Category Archives: Uncategorized

Short Bowel Syndrome and Risk of Eosinophilic Disease

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N Du, C Torres. JPGN 2024;78:1149–1154. Prevalence of eosinophilic gastrointestinal diseases in children with short bowel syndrome: A single center study

Methods: EoEdefined as ≥15 eosinophils per high powered field (HPF), eosinophilic gastritis (EoG) as ≥30 eosinophils per HPF, eosinophilic enteritis (EoGN) as >50 eosinophils per HPF, and eosinophilic colitis (EoC) as>80–100 eosinophils per HPF.

Key findings in this retrospective study (n=82):

  • The prevalence of eosinophilic esophagitis in our SBS cohort was10%, eosinophilic gastritis was 4.9%, and eosinophilic enteritis was 4.9%
  • SBS patients with history of allergy or atopy were more likely to have esophageal and intestinal eosinophilia on biopsy than patients without allergy
  • One patient had EoC

In their discussion, the authors speculate on the potential role for dysbiosis, possibly related to parenteral nutrition. They note that “rare SBS patients were on amino acid‐based formulas alone and almost all were exposed to food allergens around the same age as the general population.” I did not see any information about PPI use in this cohort.

My take: This report reinforces the fact that eosinophilic disorders are more frequent in SBS (see related post below). The exact role of altered diet/use of amino acid based formulas and the role of medications like PPIs in regards to the development of EGIDs remains unclear.

Related blog posts:

Dr. Benjamin Gold: 2024 Pediatric H pylori Guidelines (Part 2)

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We had a brilliant lecture given to our group by Dr. Benjamin Gold. I have had the good fortune of getting to know Ben and working alongside Ben for more than 15 years. Most readers of this blog are very familiar with Dr. Gold who is a leader in our field.

My notes below may contain errors in transcription and in omission.

Guidelines:

  • Bismuth-based quadruple therapy recommended when antimicrobial sensitivity testing (AST) is not available
  • Routine use of CLO test is NOT recommended during endoscopy
  • Routine testing for H pylori is NOT recommended for children with recurrent abdominal pain
  • Stool PCR testing is NOT recommended
  • Test for cure should be done at 6-8 weeks after completion of treatment

During endoscopy at CHOA in which H pylori is suspected, complete a microbiology form and ask for a culture to arrange for resistance testing.  Submit a sample (or multiple) in a sterile tube/cup.  Completed results will include clarithromycin sensitivity.  Additional testing for other antibiotic resistance can be requested subsequently.  Testing can be done with paraffin block as well.

Related blog posts:

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.

Dr. Benjamin Gold: 2024 Pediatric H pylori Guidelines (Part One)

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We had a brilliant lecture given to our group by Dr. Benjamin Gold. I have had the good fortune of getting to know Ben and working alongside Ben for more than 15 years. Most readers of this blog are very familiar with Dr. Gold who is a leader in our field.

My notes below may contain errors in transcription and in omission.

.Key points:

  • While H pylori prevalence has decreased, it is becoming more difficult to treat
  • Knowing if there is clarithromycin resistance in individuals with H pylori infection is most likely to impact treatment success. Metronidazole resistance can often be overcome with adequate dosing
  • H pylori is an infectious disease with GI manifestations (rather than a GI disease).  It needs to be treated as such, using tools like antimicrobial sensitivity
  • Improving water supply in endemic areas reduces reacquisition of infection
  • Transmission can occur from one generation to the next.  Dr. Gold (& coauthors) has published a study showing transmission from grandfather to mother to child using DNA fingerprinting
  • Eradication of H pylori lowers the risk of developing gastric cancer
  • Vonoprazan has been an effective part of treatment in adults. Pediatric studies are underway

Related blog posts:

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.

Pediatric Data for Ustekinumab Therapy in Crohn’s Disease

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D Turner et al. JPGN 2024; 79:315–324. Ustekinumab in paediatric patients with moderately to severely active Crohn’s disease: UniStar study long-term extension results

Dosing: “Patients were randomised 1:1 and stratified by body weight (<40 or ≥40 kg) to receive a single induction dose of lower- or higher-dose IV ustekinumab (lower dose: 3 mg/kg [<40 kg] and 130 mg [≥40 kg]; higher dose: 9 mg/kg [<40 kg] and 390 mg [≥40 kg]). Doses specified as higher were selected to deliver ustekinumab exposure comparable to a reference adult population with CD.7, 12 At Week 8, patients received a single SC maintenance dose of ustekinumab (2 mg/kg [<40 kg]; 90 mg [≥40 kg]).”

