Category Archives: Pediatric Gastroenterology Intestinal Disorder

How Effective are Stents for Anastomotic Esophageal Strictures in Patients with Esophageal Atresia

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O Baghdadi et al. JPGN 2022; 74: 221-226. Predictors and Outcomes of Fully Covered Stent Treatment for Anastomotic Esophageal Strictures in Esophageal Atresia

This retrospective review of 45 patients (n=92 stents) examine the effectiveness of esophageal stenting in patients with esophageal atresia (EA). All patients had multiple dilatations prior to stenting and/or had a stricture diameter that rapidly narrowed within 2 weeks of dilatation to a diameter that was the same or smaller to predilatation. Patients were observed in the hospital after stent placement and outpatient management was considered if oral nutrition was tolerated for at least 3 days.

Key findings:

  • According to the authors, the stents prevented surgical resection in 41% of patients
  • ΔD (change in diameter) of ≤4 mm (area under the curve = 0.790; 95% confidence interval: 0.655–0.924; P < 0.001) was the optimal cutoff point in differentiating stent success. If the change in diameter decreases by 4 mm or more after stent removal at endoscopic followup, it is likely that stricture contains significant scar tissue and is not amenable to dilatation/stenting.
  • Median stent duration was 11.5 days
  • The most common adverse events were erosions/ulcerations (29%), granulation tissue formation (27%), vomiting/retching (26%) and stent migration (9%). Three stents were complicated by an esophageal leak (treated medically).

My take: Complications were frequent; thus, stenting for refractory strictures requires highly-specialized technical expertise.

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Azalea trail, near the Chattahoochee River (Atlanta)

Menetrier Disease in a Pediatric Patient

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During my training, we reported a case of Menetrier’s disease in a pediatric patient associated with CMV infection (Open Access PDF: Diagnosis of Cytomegalovirus Infection in Pediatric Menetrier’s Disease by In Situ Hybridization).

Recently, JPGN’s twitter feed shared the images below from a recent report (SJ Pathak et al. JPGN January 2022 – Volume 74 – Issue 1 – p e16doi: 10.1097/MPG.0000000000003276. Menetrier Disease in a Pediatric Patient With Ulcerative Colitis and Primary Sclerosing Cholangitis)

From JPGN Twitter Feed

Related blog post: Image Only: Pediatric Menetrier’s Disease

Vedolizumab for Refractory Microscopic Colitis, Plus, Vedolizumab and Serious Infections

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LC Shipley et al. Clin Gastroenterol Hepatol 2022; 20: 455-457. Vedolizumab Therapy in Refractory Microscopic Colitis: A Single Center Case Series

In this report, the authors describe nine patients with refractory microscopic colitis (median age 55 years) who were treated with vedolizumab.

Key findings:

  • Clinical response with induction in 9 (100%); time to >50% response ranged from 1 to 7 weeks with 5 patients responding within 2 weeks.
  • Sustained response with maintenance therapy in 6 (67%); duration of follow-up ranged from 1 month to 15 months. The three patients without response had symptom duration of 10 yrs, 12 yrs, and 25 yrs prior to institution of vedolizumab.
  • Only two patients had histologic follow-up. While both had clinical response, the patient with lymphocytic colitis had histologic resolution whereas a patient with collagenous colitis had histologic persistent.

My take: Given vedolizumab’s favorable safety profile, further studies (with endoscopic endpoints) of vedolizumab are needed to define its efficacy for microscopic colitis.

Another study with vedolizumab: J Kirchgesner et al. Clin Gastroenterol Hepatol 2022; 20: 314-324. Risk of Serious Infections With Vedolizumab Versus Tumor Necrosis Factor Antagonists in Patients With Inflammatory Bowel Disease

Key finding: The risk of serious infections was not different between vedolizumab and anti-TNF in the overall IBD cohort (HR, 0.95; 95% CI, 0·79-1.13), while the risk was decreased for vedolizumab users in patients with UC (HR, 0.68; 95% CI, 0.50-0.93), but not CD (HR, 1.10; 95% CI, 0.87-1.38)

Related blog post/related article:

Disclaimer: This blog, gutsandgrowth, assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  Because of rapid advances in the medical sciences, the gutsandgrowth blog cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure.  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.

