Category Archives: Uncategorized

How Reliable is a Motilist in Interpreting Manometry and FLIP Studies?

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Correction: Yesterday’s post was updated after an astute observation from one of my colleagues (Jordan) to note that the pictured instrument was in fact a harpsichord rather than a piano. A harpsichord’s sound is derived from plucking a string whereas a piano’s sound comes after a hammer strikes a chord.

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JW Chen et al. AJG 2023; 118: 1334-1343. Interrater Reliability of Functional Lumen Imaging Probe Panometry and High-Resolution Manometry for the Assessment of Esophageal Motility Disorders

Thanks to Ben Gold for this reference. Also, congratulations to Jose Garza -our motility specialist and a coauthor of this study.

15 motility specialists completed their interpretation of 40 consecutive HRM (high resolution manometry) and 40 FLIP (functional lumen imaging probe panometry) studies. All were part of a FLIP study group. Key findings:

  • Overall, there were high levels of interrater agreement and accuracy in the interpretation of HRM and FLIP metrics and moderate-to-high levels for motility classification in FLIP
  • There were no FLIP diagnoses of normal EGJ opening in patients with established achalasia and no FLIP diagnoses of achalasia in patients with normal EGJ opening and contractility. This was true with HRM as well.
  • In non-obstructive motor disorders, raters frequently indicated that they would request alternate confirmatory testing before invasive management

My take: This is a reassuring study indicating that with the most consequential esophageal findings, there is excellent agreement among motilist interpretation. Previous studies of colonic manometry, in contrast, have found much lower levels of agreement.

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Santa Justa Lift (Lisbon)

Alpha-Gal Reaction to Infliximab

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G Polanco, et al. JPGN Reports 4(3):p e322, August 2023. Open Access! Delayed Hypersensitivity Reaction to Infliximab Due to Mammalian Meat Allergy

Briefly noted: Case report of a 17 yo with Crohn’s disease who developed urticaria and pruritus approximately 6 hours after her very first infliximab infusion; the patient was diagnosed with Alpha-Gal and responded to change to adalimumab which  is not glycosylated with alpha-gal.

Related blog post: Nonanaphylactic Alpha-Gal and Chronic Gastrointestinal Symptoms

Sigal Music Museum (Greenville, SC) -has a large number of very old harpsichords and pianos. A harpsichord plucks strings to make the sound whereas a piano has a small hammer that strikes the strings to make the sound. This musical instrument is a harpsichord.

Vaping-Induced Esophagitis

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L Pace, K McGoogan. JPGN Reports 4(3):p e327, August 2023. Open Access! Vaping Induced Severe Erosive Esophagitis

This case report describes a 17 yo with sore throat, odynophagia chest pain, and dysphagia associated with vaping. His symptoms resulted in hospitalization and he underwent an EGD on day 4 after symptoms had not improved with multiple empiric therapies.

EGD findings included .

  1. Circumferential erosive or exudative lesions) esophagitis with bleeding found throughout the entire esophagus. Given the lack of infectious etiologies, the authors made a diagnosis of vaping-associated esophagitis
  2. H pylori gastritis

My take: When adolescent patients present with esophageal symptoms, inquiring about exposure to vaping along with medications known to cause pill-esophagitis, is worthwhile.

Related blog post: Review: Infectious Esophagitis

Aerodigestive Complexity Score

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HM Horita et al. J Pediatr 2023; 261: 113549. Open Access! Development of a Medical Complexity Score for Pediatric Aerodigestive Patients

Methods: The authors in this study developed a 7-point medical complexity score .  One point was assigned for each comorbid diagnosis in the following categories: airway anomaly, neurologic, cardiac, respiratory, gastrointestinal, genetic diagnoses, and prematurity. A retrospective chart review was conducted of patients (n=234) seen in the aerodigestive clinic who had ≥2 visits between 2017 and 2021. 