Key findings:

  • Of the 34 patients who entered the LTE, 25 patients with evaluable data completed Week 48, and 41.2% (14/34) achieved clinical remission at Week 48
  • Efficacy and PK through 1 year in ustekinumab-treated paediatric patients were comparable to those previously reported in adults. No new safety or immunogenicity signals were reported through 4 years of ustekinumab treatment.

My take (borrowed in part from authors): “Overall, long-term data support the SC dose regimens of 90 mg as maintenance therapy for the treatment of CD for a paediatric population with ≥40 kg body weight. A phase 3 study of ustekinumab (ClinicalTrials.gov Identifier: NCT04673357) is ongoing to further evaluate dose regimens for paediatric patients <40 kg and ≥40 kg.” This type of data is essential to support the use of advanced therapies like ustekinumab until they receive specific regulatory approval for children (often 8-10 years after approval in adults).

Related blog posts:

IBD Updates: SMART IBD App, SC Vedolizumab Durability, Risk Factors in Acute Severe Ulcerative Colitis

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KA Hommel et al. JPGN 2024; 78:1273–1278. Pilot and feasibility of the SMART IBD mobile app to improve self-management in pediatric inflammatory bowel disease

The Self‐Management Assistance with Recommended Treatment (SMART) IBD app –Key findings:

  • Patients rated the app quality as good and accessed the app adequately overall, with some pages being used often.
  • Medication adherence increased over the course of the study and was associated with sleep duration, mood, and stool consistency and blood content.

My take: IBD Management apps could be quite helpful, especially for teens and young adults.

S Hsiang et al. Inflammatory Bowel Diseases, Volume 30, Issue 8, August 2024, Pages 1284–1294, https://doi.org/10.1093/ibd/izad166. Safety, Effectiveness, and Treatment Persistence of Subcutaneous Vedolizumab in IBD: A Multicenter Study From the United Kingdom

Methods: IBD patients (n=563) on IV vedolizumab across 11 UK sites agreed to transition to SC injections or otherwise continued IV treatment

Key findings:

  • Data from 563 patients, demonstrated no differences in disease activity, remission rates, and quality of life between the SC and IV groups at all time points
  • Drug persistence at week 52 was similar (81.1% vs 81.2%; P = .98)

Related blog posts:

CFD Li Wai Suen, et al. Inflammatory Bowel Diseases, Volume 30, Issue 8, August 2024, Pages 1389–1405, https://doi.org/10.1093/ibd/izad183. Factors Associated With Response to Rescue Therapy in Acute Severe Ulcerative Colitis 

This systematic review identified 101 completed studies were eligible for inclusion.

Related blog posts:

ESPGHAN Eosinophilic Esophagitis Guidelines

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Yesterday was “National Dog Day.” Here’s our pooch:

J Amil-Dias et al. JPGN;79:394–437. Open Access! Diagnosis and management of eosinophilic esophagitis in children: An update from the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN)

This report makes 52 statements and 44 recommendations. Overall this is a helpful report but there are many statements and recommendations that have NO value for pediatric gastroenterologists (except for those trapped in a cave for the last 10 years). Here are a couple examples of that:

  • “ESPGHAN EGID WG recommends that pediatricians should be aware of the higher incidence of EoE in relatives.”
  • “ESPGHAN EGID WG recommends that a high index of suspicion for EoE must be maintained in children with concomitant atopic disease.”
  • “ESPGHAN EGID WG recommends the peak value of 15 eos/HPF as the cut‐off value in esophageal biopsy specimens, for the histological diagnosis of EoE in an appropriate clinical context”

Some helpful recommendations:

  • “ESPGHAN EGID WG recommends against using available allergy tests to predict dietary triggers of EoE.” This is not new information but helpful to have clearly stated in guidelines.”
  • “ESPGHAN EGID WG recommends maintenance therapy to all patients after achieving histological remission” Commentary: “There are no prospective data on the best duration of maintenance therapy in pediatric EoE…[In a large study of adults} sustained untreated combined remission was seen in only 1.3% of patients who discontinued treatment.”
  • “ESPGHAN EGID WG suggests endoscopic and histological re‐evaluation after 1‐3 years during the maintenance phase in cases of stable clinical remission”
  • “ESPGHAN EGID WG recommends that dupilumab can be used in selected cases of children over1 year old weighing >15 kg with EoE refractory to conventional treatment and in those with concomitant atopic burden with approved indications for biologics”
  • “ESPGHAN EGID WG suggests that a short course of systemic steroids be considered as an alternative to dilation in the presence of moderate to severe esophageal strictures with severe symptoms.” “Treatment with short term systemic steroids can significantly reduce the need for mechanical esophageal dilation in moderate to severe strictures associated with pediatric EoE”
  • Suggested drug dosing is noted in Table 3 (see below)

Related blog posts:

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.

