient with severe aspiration or profuse drooling with a low awareness level also might be a candidate for surgery. In contrast, surgery is contraindicated if the patient has neurologic disease that is not stabilized or has other health priorities.

Multiple surgical options include submandibular duct rerouting, four-duct ligation, bilateral submandibular gland excision, and tympanic neurotomy. In a meta-analysis of surgical management studies, bilateral submandibular duct excision with parotid duct rerouting was associated with the highest subjective rating of improvement – 88% (Arch. Otolaryngol. Head. Neck Surg. 2009;135:924-31). The researchers reported four-duct ligation had the lowest such rating – 64%.

Dr. Tirado said she had no relevant financial disclosures.

MIAMI – Make sure patient and family expectations are realistic when you assess a child with a drooling disorder, Dr. Yamilet Tirado said.

"Success almost never means complete resolution of drooling," Dr. Tirado said at a pediatric update sponsored by Miami Children’s Hospital.

The use of fewer bibs, a decrease in soiled clothing, and improved socialization are examples of personal objectives that can dictate the success of conservative, medical, and/or surgical interventions to address sialorrhea, or excessive drooling. "In that context, about two-thirds of patients have some improvement," Dr. Tirado said.

Even without complete resolution of symptoms, intervention is important because sialorrhea is associated with significant quality of life impairment. "Patients and caregivers are equally affected by this problem," she said.

Patients can experience physical discomfort, skin infection, dehydration, and recurrent pneumonia that lead to stigmatization, social rejection, and isolation. Frequent patient hospitalizations, clothing changes, and laundry are among the caregiver burdens. Parents also experience anxiety, worry, and social isolation, added Dr. Tirado, a pediatric otolaryngologist at Miami Children’s Hospital.

Sialorrhea is a pathologic loss of saliva from the oral cavity. Interestingly, the majority of cases stem from an inability to swallow saliva, not from saliva overproduction, Dr. Tirado said. The etiology is often neurologic. Cerebral palsy, stroke, pseudobulbar palsy, juvenile Parkinson’s disease, and mental retardation can cause sialorrhea. Other culprits include peripheral neurologic disorders such as facial nerve palsy and glossopharyngeal nerve palsy. "Almost all affected patients have poor oral motor control and inefficient swallowing. They can also have poor head control, mouth breathing, and poor lip and tongue control," Dr. Tirado said.

A multidisciplinary approach can optimize outcomes. "Assessment and management of chronic drooling in children is best coordinated by a specialist medical team," Dr. Tirado said. The "drooling team" can consist of a pediatrician, an occupational therapist, a physical therapist, a dentist, an otolaryngologist, a pulmonologist, a neurologist, and a social worker.

The Drooling Rating Scale (Dysphagia 1988;3:73-8) is widely used to determine the frequency and severity of drooling, Dr. Tirado said. Parent questionnaires and visual analog rating scales are additional clinical assessment tools.

Consider both expectations and clinical presentation when devising your management strategy. "There are a considerable number of options for treatment depending on the age of the child and severity of the problem. The overall goal is to optimize quality of life without compromising oral or general health," she said.

Conservative approaches include wearing a bib, specific oral-motor exercises, and prescribed intraoral devices. Medical treatment usually precedes surgical intervention, but can be used as an adjunctive approach after surgery as well, Dr. Tirado said. She suggested that behavioral therapy be employed "whenever possible to reinforce techniques to improve swallowing."

Glycopyrrolate, scopolamine patches, benztropine, and ipratropium bromide are some of the medication options, "but there can be side effects," she said. Dry mouth, thick secretions, and urinary retention are examples.

Botulinum toxin type A "is now the preferred treatment when the medical treatment fails." The toxin is injected directly into the salivary gland under ultrasound guidance, usually under general anesthesia. The toxin blocks the release of acetylcholine, and the response lasts 3-6 months in the majority of patients, Dr. Tirado said. The maximum benefit occurs 4-6 weeks after injection.

About 60% of patients experience an excellent response; 20%, a good response; and the remaining 20%, a poor response – including 10% who do not respond even to high doses.

Botulinum toxin A therapy is safe and effective, Dr. Tirado said, and does not affect the adrenergic portion of salivary gland innervation. Adverse effects can include dysphagia, weak mastication, and gland infection.

Surgery is an option after medical therapy fails if a child is at least 4 years old, Dr. Tirado said. A patient with severe aspiration or profuse drooling with a low awareness level also might be a candidate for surgery. In contrast, surgery is contraindicated if the patient has neurologic disease that is not stabilized or has other health priorities.

Multiple surgical options include submandibular duct rerouting, four-duct ligation, bilateral submandibular gland excision, and tympanic neurotomy. In a meta-analysis of surgical management studies, bilateral submandibular duct excision with parotid duct rerouting was associated with the highest subjective rating of improvement – 88% (Arch. Otolaryngol. Head. Neck Surg. 2009;135:924-31). The researchers reported four-duct ligation had the lowest such rating – 64%.

