Damian McNamara is a journalist for Medscape Medical News and MDedge. He worked full-time for MDedge as the Miami Bureau covering a dozen medical specialties during 2001-2012, then as a freelancer for Medscape and MDedge, before being hired on staff by Medscape in 2018. Now the two companies are one. He uses what he learned in school – Damian has a BS in chemistry and an MS in science, health and environmental reporting/journalism. He works out of a home office in Miami, with a 100-pound chocolate lab known to snore under his desk during work hours.

Nephrologist Filters Through Febrile Infant UTI Guidelines

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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.

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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.

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Problematic Physician Behavior Can Be Cured

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MIAMI BEACH – You get along with most of your colleagues in the hospital, but there is that one physician who doesn’t follow the rules, disrupts everyone’s workday, and ramps up the tension when they walk onto the ward.

Advice on which strategies work best to change disruptive and other problematic physician behavior comes from an anesthesiologist who oversees a South Florida health system of more than 1,400 doctors.

Dr. David Lubarsky

Focusing on changing behavior "is primarily what I do now more than anything else. ... It is about getting people to all pull in one direction," said Dr. David Lubarsky, who in September 2011 became CEO of UHealth Physician Practice and associate vice president for UHealth Practice administration at the University of Miami.

Disruptive or above-the-law behavior may garner the most attention, but behaviors related to low productivity, poor resource utilization, or poor patient satisfaction scores also need to change in this era of heightened accountability, Dr. Lubarsky said at a meeting on perioperative medicine sponsored by the University of Miami.

Sometimes there is a significant disconnect between how a disruptive physician perceives their own behavior compared with the impressions of clinicians around them. In such instances, a program such as Physicians Universal Leadership Skills Survey Enhancement (P.U.L.S.E.) 360 can help. "I have referred physicians for this," Dr. Lubarsky said. Essentially, the doctor in question first rates their own workplace behavior, and then the multidisciplinary team around them also completes surveys. Next, the doctor receives very specific feedback on both positive and negative behaviors.

"When they are confronted with this and told specifically what they are doing that isn’t really working, and given alternatives about how to approach a situation – and they see their scores getting better – they get better, they feel better. The whole team operates better. It’s a very positive experience."

Another tip: Don’t expend energy getting physicians to change their behavior when a technologic solution can achieve the same result, said Dr. Lubarsky, who retains his title as Emanuel M. Papper Professor and Chair, Department of Anesthesiology, Perioperative Medicine, and Pain Management, at the University of Miami.

A computerized prompt to administer preoperative antibiotics on time is an example. If this does not happen, other preparations for surgery automatically stop. Another successful intervention addresses incomplete chart documentation. This used to be an issue at the University of Miami when support staff had to "chase down a doctor for a signature," Dr. Lubarsky said. Now, if an attending forgets their documentation, they receive a page. "Incomplete chart documentation is now close to zero."

"You have to make it easy for everyone to do the right thing," he said.

Providing incentives can improve behaviors as well, but some ideas work better than others. Find out what the physicians value to devise meaningful incentives, Dr. Lubarsky said.

Increased commitment and motivation often ensue when physicians and others participate in setting specific, measurable goals. Difficult but achievable goals generate better performance than easy or impossible ones, Dr. Lubarsky noted. "The doctor must believe the goal is within reach."

Keep the plan simple. "Three to five goals are preferable to six or more goals," he said. Avoid complex incentive plans that assign two points for writing a paper, one point for attending a conference, and one point for staying late at work because "nobody understands this."

This era of tracking benchmark data for individual providers raises the question: Why is changing how people deliver care so difficult?

Part of it stems from physician experience and attitude, Dr. Lubarsky said. "After 4 years of college, 4 years of medical school, an average 4 years of training, and an average $150,000 of debt ... you paid for the right to call yourself a physician." Professional autonomy is deeply valued by physicians, he added.

However, "benchmarking, or use of external data, is very common now and it’s very powerful," Dr. Lubarsky said.

Physicians often balk when presented with their numbers, Dr. Lubarsky said. "I have not met a doctor yet who believes they are average or below average. I hear all this time: ‘The numbers are wrong. You have to get the right data.’ " Other reactions include, "So what?" or "My numbers are worse because my patients are sicker." Or there are physicians who dismiss what they perceive as a cookbook approach to medicine: "Are you planning to really make us do all you say and not individualize it for each patient?"

Benchmarking also relates to resource utilization. Physician attitude and behaviors are important because doctors dictate about 85% of the money spent in the hospital, he said.

 

 

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

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MIAMI BEACH – You get along with most of your colleagues in the hospital, but there is that one physician who doesn’t follow the rules, disrupts everyone’s workday, and ramps up the tension when they walk onto the ward.

Advice on which strategies work best to change disruptive and other problematic physician behavior comes from an anesthesiologist who oversees a South Florida health system of more than 1,400 doctors.

Dr. David Lubarsky

Focusing on changing behavior "is primarily what I do now more than anything else. ... It is about getting people to all pull in one direction," said Dr. David Lubarsky, who in September 2011 became CEO of UHealth Physician Practice and associate vice president for UHealth Practice administration at the University of Miami.

Disruptive or above-the-law behavior may garner the most attention, but behaviors related to low productivity, poor resource utilization, or poor patient satisfaction scores also need to change in this era of heightened accountability, Dr. Lubarsky said at a meeting on perioperative medicine sponsored by the University of Miami.

Sometimes there is a significant disconnect between how a disruptive physician perceives their own behavior compared with the impressions of clinicians around them. In such instances, a program such as Physicians Universal Leadership Skills Survey Enhancement (P.U.L.S.E.) 360 can help. "I have referred physicians for this," Dr. Lubarsky said. Essentially, the doctor in question first rates their own workplace behavior, and then the multidisciplinary team around them also completes surveys. Next, the doctor receives very specific feedback on both positive and negative behaviors.

"When they are confronted with this and told specifically what they are doing that isn’t really working, and given alternatives about how to approach a situation – and they see their scores getting better – they get better, they feel better. The whole team operates better. It’s a very positive experience."

Another tip: Don’t expend energy getting physicians to change their behavior when a technologic solution can achieve the same result, said Dr. Lubarsky, who retains his title as Emanuel M. Papper Professor and Chair, Department of Anesthesiology, Perioperative Medicine, and Pain Management, at the University of Miami.

A computerized prompt to administer preoperative antibiotics on time is an example. If this does not happen, other preparations for surgery automatically stop. Another successful intervention addresses incomplete chart documentation. This used to be an issue at the University of Miami when support staff had to "chase down a doctor for a signature," Dr. Lubarsky said. Now, if an attending forgets their documentation, they receive a page. "Incomplete chart documentation is now close to zero."

"You have to make it easy for everyone to do the right thing," he said.

Providing incentives can improve behaviors as well, but some ideas work better than others. Find out what the physicians value to devise meaningful incentives, Dr. Lubarsky said.

Increased commitment and motivation often ensue when physicians and others participate in setting specific, measurable goals. Difficult but achievable goals generate better performance than easy or impossible ones, Dr. Lubarsky noted. "The doctor must believe the goal is within reach."

Keep the plan simple. "Three to five goals are preferable to six or more goals," he said. Avoid complex incentive plans that assign two points for writing a paper, one point for attending a conference, and one point for staying late at work because "nobody understands this."

This era of tracking benchmark data for individual providers raises the question: Why is changing how people deliver care so difficult?

Part of it stems from physician experience and attitude, Dr. Lubarsky said. "After 4 years of college, 4 years of medical school, an average 4 years of training, and an average $150,000 of debt ... you paid for the right to call yourself a physician." Professional autonomy is deeply valued by physicians, he added.

However, "benchmarking, or use of external data, is very common now and it’s very powerful," Dr. Lubarsky said.

Physicians often balk when presented with their numbers, Dr. Lubarsky said. "I have not met a doctor yet who believes they are average or below average. I hear all this time: ‘The numbers are wrong. You have to get the right data.’ " Other reactions include, "So what?" or "My numbers are worse because my patients are sicker." Or there are physicians who dismiss what they perceive as a cookbook approach to medicine: "Are you planning to really make us do all you say and not individualize it for each patient?"

Benchmarking also relates to resource utilization. Physician attitude and behaviors are important because doctors dictate about 85% of the money spent in the hospital, he said.

 

 

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

MIAMI BEACH – You get along with most of your colleagues in the hospital, but there is that one physician who doesn’t follow the rules, disrupts everyone’s workday, and ramps up the tension when they walk onto the ward.

Advice on which strategies work best to change disruptive and other problematic physician behavior comes from an anesthesiologist who oversees a South Florida health system of more than 1,400 doctors.

Dr. David Lubarsky

Focusing on changing behavior "is primarily what I do now more than anything else. ... It is about getting people to all pull in one direction," said Dr. David Lubarsky, who in September 2011 became CEO of UHealth Physician Practice and associate vice president for UHealth Practice administration at the University of Miami.

Disruptive or above-the-law behavior may garner the most attention, but behaviors related to low productivity, poor resource utilization, or poor patient satisfaction scores also need to change in this era of heightened accountability, Dr. Lubarsky said at a meeting on perioperative medicine sponsored by the University of Miami.