NASPGHAN Dysphagia Webinar: Dr. Khalil El-Chammas, Dr. Peter Osgood, and Dr. Jose Garza

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I signed up for this webinar mainly to hear my partner Jose Garza’s presentation (who presented last), though all the speakers were good. I took a couple screenshots on my phone during the presentations. The webinar is available/archived at NASPGHAN website.

  • Dr. El-Chammas’ presentation gave a quick review on normal swallowing physiology, modalities for evaluation (eg. VSS, FEES) and showed some cool slides particularly with regard to pharyngeal manometry.
  • Dr. Osgood reviewed the etiologies/workup for dysphagia including helpful slides on esophagrams, FLIP and manometry.
Manometry typical of Type 2 Achalasia
  • Dr. Garza provided insightful information on gastric vs supragastric belching. Supragastric belching can be treated with diaphragmatic breathing and cognitive behavioral therapy. Supragastric belching has shown poor response to pharmacologic therapy. He also explained the physiology behind the inability to burp.
Important to distinguish reason for belching as this affect management
This study shows that with gastric belching the air works its way from the stomach up and with supragastric belching air is swallowed and expelled from the esophagus

My take: Our motility colleagues have some cool toys. When the treatments are as good as the toys, being a motility specialist will be even more fun.

ENTERPRET: Vedolizumab Optimization Study in Ulcerative Colitis

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V Jairath et al. Clin Gastroenterol Hepatol 2024; 22: 1077-1086. Open Access! ENTERPRET: A Randomized Controlled Trial of Vedolizumab Dose Optimization in Patients With Ulcerative Colitis Who Have Early Nonresponse

Methods: ENTERPRET was a phase 4, open-label, randomized, controlled trial (n=278) that included patients with moderate to severe UC who had high drug clearance at week 5 (serum concentration, <50 μg/mL) and nonresponse to standard vedolizumab treatment at week 6. At week 6, eligible patients were randomized 1:1 to receive standard dosing (300 mg every 8 weeks) or dose-optimized vedolizumab (600 mg at week 6, then 300 mg every 4 weeks; or 600 mg at week 6, then 600 mg every 4 weeks [based on week 5 serum concentration]). 

After an initial clinical response of 47.5% (132/275) at week 6, 108 patients were subsequently randomized into either standard (n = 53) or dose-optimized vedolizumab (n = 55). The majority (86%) with nonresponse at week 6 had high drug clearance

Key findings at week 30:

  • Endoscopic improvement: 10 patients (18.9%) with standard vedolizumab vs 8 patients (14.5%) with dose-optimized vedolizumab
  • Clinical remission: Five patients (9.4%) with standard vedolizumab vs 5 patients (9.1%) with dose-optimized vedolizumab
  • Clinical response: 17 (32.1%) with standard vedolizumab vs 17 (30.9%) patients (30.9%) with dose-optimized vedolizumab

In their discussion, the authors note the gain of clinical response of ~16% in both groups is consistent with other studies showing “8% to 16% increase in the proportion of patients who had a clinical response between 6 and 14 weeks.” Thus, duration of treatment may be more important that serum drug level.

My take (borrowed in part from authors): “In patients with early nonresponse and high drug clearance, vedolizumab dose optimization is probably not required.” At week 30, both standard dosing and dose-optimized groups had a significant response despite lack of response at 6 weeks. So, it may be best to give a longer treatment course before reaching a conclusion on vedolizumab’s efficacy.

c Mayo endoscopic subscore </= 1.
d Reduction in total Mayo score of >/=3 points and >/= 30% from baseline with an accompanying decrease in rectal bleeding subscore of >/=1 or absolute rectal subscore of </= 1

Related blog posts:

Overuse of Tongue-Tie Surgeries

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J Thomas et al. Pediatrics 2024; https://doi.org/10.1542/peds.2024-067605. Open Access! Identification and Management of Ankyloglossia and Its Effect on Breastfeeding in Infants: Clinical Report

To examine the tongue, the authors recommend the following:

  • Assessment of tongue movement and coordination with a clean, gloved finger in the mouth to test the suck reflex and to palpate the hard and soft palate
  • Appearance of lingual frenulum, including the inability of the infant to extend tongue over the lower alveolar ridge or lift tongue midway to the palate, or a heart-shaped tongue on extension

Key Recommendations:

  • Ankyloglossia is a variation of a normal oral structure. Symptomatic ankyloglossia is defined as a restrictive lingual frenulum that causes problems with breastfeeding that are not improved with lactation support. Infants with ankyloglossia and normal feeding patterns need no intervention. Frenotomy for other problems or to prevent issues such as speech articulation or obstructive sleep apnea in the future is not evidence based.18 
  • Posterior ankyloglossia is a poorly defined term, lacking agreement from experts, and should not be used as a reason to perform surgical intervention on an infant.
  • Labial and buccal frenae are normal oral structures unrelated to breastfeeding mechanics and do not require surgical intervention to improve breastfeeding. Sucking blisters are a normal finding in newborn infants, and as such, are not suggestive of pathology.
  • Suboptimal breastfeeding is a complex issue and every nursing dyad with painful or ineffective feeding should have a complete breastfeeding assessment before any treatment is offered.65,66  Here, multidisciplinary communication and management between lactation specialists, feeding therapists, surgeons, and pediatricians are paramount for the best outcome for the family.
  • Newborn infants with possible symptomatic ankyloglossia need close monitoring, support of breastfeeding while in the hospital, early postdischarge follow-up, and monitoring of weight gain in their medical home.
  • Surgical intervention for symptomatic ankyloglossia, versus laser, can reasonably be offered after other causes of breastfeeding problems have been evaluated and treated. Frenotomy may decrease maternal nipple pain.6,17,67  Although the evidence is not strong, addressing pain is important for successful continued breastfeeding.
  • Frenotomy should be performed by a trained professional, either the medical home provider or another to whom the medical home refers the patient. The performing professional should be experienced in the medical care of newborns and older infants and should maintain needed privileges for the procedure. As with any surgical procedure, before performing a frenotomy, the performing provider should take a “time out” to:
  • Obtain a signed consent
  • Discuss alternatives, risks, and benefits of the procedure
  • Discuss and provide pain control options
  • Document previous receipt of intramuscular vitamin K
  • Provide information on postsurgical care and follow-up
  • Attention to prevention of surgical complications, hemorrhage risk, pain mitigation, and evidence-based postsurgical care is recommended. Postoperative stretching exercises are not evidence-based and are not recommended.
Number of inpatient newborn ankyloglossia diagnoses in United States by year from Wei EX, Tunkel D, Boss E, Walsh J. Ankyloglossia: update on trends in diagnosis and management in the United States. 2012–2016. Otolaryngol Head Neck Surg. 2020;163(5):1029–1031.
“(A) An example of anterior ankyloglossia with the lingual frenulum attaching at the tongue tip, limiting tongue mobility. (B) Posterior attachment of lingual frenulum. In the AAO-HNS consensus statement, consensus was not reached regarding the definition of posterior ankyloglossia. Some in the consensus group would describe this figure as an example of posterior ankyloglossia if there are objective findings of restricted tongue mobility caused by the lingual frenulum”

My take: Everyday I see infants with feeding problems that were attributed to being tongue-tied who do not improve after frenotomy. Most often, ankyloglossia does not need any intervention.

Related article: NY Times 7/2/24: Pediatricians Warn Against Overuse of Tongue-Tie Surgeries “The tongue procedures, which often cost several hundred dollars, should be done only to the small fraction of infants with severely tethered tongues, the report said.”

Bloating, Belching –Bowel Sounds Podcast with Dr. Jose Garza

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A recent episode of Bowel Sounds Podcast with my colleague Dr. Jose Garza was terrific. I scribbled a few notes afterwards but I usually listen while on a ride to an outreach clinic. So, there may be errors in omission, and in transcription.

Here’s the link: buzzsprout.com/581062/15556601

Key points:

  • Toddlers frequently have aerophagia and asymptomatic bloating
  • Nitrogen gas needs to be expelled from GI tract
  • Bloating symptoms can occur with and without distention
  • Bloating without other symptoms usually does not require an extensive workup.  Bloating with diarrhea could indicate malabsorption
  • Bloating symptoms can worsen due to constipation
  • Chronic symptoms are usually reassuring; acute changes are more concerning
  • Intermittent distention is less concerning than persistent distention
  • Abdomino-phrenic dyssynergia occurs when the diaphragm pushes down on the abdomen and results in distention (see reference below)
  • Bloating symptoms frequently occur in those with only a small amount of excess air (less than 1 oz).  There is often a degree of hypersensitivity. 
  • Treatments for bloating could include diaphragmatic breathing, peppermint products, neuromodulators and sometimes diet.  Diets can result in adverse effects including eating disorders, nutrient deficiencies, and cost
  • SIBO is over diagnosed in those without risk factors
  • pH-Impedance study, read manually, is a good test for belching: differentiates aerophagia, supragastric belching, and gastric belching
  • The podcast reviewed the inability to burp due to rRetrograde cricopharyngeus dysfunction (see prior post: Jose Garza: What’s New in Motility (Part 2))

Dr. Garza also previously did a Bowel Sounds Podcast on Why Kids Poop Their Pants

Related blog posts: