Buckyball Recall –It’s Official

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From NASPGHAN twitter feed, 5/13/14: consumerfed.org/news/785

Consumer advocates and pediatric gastroenterologists applaud the Consumer Product Safety Commission’s announcement today that a settlement has been reached with Craig Zucker, the former CEO and President of the company that manufactured Buckyballs and Buckycubes, Maxfield and Oberton, which was dissolved in 2012.

High powered magnets, such as Buckyballs or Buckycubes, are bb-shaped smooth balls or cubes that connect to one another with a strong magnetic bond.  The magnets are individual balls or cubes that are sold in packages of many individual balls.  These products were originally sold as toys to children over 13 years of age, but after a recall in 2010, these products are sold to teens and adults age14 and older.  The new warning label that has appeared on the package of Buckyballs since the recall has not resulted in a decrease in serious injuries to children.  In November of 2011, CPSC issued a safety warning to consumers about this product but the injuries continue to occur.  In July of 2012, CPSC filed a law suit against the manufacturer of Buckyballs to issue a recall of these products. The settlement announced today is a resolution of that complaint…

Consumers will have six months to participate in the recall by requesting a refund.  The recall trust will be funded by Craig Zucker and will be overseen by the CPSC.

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Diarrhea -a Universal Experience

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To be clear, by the term “universal” I am in no way referring to a theme park.  A recent review in the NEJM (N Engl J Med 2014; 370:1532-1540) discusses acute diarrhea, http://nej.md/1l2bAIn .

“In the United States, there are approximately 179 million cases of acute diarrhea per year. This update on the diagnosis and management of acute diarrhea in immunocompetent adults gives particular attention to the roles of noroviruses and Clostridium difficile.”

This post is mainly to provide an up-to-date useful reference.

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.

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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.

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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.

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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.

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BRIC, PFIC, and nasobiliary drainage | gutsandgrowth