Sometimes there is a significant disconnect between how a disruptive physician perceives their own behavior compared with the impressions of clinicians around them. In such instances, a program such as Physicians Universal Leadership Skills Survey Enhancement (P.U.L.S.E.) 360 can help. "I have referred physicians for this," Dr. Lubarsky said. Essentially, the doctor in question first rates their own workplace behavior, and then the multidisciplinary team around them also completes surveys. Next, the doctor receives very specific feedback on both positive and negative behaviors.

"When they are confronted with this and told specifically what they are doing that isn’t really working, and given alternatives about how to approach a situation – and they see their scores getting better – they get better, they feel better. The whole team operates better. It’s a very positive experience."

Another tip: Don’t expend energy getting physicians to change their behavior when a technologic solution can achieve the same result, said Dr. Lubarsky, who retains his title as Emanuel M. Papper Professor and Chair, Department of Anesthesiology, Perioperative Medicine, and Pain Management, at the University of Miami.

A computerized prompt to administer preoperative antibiotics on time is an example. If this does not happen, other preparations for surgery automatically stop. Another successful intervention addresses incomplete chart documentation. This used to be an issue at the University of Miami when support staff had to "chase down a doctor for a signature," Dr. Lubarsky said. Now, if an attending forgets their documentation, they receive a page. "Incomplete chart documentation is now close to zero."

"You have to make it easy for everyone to do the right thing," he said.

Providing incentives can improve behaviors as well, but some ideas work better than others. Find out what the physicians value to devise meaningful incentives, Dr. Lubarsky said.

Increased commitment and motivation often ensue when physicians and others participate in setting specific, measurable goals. Difficult but achievable goals generate better performance than easy or impossible ones, Dr. Lubarsky noted. "The doctor must believe the goal is within reach."

Keep the plan simple. "Three to five goals are preferable to six or more goals," he said. Avoid complex incentive plans that assign two points for writing a paper, one point for attending a conference, and one point for staying late at work because "nobody understands this."

This era of tracking benchmark data for individual providers raises the question: Why is changing how people deliver care so difficult?

Part of it stems from physician experience and attitude, Dr. Lubarsky said. "After 4 years of college, 4 years of medical school, an average 4 years of training, and an average $150,000 of debt ... you paid for the right to call yourself a physician." Professional autonomy is deeply valued by physicians, he added.

However, "benchmarking, or use of external data, is very common now and it’s very powerful," Dr. Lubarsky said.

Physicians often balk when presented with their numbers, Dr. Lubarsky said. "I have not met a doctor yet who believes they are average or below average. I hear all this time: ‘The numbers are wrong. You have to get the right data.’ " Other reactions include, "So what?" or "My numbers are worse because my patients are sicker." Or there are physicians who dismiss what they perceive as a cookbook approach to medicine: "Are you planning to really make us do all you say and not individualize it for each patient?"

Benchmarking also relates to resource utilization. Physician attitude and behaviors are important because doctors dictate about 85% of the money spent in the hospital, he said.

 

 

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

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Strategies to Help Kids With IBD Thrive

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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.

Dr. Alisa Muniz Crim

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.

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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.

Dr. Alisa Muniz Crim

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.

Dr. Alisa Muniz Crim

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.

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Genetic Microarrays Grow for Neurodevelopmental Diagnosis

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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.

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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.

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Physician-Entrepreneurs Share Lessons Learned

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Think you’ve got a great technology idea to improve your clinical work experience and optimize patient care?

You’re probably not alone. But identifying an opportunity and taking it to the next level – a successful business solution adopted by many of your colleagues – are a long, arduous, and complex process, according to two hospitalists who are developing apps designed to improve productivity in daily practice, optimize patient care, and save money.

Courtesy Dr. William Morris
Dr. William Morris (Cleveland Clinic) holds a mobile device displaying his new EMR interface.

Dr. William Morris, vice chair of clinical informatics and director of the Clinical Solutions Group at the Cleveland Clinic, is in the final phases of testing a novel electronic medical record (EMR) interface, which he developed along with a cardiothoracic surgeon and an orthopedist at the Cleveland Clinic.

Dr. Elizabeth Farrell of Beth Israel Deaconess Medical Center in Boston has just released an iPhone app that uses Bayes nomograms to inform decision-making on testing and treatment. The app draws on a database of likelihood ratios and performs the calculations at the point of care.

Both physicians saw gaps in their daily practices – things that didn’t work well or were inefficient – and devised app-based solutions to solve those problems.

Dr. Morris saw the limitations in the existing EMRs being used in his hospital. The EMRs contained a lot of data, but provided little clinical information in a context that mattered according to the type of provider, the particular setting within the hospital, and the particular patient status, he explained.

"For example, if you open any electronic medical record, a normal value for creatinine is set across the enterprise," Dr. Morris said. The value appears in red if the patient has kidney dysfunction. "But a patient with end-stage renal disease should be red – you already know that."

Dr. Morris and his colleagues realized that the EMR needed to be taken to the next level. They began by devising an interface that synthesizes and presents the newest and most relevant information at the point of care. The experience is "like having a seasoned resident standing right next to you who is only going to give you those pertinent things" needed to have an impact on that patient’s care.

To get that depth of information, the physicians developed several "novel data presentation layers" that work with the EMR in real time to present data that is tailored to the specific clinical situation – it is context-, provider-, and patient-aware. Clinical items that a physician typically puts together now "cohabitate" the screen. As an example, a chest x-ray might come up along with a trend line showing a patient’s oxygen saturation.

The app designed by Dr. Farrell grew out of her experience teaching on medical rounds at Beth Israel several years ago. Multiple providers varied widely in how they were accessing and interpreting data for individual patients.

As an initial solution, Dr. Farrell printed out index cards with Bayes nomograms that internists, residents, and medical students could use on rounds to convert a pretest probability to a post-test probability for a particular patient.

But the low-tech approach was too limited in its scope. "I ran into a lot of problems because someone would lose their cards or leave them in their white coat back in the workroom," Dr. Farrell said. "Sometimes a test would come up that we wouldn’t have the likelihood ratio for. It got a little frustrating."

Images courtesy Alan Nawoj/Beacon Mobile
Graphical depiction of a Bayes nomogram, showing how the user's pre-test probability for a particular diagnosis combined with the appropriate likelihood ratio for the diagnostic test produces a corresponding post-test probability.

Dr. Farrell realized that iPhones were ubiquitous among the medical staff, many of whom were already using the devices to seek out information for patient management. "Once I thought about putting the nomograms on the iPhone and having the app perform the calculations, the idea came to me that building a database of likelihood ratios would be even more useful," said Dr. Farrell, staff internist in Beth Israel’s Hospitalist Medicine Program.

And both hospitalists credit their success to strong business cases built in collaboration with their hospitals.

It didn’t hurt that Dr. Farrell’s husband, Alan Nawoj, is a software developer who has started a business developing iPhone apps. "We felt like this would be a great way for us to work together and partner my content expertise with his software development skills to create a new product," Dr. Farrell said.

 

 

Mr. Nawoj’s advice for would-be physician app entrepreneurs? Before you start, clearly define the goals of the application, including potential end users and where they will likely use the technology. Meet with developers, physicians, and any other end users in person to brainstorm. Draw out – literally on a whiteboard – everyone’s expectations for the product. Development of a new app through e-mail and telephone calls is much less time efficient in the end, he said.

The in-person meeting also is important because "a developer’s perspective and a physician’s perspective are going to be very different," Dr. Farrell said.

Be sure what you choose is something you are passionate about, Dr. Farrell said, "because if you’re not, it’s just going to be in the way of other things you’d rather be doing." The ideal situation is to develop a technology you yourself will ultimately use on a daily basis, she said. "That keeps up your motivation and keeps you interested and excited."

Also, solicit ongoing feedback from likely users as the product is further and further refined, added Mr. Nawoj, founder and chief software architect at Beacon Mobile.

Dr. Morris also outlined advice for physicians seeking to develop a health care business idea. "Find someone in your organization to mentor you, someone who has been successful in finding an opportunity and executing their idea."

In addition, realize that the problem you’re trying to solve rarely is a gap in technology alone, Dr. Morris said. More often it’s a "people-process gap," which makes it all the more important to know your stakeholders. "The fact that you’re a doctor is not enough. You can get your blinders on. You may veer off if you don’t have frequent interactions with other clinicians or trusted advisers."

A willingness to take some calculated risk is essential if you’re going to become an entrepreneur, Dr. Morris said. "It’s a little unnerving. This is real money, health care dollars, and a real investment in time."

When he and his colleagues at the Cleveland Clinic decided to develop technology to make the institution’s EMRs make more sense from a user standpoint, they spent "an enormous amount of time developing a business case," he said. They had to prove a demonstrable return on investment that would support development of the new EMR interface.

Dr. Farrell said that for her, and probably for any medical entrepreneur, navigating potential conflict-of-interest issues was a challenge.