Dr. Tirado said she had no relevant financial disclosures.

MIAMI – Make sure patient and family expectations are realistic when you assess a child with a drooling disorder, Dr. Yamilet Tirado said.

"Success almost never means complete resolution of drooling," Dr. Tirado said at a pediatric update sponsored by Miami Children’s Hospital.

The use of fewer bibs, a decrease in soiled clothing, and improved socialization are examples of personal objectives that can dictate the success of conservative, medical, and/or surgical interventions to address sialorrhea, or excessive drooling. "In that context, about two-thirds of patients have some improvement," Dr. Tirado said.

Even without complete resolution of symptoms, intervention is important because sialorrhea is associated with significant quality of life impairment. "Patients and caregivers are equally affected by this problem," she said.

Patients can experience physical discomfort, skin infection, dehydration, and recurrent pneumonia that lead to stigmatization, social rejection, and isolation. Frequent patient hospitalizations, clothing changes, and laundry are among the caregiver burdens. Parents also experience anxiety, worry, and social isolation, added Dr. Tirado, a pediatric otolaryngologist at Miami Children’s Hospital.

Sialorrhea is a pathologic loss of saliva from the oral cavity. Interestingly, the majority of cases stem from an inability to swallow saliva, not from saliva overproduction, Dr. Tirado said. The etiology is often neurologic. Cerebral palsy, stroke, pseudobulbar palsy, juvenile Parkinson’s disease, and mental retardation can cause sialorrhea. Other culprits include peripheral neurologic disorders such as facial nerve palsy and glossopharyngeal nerve palsy. "Almost all affected patients have poor oral motor control and inefficient swallowing. They can also have poor head control, mouth breathing, and poor lip and tongue control," Dr. Tirado said.

A multidisciplinary approach can optimize outcomes. "Assessment and management of chronic drooling in children is best coordinated by a specialist medical team," Dr. Tirado said. The "drooling team" can consist of a pediatrician, an occupational therapist, a physical therapist, a dentist, an otolaryngologist, a pulmonologist, a neurologist, and a social worker.

The Drooling Rating Scale (Dysphagia 1988;3:73-8) is widely used to determine the frequency and severity of drooling, Dr. Tirado said. Parent questionnaires and visual analog rating scales are additional clinical assessment tools.

Consider both expectations and clinical presentation when devising your management strategy. "There are a considerable number of options for treatment depending on the age of the child and severity of the problem. The overall goal is to optimize quality of life without compromising oral or general health," she said.

Conservative approaches include wearing a bib, specific oral-motor exercises, and prescribed intraoral devices. Medical treatment usually precedes surgical intervention, but can be used as an adjunctive approach after surgery as well, Dr. Tirado said. She suggested that behavioral therapy be employed "whenever possible to reinforce techniques to improve swallowing."

Glycopyrrolate, scopolamine patches, benztropine, and ipratropium bromide are some of the medication options, "but there can be side effects," she said. Dry mouth, thick secretions, and urinary retention are examples.

Botulinum toxin type A "is now the preferred treatment when the medical treatment fails." The toxin is injected directly into the salivary gland under ultrasound guidance, usually under general anesthesia. The toxin blocks the release of acetylcholine, and the response lasts 3-6 months in the majority of patients, Dr. Tirado said. The maximum benefit occurs 4-6 weeks after injection.

About 60% of patients experience an excellent response; 20%, a good response; and the remaining 20%, a poor response – including 10% who do not respond even to high doses.

Botulinum toxin A therapy is safe and effective, Dr. Tirado said, and does not affect the adrenergic portion of salivary gland innervation. Adverse effects can include dysphagia, weak mastication, and gland infection.

Surgery is an option after medical therapy fails if a child is at least 4 years old, Dr. Tirado said. A patient with severe aspiration or profuse drooling with a low awareness level also might be a candidate for surgery. In contrast, surgery is contraindicated if the patient has neurologic disease that is not stabilized or has other health priorities.

Multiple surgical options include submandibular duct rerouting, four-duct ligation, bilateral submandibular gland excision, and tympanic neurotomy. In a meta-analysis of surgical management studies, bilateral submandibular duct excision with parotid duct rerouting was associated with the highest subjective rating of improvement – 88% (Arch. Otolaryngol. Head. Neck Surg. 2009;135:924-31). The researchers reported four-duct ligation had the lowest such rating – 64%.

Dr. Tirado said she had no relevant financial disclosures.

MIAMI BEACH – With 92 recommendations and 340 references that span 51 pages, clinical practice guidelines for management of infants and children with community-acquired pneumonia might at first seem overwhelming, but they are worth consideration, according to Dr. Mary Anne Jackson.

"Don’t be daunted about getting into it. It actually divides out pneumonia in ways that will be relevant to your particular practice," she said.

The guidelines address management of otherwise healthy children with community-acquired pneumonia (CAP) – not hospital-acquired – in both the outpatient and inpatient settings (Clin. Infect. Disease 2011;53:e25-76). "This is the first time the Pediatric Infectious Diseases Society has been asked to make pediatric-specific recommendations. This is a big step," Dr. Jackson said.