Improvements were followed in the Functional Oral Intake Scale (FOIS)–assigned by aerodigestive feeding therapists.6 The FOIS scale is as follows:

  • •1 = Nothing by mouth
  • •2 = Tube-dependent with minimal attempts of food or liquids (<10%)
  • •3 = Tube-dependent with consistent oral intake of food or liquids
  • •4 = Total oral diet of a single consistency
  • •5 = Total oral diet with multiple consistencies, but requiring special preparations or compensations
  • •6 = Total oral diet with multiple consistencies without special preparation, but with specific food limitations
  • •7 = Total oral diet with no restriction, or <12 months of age on age-appropriate diet

Key findings:

  • At presentation, 69.5% were not at unrestricted age-appropriate diet; 22.7% of the cohort (n = 53) were completely tube dependent
  • There were 165 patients who were not at unrestricted total age-appropriate oral diet at presentation, and the majority (54% [n = 90]) showed improvement in their FOIS scores after aerodigestive team intervention.
  • “For each 1-unit increase in complexity score, there was a 33% decrease in the odds of improvement in FOIS scores (OR, 0.66; 95% CI, 0.51-0.84; P = .001);” however, only neurological comorbidity (OR, 0.26; 95% CI, 0.13-0.53; P < .001) and airway anomaly (OR, 0.35; 95% CI, 0.15-0.79; P = .01) were significantly associated with decreased likelihood to progress in feeding based on FOIS scores
  • Of the 125 patients who were tube fed at initial presentation, 20% (n = 25) were able to achieve full oral feeding after intervention

My take: While the complexity score did correlate with likelihood of progressing with oral feedings, it appears that this score is unnecessary as likelihood of progressing is mainly related to two factors: neurological comorbidities and airway anomalies.

Related blog posts:

Current Approach for FPIES

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Our excellent nutritionist, Bailey Koch, recently gave our group a terrific update on FPIES. Bailey is part of the medical advisory board for THE FPIES Foundation, as is Dr. Benjamin Gold from our group. Here are many of the slides from her lecture.

Link: FPIES foundation action plan sheet:

From International guidelines:

Nowak-Węgrzyn A, Chehade M, Groetch ME, et al. Open Access: International consensus guidelines for the diagnosis and management of food protein-induced enterocolitis syndrome: Executive summary-Workgroup Report of the Adverse Reactions to Foods Committee, American Academy of Allergy, Asthma & Immunology. J Allergy Clin Immunol. 2017;139:1111-26.

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From FPIES 2017 Guidelines:

  • #4. “Consider specific IgE testing of children with FPIES to their trigger food because comorbid IgE-mediated sensitization to triggers, such as CM [cow’s milk], can infer a greater chance of persistent disease.
  • ”#8. Conduct food challenges “in patients with suspected FPIES in medically supervised settings in which access to rapid fluid resuscitation is available and prolonged observation can be provided, if necessary.”
  • #14. Do not routinely obtain endoscopic evaluation as part of the evaluation of FPIES.
  • #17. Acute FPIES should be considered a medical emergency. “Approximately 15% of patients can have hypovolemic shock.”
  • #19. Consider ondansetron treatment as an adjunct (if >6 months of age)
  • #21. Do not recommend routine maternal dietary elimination of offending triggers while breast-feeding if the infant is asymptomatic.
  • #23. FPIES can occur to multiple foods.  “The majority of children (65% to 80%) have FPIES to a single food, most commonly CM.”  In one study, 5% to 10% of children reacted to more than 3 foods.
  • #26. Use hypoallergenic formula in infants who can no longer breast-feed and are given a diagnosis of FPIES caused by CM. Most will tolerate extensively hydrolyzed formulas; some may require an amino acid based formula
  • #29. Reviews natural history.  “The age of CM tolerance appears to be around 3 years” but there has been variability in reports. For FPIES due to grains, average age of tolerance is 35 months and other solid foods is 42 months.  The average age for soy is 12 months (later in some studies), for rice 4.7 years and 4.0 years for oats. For CM-FPIES with positive SPT response, a much protracted course has been reported, with older age of tolerance (~13.8 years)

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.

Why It is Still Not a Good Idea to Test Healthy Children for Enteric Pathogens & Infant Mortality Rates Rising in Georgia (& much of U.S.)

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BR Lee et al. J Pediatr 2023; 261: 113551. A Comparison of Pathogen Detection and Risk Factors among Symptomatic Children with Gastroenteritis Compared with Asymptomatic Children in the Post-rotavirus Vaccine Era

Patients (<11 yrs old) with acute gastroenteritis (AGE, n=2503) and healthy controls (HC, n=537) old enrolled in the New Vaccine Surveillance Network study between December 2011 to June 2016. Key findings:

  • One or more organisms was detected in 1159 of 2503 children (46.3%) with AGE compared with 99 of 537 HC (17.3%).
  • Norovirus was detected most frequently among AGE (n = 568 [22.7%]). The other frequent pathogens detected were rotavirus 7.8% (despite ~75% vaccinated population), adenovirus 4.8%, C difficile 5.3%, Salmonella 6.4%, and Shigella 4.5%. 63.5% of all pathogens detected were viruses.
  • C difficile was detected more frequently in the HC population (7% vs 5.3%). E coli infections, likewise, were very commonly observed in the HC population (2.1% vs 1.1%). The false positive rates for C difficile pathogenicity would have been higher if the authors had not restricted their analysis to >12 months for C diff. The rates of Norovirus and Rotavirus in the HC group was 6.8% and 2.6% respectively.
  • Codetection of multiple pathogens was common. For example, with norovirus, 20.8% had a copathogen detected. Salmonella and C difficile had the highest codection rates of 53.5% and 54.5% respectively.

This study shows substantial improvement in rotavirus infections with a drop from 26% in detection prior to vaccine era to 6% afterwards.

My take: These muliplex molecular assays are quite useful and have improved our ability to determine underlying infections. This is particularly useful in children with underlying diseases (eg. IBD, malignancy). However, this report serves as a cautionary note that many pathogens, including C diff and E coli, are frequently identified with PCR assays in healthy children

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In 2022, 892 infants died in Georgia, an increase of 116 from prior year. About 7 infants dying for every 1000 births. AJC 11/1/23: CDC: Georgia’s infant mortality increase is among the worst in U.S.

Bromelia

Surgery Compared to Biologic Therapy for Crohn’s Disease

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M Agrawal et al. Gastroenterol 2023; 165: 976-985. Open Access! Early Ileocecal Resection for Crohn’s Disease Is Associated With Improved Long-term Outcomes Compared With Anti-Tumor Necrosis Factor Therapy: A Population-Based Cohort Study

In this study from Denmark (2003-2018) using the Danish National Patient and Prescription Registries, the role of early ileocecal resection (CR), n=581, was compared with anti-TNF treatment, n=698. The primary outcome was a composite of ≥1 of the following: CD-related hospitalization, systemic corticosteroid exposure, CD-related surgery, and perianal CD. Only 178 patients (13.9%) were less than 17 years old. ICR cohort had surgery within 5 months of diagnosis in 85%, and in all within 1 yr of diagnosis. Key findings:

  • The risk of the composite outcome was 33% lower with ICR compared with anti-TNF (adjusted hazard ratio, 0.67; 95% confidence interval, 0.54–0.83). ICR was associated with reduced risk of systemic corticosteroid exposure and CD-related surgery, but not other secondary outcomes
  • Of individuals who underwent ICR, the Kaplan-Meier estimate of the proportion who postoperatively initiated IMM, initiated anti-TNF treatment, underwent another intestinal resection, or were on no treatment at 5 years of postoperative follow-up was 46.3%, 16.8%, 1.8% and 49.7%,
  • Of those who were initiated on infliximab as primary therapy, the Kaplan-Meier estimate of the proportion who underwent ICR, switched to a different biologic agent, or continued infliximab at 5 years of follow-up was 17.7%, 40.8%, and 47.3%

The authors note that their cohort had infrequent complicated disease: “Before ICR, 21% were diagnosed with a stricture, ileus, internal fistula, or abscess, indicative of complicated CD and representative of the real world. In contrast, only 1.7% in the anti-TNF group had complicated CD.”

My take: This study suggests that ICR may be a reasonable option in many cases of Crohn’s disease at an earlier stage rather than reserved for only those with refractory disease and complications. However, in pediatric patients, I would be more reluctant to start with a surgical approach given even longer time frame in which further surgery could be needed.