"You certainly need to be transparent. This is something my husband’s company has developed, and though I’m specifically not the one receiving the money, that is obviously very close to home. The wife-husband team spoke with the intellectual property personnel at Beth Israel. "Because they fund my salary, [the institution] would be at least able to make an argument that they would own part of the rights to the product as well." The same would be true for any physician developing a product related to the work a hospital is paying them to do, Dr. Farrell said.

Courtesy Dr. Elizabeth Farrell
Dr. Elizabeth Farrell and her husband Alan Nowaj working together in their home office, where they spent many hours developing the Medicine Toolkit.

"Beth Israel decided that because of all the liability associated with a medical application, and because Apple’s policy is that they do not accept any of the liability [for any applications used on their products], it all falls on the developers." That process took 5 months, Dr. Farrell added, because like Dr. Morris’ interface, the situation was a first for the institution.

Throughout the process, one of the most important things to Dr. Farrell was "to find that balance where I don’t feel like I’m self-promoting and trying to make more money, but at the same time not holding back on something I think would be really useful for everyone."

App’s Profit Measured in Rewards Not Dollars

Bettering medicine aside, what’s the payoff for following the entrepreneurial muse? Measuring a profit can be complicated, says the team behind the Medicine Toolkit app.

"I think it would be hard to define what a ‘profit’ would be. It’s hard to put a price tag on the ‘costs’ that went into making the app, in regards to the amount of our free time we spent developing it, and the ongoing time investment that will be required to keep it updated," says Dr. Farrell.

In a similar vein, her husband, whose software company, Beacon Mobile, developed the app adds: "In general, my business (Beacon Mobile) is a for-profit enterprise ... but when Elizabeth and I partnered together to create the Medicine Toolkit app, the primary goal for this particular product was not around profit but more around helping to make teaching opportunities between med students, residents, and attendings more efficient and effective ... starting with the members of Elizabeth’s team. The fact that others have also shown an interest in the app and found it to be useful in their daily work has been very rewarding for both of us."

 

 

Dr. Farrell says that several of her current and former colleagues have used the app at Massachusetts General Hospital, Georgetown, and University of California, San Francisco.

Developing an app for the iPhone/iPad can be "in the several-thousand-dollar range" for a "basic app that doesn’t have much functionality" to "hundreds of thousands of dollars" for one that can easily interface with other systems," Mr. Nawoj estimates. He lists the individuals typically involved with developing an app: "graphic designer(s) to create all of the images that go into the app, software engineer(s) to write the code that brings the app to life, a project manager to keep the project on track ... and possibly a sound-effects engineer to develop any custom sounds/music/etc."

He adds this bit of wisdom: "As with many other disciplines, the old saying that "you get what you pay for" almost always holds true when you are talking about developing any type of software application."

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Think you’ve got a great technology idea to improve your clinical work experience and optimize patient care?

You’re probably not alone. But identifying an opportunity and taking it to the next level – a successful business solution adopted by many of your colleagues – are a long, arduous, and complex process, according to two hospitalists who are developing apps designed to improve productivity in daily practice, optimize patient care, and save money.

Courtesy Dr. William Morris
Dr. William Morris (Cleveland Clinic) holds a mobile device displaying his new EMR interface.

Dr. William Morris, vice chair of clinical informatics and director of the Clinical Solutions Group at the Cleveland Clinic, is in the final phases of testing a novel electronic medical record (EMR) interface, which he developed along with a cardiothoracic surgeon and an orthopedist at the Cleveland Clinic.

Dr. Elizabeth Farrell of Beth Israel Deaconess Medical Center in Boston has just released an iPhone app that uses Bayes nomograms to inform decision-making on testing and treatment. The app draws on a database of likelihood ratios and performs the calculations at the point of care.

Both physicians saw gaps in their daily practices – things that didn’t work well or were inefficient – and devised app-based solutions to solve those problems.

Dr. Morris saw the limitations in the existing EMRs being used in his hospital. The EMRs contained a lot of data, but provided little clinical information in a context that mattered according to the type of provider, the particular setting within the hospital, and the particular patient status, he explained.

"For example, if you open any electronic medical record, a normal value for creatinine is set across the enterprise," Dr. Morris said. The value appears in red if the patient has kidney dysfunction. "But a patient with end-stage renal disease should be red – you already know that."

Dr. Morris and his colleagues realized that the EMR needed to be taken to the next level. They began by devising an interface that synthesizes and presents the newest and most relevant information at the point of care. The experience is "like having a seasoned resident standing right next to you who is only going to give you those pertinent things" needed to have an impact on that patient’s care.

To get that depth of information, the physicians developed several "novel data presentation layers" that work with the EMR in real time to present data that is tailored to the specific clinical situation – it is context-, provider-, and patient-aware. Clinical items that a physician typically puts together now "cohabitate" the screen. As an example, a chest x-ray might come up along with a trend line showing a patient’s oxygen saturation.

The app designed by Dr. Farrell grew out of her experience teaching on medical rounds at Beth Israel several years ago. Multiple providers varied widely in how they were accessing and interpreting data for individual patients.

As an initial solution, Dr. Farrell printed out index cards with Bayes nomograms that internists, residents, and medical students could use on rounds to convert a pretest probability to a post-test probability for a particular patient.

But the low-tech approach was too limited in its scope. "I ran into a lot of problems because someone would lose their cards or leave them in their white coat back in the workroom," Dr. Farrell said. "Sometimes a test would come up that we wouldn’t have the likelihood ratio for. It got a little frustrating."

Images courtesy Alan Nawoj/Beacon Mobile
Graphical depiction of a Bayes nomogram, showing how the user's pre-test probability for a particular diagnosis combined with the appropriate likelihood ratio for the diagnostic test produces a corresponding post-test probability.

Dr. Farrell realized that iPhones were ubiquitous among the medical staff, many of whom were already using the devices to seek out information for patient management. "Once I thought about putting the nomograms on the iPhone and having the app perform the calculations, the idea came to me that building a database of likelihood ratios would be even more useful," said Dr. Farrell, staff internist in Beth Israel’s Hospitalist Medicine Program.

And both hospitalists credit their success to strong business cases built in collaboration with their hospitals.

It didn’t hurt that Dr. Farrell’s husband, Alan Nawoj, is a software developer who has started a business developing iPhone apps. "We felt like this would be a great way for us to work together and partner my content expertise with his software development skills to create a new product," Dr. Farrell said.

 

 

Mr. Nawoj’s advice for would-be physician app entrepreneurs? Before you start, clearly define the goals of the application, including potential end users and where they will likely use the technology. Meet with developers, physicians, and any other end users in person to brainstorm. Draw out – literally on a whiteboard – everyone’s expectations for the product. Development of a new app through e-mail and telephone calls is much less time efficient in the end, he said.

The in-person meeting also is important because "a developer’s perspective and a physician’s perspective are going to be very different," Dr. Farrell said.

Be sure what you choose is something you are passionate about, Dr. Farrell said, "because if you’re not, it’s just going to be in the way of other things you’d rather be doing." The ideal situation is to develop a technology you yourself will ultimately use on a daily basis, she said. "That keeps up your motivation and keeps you interested and excited."

Also, solicit ongoing feedback from likely users as the product is further and further refined, added Mr. Nawoj, founder and chief software architect at Beacon Mobile.

Dr. Morris also outlined advice for physicians seeking to develop a health care business idea. "Find someone in your organization to mentor you, someone who has been successful in finding an opportunity and executing their idea."

In addition, realize that the problem you’re trying to solve rarely is a gap in technology alone, Dr. Morris said. More often it’s a "people-process gap," which makes it all the more important to know your stakeholders. "The fact that you’re a doctor is not enough. You can get your blinders on. You may veer off if you don’t have frequent interactions with other clinicians or trusted advisers."

A willingness to take some calculated risk is essential if you’re going to become an entrepreneur, Dr. Morris said. "It’s a little unnerving. This is real money, health care dollars, and a real investment in time."

When he and his colleagues at the Cleveland Clinic decided to develop technology to make the institution’s EMRs make more sense from a user standpoint, they spent "an enormous amount of time developing a business case," he said. They had to prove a demonstrable return on investment that would support development of the new EMR interface.

Dr. Farrell said that for her, and probably for any medical entrepreneur, navigating potential conflict-of-interest issues was a challenge.

"You certainly need to be transparent. This is something my husband’s company has developed, and though I’m specifically not the one receiving the money, that is obviously very close to home. The wife-husband team spoke with the intellectual property personnel at Beth Israel. "Because they fund my salary, [the institution] would be at least able to make an argument that they would own part of the rights to the product as well." The same would be true for any physician developing a product related to the work a hospital is paying them to do, Dr. Farrell said.

Courtesy Dr. Elizabeth Farrell
Dr. Elizabeth Farrell and her husband Alan Nowaj working together in their home office, where they spent many hours developing the Medicine Toolkit.

"Beth Israel decided that because of all the liability associated with a medical application, and because Apple’s policy is that they do not accept any of the liability [for any applications used on their products], it all falls on the developers." That process took 5 months, Dr. Farrell added, because like Dr. Morris’ interface, the situation was a first for the institution.

Throughout the process, one of the most important things to Dr. Farrell was "to find that balance where I don’t feel like I’m self-promoting and trying to make more money, but at the same time not holding back on something I think would be really useful for everyone."