The clinical practice guidelines, developed in conjunction with the Infectious Disease Society of America, address "looking at a child with signs and symptoms and being able to predict pneumonia," said Dr. Jackson, chief of the pediatric infectious diseases section at Children’s Mercy Hospitals and Clinics in Kansas City.

Nasal flaring in an infant less than 12 months, for example, had the best positive predictive value. Oxygen saturation less than 94% at sea level "is also clearly predictive," she said. Less surprising predictors include tachypnea and retractions. In contrast, absence of tachypnea or other respiratory signs had the best negative predictive value.

Also addressed within the scope of the evidence-based guidelines are site of care, use of diagnostic testing, appropriate initial anti-infective treatment, adjunctive therapy, management of a child unresponsive to therapy, appropriate discharge criteria, and prevention of community-acquired pneumonia.

"Let’s look at a little of the guidelines and see how these recommendations might influence your practice," Dr. Jackson said at a pediatric update sponsored by Miami Children’s Hospital.

Blood cultures are not necessary for the nontoxic, immunized child who is going to be managed as outpatient, for example. "The guidelines will tell you that less than 1% of the individuals who meet these guidelines will have a positive blood culture if they have been effectively immunized," said Dr. Jackson, who was not one of the 13 authors of the guidelines.

Blood cultures, however, are indicated for the infant or child who requires hospital admission or who has evidence of empyema. An estimated 14%- 27% of children with a complicated pneumonia will have a positive blood culture, she said. "This will help you decide about anti-infective therapy [and] help you provide definitive therapy."

In terms of diagnostic testing, radiography should be obtained for the child sick enough to be admitted to the hospital, for the child who is hypoxic with significant respiratory distress, for the child with an infection that is prolonged or unresponsive, and "certainly if you suspect an empyema."

Think empyema when you suspect CAP, particularly in the setting of prolonged or recurrent fever, Dr. Jackson said. Patients with empyema often report chest and abdominal pain as well. Check their medication history too, she advised. "They may have received azithromycin, which I will tell you is not a good choice for pneumococcal infection or respiratory infection in general (unless you suspect Mycoplasma pneumoniae) in the pediatric population."

The guideline authors outline scenarios where hospitalization is recommended. For example, admission is indicated for suspected bacterial pneumonia in children aged 3-6 months of age or any infant or any child who is not fully immunized, who has oxygen saturation below 90%, or who cannot comply with oral therapy.

Admission is also supported for suspected methicillin-resistant Staphylococcus aureus (MRSA) infection. "I have a very high index of suspicion in the sick infant (particularly those under 1 year of age) with bacterial pneumonia [with] MRSA as a pathogen," Dr. Jackson said.

The guidelines also outline antibiotic treatment. Treatment is generally 10 days for CAP or longer with empyema.

The previously healthy and immunized preschool age child treated as an outpatient should receive amoxicillin, the guideline authors stated. They prefer clindamycin for penicillin-allergic patients but offer alternative options. "You should check your antibiogram in your own institution so you know what your clindamycin resistance rates are for pneumococcus," Dr. Jackson said.

In the inpatient setting, an immunized infant or child with CAP can be treated with ampicillin or penicillin G. "Once again, you must know what your local rates of penicillin resistance are in your institution," she said.

Empiric therapy with a third generation parenteral cephalosporin such as ceftriaxone or cefotaxime is recommended for hospitalized infants and children who are not fully immunized in regions with a higher penicillin resistance rate or for children with a life-threatening infection. "I will tell you in those instances you are going to have a higher risk of staphylococcal infection, and ceftriaxone and cefotaxime lack staphylococcal coverage," Dr. Jackson said. "So you’re going to have to broaden your coverage for those patients."

The guideline authors also address prevention of CAP. "Prevention is all about relying on our classic American Academy of Pediatrics policies: who should get palivizumab (Synagis), endorsing immunization of parents and other caregivers, and making sure vaccines are given appropriately to the infant or child," Dr. Jackson said.

The American Academy of Pediatrics endorsed these CAP clinical practice guidelines (Pediatrics 2011;128:e1677).

Dr. Jackson disclosed she is a member of the American Academy of Pediatrics Committee on Infectious Diseases, secretary-treasurer of the Pediatric Infectious Diseases Society, and a member of the Infectious Diseases Society of America Education Committee. She reported that she had no relevant financial disclosures.

MIAMI BEACH – With 92 recommendations and 340 references that span 51 pages, clinical practice guidelines for management of infants and children with community-acquired pneumonia might at first seem overwhelming, but they are worth consideration, according to Dr. Mary Anne Jackson.

"Don’t be daunted about getting into it. It actually divides out pneumonia in ways that will be relevant to your particular practice," she said.

