Declining Role of Fundoplication in Esophageal Atresia, Too

A recent study (SA Pellegrino et al. J Pediatr 2018; 198: 60-6) examines the likelihood of redo fundoplication for esophageal atresia (EA) in comparison to redo fundoplication for other indications.

Key findings:

  • Among all EA cases, n=344 (1994-2013), 85 underwent fundoplication (single-center study from Melbourne)
  • Rates of fundoplication were declining over study period: there was a 37% drop from 2010-2013 compared to 1994-97.
  • Overall, 767 patients had a fundoplication (n=682 without EA).  The rates of redo fundoplication were similar 11/85 compared with 53/682, despite the fact that EA patients had earlier surgery with median ages of 7.2 months versus 23 months, respectively.

Factors leading to fewer fundoplications:

  • Improving medical therapies, including proton pump inhibitors and use of jejunal feedings
  • Awareness that fundoplication may not be curative and is associated with significant morbidity

Anecdotally, I have had some EA patients whose lives were transformed positively by fundoplication, though many are difficult operations due to anatomic factors like small gastric volumes and pulmonary issues.  Careful selection and surgical expertise are essential to good outcomes.

My take: The authors note that “this study challenges the assertion that fundoplicaiton is less successful in patients with EA.”

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Gibbs Gardens

Costs/Yield of Diagnosing Cyclic Vomiting Syndrome

A recent retrospective study (CJ Lucia-Casadonte et al. JPGN 2018; 67: 13-17) examined the costs and yield of testing for Cyclic Vomiting Sydrome (CVS).

As a bonus –this is a study with CME available (& ABP MOC): NASPGHAN-JPGN CME The full text can be obtained at CME website.

This study looked at 503 charts from a single center using ICD-9 coding to identify patients. In this group, 165 (33%) had a diagnosis of CVS with 135 of this group (82%) meeting NASPGHAN diagnostic criteria with a mean age of 7.7 years.

Key findings:

  • 6 (4%) had a change in management based on CVS evaluation
  • The mean cost for screening was $6125.02 per patient
  • Atypical symptoms included bilious emesis in 9 (7%), abdominal pain in 67 (50%), attacks precipitated by fasting 1 (0.7%), and neurologic abnormalities in 3 (2%).
  • Brain MRI was performed in 68 patients and 10 were considered abnormal; though, only 1 (0.7%) had a change in management related to increased intracranial pressure (this patient had hx/o hydrocephalus). Other findings included Chiari I malformation, cerebral cyst, macrocephaly, and abnormal myelination pattern.
  • Other underlying diagnosis: UPJ obstruction (n=1), unspecified metabolic condition with carnitine deficiency (n=1), and eosinophilic esophagitis.
  • Given the costs involved, the authors reiterate NASPGHAN recommendations to avoid a ‘shotgun’ approach and note that it has previously been shown that “the most cost effective therapy in the management of CVS to be UGI with small bowel follow through (SBFT) with empiric treatment.”  Additional evaluation would be indicated for those with red flags and/or progressive or a changing pattern of vomiting episodes.
  • The authors indicate that endoscopy was the most costly evaluation tool at their institution ($11,500) and only used in 36 patients and was considered to have a low yield.

My take: This study underscores the low yield and expense involved in the evaluation of pediatric CVS; yet, it remains difficult to balance this with the concern of overlooking some anatomic and metabolic problems which can benefit from a timely diagnosis.

Related blog post:

Gibbs Gardens 2018

 

Common Sense Media Web Site

“Common sense is not so common.” Voltaire,, Dictionnaire Philosophique 1764

A website that I learned about recently from the Journal of Pediatrics article, “The Elephant in the Examination Room: Addressing Parent and Child Mobile Device Use as a Teachable Moment:”  commonsensemedia.org

“Common Sense is the nation’s leading nonprofit organization dedicated to improving the lives of kids and families by providing the trustworthy information, education, and independent voice they need to thrive in the 21st century.”
This website has extensive resources for families regarding all forms of media.  This includes advice on apps, age for using smartphones, encouragement for device-free dinners, movie/TV reviews and more.
The AAP also has a media use plan tool: www.healthychildren.org/mediauseplan based on children’s ages.

Dietary Patterns in First Year of Life May Increase Risk of Celiac Autoimmunity

M Barroso et al. Gastroenterol 2018; 154: 2087-96.