App’s Profit Measured in Rewards Not Dollars

Bettering medicine aside, what’s the payoff for following the entrepreneurial muse? Measuring a profit can be complicated, says the team behind the Medicine Toolkit app.

"I think it would be hard to define what a ‘profit’ would be. It’s hard to put a price tag on the ‘costs’ that went into making the app, in regards to the amount of our free time we spent developing it, and the ongoing time investment that will be required to keep it updated," says Dr. Farrell.

In a similar vein, her husband, whose software company, Beacon Mobile, developed the app adds: "In general, my business (Beacon Mobile) is a for-profit enterprise ... but when Elizabeth and I partnered together to create the Medicine Toolkit app, the primary goal for this particular product was not around profit but more around helping to make teaching opportunities between med students, residents, and attendings more efficient and effective ... starting with the members of Elizabeth’s team. The fact that others have also shown an interest in the app and found it to be useful in their daily work has been very rewarding for both of us."

 

 

Dr. Farrell says that several of her current and former colleagues have used the app at Massachusetts General Hospital, Georgetown, and University of California, San Francisco.

Developing an app for the iPhone/iPad can be "in the several-thousand-dollar range" for a "basic app that doesn’t have much functionality" to "hundreds of thousands of dollars" for one that can easily interface with other systems," Mr. Nawoj estimates. He lists the individuals typically involved with developing an app: "graphic designer(s) to create all of the images that go into the app, software engineer(s) to write the code that brings the app to life, a project manager to keep the project on track ... and possibly a sound-effects engineer to develop any custom sounds/music/etc."

He adds this bit of wisdom: "As with many other disciplines, the old saying that "you get what you pay for" almost always holds true when you are talking about developing any type of software application."

Think you’ve got a great technology idea to improve your clinical work experience and optimize patient care?

You’re probably not alone. But identifying an opportunity and taking it to the next level – a successful business solution adopted by many of your colleagues – are a long, arduous, and complex process, according to two hospitalists who are developing apps designed to improve productivity in daily practice, optimize patient care, and save money.

Courtesy Dr. William Morris
Dr. William Morris (Cleveland Clinic) holds a mobile device displaying his new EMR interface.

Dr. William Morris, vice chair of clinical informatics and director of the Clinical Solutions Group at the Cleveland Clinic, is in the final phases of testing a novel electronic medical record (EMR) interface, which he developed along with a cardiothoracic surgeon and an orthopedist at the Cleveland Clinic.

Dr. Elizabeth Farrell of Beth Israel Deaconess Medical Center in Boston has just released an iPhone app that uses Bayes nomograms to inform decision-making on testing and treatment. The app draws on a database of likelihood ratios and performs the calculations at the point of care.

Both physicians saw gaps in their daily practices – things that didn’t work well or were inefficient – and devised app-based solutions to solve those problems.

Dr. Morris saw the limitations in the existing EMRs being used in his hospital. The EMRs contained a lot of data, but provided little clinical information in a context that mattered according to the type of provider, the particular setting within the hospital, and the particular patient status, he explained.

"For example, if you open any electronic medical record, a normal value for creatinine is set across the enterprise," Dr. Morris said. The value appears in red if the patient has kidney dysfunction. "But a patient with end-stage renal disease should be red – you already know that."

Dr. Morris and his colleagues realized that the EMR needed to be taken to the next level. They began by devising an interface that synthesizes and presents the newest and most relevant information at the point of care. The experience is "like having a seasoned resident standing right next to you who is only going to give you those pertinent things" needed to have an impact on that patient’s care.

To get that depth of information, the physicians developed several "novel data presentation layers" that work with the EMR in real time to present data that is tailored to the specific clinical situation – it is context-, provider-, and patient-aware. Clinical items that a physician typically puts together now "cohabitate" the screen. As an example, a chest x-ray might come up along with a trend line showing a patient’s oxygen saturation.

The app designed by Dr. Farrell grew out of her experience teaching on medical rounds at Beth Israel several years ago. Multiple providers varied widely in how they were accessing and interpreting data for individual patients.

As an initial solution, Dr. Farrell printed out index cards with Bayes nomograms that internists, residents, and medical students could use on rounds to convert a pretest probability to a post-test probability for a particular patient.

But the low-tech approach was too limited in its scope. "I ran into a lot of problems because someone would lose their cards or leave them in their white coat back in the workroom," Dr. Farrell said. "Sometimes a test would come up that we wouldn’t have the likelihood ratio for. It got a little frustrating."

Images courtesy Alan Nawoj/Beacon Mobile
Graphical depiction of a Bayes nomogram, showing how the user's pre-test probability for a particular diagnosis combined with the appropriate likelihood ratio for the diagnostic test produces a corresponding post-test probability.

Dr. Farrell realized that iPhones were ubiquitous among the medical staff, many of whom were already using the devices to seek out information for patient management. "Once I thought about putting the nomograms on the iPhone and having the app perform the calculations, the idea came to me that building a database of likelihood ratios would be even more useful," said Dr. Farrell, staff internist in Beth Israel’s Hospitalist Medicine Program.

And both hospitalists credit their success to strong business cases built in collaboration with their hospitals.

It didn’t hurt that Dr. Farrell’s husband, Alan Nawoj, is a software developer who has started a business developing iPhone apps. "We felt like this would be a great way for us to work together and partner my content expertise with his software development skills to create a new product," Dr. Farrell said.

 

 

Mr. Nawoj’s advice for would-be physician app entrepreneurs? Before you start, clearly define the goals of the application, including potential end users and where they will likely use the technology. Meet with developers, physicians, and any other end users in person to brainstorm. Draw out – literally on a whiteboard – everyone’s expectations for the product. Development of a new app through e-mail and telephone calls is much less time efficient in the end, he said.

The in-person meeting also is important because "a developer’s perspective and a physician’s perspective are going to be very different," Dr. Farrell said.

Be sure what you choose is something you are passionate about, Dr. Farrell said, "because if you’re not, it’s just going to be in the way of other things you’d rather be doing." The ideal situation is to develop a technology you yourself will ultimately use on a daily basis, she said. "That keeps up your motivation and keeps you interested and excited."

Also, solicit ongoing feedback from likely users as the product is further and further refined, added Mr. Nawoj, founder and chief software architect at Beacon Mobile.

Dr. Morris also outlined advice for physicians seeking to develop a health care business idea. "Find someone in your organization to mentor you, someone who has been successful in finding an opportunity and executing their idea."

In addition, realize that the problem you’re trying to solve rarely is a gap in technology alone, Dr. Morris said. More often it’s a "people-process gap," which makes it all the more important to know your stakeholders. "The fact that you’re a doctor is not enough. You can get your blinders on. You may veer off if you don’t have frequent interactions with other clinicians or trusted advisers."

A willingness to take some calculated risk is essential if you’re going to become an entrepreneur, Dr. Morris said. "It’s a little unnerving. This is real money, health care dollars, and a real investment in time."

When he and his colleagues at the Cleveland Clinic decided to develop technology to make the institution’s EMRs make more sense from a user standpoint, they spent "an enormous amount of time developing a business case," he said. They had to prove a demonstrable return on investment that would support development of the new EMR interface.

Dr. Farrell said that for her, and probably for any medical entrepreneur, navigating potential conflict-of-interest issues was a challenge.

"You certainly need to be transparent. This is something my husband’s company has developed, and though I’m specifically not the one receiving the money, that is obviously very close to home. The wife-husband team spoke with the intellectual property personnel at Beth Israel. "Because they fund my salary, [the institution] would be at least able to make an argument that they would own part of the rights to the product as well." The same would be true for any physician developing a product related to the work a hospital is paying them to do, Dr. Farrell said.

Courtesy Dr. Elizabeth Farrell
Dr. Elizabeth Farrell and her husband Alan Nowaj working together in their home office, where they spent many hours developing the Medicine Toolkit.

"Beth Israel decided that because of all the liability associated with a medical application, and because Apple’s policy is that they do not accept any of the liability [for any applications used on their products], it all falls on the developers." That process took 5 months, Dr. Farrell added, because like Dr. Morris’ interface, the situation was a first for the institution.

Throughout the process, one of the most important things to Dr. Farrell was "to find that balance where I don’t feel like I’m self-promoting and trying to make more money, but at the same time not holding back on something I think would be really useful for everyone."

App’s Profit Measured in Rewards Not Dollars

Bettering medicine aside, what’s the payoff for following the entrepreneurial muse? Measuring a profit can be complicated, says the team behind the Medicine Toolkit app.

"I think it would be hard to define what a ‘profit’ would be. It’s hard to put a price tag on the ‘costs’ that went into making the app, in regards to the amount of our free time we spent developing it, and the ongoing time investment that will be required to keep it updated," says Dr. Farrell.

In a similar vein, her husband, whose software company, Beacon Mobile, developed the app adds: "In general, my business (Beacon Mobile) is a for-profit enterprise ... but when Elizabeth and I partnered together to create the Medicine Toolkit app, the primary goal for this particular product was not around profit but more around helping to make teaching opportunities between med students, residents, and attendings more efficient and effective ... starting with the members of Elizabeth’s team. The fact that others have also shown an interest in the app and found it to be useful in their daily work has been very rewarding for both of us."