The guidelines address management of otherwise healthy children with community-acquired pneumonia (CAP) – not hospital-acquired – in both the outpatient and inpatient settings (Clin. Infect. Disease 2011;53:e25-76). "This is the first time the Pediatric Infectious Diseases Society has been asked to make pediatric-specific recommendations. This is a big step," Dr. Jackson said.

The clinical practice guidelines, developed in conjunction with the Infectious Disease Society of America, address "looking at a child with signs and symptoms and being able to predict pneumonia," said Dr. Jackson, chief of the pediatric infectious diseases section at Children’s Mercy Hospitals and Clinics in Kansas City.

Nasal flaring in an infant less than 12 months, for example, had the best positive predictive value. Oxygen saturation less than 94% at sea level "is also clearly predictive," she said. Less surprising predictors include tachypnea and retractions. In contrast, absence of tachypnea or other respiratory signs had the best negative predictive value.

Also addressed within the scope of the evidence-based guidelines are site of care, use of diagnostic testing, appropriate initial anti-infective treatment, adjunctive therapy, management of a child unresponsive to therapy, appropriate discharge criteria, and prevention of community-acquired pneumonia.

"Let’s look at a little of the guidelines and see how these recommendations might influence your practice," Dr. Jackson said at a pediatric update sponsored by Miami Children’s Hospital.

Blood cultures are not necessary for the nontoxic, immunized child who is going to be managed as outpatient, for example. "The guidelines will tell you that less than 1% of the individuals who meet these guidelines will have a positive blood culture if they have been effectively immunized," said Dr. Jackson, who was not one of the 13 authors of the guidelines.

Blood cultures, however, are indicated for the infant or child who requires hospital admission or who has evidence of empyema. An estimated 14%- 27% of children with a complicated pneumonia will have a positive blood culture, she said. "This will help you decide about anti-infective therapy [and] help you provide definitive therapy."

In terms of diagnostic testing, radiography should be obtained for the child sick enough to be admitted to the hospital, for the child who is hypoxic with significant respiratory distress, for the child with an infection that is prolonged or unresponsive, and "certainly if you suspect an empyema."

Think empyema when you suspect CAP, particularly in the setting of prolonged or recurrent fever, Dr. Jackson said. Patients with empyema often report chest and abdominal pain as well. Check their medication history too, she advised. "They may have received azithromycin, which I will tell you is not a good choice for pneumococcal infection or respiratory infection in general (unless you suspect Mycoplasma pneumoniae) in the pediatric population."

The guideline authors outline scenarios where hospitalization is recommended. For example, admission is indicated for suspected bacterial pneumonia in children aged 3-6 months of age or any infant or any child who is not fully immunized, who has oxygen saturation below 90%, or who cannot comply with oral therapy.

Admission is also supported for suspected methicillin-resistant Staphylococcus aureus (MRSA) infection. "I have a very high index of suspicion in the sick infant (particularly those under 1 year of age) with bacterial pneumonia [with] MRSA as a pathogen," Dr. Jackson said.

The guidelines also outline antibiotic treatment. Treatment is generally 10 days for CAP or longer with empyema.

The previously healthy and immunized preschool age child treated as an outpatient should receive amoxicillin, the guideline authors stated. They prefer clindamycin for penicillin-allergic patients but offer alternative options. "You should check your antibiogram in your own institution so you know what your clindamycin resistance rates are for pneumococcus," Dr. Jackson said.

In the inpatient setting, an immunized infant or child with CAP can be treated with ampicillin or penicillin G. "Once again, you must know what your local rates of penicillin resistance are in your institution," she said.

Empiric therapy with a third generation parenteral cephalosporin such as ceftriaxone or cefotaxime is recommended for hospitalized infants and children who are not fully immunized in regions with a higher penicillin resistance rate or for children with a life-threatening infection. "I will tell you in those instances you are going to have a higher risk of staphylococcal infection, and ceftriaxone and cefotaxime lack staphylococcal coverage," Dr. Jackson said. "So you’re going to have to broaden your coverage for those patients."

The guideline authors also address prevention of CAP. "Prevention is all about relying on our classic American Academy of Pediatrics policies: who should get palivizumab (Synagis), endorsing immunization of parents and other caregivers, and making sure vaccines are given appropriately to the infant or child," Dr. Jackson said.

The American Academy of Pediatrics endorsed these CAP clinical practice guidelines (Pediatrics 2011;128:e1677).

Dr. Jackson disclosed she is a member of the American Academy of Pediatrics Committee on Infectious Diseases, secretary-treasurer of the Pediatric Infectious Diseases Society, and a member of the Infectious Diseases Society of America Education Committee. She reported that she had no relevant financial disclosures.

MIAMI BEACH – With 92 recommendations and 340 references that span 51 pages, clinical practice guidelines for management of infants and children with community-acquired pneumonia might at first seem overwhelming, but they are worth consideration, according to Dr. Mary Anne Jackson.

"Don’t be daunted about getting into it. It actually divides out pneumonia in ways that will be relevant to your particular practice," she said.

The guidelines address management of otherwise healthy children with community-acquired pneumonia (CAP) – not hospital-acquired – in both the outpatient and inpatient settings (Clin. Infect. Disease 2011;53:e25-76). "This is the first time the Pediatric Infectious Diseases Society has been asked to make pediatric-specific recommendations. This is a big step," Dr. Jackson said.

The clinical practice guidelines, developed in conjunction with the Infectious Disease Society of America, address "looking at a child with signs and symptoms and being able to predict pneumonia," said Dr. Jackson, chief of the pediatric infectious diseases section at Children’s Mercy Hospitals and Clinics in Kansas City.

Nasal flaring in an infant less than 12 months, for example, had the best positive predictive value. Oxygen saturation less than 94% at sea level "is also clearly predictive," she said. Less surprising predictors include tachypnea and retractions. In contrast, absence of tachypnea or other respiratory signs had the best negative predictive value.

Also addressed within the scope of the evidence-based guidelines are site of care, use of diagnostic testing, appropriate initial anti-infective treatment, adjunctive therapy, management of a child unresponsive to therapy, appropriate discharge criteria, and prevention of community-acquired pneumonia.

"Let’s look at a little of the guidelines and see how these recommendations might influence your practice," Dr. Jackson said at a pediatric update sponsored by Miami Children’s Hospital.

Blood cultures are not necessary for the nontoxic, immunized child who is going to be managed as outpatient, for example. "The guidelines will tell you that less than 1% of the individuals who meet these guidelines will have a positive blood culture if they have been effectively immunized," said Dr. Jackson, who was not one of the 13 authors of the guidelines.

Blood cultures, however, are indicated for the infant or child who requires hospital admission or who has evidence of empyema. An estimated 14%- 27% of children with a complicated pneumonia will have a positive blood culture, she said. "This will help you decide about anti-infective therapy [and] help you provide definitive therapy."

In terms of diagnostic testing, radiography should be obtained for the child sick enough to be admitted to the hospital, for the child who is hypoxic with significant respiratory distress, for the child with an infection that is prolonged or unresponsive, and "certainly if you suspect an empyema."

Think empyema when you suspect CAP, particularly in the setting of prolonged or recurrent fever, Dr. Jackson said. Patients with empyema often report chest and abdominal pain as well. Check their medication history too, she advised. "They may have received azithromycin, which I will tell you is not a good choice for pneumococcal infection or respiratory infection in general (unless you suspect Mycoplasma pneumoniae) in the pediatric population."

The guideline authors outline scenarios where hospitalization is recommended. For example, admission is indicated for suspected bacterial pneumonia in children aged 3-6 months of age or any infant or any child who is not fully immunized, who has oxygen saturation below 90%, or who cannot comply with oral therapy.

Admission is also supported for suspected methicillin-resistant Staphylococcus aureus (MRSA) infection. "I have a very high index of suspicion in the sick infant (particularly those under 1 year of age) with bacterial pneumonia [with] MRSA as a pathogen," Dr. Jackson said.

The guidelines also outline antibiotic treatment. Treatment is generally 10 days for CAP or longer with empyema.

The previously healthy and immunized preschool age child treated as an outpatient should receive amoxicillin, the guideline authors stated. They prefer clindamycin for penicillin-allergic patients but offer alternative options. "You should check your antibiogram in your own institution so you know what your clindamycin resistance rates are for pneumococcus," Dr. Jackson said.

In the inpatient setting, an immunized infant or child with CAP can be treated with ampicillin or penicillin G. "Once again, you must know what your local rates of penicillin resistance are in your institution," she said.

Empiric therapy with a third generation parenteral cephalosporin such as ceftriaxone or cefotaxime is recommended for hospitalized infants and children who are not fully immunized in regions with a higher penicillin resistance rate or for children with a life-threatening infection. "I will tell you in those instances you are going to have a higher risk of staphylococcal infection, and ceftriaxone and cefotaxime lack staphylococcal coverage," Dr. Jackson said. "So you’re going to have to broaden your coverage for those patients."

The guideline authors also address prevention of CAP. "Prevention is all about relying on our classic American Academy of Pediatrics policies: who should get palivizumab (Synagis), endorsing immunization of parents and other caregivers, and making sure vaccines are given appropriately to the infant or child," Dr. Jackson said.

The American Academy of Pediatrics endorsed these CAP clinical practice guidelines (Pediatrics 2011;128:e1677).

Dr. Jackson disclosed she is a member of the American Academy of Pediatrics Committee on Infectious Diseases, secretary-treasurer of the Pediatric Infectious Diseases Society, and a member of the Infectious Diseases Society of America Education Committee. She reported that she had no relevant financial disclosures.

MIAMI – Some hallmark signs of dermatologic problems in children – especially erythema and hyperpigmentation – often are less obvious in children with skin of color and can require more clinical detective work to diagnose.

Dr. Patricia A. Treadwell narrowed down the most likely dermatoses a pediatrician will encounter in this patient population at a pediatric update sponsored by Miami Children’s Hospital. Atopic and contact dermatitis, phytophotodermatitis, transient neonatal pustular melanosis, and neonatal lupus erythematosus are among the noteworthy clinical challenges, she said at the meeting.