“Separating Fact from Fiction in the Diagnosis and Management of Food Allergy”

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This great article/commentary is available as open access: Separating Fact from Fiction in the Diagnosis and Management of Food Allergy EM Abrams et al. J Pediatr 2022; 241: 221-228.

Key points:

  • “The risk of fatal anaphylaxis is about 1 in 10 million (about equivalent to being struck by lightning)..and fatality on first ingestion of an allergen in infancy has never been described”
  • Food allergy causes considerable morbidity/worsens quality of life (QOL): bullying, meal preparation, stress, social activities, and school attendance (with 10% choosing home schooling because of a food allergy). Some of this detriment on QOL is due to fear “that accidental exposure could result in fatal anaphylaxis”
  • “The impact of peanut allergy on QoL has been noted to be significantly worse than the impact of other chronic childhood diseases such as rheumatologic disease and type 1 diabetes mellitus”
  • Uriticaria does not equal allergy. “Although urticaria is commonly equated with a food allergy, food allergy accounts for less than 10% of the causes of all acute urticaria and is not a cause of chronic urticaria.” Urticaria can be triggered by an acute infection (most common etiology)
  • “Food proteins are digested within several hours; therefore, urticaria associated with a food ingestion will occur within minutes to 2 hours of ingestion, but typically last no more than a few hours (and less with treatment)” (an exception is meat anaphylaxis owing to galactose-α-1,3-galactose allergy)
  • Other common symptoms that are often incorrectly attributed to food allergy include headaches, chronic behavioral symptoms, or chronic nonspecific abdominal pain
  • Allergy testing: “the presence of a food-specific IgE is not itself indicative of an allergy…Although both skin prick testing and food-specific IgE testing are highly sensitive (>90% for skin prick tests, 70%-90% for sIgE), the specificity and positive predictive values of testing are often very low. The rate of false-positive tests is up to 40%.” 
  • Broad “panel testing” (eg, to a panel of “common” foods), or any food allergy diagnostic testing in the absence of a convincing clinical history has high potential to overdiagnose food allergy and result in unnecessary food avoidances
  • Don’t do IgG testing. “There is no evidence that IgG testing marks food sensitivity; in fact, the presence of IgG is both expected and is a potential indicator of ongoing tolerance”
  • For first-line management anaphylaxis, (intramuscular) epinephrine is the “only life-saving intervention available.” Antihistamines are useful only in the treatment of cutaneous symptoms/urticaria.
  • “Although oral corticosteroids are often administered in the setting of anaphylaxis, there is no convincing evidence that their use prevents a biphasic reaction…their routine use is not recommended” (for anaphylaxis)

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Siesta Beach, FL (2021)

Too Good To Be True: Two Lessons For Eosinophilic Esophagitis

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LE Irastorza et al. JPGN 2022; 74: 267-271. Eosinophil-Derived Neurotoxin Predicts Response to Proton-Pump Inhibitor Treatment in Pediatric Eosinophilic Esophagitis

In this prospective study, the authors compared Eosinophil-Derived Neurotoxin (EDN) levels in pediatric patients with eosinophilic esophagitis (EoE) who responded PPIs (n=15) to those who did not respond to PPIs (n=21). The publication states that EDN levels of 10 mcg/mL or greater are diagnostic for EoE (sensitivity 97%, specificity 89%) but EDN levels have not previously been studied as a marker for PPI responsiveness.

Key finding: EDN concentration was significantly higher in the PPI-nonresponsive group than in the PPI-responsive group (219.1 ± 229 mcg/mL vs 75.7 ± 60 mcg/mL, respectively, P = 0.036).