 

 

Dr. Farrell says that several of her current and former colleagues have used the app at Massachusetts General Hospital, Georgetown, and University of California, San Francisco.

Developing an app for the iPhone/iPad can be "in the several-thousand-dollar range" for a "basic app that doesn’t have much functionality" to "hundreds of thousands of dollars" for one that can easily interface with other systems," Mr. Nawoj estimates. He lists the individuals typically involved with developing an app: "graphic designer(s) to create all of the images that go into the app, software engineer(s) to write the code that brings the app to life, a project manager to keep the project on track ... and possibly a sound-effects engineer to develop any custom sounds/music/etc."

He adds this bit of wisdom: "As with many other disciplines, the old saying that "you get what you pay for" almost always holds true when you are talking about developing any type of software application."

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Heeding the Need to Feed Kids with CF

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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.

Dr. William I. Muinos

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.

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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.

Dr. William I. Muinos

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.

Dr. William I. Muinos

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.

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EXPERT ANALYSIS FROM A PEDIATRIC UPDATE SPONSORED BY MIAMI CHILDREN'S HOSPITAL

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Don't Rush Psoriasis Diagnosis in Children

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MIAMI BEACH – A diagnosis of pediatric psoriasis is a major event for a child and their family – especially given the long-term medical and psychosocial implications – so proceed cautiously, said Dr. Ronald C. Hansen.

"I am never in a big hurry to make the diagnosis of psoriasis if I am not sure. When you make the diagnosis, you are pretty much saying: ‘You are going to have some degree of psoriasis the rest of your life," Dr. Hansen said at the South Beach Symposium.

And, "I never ever underestimate the impact of psoriasis on the child’s life," he said. "I’ve had 5-year-olds already psychologically stricken."

Affected children – particularly those with more severe disease – will be self-conscious and avoid undressing before gym class or joining others to swim at a pool, said Dr. Hansen, chief of pediatric dermatology at Phoenix Children’s Hospital. "These kids end up loathing their bodies." Psoriasis often has long-term impacts on relationships and intimacy as well.

"The psychosocial impacts ... are immense," agreed session moderator Dr. Lawrence A. Schachner. Because of this, consider whether your patient needs psychosocial counseling when you diagnose psoriasis, added Dr. Schachner, who is director of pediatric dermatology at the University of Miami.

Pediatric psoriasis impacts the whole family. Counsel parents that psoriasis will require a long-term commitment to provide care for their child.

The onset of childhood psoriasis can occur at any age, even at birth. "It is genetically loaded," Dr. Hansen said.

For example, a child born to unaffected parents has about a 4% chance of developing psoriasis, he said. In contrast, a child born to one parent with psoriasis has a 28% likelihood of also developing psoriasis, and if both parents are affected, it jumps to 65%. The chances are even greater if the child has a sibling with psoriasis.

A clinical tip is to ask parents about a history of diaper dermatitis. In his experience, when Dr. Hansen suspects childhood psoriasis, he asks families about whether the child has had difficult diaper rashes. "The parents roll their eyes and say, ‘Yes, diaper rashes from hell.’ This is one of the things I hear routinely when I make the diagnosis in a 4-year-old."

Umbilical and scalp involvement often suggest psoriasis. Severe seborrheic dermatitis, for example, is another diagnostic clue. "We all know about the flaky, persistent scalp dermatitis, sometimes misdiagnosed as seborrheic dermatitis, but again it’s the seborrheic dermatitis from hell. It doesn’t respond to usual treatments," he said.

Even with a rash that looks like psoriasis, most children will have something else: seborrheic dermatitis, atopic dermatitis, or a candidal infection. "If it’s the first time I see this rash, I don’t make the diagnosis of psoriasis. ... Maybe about 20% of them will end up with psoriasis," Dr. Hansen said.

In contrast to adults with psoriasis, pediatric patients can present with prominent, full facial involvement. Flexural involvement is common in all ages, and the lesions can be thick and white or erythematous.

Be particularly thorough with your differential diagnosis of annular psoriasis. "The annular form can fool us. There are a lot of things that cause rings," Dr. Hansen said. A misdiagnosis of extensive tinea can occur, for example.

Some children with psoriasis can have extensive nail involvement. But "nail pits typify psoriasis. ... You can only use nail pits as a diagnostic [criterion] for psoriasis if the cuticle and proximal nail fold are intact," he said.

Pustular psoriasis is rare but important to diagnose in children, Dr. Hansen said. "These patients can be physically quite ill, and treatment has to be instituted right away."

Acute generalized pustular psoriasis is a severe form. Patients can present with fevers, polyarthritis, alopecia, cholestatic jaundice, acute respiratory distress syndrome, eye complications, conjunctivitis, and other adverse signs and comorbidities. "These kids tend to be medical emergencies," Dr. Hansen said. "Consider hospitalization if they have fever."

Many potential factors can elicit this condition. Acute generalized pustular psoriasis can be triggered by an upper respiratory infection or urinary tract infection. "Infections can open the door to anyone already predisposed to get psoriasis," Dr. Hansen said. Withdrawal from systemic or topical steroids and sunburn are other triggers. "Interestingly enough, the [tumor necrosis factor] antagonists which we use to treat psoriasis can also precipitate generalized pustular psoriasis. This confuses most people," he said.

Dr. Hansen disclosed that is a researcher for Novartis. Dr. Schachner disclosed that he is a consultant for Beiersdorf and a researcher/investigator for Astellas, Ferndale, Novartis, Organogenesis, and Stiefel. Both receive royalties from Elsevier, which also owns this news organization.

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MIAMI BEACH – A diagnosis of pediatric psoriasis is a major event for a child and their family – especially given the long-term medical and psychosocial implications – so proceed cautiously, said Dr. Ronald C. Hansen.

"I am never in a big hurry to make the diagnosis of psoriasis if I am not sure. When you make the diagnosis, you are pretty much saying: ‘You are going to have some degree of psoriasis the rest of your life," Dr. Hansen said at the South Beach Symposium.

And, "I never ever underestimate the impact of psoriasis on the child’s life," he said. "I’ve had 5-year-olds already psychologically stricken."

Affected children – particularly those with more severe disease – will be self-conscious and avoid undressing before gym class or joining others to swim at a pool, said Dr. Hansen, chief of pediatric dermatology at Phoenix Children’s Hospital. "These kids end up loathing their bodies." Psoriasis often has long-term impacts on relationships and intimacy as well.

"The psychosocial impacts ... are immense," agreed session moderator Dr. Lawrence A. Schachner. Because of this, consider whether your patient needs psychosocial counseling when you diagnose psoriasis, added Dr. Schachner, who is director of pediatric dermatology at the University of Miami.

Pediatric psoriasis impacts the whole family. Counsel parents that psoriasis will require a long-term commitment to provide care for their child.

The onset of childhood psoriasis can occur at any age, even at birth. "It is genetically loaded," Dr. Hansen said.

For example, a child born to unaffected parents has about a 4% chance of developing psoriasis, he said. In contrast, a child born to one parent with psoriasis has a 28% likelihood of also developing psoriasis, and if both parents are affected, it jumps to 65%. The chances are even greater if the child has a sibling with psoriasis.

A clinical tip is to ask parents about a history of diaper dermatitis. In his experience, when Dr. Hansen suspects childhood psoriasis, he asks families about whether the child has had difficult diaper rashes. "The parents roll their eyes and say, ‘Yes, diaper rashes from hell.’ This is one of the things I hear routinely when I make the diagnosis in a 4-year-old."

Umbilical and scalp involvement often suggest psoriasis. Severe seborrheic dermatitis, for example, is another diagnostic clue. "We all know about the flaky, persistent scalp dermatitis, sometimes misdiagnosed as seborrheic dermatitis, but again it’s the seborrheic dermatitis from hell. It doesn’t respond to usual treatments," he said.

Even with a rash that looks like psoriasis, most children will have something else: seborrheic dermatitis, atopic dermatitis, or a candidal infection. "If it’s the first time I see this rash, I don’t make the diagnosis of psoriasis. ... Maybe about 20% of them will end up with psoriasis," Dr. Hansen said.

In contrast to adults with psoriasis, pediatric patients can present with prominent, full facial involvement. Flexural involvement is common in all ages, and the lesions can be thick and white or erythematous.

Be particularly thorough with your differential diagnosis of annular psoriasis. "The annular form can fool us. There are a lot of things that cause rings," Dr. Hansen said. A misdiagnosis of extensive tinea can occur, for example.

Some children with psoriasis can have extensive nail involvement. But "nail pits typify psoriasis. ... You can only use nail pits as a diagnostic [criterion] for psoriasis if the cuticle and proximal nail fold are intact," he said.

Pustular psoriasis is rare but important to diagnose in children, Dr. Hansen said. "These patients can be physically quite ill, and treatment has to be instituted right away."

Acute generalized pustular psoriasis is a severe form. Patients can present with fevers, polyarthritis, alopecia, cholestatic jaundice, acute respiratory distress syndrome, eye complications, conjunctivitis, and other adverse signs and comorbidities. "These kids tend to be medical emergencies," Dr. Hansen said. "Consider hospitalization if they have fever."

Many potential factors can elicit this condition. Acute generalized pustular psoriasis can be triggered by an upper respiratory infection or urinary tract infection. "Infections can open the door to anyone already predisposed to get psoriasis," Dr. Hansen said. Withdrawal from systemic or topical steroids and sunburn are other triggers. "Interestingly enough, the [tumor necrosis factor] antagonists which we use to treat psoriasis can also precipitate generalized pustular psoriasis. This confuses most people," he said.

Dr. Hansen disclosed that is a researcher for Novartis. Dr. Schachner disclosed that he is a consultant for Beiersdorf and a researcher/investigator for Astellas, Ferndale, Novartis, Organogenesis, and Stiefel. Both receive royalties from Elsevier, which also owns this news organization.

MIAMI BEACH – A diagnosis of pediatric psoriasis is a major event for a child and their family – especially given the long-term medical and psychosocial implications – so proceed cautiously, said Dr. Ronald C. Hansen.

"I am never in a big hurry to make the diagnosis of psoriasis if I am not sure. When you make the diagnosis, you are pretty much saying: ‘You are going to have some degree of psoriasis the rest of your life," Dr. Hansen said at the South Beach Symposium.

And, "I never ever underestimate the impact of psoriasis on the child’s life," he said. "I’ve had 5-year-olds already psychologically stricken."

Affected children – particularly those with more severe disease – will be self-conscious and avoid undressing before gym class or joining others to swim at a pool, said Dr. Hansen, chief of pediatric dermatology at Phoenix Children’s Hospital. "These kids end up loathing their bodies." Psoriasis often has long-term impacts on relationships and intimacy as well.

"The psychosocial impacts ... are immense," agreed session moderator Dr. Lawrence A. Schachner. Because of this, consider whether your patient needs psychosocial counseling when you diagnose psoriasis, added Dr. Schachner, who is director of pediatric dermatology at the University of Miami.

Pediatric psoriasis impacts the whole family. Counsel parents that psoriasis will require a long-term commitment to provide care for their child.

The onset of childhood psoriasis can occur at any age, even at birth. "It is genetically loaded," Dr. Hansen said.

For example, a child born to unaffected parents has about a 4% chance of developing psoriasis, he said. In contrast, a child born to one parent with psoriasis has a 28% likelihood of also developing psoriasis, and if both parents are affected, it jumps to 65%. The chances are even greater if the child has a sibling with psoriasis.

A clinical tip is to ask parents about a history of diaper dermatitis. In his experience, when Dr. Hansen suspects childhood psoriasis, he asks families about whether the child has had difficult diaper rashes. "The parents roll their eyes and say, ‘Yes, diaper rashes from hell.’ This is one of the things I hear routinely when I make the diagnosis in a 4-year-old."

Umbilical and scalp involvement often suggest psoriasis. Severe seborrheic dermatitis, for example, is another diagnostic clue. "We all know about the flaky, persistent scalp dermatitis, sometimes misdiagnosed as seborrheic dermatitis, but again it’s the seborrheic dermatitis from hell. It doesn’t respond to usual treatments," he said.

Even with a rash that looks like psoriasis, most children will have something else: seborrheic dermatitis, atopic dermatitis, or a candidal infection. "If it’s the first time I see this rash, I don’t make the diagnosis of psoriasis. ... Maybe about 20% of them will end up with psoriasis," Dr. Hansen said.

In contrast to adults with psoriasis, pediatric patients can present with prominent, full facial involvement. Flexural involvement is common in all ages, and the lesions can be thick and white or erythematous.

Be particularly thorough with your differential diagnosis of annular psoriasis. "The annular form can fool us. There are a lot of things that cause rings," Dr. Hansen said. A misdiagnosis of extensive tinea can occur, for example.

Some children with psoriasis can have extensive nail involvement. But "nail pits typify psoriasis. ... You can only use nail pits as a diagnostic [criterion] for psoriasis if the cuticle and proximal nail fold are intact," he said.

Pustular psoriasis is rare but important to diagnose in children, Dr. Hansen said. "These patients can be physically quite ill, and treatment has to be instituted right away."

Acute generalized pustular psoriasis is a severe form. Patients can present with fevers, polyarthritis, alopecia, cholestatic jaundice, acute respiratory distress syndrome, eye complications, conjunctivitis, and other adverse signs and comorbidities. "These kids tend to be medical emergencies," Dr. Hansen said. "Consider hospitalization if they have fever."

Many potential factors can elicit this condition. Acute generalized pustular psoriasis can be triggered by an upper respiratory infection or urinary tract infection. "Infections can open the door to anyone already predisposed to get psoriasis," Dr. Hansen said. Withdrawal from systemic or topical steroids and sunburn are other triggers. "Interestingly enough, the [tumor necrosis factor] antagonists which we use to treat psoriasis can also precipitate generalized pustular psoriasis. This confuses most people," he said.

Dr. Hansen disclosed that is a researcher for Novartis. Dr. Schachner disclosed that he is a consultant for Beiersdorf and a researcher/investigator for Astellas, Ferndale, Novartis, Organogenesis, and Stiefel. Both receive royalties from Elsevier, which also owns this news organization.

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Hyponatremia: What to Worry About in the Hospital

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MIAMI BEACH – When you get a call in the hospital to evaluate a patient with postoperative hyponatremia, you need to worry primarily about three causes, according to an expert.

Increased free water intake, sodium loss that exceeds free water loss, and – "what we see all the time" – an inability to excrete free water will be your main concerns with these inpatients, Dr. Rachel E. Thompson said.

Initially rule out the most serious potential consequences of hyponatremia. "Assess mental status first to rule out increased intracranial pressure," said Dr. Thompson, founder and director of the Medicine Consult Service at Harborview Medical Center in Seattle. She spoke at a meeting on perioperative medicine sponsored by the University of Miami. "We have rigid skulls. There is very little space for tissue expansion." Left unchecked, intracranial hypertension can lead to brain damage, herniation, and respiratory arrest. "You can die from this."

It’s also important to distinguish neurology patients from others, Dr. Thompson said. Neurologic surgeons often want patients to have sodium levels above 140 mEq/L. "However, in other populations, if there is not brain damage that is going to be worsened by this, we are a lot more laissez faire. We might allow it to go lower, and people don’t get too worried about it until you’re in the mid to low 120s."

Dr. Thompson cited a neurosurgery patient as a case example. You get a call about a 66-year-old woman currently in the rehabilitation unit following endovascular coiling for aneurysmal subarachnoid hemorrhage repair two weeks earlier. The rehabilitation specialist reports her sodium is low at 126 mEq/L (it was 142 mEq/L when she left the neurosurgery unit 7 days earlier).

Dr. Thompson electronically polled meeting attendees regarding the next appropriate step. Of the total 91 responses, 33% would evaluate the patient’s volume status, 29% would evaluate mental status, 24% would check serum osmolality, and the remaining 14% would recheck the sodium level.

"I like that you want to see the volume status, and another third of you want to check the mental status. That means you are going to go see the patient. That is great," Dr. Thompson said.

Early assessment of mental status is key in Dr. Thompson’s four-step practical model for perioperative providers caring for hyponatremia patients:

• Step 1. Go to the bedside to assess mental status. Does it reflect what you expect for that sodium level? Assess acuity and severity, check which fluids are running, determine if the patient is hypovolemic, and find out if the patient had any recent vomiting or diarrhea that could facilitate volume loss.

• Step 2. Review medications. Did the patient take any diuretics or medications known to cause syndrome of inappropriate antidiuretic hormone (SIADH)? For more information on this syndrome, see (Nephron. Clin. Pract. 2011;119:c62-73).

Step 3. Order appropriate studies. You might repeat the sodium level and check serum osmolality; also measure urine osmolality and sodium. Which brings us to the final step ...

• Step 4. "The urine is going to be where your answers are," Dr. Thompson said. If the urine is dilute, think primary polydipsia or "tea and toast diet syndrome." If the urine is concentrated, causes include chronic kidney disease, thiazides, hypothyroidism, or glucocorticoid deficiency.

It is important to be thorough and closely monitor hyponatremia patients either way, Dr. Thompson said.

Hyponatremia comes with risks. In one retrospective cohort study, hospital-acquired hyponatremia was associated with increased in-hospital mortality (adjusted odds ratio, 1.66); a 64% adjusted increase in length of stay; and a greater likelihood of discharge to a short- or long-term care facility (OR, 1.64) compared to unaffected patients (Arch. Intern. Med. 2010;8:294-302). The researchers found 38% of more than 55,000 inpatients at a single center over 7 years had hospital-acquired hyponatremia, defined as a decrease in serum sodium to below 138 mEq/L following normal levels at admission.

Although management of inpatients with hyponatremia might appear complex, there are two main treatment choices. "You either give fluids or you restrict fluids," Dr. Thompson said.

Give fluids to patients who are hypovolemic, have cerebral salt wasting, or display acute mental status changes. Go slow with treatment, she added, with a goal correction of no more than 0.5 mEq/L per hour or 12 mEq/L every 24 hours. Some clinicians recommend a more conservative increase of 6 mEq/L per 24 hours, she added.

Restrict fluids to patients with SIADH or primary polydipsia. Restriction to 1L to 2L free water is generally appropriate, Dr. Thompson said.

Sodium chloride tablets and vasopressin antagonists are two additional treatment options. Consider administration of sodium chloride tablets to patients with cerebral salt wasting, Dr. Thompson said. Some clinicians are reluctant to treat with salt tablets. "I was one of the people who used to think this was a terrible thing, but it actually works in cerebral salt wasting." The tablets also are indicated as a treatment for chronic SIADH, she said.

 

 

Vasopressin antagonists can be given I.V. or oral and are considered a treatment of last resort, she added. "Save them for the severely symptomatic patient. Yes, the sodium comes up, but there are no data on better outcomes in the acute setting." At an estimated daily cost of $260-$550, "they are extremely expensive." She cautioned that patients need to be watched for rapid overcorrection with these agents.

Dr. Thompson did not have any relevant disclosures.

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MIAMI BEACH – When you get a call in the hospital to evaluate a patient with postoperative hyponatremia, you need to worry primarily about three causes, according to an expert.

Increased free water intake, sodium loss that exceeds free water loss, and – "what we see all the time" – an inability to excrete free water will be your main concerns with these inpatients, Dr. Rachel E. Thompson said.

Initially rule out the most serious potential consequences of hyponatremia. "Assess mental status first to rule out increased intracranial pressure," said Dr. Thompson, founder and director of the Medicine Consult Service at Harborview Medical Center in Seattle. She spoke at a meeting on perioperative medicine sponsored by the University of Miami. "We have rigid skulls. There is very little space for tissue expansion." Left unchecked, intracranial hypertension can lead to brain damage, herniation, and respiratory arrest. "You can die from this."

It’s also important to distinguish neurology patients from others, Dr. Thompson said. Neurologic surgeons often want patients to have sodium levels above 140 mEq/L. "However, in other populations, if there is not brain damage that is going to be worsened by this, we are a lot more laissez faire. We might allow it to go lower, and people don’t get too worried about it until you’re in the mid to low 120s."

Dr. Thompson cited a neurosurgery patient as a case example. You get a call about a 66-year-old woman currently in the rehabilitation unit following endovascular coiling for aneurysmal subarachnoid hemorrhage repair two weeks earlier. The rehabilitation specialist reports her sodium is low at 126 mEq/L (it was 142 mEq/L when she left the neurosurgery unit 7 days earlier).

Dr. Thompson electronically polled meeting attendees regarding the next appropriate step. Of the total 91 responses, 33% would evaluate the patient’s volume status, 29% would evaluate mental status, 24% would check serum osmolality, and the remaining 14% would recheck the sodium level.

"I like that you want to see the volume status, and another third of you want to check the mental status. That means you are going to go see the patient. That is great," Dr. Thompson said.

Early assessment of mental status is key in Dr. Thompson’s four-step practical model for perioperative providers caring for hyponatremia patients:

• Step 1. Go to the bedside to assess mental status. Does it reflect what you expect for that sodium level? Assess acuity and severity, check which fluids are running, determine if the patient is hypovolemic, and find out if the patient had any recent vomiting or diarrhea that could facilitate volume loss.

• Step 2. Review medications. Did the patient take any diuretics or medications known to cause syndrome of inappropriate antidiuretic hormone (SIADH)? For more information on this syndrome, see (Nephron. Clin. Pract. 2011;119:c62-73).

Step 3. Order appropriate studies. You might repeat the sodium level and check serum osmolality; also measure urine osmolality and sodium. Which brings us to the final step ...

• Step 4. "The urine is going to be where your answers are," Dr. Thompson said. If the urine is dilute, think primary polydipsia or "tea and toast diet syndrome." If the urine is concentrated, causes include chronic kidney disease, thiazides, hypothyroidism, or glucocorticoid deficiency.

It is important to be thorough and closely monitor hyponatremia patients either way, Dr. Thompson said.

Hyponatremia comes with risks. In one retrospective cohort study, hospital-acquired hyponatremia was associated with increased in-hospital mortality (adjusted odds ratio, 1.66); a 64% adjusted increase in length of stay; and a greater likelihood of discharge to a short- or long-term care facility (OR, 1.64) compared to unaffected patients (Arch. Intern. Med. 2010;8:294-302). The researchers found 38% of more than 55,000 inpatients at a single center over 7 years had hospital-acquired hyponatremia, defined as a decrease in serum sodium to below 138 mEq/L following normal levels at admission.

Although management of inpatients with hyponatremia might appear complex, there are two main treatment choices. "You either give fluids or you restrict fluids," Dr. Thompson said.

Give fluids to patients who are hypovolemic, have cerebral salt wasting, or display acute mental status changes. Go slow with treatment, she added, with a goal correction of no more than 0.5 mEq/L per hour or 12 mEq/L every 24 hours. Some clinicians recommend a more conservative increase of 6 mEq/L per 24 hours, she added.

Restrict fluids to patients with SIADH or primary polydipsia. Restriction to 1L to 2L free water is generally appropriate, Dr. Thompson said.

Sodium chloride tablets and vasopressin antagonists are two additional treatment options. Consider administration of sodium chloride tablets to patients with cerebral salt wasting, Dr. Thompson said. Some clinicians are reluctant to treat with salt tablets. "I was one of the people who used to think this was a terrible thing, but it actually works in cerebral salt wasting." The tablets also are indicated as a treatment for chronic SIADH, she said.

 

 

Vasopressin antagonists can be given I.V. or oral and are considered a treatment of last resort, she added. "Save them for the severely symptomatic patient. Yes, the sodium comes up, but there are no data on better outcomes in the acute setting." At an estimated daily cost of $260-$550, "they are extremely expensive." She cautioned that patients need to be watched for rapid overcorrection with these agents.

Dr. Thompson did not have any relevant disclosures.

MIAMI BEACH – When you get a call in the hospital to evaluate a patient with postoperative hyponatremia, you need to worry primarily about three causes, according to an expert.

Increased free water intake, sodium loss that exceeds free water loss, and – "what we see all the time" – an inability to excrete free water will be your main concerns with these inpatients, Dr. Rachel E. Thompson said.

Initially rule out the most serious potential consequences of hyponatremia. "Assess mental status first to rule out increased intracranial pressure," said Dr. Thompson, founder and director of the Medicine Consult Service at Harborview Medical Center in Seattle. She spoke at a meeting on perioperative medicine sponsored by the University of Miami. "We have rigid skulls. There is very little space for tissue expansion." Left unchecked, intracranial hypertension can lead to brain damage, herniation, and respiratory arrest. "You can die from this."

It’s also important to distinguish neurology patients from others, Dr. Thompson said. Neurologic surgeons often want patients to have sodium levels above 140 mEq/L. "However, in other populations, if there is not brain damage that is going to be worsened by this, we are a lot more laissez faire. We might allow it to go lower, and people don’t get too worried about it until you’re in the mid to low 120s."

Dr. Thompson cited a neurosurgery patient as a case example. You get a call about a 66-year-old woman currently in the rehabilitation unit following endovascular coiling for aneurysmal subarachnoid hemorrhage repair two weeks earlier. The rehabilitation specialist reports her sodium is low at 126 mEq/L (it was 142 mEq/L when she left the neurosurgery unit 7 days earlier).

Dr. Thompson electronically polled meeting attendees regarding the next appropriate step. Of the total 91 responses, 33% would evaluate the patient’s volume status, 29% would evaluate mental status, 24% would check serum osmolality, and the remaining 14% would recheck the sodium level.

"I like that you want to see the volume status, and another third of you want to check the mental status. That means you are going to go see the patient. That is great," Dr. Thompson said.

Early assessment of mental status is key in Dr. Thompson’s four-step practical model for perioperative providers caring for hyponatremia patients:

• Step 1. Go to the bedside to assess mental status. Does it reflect what you expect for that sodium level? Assess acuity and severity, check which fluids are running, determine if the patient is hypovolemic, and find out if the patient had any recent vomiting or diarrhea that could facilitate volume loss.

• Step 2. Review medications. Did the patient take any diuretics or medications known to cause syndrome of inappropriate antidiuretic hormone (SIADH)? For more information on this syndrome, see (Nephron. Clin. Pract. 2011;119:c62-73).

Step 3. Order appropriate studies. You might repeat the sodium level and check serum osmolality; also measure urine osmolality and sodium. Which brings us to the final step ...

• Step 4. "The urine is going to be where your answers are," Dr. Thompson said. If the urine is dilute, think primary polydipsia or "tea and toast diet syndrome." If the urine is concentrated, causes include chronic kidney disease, thiazides, hypothyroidism, or glucocorticoid deficiency.

It is important to be thorough and closely monitor hyponatremia patients either way, Dr. Thompson said.

Hyponatremia comes with risks. In one retrospective cohort study, hospital-acquired hyponatremia was associated with increased in-hospital mortality (adjusted odds ratio, 1.66); a 64% adjusted increase in length of stay; and a greater likelihood of discharge to a short- or long-term care facility (OR, 1.64) compared to unaffected patients (Arch. Intern. Med. 2010;8:294-302). The researchers found 38% of more than 55,000 inpatients at a single center over 7 years had hospital-acquired hyponatremia, defined as a decrease in serum sodium to below 138 mEq/L following normal levels at admission.

Although management of inpatients with hyponatremia might appear complex, there are two main treatment choices. "You either give fluids or you restrict fluids," Dr. Thompson said.

Give fluids to patients who are hypovolemic, have cerebral salt wasting, or display acute mental status changes. Go slow with treatment, she added, with a goal correction of no more than 0.5 mEq/L per hour or 12 mEq/L every 24 hours. Some clinicians recommend a more conservative increase of 6 mEq/L per 24 hours, she added.

Restrict fluids to patients with SIADH or primary polydipsia. Restriction to 1L to 2L free water is generally appropriate, Dr. Thompson said.

Sodium chloride tablets and vasopressin antagonists are two additional treatment options. Consider administration of sodium chloride tablets to patients with cerebral salt wasting, Dr. Thompson said. Some clinicians are reluctant to treat with salt tablets. "I was one of the people who used to think this was a terrible thing, but it actually works in cerebral salt wasting." The tablets also are indicated as a treatment for chronic SIADH, she said.

 

 

Vasopressin antagonists can be given I.V. or oral and are considered a treatment of last resort, she added. "Save them for the severely symptomatic patient. Yes, the sodium comes up, but there are no data on better outcomes in the acute setting." At an estimated daily cost of $260-$550, "they are extremely expensive." She cautioned that patients need to be watched for rapid overcorrection with these agents.

Dr. Thompson did not have any relevant disclosures.

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Tips to Dry Up Drooling Disorders

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Tips to Dry Up Drooling Disorders

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.

Dr. Yamilet Tirado

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.

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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.

Dr. Yamilet Tirado

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.

Dr. Yamilet Tirado

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.

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Be Skeptical of Nail Fungus Treatment Products

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MIAMI BEACH – Remain skeptical about new devices or drugs that claim the ability to target fungal infections, recommended Dr. Alan B. Fleischer Jr.

"You are probably going to be barraged by devices that do something for nail fungus," Dr. Fleischer said. "As a physician, try to focus on curing the fungus and not just making them [patients] look better," he said at the South Beach Symposium.

For example, enrollment is underway for a study to assess a 1320-nm Nd:YAG device to fight onychomycosis. However, the primary outcome in the trial is "improved appearance," not fungal cure, he said. "It is confusing ... to a consumer. Improving the appearance sounds similar, but it is not the same."

Photo (c) 1993, Elsevier
"New drugs and modalities [for treating nail fungus] are continually evolving," Dr. Alan B. Fleisher Jr. said.    

When it comes to topical treatments for onychomycosis, efinaconazole is in development for treating mild to moderate forms of toenail infection, noted Dr. Fleischer. The manufacturer, Valeant Pharmaceuticals, reported promising results in two international phase III studies in December 2011. However, Valeant has not released mycology results.

"Pay attention to the cure rate when it is approved, probably sometime in the next year," said Dr. Fleischer, professor of dermatology at Wake Forest University in Winston-Salem, N.C.

Patients may ask about natural cures for fungal infections, but the data are generally unconvincing or lacking, Dr. Fleischer said. For example, some people advocate melaleuca oil from the tea tree to clear tinea pedis. However, when 104 patients were randomized to creams containing 10% melaleuca oil, 1% tolnaftate, or placebo, "melaleuca oil cleared 30% of subjects of tinea pedis. That is very low," he said (Australas. J. Dermatol. 1992;33:145-9). Of the tolnaftate-treated patients, 85% had a negative culture at the end of therapy, and 21% of placebo patients were considered cleared.

Other patients might ask about using vinegar to cure foot fungus. They may have read about this natural remedy on the Internet, where a Google search for the keywords "vinegar" and "foot fungus" yields about 116,000 results. "Although there are no data, topical vinegar immersion probably does something," Dr. Fleischer said.

"Fungal infections are still out there, but new drugs and modalities are continually evolving," he added.

Dr. Fleischer disclosed that he is a consultant for Intendis and Upsher-Smith Laboratories. He is also a researcher for Abbott, Amgen, Astellas, Galderma, GlaxoSmithKline, Intendis, and Pfizer. He receives a salary from Merz Pharma.

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MIAMI BEACH – Remain skeptical about new devices or drugs that claim the ability to target fungal infections, recommended Dr. Alan B. Fleischer Jr.

"You are probably going to be barraged by devices that do something for nail fungus," Dr. Fleischer said. "As a physician, try to focus on curing the fungus and not just making them [patients] look better," he said at the South Beach Symposium.

For example, enrollment is underway for a study to assess a 1320-nm Nd:YAG device to fight onychomycosis. However, the primary outcome in the trial is "improved appearance," not fungal cure, he said. "It is confusing ... to a consumer. Improving the appearance sounds similar, but it is not the same."

Photo (c) 1993, Elsevier
"New drugs and modalities [for treating nail fungus] are continually evolving," Dr. Alan B. Fleisher Jr. said.    

When it comes to topical treatments for onychomycosis, efinaconazole is in development for treating mild to moderate forms of toenail infection, noted Dr. Fleischer. The manufacturer, Valeant Pharmaceuticals, reported promising results in two international phase III studies in December 2011. However, Valeant has not released mycology results.

"Pay attention to the cure rate when it is approved, probably sometime in the next year," said Dr. Fleischer, professor of dermatology at Wake Forest University in Winston-Salem, N.C.

Patients may ask about natural cures for fungal infections, but the data are generally unconvincing or lacking, Dr. Fleischer said. For example, some people advocate melaleuca oil from the tea tree to clear tinea pedis. However, when 104 patients were randomized to creams containing 10% melaleuca oil, 1% tolnaftate, or placebo, "melaleuca oil cleared 30% of subjects of tinea pedis. That is very low," he said (Australas. J. Dermatol. 1992;33:145-9). Of the tolnaftate-treated patients, 85% had a negative culture at the end of therapy, and 21% of placebo patients were considered cleared.

Other patients might ask about using vinegar to cure foot fungus. They may have read about this natural remedy on the Internet, where a Google search for the keywords "vinegar" and "foot fungus" yields about 116,000 results. "Although there are no data, topical vinegar immersion probably does something," Dr. Fleischer said.

"Fungal infections are still out there, but new drugs and modalities are continually evolving," he added.

Dr. Fleischer disclosed that he is a consultant for Intendis and Upsher-Smith Laboratories. He is also a researcher for Abbott, Amgen, Astellas, Galderma, GlaxoSmithKline, Intendis, and Pfizer. He receives a salary from Merz Pharma.

MIAMI BEACH – Remain skeptical about new devices or drugs that claim the ability to target fungal infections, recommended Dr. Alan B. Fleischer Jr.

"You are probably going to be barraged by devices that do something for nail fungus," Dr. Fleischer said. "As a physician, try to focus on curing the fungus and not just making them [patients] look better," he said at the South Beach Symposium.

For example, enrollment is underway for a study to assess a 1320-nm Nd:YAG device to fight onychomycosis. However, the primary outcome in the trial is "improved appearance," not fungal cure, he said. "It is confusing ... to a consumer. Improving the appearance sounds similar, but it is not the same."

Photo (c) 1993, Elsevier
"New drugs and modalities [for treating nail fungus] are continually evolving," Dr. Alan B. Fleisher Jr. said.    

When it comes to topical treatments for onychomycosis, efinaconazole is in development for treating mild to moderate forms of toenail infection, noted Dr. Fleischer. The manufacturer, Valeant Pharmaceuticals, reported promising results in two international phase III studies in December 2011. However, Valeant has not released mycology results.

"Pay attention to the cure rate when it is approved, probably sometime in the next year," said Dr. Fleischer, professor of dermatology at Wake Forest University in Winston-Salem, N.C.

Patients may ask about natural cures for fungal infections, but the data are generally unconvincing or lacking, Dr. Fleischer said. For example, some people advocate melaleuca oil from the tea tree to clear tinea pedis. However, when 104 patients were randomized to creams containing 10% melaleuca oil, 1% tolnaftate, or placebo, "melaleuca oil cleared 30% of subjects of tinea pedis. That is very low," he said (Australas. J. Dermatol. 1992;33:145-9). Of the tolnaftate-treated patients, 85% had a negative culture at the end of therapy, and 21% of placebo patients were considered cleared.

Other patients might ask about using vinegar to cure foot fungus. They may have read about this natural remedy on the Internet, where a Google search for the keywords "vinegar" and "foot fungus" yields about 116,000 results. "Although there are no data, topical vinegar immersion probably does something," Dr. Fleischer said.

"Fungal infections are still out there, but new drugs and modalities are continually evolving," he added.

Dr. Fleischer disclosed that he is a consultant for Intendis and Upsher-Smith Laboratories. He is also a researcher for Abbott, Amgen, Astellas, Galderma, GlaxoSmithKline, Intendis, and Pfizer. He receives a salary from Merz Pharma.

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