"Children with increased pigmentation in their skin may end up testing your knowledge in terms of looking at their dermatitis and being able to diagnose that. Keep in mind it may be a little bit different in terms of the clinical presentation, but it’s important to identify it and start the proper treatment," said Dr. Treadwell, a pediatric dermatologist at Indiana University Health and Riley Hospital for Children in Indianapolis.

Overcoming this "masking" of a condition by skin pigmentation can be important, Dr. Treadwell said. She cited a patient born with a port-wine stain that went undiagnosed. The infant had subtle erythema and some asymmetry related to the overgrowth of the lesion. "This was not diagnosed based on the fact that the erythema was not apparent. The patient later developed a pyogenic granuloma, which is a complication that can be seen in patients with port-wine stains as they get older."

Photos courtesy Dr. Patricia A. Treadwell

Erythema can be missed in children of color with atopic dermatitis as well. For this reason, atopic dermatitis may be underdiagnosed in this population overall, she said. Another challenge is the common practice of grading the severity of atopic dermatitis in lighter-skinned patients based on the degree of erythema. "In children with a fair amount of pigmentation in their skin, the erythema may not be noted and the severity will not be recognized."

Similarly, you might need a higher index of clinical suspicion to diagnose a child of color with contact dermatitis. Again, the erythema can be subtle. In contrast, "contact dermatitis can be very clear in a Caucasian patient. But the lesions are the same – linear, asymmetrical, and occurring on exposed areas," Dr. Treadwell said. Pruritus is common, and edema and swelling also occur. Watch for development of vesiculobullous lesions.

Phytophotodermatitis is another dermatologic condition that may require some additional detective work in children of color. Dr. Treadwell described a girl with a unique hyperpigmentation pattern on her legs and arms. She was referred following a vacation in Cancun, Mexico, with her family, and there was a concern about an autoimmune process. "They asked if she needed blood work. I said no, she was eating a mango and went out in the sun." Some of the mango juice splashed on her legs and arms.

Phytophotodermatitis occurs when furocoumarins from tropical fruit, citrus, celery, fennel, or parsnip come into contact with skin subsequently exposed to the sun.

"This condition can have a fairly bizarre pattern of presentation," Dr. Treadwell said. "Again, if there is more pigment in the skin, hyperpigmentation can present in a less common way than might be expected."

Some dermatoses are noted more commonly in children of color, she said. For example, the hyperpigmented macules or pustules that characterize transient neonatal pustular melanosis are reported in 4.4% of African American infants and 0.2% of Caucasian infants. "The percentages here may be related to the pigmentation in the skin. This does occur in Caucasian infants, but it may not be as noticeable."

The condition can be present at birth. The macules and pustules can appear anywhere on the body, but most often on the chin, neck, upper chest, and/or lower back. The good news is they fade over time and are benign, so no treatment is necessary. The differential diagnosis from such conditions as herpes simplex and erythema toxicum is important, however, said Dr. Treadwell. One tip is to check for lesions on the palms and soles, which can be diagnostic in transient neonatal pustular melanosis, but not for erythema toxicum. A biopsy can confirm your clinical suspicions.

The cutaneous manifestations of neonatal lupus erythematosus can tip you off to this condition, she said. Skin lesions can be annular, discoid, or atrophic. Some children present with "raccoon eyes." Because this condition is related to maternal antibodies passed through the placenta during gestation, lesions generally clear by 6 months to 1 year of age.

The mother may have a diagnosis of an autoimmune disease, or she may be completely asymptomatic. In one instance, a mother brought her newborn to Dr. Treadwell’s clinic. He had annular lesions on his forehead with some erythema. The lesions were more edematous around the edges. He actually got some sun exposure between his first and second visits, and developed more-discoid lesions in his sun-exposed areas. He also presented with lesions in non–sun exposed areas.

"The mother had a positive ANA [antinuclear antibody] test. I told her she should go to her doctor for urine and blood pressure monitoring," Dr. Treadwell said. "She had no symptoms and thought I didn’t know what I was talking about." Two years later, the woman returned with a newborn daughter who also had neonatal lupus with lesions on her face and some patchy alopecia on her scalp.

"The brother came back in, ... and he had telangiectasias already present at age 2 years."

Your evaluation can be more family centered. Consider testing the parent and affected children for anti-Rho, anti-La, and anti-RNP.

"I usually treat them with sun avoidance, sun protection, and possibly hydrocortisone," Dr. Treadwell said. She also recommends one electrocardiogram to rule out any cardiac consequences.

Dr. Treadwell reported that she had no relevant financial disclosures.

MIAMI – Some hallmark signs of dermatologic problems in children – especially erythema and hyperpigmentation – often are less obvious in children with skin of color and can require more clinical detective work to diagnose.

