Author Archives: gutsandgrowth

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About gutsandgrowth

I am a pediatric gastroenterologist at GI Care for Kids (previously called CCDHC) in Atlanta, Georgia. The goal of my blog is to share some of my reading in my field more broadly. In addition, I wanted to provide my voice to a wide range of topics that often have inaccurate or incomplete information. Before starting this blog in 2011, I would tear out articles from journals and/or keep notes in a palm pilot. This blog helps provide an updated source of information that is easy to access and search, along with links to useful multimedia sources. I was born and raised in Chattanooga. After graduating from the University of Virginia, I attended Baylor College of Medicine. I completed residency and fellowship training at the University of Cincinnati at the Children’s Hospital Medical Center. I received funding from the National Institutes of Health for molecular biology research of the gastrointestinal tract. During my fellowship, I had the opportunity to work with some of the most amazing pediatric gastroenterologists and mentors. Some of these individuals included Mitchell Cohen, William Balistreri, James Heubi, Jorge Bezerra, Colin Rudolph, John Bucuvalas, and Michael Farrell. I am grateful for their teaching and their friendship. During my training with their help, I received a nationwide award for the best research by a GI fellow. I have authored numerous publications/presentations including original research, case reports, review articles, and textbook chapters on various pediatric gastrointestinal problems. In addition, I have been recognized by Atlanta Magazine as a "Top Doctor" in my field multiple times. Currently, I am the vice chair of the section of nutrition for the Georgia Chapter of the American Academy of Pediatrics. In addition, I am an adjunct Associate Clinical Professor of Pediatrics at Emory University School of Medicine. Other society memberships have included the North American Society for Pediatric Gastroenterology Hepatology and Nutrition (NASPGHAN), American Academy of Pediatrics, the Food Allergy Network, the American Gastroenterology Association, the American Association for the Study of Liver Diseases, and the Crohn’s and Colitis Foundation. As part of a national pediatric GI organization called NASPGHAN (and its affiliated website GIKids), I have helped develop educational materials on a wide-range of gastrointestinal and liver diseases which are used across the country. Also, I have been an invited speaker for national campaigns to improve the evaluation and treatment of gastroesophageal reflux disease, celiac disease, eosinophilic esophagitis, hepatitis C, and inflammatory bowel disease (IBD). Some information on these topics has been posted at my work website, www.gicareforkids.com, which has links to multiple other useful resources. I am fortunate to work at GI Care For Kids. Our group has 17 terrific physicians with a wide range of subspecialization, including liver diseases, feeding disorders, eosinophilic diseases, inflammatory bowel disease, cystic fibrosis, DiGeorge/22q, celiac disease, and motility disorders. Many of our physicians are recognized nationally for their achievements. Our group of physicians have worked closely together for many years. None of the physicians in our group have ever left to join other groups. I have also worked with the same nurse (Bernadette) since I moved to Atlanta in 1997. For many families, more practical matters about our office include the following: – 14 office/satellite locations – physicians who speak Spanish – cutting edge research – on-site nutritionists – on-site psychology support for abdominal pain and feeding disorders – participation in ImproveCareNow to better the outcomes for children with inflammatory bowel disease – office endoscopy suite (lower costs and easier scheduling) – office infusion center (lower costs and easier for families) – easy access to nursing advice (each physician has at least one nurse) I am married and have two sons (both adults). I like to read, walk/hike, bike, swim, and play tennis with my free time. I do not have any financial relationships with pharmaceutical companies or other financial relationships to disclose. I have helped enroll patients in industry-sponsored research studies.

The Bigger Picture -Mammography as an Example

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This week, a commentary makes a strong case for eliminating mammography (N Engl J Med 2014; 370:1965-1967):  “Abolishing Mammography Screening Programs? A View from the Swiss Medical Board”

Here’s a link from the NEJM: nej.md/1hV8q0L

What is fascinating is how ingrained mammography has become in our medical culture and how most individuals believe that mammography is so beneficial.  Take a look at the figure in the link to get a better perspective.  While women think that mammography may save 80 lives out of a thousand screened, according to the commentary, the data suggest that 1 woman may be saved.  The main problem: “for every breast-cancer death prevented in U.S. women over a 10-year course of annual screening beginning at 50 years of age, 490 to 670 women are likely to have a false positive mammogram with repeat examination; 70 to 100, an unnecessary biopsy; and 3 to 14, an overdiagnosed breast cancer that would never have become clinically apparent.”

If a well-established screening measure like mammography is not so beneficial, what else could be on the chopping block?  As noted in a previous blog post (Do you know about the “Choosing Wisely … – gutsandgrowth), even the annual physical exam has been deemed a low-value service.

Another related blog post:

“There is More to Life Than Death” | gutsandgrowth

 

The Latest on EoE and PPI-REE

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A recent study shows similar clinical, endoscopic and histologic findings between eosinophilic esophagitis (EoE) and proton pump inhibitor-responsive esophageal eosinophilia (PPI-REE) (Aliment Pharmacol There 2014; 39: 603-08 -thanks to Seth Marcus for this reference).

The authors used two databases: one from Walter Reed and one from the Swiss EoE database.  All of these patients were >/=18 years.  Response to PPI was defined as achieving less than 15 eos/hpf and a 50% decrease from baseline following at least 6-weeks of PPI treatment.

Demographics: 63 EoE patients, 40 PPI-REE, mean age 40 years (75% male, 89% Caucasian).

Findings:

  • Similar dysphagia 97% vs. 100% (in EoE and  of PPI-REE cohorts)
  • Similar food impaction 43% vs. 35% (in EoE and  of PPI-REE cohorts)
  • Similar heartburn 33% vs. 32% (in EoE and  of PPI-REE cohorts)
  • Similar duration of symptoms: 6.0 years vs 5.8 years (in EoE and  of PPI-REE cohorts)
  • Similar endoscopic findings too: rings 68% in both groups, furrows 70% in both groups, strictures 49% vs 30% (in EoE and  of PPI-REE cohorts)
  • Similar histology: proximal esophagus 39 vs 38 eos/hpf and distal esophagus 50 vs 43 eos/hpf

Take-home message: EoE and PPI-REE are very similar in presentation and indistinguishable without a PPI trial.

Related blog posts:

 

 

SEED Journal Club: FPIES

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The main topic at this month’s SEED (SouthEast Eosinophilic Disease) Center Journal club was Food Protein-Induced Enterocolitis Syndrome (FPIES) with two featured articles:

  • J Allergy Clin Immunol In Practice 2013; 1: 317-22. Review
  • J Allergy Clin Immunol In Practice 2013; 1: 343-9. Original Study

The review covers the key issues including presentation, diagnosis, differential diagnosis, outcome and management. Some of the key points:

  • FPIES is characterized by repetitive emesis (often to the point of dehydration and lethargy) and sometimes diarrhea.  It typically starts within the first 3-6 months of life.
  • Trigger foods are most commonly milk, then soy, then grains (rice cereal). FPIES in exclusively breastfed babies is extremely rare.
  • FPIES is a non-IgE mediated reaction; thus testing with skin prick tests or serum for food-specific IgE has poor utility.
  • Differential diagnosis (Table E4): gastroenteritis/food poisoning, sepsis, anaphylaxis, inborn errors of metabolism, intussception, Hirschsprung’s, necrotizing enterocolitis, and proctocolitis
  • Variable time to resolution  (Table E6): Cow’s milk resolution up to 60% resolution by 10 months, though some studies report 60% resolution at 3 years.  Soy resolution as much as 90% by 10 months of age (less in other studies).  Solids -resolution in 67% by 3 years.
  • Management: Avoid trigger foods. If supplementing breastmilk, consider hydrolyzed (or amino acid based) formula.  Conduct food challenges in supervised medical setting (often inpatient).  Acute management: Consider intravenous fluids and methylprednisolone (1 gm/kg) during bouts

2nd Article: Retrospective chart review of 462 patients with FPIES from CHOP (Philadelphia).  Inclusion criteria: “classic reaction of prolonged vomiting and diarrhea that occurred 2-6 hours after ingestion of the food.”