Background: “Western-like diets –mainly characterized by high intake of red and processed meats, refined grains, simple sugars, and saturated fats and low intake of fruits, vegetables, and whole grains– have been associated with low-grade chronic inflammation, which is involved in the etiology of inflammatory conditions.” Ref: Br J Nutr 2015; 114: 999-1012.

To examine how diet may influence the development of celiac autoimmunity, defined by TG2A positivity, the authors examined a subset of patients (n=1997) from the prospective Generation R study (Netherlands); 27 in this cohort developed celiac autoimmunity (1.4%).

Key finding:

  • Higher adherence to a “prudent” diet which had a higher intake of vegetables, vegetable oils, pasta, and grains and low consumption of refined cereals and sweet beverages at 1 year of age was associated with a lower odds of celiac autoimmunity at 6 years of age with an odds ratio of 0.67.

This study is limited by the relatively low number who had celiac autoimmunity and by its use of a food questionnaire.

My take: This study indicates that diet plays a role in the development of celiac along with other disease, but this likely involves a complex mix of components rather than a single toxic agent.

Related blog posts:

How Many Eosinophils Indicate Eosinophilic Gastroenteritis or Colitis?

A recent study (Z Kiss et al. JPGN 2018; 67: 6-12) provides more data on normative values for eosinophil counts in the GI tract.  For their report, the authors reviewed 3 databases for a systematic search of the literature. They screened 1316 abstracts but found only 8 articles with complete/relevant data.  Among these 8 articles, data regarding each segment of the GI tract was present in as few as 3 articles and as many as 6 articles. The authors provide confidence intervals (CIs) and prediction intervals (PIs); the latter account for the wider uncertainty due to insufficient data.

Key points:

Normal eosinophil cell number per high-power field (HPF area = 0.2 mm squared):

  • Duodenum 8.26 with CI 4.71-11.8 and PI of 0 to 20.57
  • Terminal ileum 11.52 with CI 7.21-15.83 and PI of 0 to 60.64
  • Cecum 14.12 with CI 9.05-19.19 and PI of 0 to 38.64
  • Ascending colon 13.25 with CI 8.65-17.86 and PI of 0 to 35.42
  • Transverse colon 11.52 with CI 7.80-15.23 and PI of 0 to 25.85
  • Descending colon 10.32 with CI 7.22-13.42 and PI of 0 to 49.10
  • Sigmoid colon 8.80 with CI 6.82-10.77 and PI of 0 to 32.49
  • Rectum 7.39 with CI 4.20-10.59 and PI of 0 to 22.33

Other points:

  • The authors note that eos/HPFis a flawed measurement due to technical parameters of the microscope.  Some HPFs are bigger than others –this could affect eosinophil count up to 5-fold.  The authors specify an HPF to be =0.2 mm squared.
  • Obtaining appropriate mucosal samples for normal number of eosinophil counts can be difficult.  Even patients with functional disorders like irritable bowel syndrome and nonulcer dyspepsia could have abnormal numbers of eosinophils.

My take: These numbers of expected eosinophil counts for pediatric histology are a good starting point.  The prediction intervals remain large due to insufficient data.

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Gibbs Gardens

Pediatric NAFLD: You Don’t Have to be Obese/Overweight to Have Fatty Liver Disease (but it helps)

A recent study (P Kumar et al. JPGN 2018; 67: 75-9) examined suspected NAFLD in 12 to 18 year olds using data from NHANES. In the analysed cohort, there were 124 suspected NAFLD and 1385 without suspicion of NAFLD.  This subset was weight to represent a U.S. population of over 18 million.

Key definitions:

  • Suspected NAFLD was defined by abnormal ALT (>25.8 U/L for boys and >22.1 U/L for girls) who did not have another explanation (eg. viral hepatitis, medication)
  • Lean BMI was defined by BMI less than 85th% for age
  • Hypertriglyceridemia ≥ 150
  • Low HDL ≤ 40 mg/dL
  • HOMA-IR =fasting glucose x insulin (microU/mL) divided by 405. Insulin resistance was defined as HOMA-IR ≥ 3

Key findings:

  • Suspected NAFLD affects ~8% of lean adolescents in the U.S.
  • Hypertriglyceridemia was noted in 10 of 124 suspected NAFLD and was a risk factor (P=0.028) as was Low HDL which occurred in 15 (P=0.016) and IR which occurred in 43 (P=0.053)

My take: Elevated ALT, a marker for fatty liver disease, is common even in adolescents without obesity. Elevated triglycerides, low HDL, and insulin resistance are all risk factors for suspected NAFLD in non-overweight/non-obese teens.