Dr. Patricia A. Treadwell narrowed down the most likely dermatoses a pediatrician will encounter in this patient population at a pediatric update sponsored by Miami Children’s Hospital. Atopic and contact dermatitis, phytophotodermatitis, transient neonatal pustular melanosis, and neonatal lupus erythematosus are among the noteworthy clinical challenges, she said at the meeting.

"Children with increased pigmentation in their skin may end up testing your knowledge in terms of looking at their dermatitis and being able to diagnose that. Keep in mind it may be a little bit different in terms of the clinical presentation, but it’s important to identify it and start the proper treatment," said Dr. Treadwell, a pediatric dermatologist at Indiana University Health and Riley Hospital for Children in Indianapolis.

Overcoming this "masking" of a condition by skin pigmentation can be important, Dr. Treadwell said. She cited a patient born with a port-wine stain that went undiagnosed. The infant had subtle erythema and some asymmetry related to the overgrowth of the lesion. "This was not diagnosed based on the fact that the erythema was not apparent. The patient later developed a pyogenic granuloma, which is a complication that can be seen in patients with port-wine stains as they get older."

Photos courtesy Dr. Patricia A. Treadwell

Erythema can be missed in children of color with atopic dermatitis as well. For this reason, atopic dermatitis may be underdiagnosed in this population overall, she said. Another challenge is the common practice of grading the severity of atopic dermatitis in lighter-skinned patients based on the degree of erythema. "In children with a fair amount of pigmentation in their skin, the erythema may not be noted and the severity will not be recognized."

Similarly, you might need a higher index of clinical suspicion to diagnose a child of color with contact dermatitis. Again, the erythema can be subtle. In contrast, "contact dermatitis can be very clear in a Caucasian patient. But the lesions are the same – linear, asymmetrical, and occurring on exposed areas," Dr. Treadwell said. Pruritus is common, and edema and swelling also occur. Watch for development of vesiculobullous lesions.

Phytophotodermatitis is another dermatologic condition that may require some additional detective work in children of color. Dr. Treadwell described a girl with a unique hyperpigmentation pattern on her legs and arms. She was referred following a vacation in Cancun, Mexico, with her family, and there was a concern about an autoimmune process. "They asked if she needed blood work. I said no, she was eating a mango and went out in the sun." Some of the mango juice splashed on her legs and arms.

Phytophotodermatitis occurs when furocoumarins from tropical fruit, citrus, celery, fennel, or parsnip come into contact with skin subsequently exposed to the sun.

"This condition can have a fairly bizarre pattern of presentation," Dr. Treadwell said. "Again, if there is more pigment in the skin, hyperpigmentation can present in a less common way than might be expected."

Some dermatoses are noted more commonly in children of color, she said. For example, the hyperpigmented macules or pustules that characterize transient neonatal pustular melanosis are reported in 4.4% of African American infants and 0.2% of Caucasian infants. "The percentages here may be related to the pigmentation in the skin. This does occur in Caucasian infants, but it may not be as noticeable."

The condition can be present at birth. The macules and pustules can appear anywhere on the body, but most often on the chin, neck, upper chest, and/or lower back. The good news is they fade over time and are benign, so no treatment is necessary. The differential diagnosis from such conditions as herpes simplex and erythema toxicum is important, however, said Dr. Treadwell. One tip is to check for lesions on the palms and soles, which can be diagnostic in transient neonatal pustular melanosis, but not for erythema toxicum. A biopsy can confirm your clinical suspicions.

The cutaneous manifestations of neonatal lupus erythematosus can tip you off to this condition, she said. Skin lesions can be annular, discoid, or atrophic. Some children present with "raccoon eyes." Because this condition is related to maternal antibodies passed through the placenta during gestation, lesions generally clear by 6 months to 1 year of age.

The mother may have a diagnosis of an autoimmune disease, or she may be completely asymptomatic. In one instance, a mother brought her newborn to Dr. Treadwell’s clinic. He had annular lesions on his forehead with some erythema. The lesions were more edematous around the edges. He actually got some sun exposure between his first and second visits, and developed more-discoid lesions in his sun-exposed areas. He also presented with lesions in non–sun exposed areas.

"The mother had a positive ANA [antinuclear antibody] test. I told her she should go to her doctor for urine and blood pressure monitoring," Dr. Treadwell said. "She had no symptoms and thought I didn’t know what I was talking about." Two years later, the woman returned with a newborn daughter who also had neonatal lupus with lesions on her face and some patchy alopecia on her scalp.

"The brother came back in, ... and he had telangiectasias already present at age 2 years."

Your evaluation can be more family centered. Consider testing the parent and affected children for anti-Rho, anti-La, and anti-RNP.

"I usually treat them with sun avoidance, sun protection, and possibly hydrocortisone," Dr. Treadwell said. She also recommends one electrocardiogram to rule out any cardiac consequences.

Dr. Treadwell reported that she had no relevant financial disclosures.

MIAMI – Some hallmark signs of dermatologic problems in children – especially erythema and hyperpigmentation – often are less obvious in children with skin of color and can require more clinical detective work to diagnose.