Key findings:

  • Diarrhea occurred in about 50%.
  • Mean age of onset was 7 months for milk or soy compared with 12 months for grains
  • 43% of patients with milk-triggered FPIES react to soy as well
  • 42% of patients with a grain trigger react to two or more grains
  • More than 85% outgrew FPIES by 5 years of age.  35% outgrew their FPIES by age 2, 70% by age 3, and 80% by age 4.

Journal club discussion:

  • It was noted in the group discussion that FPIES in adults is most often triggered by shellfish/fish and eggs.
  • FPIES does not “run” in families. Though, atopic patients have increased risk. (As an aside: If you have diarrhea, it might be genetic –it might run in your jeans.)
  • The nomenclature of FPIES is problematic.  How come only ~50% have diarrhea if this is an “Enterocolitis” disorder?
  • Typically, trigger foods would not be reintroduced for a minimum of 12-18 months after last exposure/reaction.

Take home message: FPIES is a clinical diagnosis.  Be careful with oral challenges.

Related blog posts:

Looking Beyond the Headline for Ultra-Short Bowel Syndrome

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A quick glance at a recent study (JPGN 2014; 58: 438-42) suggests a favorable outlook for patients with ultra-short bowel syndrome (U-SBS). U-SBS has been defined as having a residual small bowel length <10 cm distal to the ligament of Treitz.  A more cynical definition by a colleague years ago was that U-SBS was when patients can fart and burp at the same time.

Looking at the details:  This study enrolled 11 patients into a prospective Italian database since 2000 and examined their outcomes.  Inclusion criteria included U-SBS diagnosed in the neonatal period (<28 days) and necessitating home parenteral nutrition at discharge.

The demographics note that these patients were bigger at birth and less premature than typical series of patients with SBS:

  • Only one of the patients had necrotizing enterocolitis as the sole underlying disease and six patients had volvulus.
  • All but two had ≥50% of their colons, with five having their entire colon.
  • All but one of these patients had gestational age ≥32 weeks and only two  patients had documented birth weight less than 2300 gm.

The authors note that these patients currently receive SMOFlipid as outpatients and Omegaven as inpatients.  All patients receive some enteral feedings.  Loperamide is used selectively.

Results:

  • Inpatient hospital care ranged from 23 to 104 days/year, but had improved during the last year of followup.
  • With >5 years of followup, 2 of the 11 patients had died.  One of these patients had severe intestinal failure associated liver disease (IFALD) despite use of Omegaven.
  • One patient underwent isolated intestinal transplantation.
  • No children in this series underwent a bowel-lengthening…”given the shortness of the residual small bowel, the gain of length after any procedure will not significantly improve absorption.”

Given their results, the authors note that despite recommendations for early referral for intestinal transplantation in patients with U-SBS, this may not result in a survival benefit.  They note a study by Pironi et al (Gut 2011; 60: 17-25) that showed that among 80 intestinal transplant candidates, 5-year survival was greater in those who were not transplanted.

Bottomline: This small cohort shows that certain populations of U-SBS may do well clinically for a long time with medical management. Caution should be used in extrapolating these results to SBS patients with different demographics.

Fructose Malabsorption and Recurrent Abdominal Pain

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Even before the popularity of a low FODMAPs diet, most pediatric gastroenterologists were aware that a significant number of children would develop pain if their diet included too much high fructose corn syrup.  A recent study shows that a breath hydrogen test (BHT) for fructose malabsorption may predict patients who will improve with a low-fructose diet (JPGN 2014; 58: 498-501).  Here’s a link to the abstract: 

Design: Retrospective study reviewed a single center experience.  Fructose BHT (1 g/kg fructose to max of 25 g) was administered to 222 patients who presented with recurrent abdominal pain.  An abnormal test was defined by a breath hydrogen >20 ppm over baseline.  If positive, families met with a nutritionist for instruction on a low-fructose diet.

Key result: 121 of 222 (54.5%) had positive BHT.  Of these 121 patients, 93 (77%) reported resolution of symptoms on a low-fructose diet.  Among those with a negative BHT, 54% reported resolution of symptoms without a low-fructose diet.

Take-home message: High fructose corn syrup and fructose malabsorption can contribute to abdominal pain; though, in clinical practice, a BHT is not needed to institute a trial of a low-fructose diet.

 

A Leading Cause of Mortality in U.S….

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Being uninsured.  A recent article in Annals of Intern Medicine (2014; 160: 585-93) explored the reduction in mortality associated with expansion of Medicaid.

Key result: “Reform in Massachusetts was associated with a significant decrease in all-cause mortality compared with the control group (−2.9%; P = 0.003, or an absolute decrease of 8.2 deaths per 100 000 adults).”  The control group consisted of similar patients from other states.

An editorial (advbd.co/1lWHfsu) on this subject (from Atul Gawande retweet), notes the following: “The takeaway: Every 830 additional people who got insurance under Massachusetts’ health reforms prevented roughly one death….

A 2012 Urban Institute report estimated that 15.1 million uninsured adults could gain coverage if every state expanded Medicaid. Using the 830 figure from the Massachusetts study, and acknowledging that the state’s coverage wasn’t exactly equivalent to Medicaid, that would translate to 18,193 deaths prevented per year.

For a sense of comparison—that would make the Medicaid coverage gap the number five leading cause of preventable death in the United States”

1 “Medicaid Coverage Gap” based on 2012 estimate; other causes based on 2010 data.

Bottomline: Not having health insurance can be very bad for your health.

Related blog post:

45,000 Unnecessary Deaths Per Year | gutsandgrowth

Parental Immunity (to Education) and Vaccine Decision-Making

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A recent NY Times editorial by the lead author of a provocative study in Pediatrics (Published online March 3, 2014  (doi: 10.1542/peds.2013-2365) argues that educational efforts to inform parents may not improve vaccination rates in children.

Here’s a link: http://nyti.ms/1sq4X2s  and here’s an excerpt:

“we found that parents with mixed or negative feelings toward vaccines actually became less likely to say they would vaccinate a future child after receiving information debunking the myth that vaccines cause autism.

Surprising as this may seem, our finding is consistent with a great deal of research on how people react to their beliefs being challenged. People frequently resist information that contradicts their views, such as corrective information— for example, by bringing to mind reasons to maintain their belief — and in some cases actually end up believing it more strongly as a result….

A more promising approach would require parents to consult with their health care provider, as the Oregon law also allows them to do. Parents name their children’s doctor as their most trusted source of vaccine information. That trust might allow doctors to do what evidence alone cannot: persuade parents to protect their children as well as yours and mine.

Related blog posts:

Emerging Treatment for PFIC-2

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A great example of “bench to bedside” research was recently published (J Pediatr 2014; 164: 1219-27). While this research involved treatment of a rare condition, progressive familial intrahepatic cholestasis type 2 (PFIC2), the way the authors used a series of convincing experiments to determine the effect of a new medication shows how important a single patient can be in advancing medical treatment.  For PFIC2, the implications of this study are more direct since there are no established medications.

Previous experimental evidence has indicated that 4-phenylbutyrate (4PB), a drug used to treat ornithine transcarbamylase deficiency (OTCD), can increase the expression of the bile salt export pump (BSEP). Since BSEP, encoded by ABCB11 gene, is defective in PFIC2, the authors sought to determine whether 4PB would be effective for patients with PFIC2 who showed a reduced (but not absent) BSEP expression.

They identified a jaundiced infant female at 2 months of age with normal GGT who was diagnosed with PFIC2 due to the presence of the c.3692G>A (p.R1231Q) mutation in both alleles of ABCB11.

The authors then treated this infant with 200 mg/kg/day (into 4 doses a day) with 4PB; gradually the dosage was increased to 500 mg/kg/day.  The authors performed elegant in vitro studies from genomic DNA from peripheral leukocytes along with histologic studies from liver biopsy specimens.

Key Findings:

At the 500 mg/kg/day dosage, BSEP expression at the canalicular membrane was partially restored and this coincided with improved liver tests, improved liver histology, and relief of pruritus.

Conclusion: 4PB retards degradation of the canalicular BSEP which resulted in biochemical and histologic improvement.  This study involved only one patient; thus, further studies will be needed.

Related blog post:

BRIC, PFIC, and nasobiliary drainage | gutsandgrowth

Questions and Answers about MERS

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Recently two cases of Middle Eastern Respiratory Syndrome (MERS) have been reported in the U.S.  For more information -the following link (thx to Mike Hart) may be useful:  www.cdc.gov/coronavirus/MERS/index.html<http://www.cdc.gov/coronavirus/MERS/index.html

From the CDC: Middle East Respiratory Syndrome (MERS) is viral respiratory illness first reported in Saudi Arabia in 2012. It is caused by a coronavirus called MERS-CoV. Most people who have been confirmed to have MERS-CoV infection developed severe acute respiratory illness. They had fever, cough, and shortness of breath. About 30% of these people died.

So far, all the cases have been linked to countries in the Arabian Peninsula. This virus has spread from ill people to others through close contact, such as caring for or living with an infected person. However, there is no evidence of sustained spreading in community settings.

Therapeutic Inertia in U.S Neonatal Units (vis-a-vis Probiotics)

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“More than 90% of very low birth weight (VLBW) infants receive substandard care” could be the headline of a recent article/editorial (J Pediatr 2014; 164: 980-5 & 959-60).  Instead they are titled: “Cohort Study of Probiotics in a North American Neonatal Intensive Care Unit” and “Probiotic Supplementation in Preterm Infants: It is Time to Change Practice.”

In the article introduction, the authors state: “In 2011, faced with overwhelming evidence that probiotics could decrease NEC in preterm infants, and because there were no significant risks described in the extensive literature, we decided to introduce probiotics as routine care for the prevention of NEC.”

Methods: Prospective cohort study of infants at a single center NICU.  Examined rates of necrotizing enterocolitis (NEC) and death for 17 months before and after introduction of a probiotic (FloraBABY).  This probiotic (0.5 g) was mixed with water and administered just before milk once a day.  It was started at the first feeding and continued until the infant reached 34 weeks postmenstrual age.

Key findings:

  • Probiotics reduced NEC from 9.8% to 5.4% (OR for NEC 0.51)
  • Probiotics reduced combined outcome for NEC or death from 17% to 10.5% (OR 0.56).  Reduction in death by itself did not meet statistical significance.

Why, in 2012, were probiotics only used in 8-9% of VLBW?

Potential profits for probiotics are small which has limited studies of specific strains.  The probiotic, FloraBABY, in this study cost 11 cents per day in amount used; however, since the probiotic came in a tub, the actual cost was $12.79 for a 60-g tub for each patient.  Thus, manufacturers are unlikely to support studies to garner FDA approval.

Yet, there have been 22 randomized controlled trials published which “showed substantial benefits of probiotics and no adverse events.” A recently completed ProPrems trial (Jacob S et al, presented at 2013 PAS Annual Meeting) used a probiotic called ABC Dophilus Probiotic Powder for Infants.  This trial showed “a significant, >50%, reduction in NEC despite an incidence in their control patients of only 4.4%” and despite the fact that >95% of infants received breast milk.

“Good quality control and confirmation of the contents of the preparation are essential…There seems to be no further reason to delay the introduction of this evidence-based therapy in the NICU.”  The adoption of probiotics could avoid 2500 cases of NEC every year in North America.

The editorial notes that the evidence for probiotics is much better than many other therapies used in NICUs.  They note that some have argued that “the evidence that probiotics reduce mortality rates is as conclusive as that for surfactant for respiratory distress syndrome.”  A recent Cochrane review of 17 trials and >4900 VLBW infants showed that the RR of severe NEC for probiotics versus control was 0.41.

If people really understood this issue, there would be outrage over this issue.  In the U.S., there was recently extensive coverage over inaction about a faulty ignition switch which has been linked to at least 13 deaths.  The potential reduction in NEC and deaths with probiotics is likely much greater.

While the editorial recommends involving parent representative groups, I recommend discussing this issue with your neonatology colleagues along with your “quality care” team to find out what they are going to do about it.  Given the enormous costs in most NICUs, it is likely that each unit could self-fund a quality project (with consented patients) to provide probiotics to this vulnerable population.

Bottomline: Probiotics have excellent evidence as prophylaxis for NEC in VLBW infants.  Physicians need to advocate for their usage to “avoid years of therapeutic inertia.”

Related blog post: One More Day Syndrome & Necrotizing Enterocolitis …

Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.