However, Figures 1 (see below) and 2 show that EDN levels while generally higher in those who did not respond to PPIs are not likely to help much at all in predicting who will respond to PPIs, mainly due to a lot of overlap in the levels. While very elevated levels (above ~300 mcg/mL) all occurred in PPI non-responders, this only accounted for 5 patients out of 36 in the entire cohort.

My take: This article’s title is quite misleading. EDN levels are generally higher in PPI-nonresponders but they do not predict response.

Figure 1

AA Wenzel et al. JPGN 2022; 74: e31-e34. Continued Basal Zone Expansion After Resolution of Eosinophilia in a Child With Eosinophilic Esophagitis on Benralizumab

This case report examined the effect of benralizumab, a monoclonal antibody against the interleukin-5 receptor (IL5Rα) on eosinophils in 20 year old with asthma and EoE. Histology was notable for resolution of esophageal eosinophilia but demonstrated marked basal zone hyperplasia (BZH) in association with high numbers of CD3+ T cells and tryptase+ mast cells. Subsequently, she improved with the institution of dupilumab with resolution of BZH and mast cell inflammation with significant reduction in T cells.

My take: Even with resolution of eosinophilia, mast cells and T cells appear to be capable of coordinating mucosal inflammation and symptoms of EoE (at least in some patients). This study mirrors my limited experience, in which patients receiving benralizumab had a grossly abnormal-appearing esophagus but resolution of the eosinophils.

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Mitigation Efforts for Button Batteries

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EM Sinclair et al. JPGN 2022; 74: 236-243. This retrospective study (n=63) describes the increased utilization of cross-sectional imaging, adoption of acetic acid irrigations, increased intensive care/hospitalizations after the implementation of consensus institutional guidelines for button battery management (see visual abstract below). An estimate in the increase in costs would have been a good addition to this study.

One of the references (EM Sinclair et al. J Pediatr Surg Case Rep 2021; 66: 101782. doi: 10.1016/j.epsc.2021.101782. Open Access: Development and repair of aorto-esophageal fistula following esophageal button battery impaction: A case report) describes one of the goals of prolonged hospitalization, namely preventing catastrophic bleeding. In this case report, though, the 6 yo had been discharged 12 days after presentation and represented on day 25 with hematemesis from a new aorto-esophageal fistula, requiring emergent cardiac catheterization with successful, life-saving aortic stent placement; “however a multidisciplinary approach to procedure planning is necessary with availability of surgical support for open repair if necessary.” This report has a lot of good images. The discussion notes that the National Capital Poison Center (NCPC) database reports a total 64 deaths in children following button battery ingestion worldwide since 1977; 61% (39/64) of which were due to documented arterio-esophageal fistulae (the actual numbers of deaths is likely much higher). This report also highlights the fact that serial MRIs “may not predict the development of severe complications.”

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Trends in Pediatric IBD Epidemiology & More on Formula Recall

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E Kuenzig et al. Gastroenterology 2022; DOI:https://doi.org/10.1053/j.gastro.2021.12.282. Open Access: Twenty-first Century Trends in the Global Epidemiology of Pediatric-Onset Inflammatory Bowel Disease: Systematic Review

Key finding:  Among studies evaluating trends over time, most (31 of 37, 84%) studies reported significant increases in incidence and all (7 of 7) reported significant increases in prevalence

App Website: The Global Epidemiology of Pediatric Inflammatory Bowel Disease

Yesterday, this blog noted the recall of several formulas (FDA Warns Consumers Not to Use Certain Powdered Infant Formula Produced in Abbott Nutrition’s Facility in Sturgis, Michigan). Our office has made a substitution table for the Abbott formulas:

Maintenance Topical Steroid Dosing for Eosinophilic Esophagitis

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T Greuter et al. Clin Gastroenterol Hepatol 2021; 19: 2514-2523. Open Access: Effectiveness and Safety of High- vs Low-Dose Swallowed Topical Steroids for Maintenance Treatment of Eosinophilic Esophagitis: A Multicenter Observational Study