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Cumberland Island 2018

MRE Does Not Fare Well at Detecting Lesions Evident on Upper Endoscopy

A recent study (PC Church et al. JPGN 2018; 67: 53-8) examined how well EGD findings were detected by MRE in 188 children (mean age 14 years).

Key findings:

  • EGD was macroscopically abnormal in 93 (49%) with ulcerations being the most common abnormality in 66 (35%).
  • In contrast, the local radiologist identified UGI inflammation in 7 (4%) and the central radiologists identied UGI inflammation in 20 (22%).  “There was no agreement between local and central radiologists when examining the UGI as a whole (κ=-0.02, P-0.59)”
  • The local radiologists “correctly identified only 5 of 93 (8%) patients with UGI findings on EGD.”  The central radiologists “correctly identified 9 of 45 (30%) patients with UGI findings on EGD.”

The authors state that “the Porto criteria mandate the performance of EGD for all pediatric patients suspected of having IBD. Our study has demonstrated that MRE cannot be relied upon as the sole method of evaluating the UGI.”

My take: For those who take care of children with IBD, this study will not come as a surprise as many of the UGI findings (found at endoscopy) are subtle.  This study does quantify the much higher sensitivity of endoscopic evaluation and is similar to studies that have compared capsule endoscopy to MRE.

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Cumberland Island 2018

Pediatric Pancreatitis -Working Group Nutritional Recommendations

Abstract Link: Nutritional Considerations in Pediatric Pancreatitis: A Position Paper from the NASPHAN Pancreas Committee and ESPHAN Cystic Fibrosis/Pancreas Working Group.

M Abu-El-Haija et al. JPGN 2018; 67: 131-43.  This working group made ~27 recommendations (summarized in Table 1) and indicated the quality of evidence supporting the recommendation as well as the agreement among team members –virtually all received at least 12 of 13 votes.

Here are the ones that grabbed my attention:

For Acute Pancreatitis (AP):

  • 1a & 1aa. Children with mild AP should be started on a regular diet –preferably via mouth as compared to nasogastric route
  • 1b. Enteral nutrition (EN) should be attempted in children with severe AP within 72 hours from presentation, once deemed hemodynamically stable.
  • 1.4 Even in severe AP, jejunal tube feeding should be reserved for those unable to tolerate oral or NG tube feeding

For Acute Recurrent Pancreatitis (ARP):

  • 2.1a & 2.1b. Children should receive a regular-fat diet in between bouts of ARP and a regular-fat diet can safely be started within 1 week after the onset of a bout of AP (except in those with very elevated triglycerids (>1000 mg/dL)
  • 2.2a & 2.3a. PERT is NOT recommended in children with ARP without eocrine pancreatic insufficiency (EPI). Antioxidants are NOT recommended (insufficient supporting evidence)

For Chronic Pancreatitis (CP):

  • 3.1b & 3.12a. Recommends routine followup every 3-6 months and a regular diet
  • 3.3a, 3.4a, & 3.5a Monitoring: recommends checking fat-soluble vitamin levels every 6 to 12 months, checking for EPI with elastase (or 72 hr fecal fat) every 6-12 months, and BMD (bone mineral density) if CP and malnutrition (especially if Vit D deficiency or hx/o fractures)

My take: This report provides a methodical approach for the care of children with these pancreatic disorders.

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Tide pools and wide beach at Cumberland Island 2018

Global Prevalence of Celiac Disease

Briefly noted: P Singh et al. Clin Gastroenterol Hepatol 2018; 16: 823-36. After a systemic review which selected 96 articles from a pool of 3843 published between 1991 through 2016, the authors determined a pooled global prevalence of 1.4% in 275,818 individuals based on seroprevalence (positive TTG or EMA).  Biopsy-confirmed celiac disease was noted in 0.7% in 138,792 individuals.

In their study, biopsy-proven disease was most prevalent in Argentina, Egypt, Hungary, Finland, Sweden, New Zealand, and India.

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