Dr. Patricia A. Treadwell narrowed down the most likely dermatoses a pediatrician will encounter in this patient population at a pediatric update sponsored by Miami Children’s Hospital. Atopic and contact dermatitis, phytophotodermatitis, transient neonatal pustular melanosis, and neonatal lupus erythematosus are among the noteworthy clinical challenges, she said at the meeting.

"Children with increased pigmentation in their skin may end up testing your knowledge in terms of looking at their dermatitis and being able to diagnose that. Keep in mind it may be a little bit different in terms of the clinical presentation, but it’s important to identify it and start the proper treatment," said Dr. Treadwell, a pediatric dermatologist at Indiana University Health and Riley Hospital for Children in Indianapolis.

Overcoming this "masking" of a condition by skin pigmentation can be important, Dr. Treadwell said. She cited a patient born with a port-wine stain that went undiagnosed. The infant had subtle erythema and some asymmetry related to the overgrowth of the lesion. "This was not diagnosed based on the fact that the erythema was not apparent. The patient later developed a pyogenic granuloma, which is a complication that can be seen in patients with port-wine stains as they get older."

Photos courtesy Dr. Patricia A. Treadwell

Erythema can be missed in children of color with atopic dermatitis as well. For this reason, atopic dermatitis may be underdiagnosed in this population overall, she said. Another challenge is the common practice of grading the severity of atopic dermatitis in lighter-skinned patients based on the degree of erythema. "In children with a fair amount of pigmentation in their skin, the erythema may not be noted and the severity will not be recognized."

Similarly, you might need a higher index of clinical suspicion to diagnose a child of color with contact dermatitis. Again, the erythema can be subtle. In contrast, "contact dermatitis can be very clear in a Caucasian patient. But the lesions are the same – linear, asymmetrical, and occurring on exposed areas," Dr. Treadwell said. Pruritus is common, and edema and swelling also occur. Watch for development of vesiculobullous lesions.

Phytophotodermatitis is another dermatologic condition that may require some additional detective work in children of color. Dr. Treadwell described a girl with a unique hyperpigmentation pattern on her legs and arms. She was referred following a vacation in Cancun, Mexico, with her family, and there was a concern about an autoimmune process. "They asked if she needed blood work. I said no, she was eating a mango and went out in the sun." Some of the mango juice splashed on her legs and arms.

Phytophotodermatitis occurs when furocoumarins from tropical fruit, citrus, celery, fennel, or parsnip come into contact with skin subsequently exposed to the sun.

"This condition can have a fairly bizarre pattern of presentation," Dr. Treadwell said. "Again, if there is more pigment in the skin, hyperpigmentation can present in a less common way than might be expected."

Some dermatoses are noted more commonly in children of color, she said. For example, the hyperpigmented macules or pustules that characterize transient neonatal pustular melanosis are reported in 4.4% of African American infants and 0.2% of Caucasian infants. "The percentages here may be related to the pigmentation in the skin. This does occur in Caucasian infants, but it may not be as noticeable."

The condition can be present at birth. The macules and pustules can appear anywhere on the body, but most often on the chin, neck, upper chest, and/or lower back. The good news is they fade over time and are benign, so no treatment is necessary. The differential diagnosis from such conditions as herpes simplex and erythema toxicum is important, however, said Dr. Treadwell. One tip is to check for lesions on the palms and soles, which can be diagnostic in transient neonatal pustular melanosis, but not for erythema toxicum. A biopsy can confirm your clinical suspicions.

The cutaneous manifestations of neonatal lupus erythematosus can tip you off to this condition, she said. Skin lesions can be annular, discoid, or atrophic. Some children present with "raccoon eyes." Because this condition is related to maternal antibodies passed through the placenta during gestation, lesions generally clear by 6 months to 1 year of age.

The mother may have a diagnosis of an autoimmune disease, or she may be completely asymptomatic. In one instance, a mother brought her newborn to Dr. Treadwell’s clinic. He had annular lesions on his forehead with some erythema. The lesions were more edematous around the edges. He actually got some sun exposure between his first and second visits, and developed more-discoid lesions in his sun-exposed areas. He also presented with lesions in non–sun exposed areas.

"The mother had a positive ANA [antinuclear antibody] test. I told her she should go to her doctor for urine and blood pressure monitoring," Dr. Treadwell said. "She had no symptoms and thought I didn’t know what I was talking about." Two years later, the woman returned with a newborn daughter who also had neonatal lupus with lesions on her face and some patchy alopecia on her scalp.

"The brother came back in, ... and he had telangiectasias already present at age 2 years."

Your evaluation can be more family centered. Consider testing the parent and affected children for anti-Rho, anti-La, and anti-RNP.

"I usually treat them with sun avoidance, sun protection, and possibly hydrocortisone," Dr. Treadwell said. She also recommends one electrocardiogram to rule out any cardiac consequences.

Dr. Treadwell reported that she had no relevant financial disclosures.