In this multicenter, retrospective study with 82 participants (mean age 37 years), the authors examined swallowed topical corticosteroids (STC) for maintenance of histologic remission (<15 eos/hpf). Low dose STC (22 budesonide, 60 fluticasone) was considered 0.5 mg/day or less. Key findings:

  • Histological relapse occurred in 67% of patients. This rate was comparable in patients treated with low-dose (72%) and high-dose (54%) STCs.
  • Histological relapse occurred significantly earlier with low dose STC (1.0 vs 1.8 years, P = .030)
  • Esophageal candidiasis was identified in 6% of subjects

The authors conclude that most of the histological relapse that occurred was due to true steroid failure since “low adherence and treatment cessation during follow-up were exclusion criteria.” Also, they note that “the recently finished but not yet fully published Maintenance of Remission With Budesonide Orodispersible Tablets vs Placebo in Eosinophilic Eosphagitis (EOS2 trial) (NCT02493335) comparing budesonide maintenance doses of 2 mg/d vs 1 mg/d suggest that there is no additional benefit of daily doses higher than 1 mg (1-year remission rates of 75.0% and 73.5%, respectively).

My take: Low dose STCs do not appear to be as effective in maintaining histologic remission; however, there is a high rate of relapse even in those with higher doses.

Related blog posts:

Kaplan Meier curve for time to histological relapse in patients with deep
histological remission at baseline stratified by steroid dose groups

Alcohol Burden in Hepatology

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As an outside observer, I wonder how practitioners in the field of adult hepatology feel about the changing epidemiology of severe liver disease.

Case (article) in point: G Cholankeril et al. Hepatology 2021; 74: 3316-3329. Open Access: Impact of COVID-19 Pandemic on Liver Transplantation and Alcohol-Associated Liver Disease in the USA

This retrospective study utilized UNOS adult data from 6/1/19 to 3/1/21. This included 9528 in the pre-COVID era and 9259 in the COVID era.

Key findings:

  • There was “a significant reduction in the monthly listing rates for HCV (−21.69%, P < 0.001) and NASH (−13.18%; P < 0.001).” However, there “was a significant increase in ALD [alcohol-associated liver disease] listing (+7.26%; P < 0.001) and LT (10.67%; P < 0.001) during the pandemic.”
  • “In the COVID era, ALD (40.1%) accounted for more listings than those due to HCV (12.4%) and NASH (23.4%) combined. The greatest increase in ALD occurred in young adults (+33%) and patients with severe alcohol-associated hepatitis (+50%).”
  • Interestingly, “patients with ALD had a 50% higher probability rate of LT [liver transplantation] than patients with other liver disease.”

The authors note that patients with alcohol use disorder (AUD) and ALD, during the pandemic, “may no longer have structured non-alcohol-related activities and in-person behavioral counseling…coupled with the delay in routine health care…Few patients with ALD receive recommended care for AUD.”

My take: Due to the cumulative effects of ALD, there is likely to continued (worsening) high rates of liver failure due to ALD. Given the difficulties in managing ALD, aside from managing liver complications, this must be a huge emotional burden for many healthcare providers watching this tragedy play out on a continual basis.

Cumulative incidence rates for LT among patients listed for ALD and non-ALD
in the pre-COVID and COVID eras.

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Epidemiology of Gastroparesis in Adults

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Briefly noted: Y Ye et al. Gastroenterol 2022; 162: 109-121. Open Access: Epidemiology, Etiology, and Treatment of Gastroparesis: Real-World Evidence From a Large US National Claims Database

Key findings:

  • The overall standardized prevalence of gastroparesis was 267.7 per 100,000 US adults, whereas prevalence of “definite” gastroparesis (individuals diagnosed within 3 months of gastric emptying scintigraphy testing with persistent symptoms for more than 3 months) was 21.5 per 100,000
  • Etiology was most commonly due to diabetes (57.4%; type 1, 5.7% and type 2, 51.7%), followed by postsurgical (15.0%), drug-induced (11.8%), and idiopathic (11.3%) etiologies

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