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#NASPGHAN23 Year in Review 2023

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Yesterday’s post was meant to be published on Halloween –will need to fire my editor.

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One of the highlights of NASPGHAN’s annual meeting is the Year in Review lecture. Sandeep Gupta, the editor of JPGN, provided a fantastic review. Here are some of the slides:

AAP has recommended pharmacotherapy and bariatric surgery in selected adolescents at 12 yr and 13 yr respectively. Related blog post: Meds for Obesity: AAP Guidelines
Related blog post: Semaglutide in Adolescent Obesity
Related blog post: Oral GLP-1 Receptor Agonist for Obesity: Orforglipron
etinoids may reduce risk of asparginase-associated pancreatitis
Related blog post: Nonspecific Duodenal Histologic Findings Common in Children with Trisomy 21
Related blog post: Updated Nomenclature for Eosinophilic Gastrointestinal Diseases
Related blog post: I-SEE for Eosinophilic Esophagitis
Related blog post: When to Use Dupilumab for Eosinophilic Esophagitis: Multispecialty Guidelines
Related blog post: Short Bowel Syndrome is a Full Time Job
Related blog post: You No Longer Have Fatty Liver Disease-You Have Steatotic Liver Disease!
Related blog post: RNA Interference (Fazirsiran) for Liver Disease Associated with Alpha-1-Antitrypsin Deficiency
Related blog post: Which Diet is Best for Irritable Bowel Syndrome? A Randomized Trial
Related blog post: Constipation Action Plan: Better Instructions, Fewer Phone Calls
Related blog posts: Foreign Body Retrieval: You Never Know What You Will See, Foreign Bodies in Children -Expert Guidance, Sharp Objects in GI Tract & Good Outcomes

Thiopurines Efficacy in Children with Ulcerative Colitis

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FA Hanna et al. JPGN 2023; 77: 505-511. Thiopurines Maintenance Therapy in Children With Ulcerative Colitis: A Multicenter Retrospective Study

In this retrospective study with 133 children (2008-2019), typical dosing of thiopurines: azathioprine 2-2.5 mg/kg/day and 6-mercaptopurine 1.5 mg/kg/day. Patients with previous or concomitant treatment with 5-ASA were allowed in the study. 62% (n=83) of the cohort had pancolitis. Key Findings:

  • Seventy-four patients (56%) had CS-free clinical remission at week 52 without rescue therapy
  • In the cohort in clinical remission, 67 and 51 patients had both CRP and calprotectin measurements at 1 year and end of follow-up. Sufficient biomarker response (CRP <1 mg/dL, calprotectin <250 mcg/g) was achieved by 44 (66%) and 44 (86%) at those two time points.
  • The likelihood of remaining free of rescue therapy among thiopurines-treated patients was 83%, 62%, 45%, and 37% at 1, 2, 3, and 4 years, respectively
  • 8 of 133 (6%) stopped thiopurine therapy due to adverse effects

In their discussion, the authors make several points regarding efficacy and safety of thiopurines.

  • Many experts have advocated use of anti-TNF therapy agents for ulcerative colitis especially when 5-ASA medications are not effective.. This is based on higher efficacy and safety. With regard to safety, the authors note an “extremely low risk of lymphoma” citing a study from Israel in which children were followed until age of 30 years. No cases of hepatosplenic T-cell lymphoma were identified and the lymphoma rate was not statistically significant (O Atia et al. J Crohns Coliitis 2022; 16: 786-795 Open Access! Risk of Cancer in Paediatric onset Inflammatory Bowel Diseases: A Nation-wide Study From the epi-IIRN).
  • The authors note a recent review “rejected the hypothesis that initiation of biologic treatment later in the disease course correlates with lower response and remission rates in UC patients.”
  • Based on the efficacy and safety, the authors advocate for use of thiopurines “either early in the treatment course or as part of a de-escalation therapy…Thiopurines should be considered in the treatment of UC patients before the initiation of biologic drugs in most children.”

My take: In the U.S., it appears that thiopurine monotherapy, and even combination therapy, in pediatrics with IBD is used infrequently. Anti-TNF therapy with therapeutic drug monitoring is used routinely in patients if a 5-ASA is ineffective or not a good option. This article is a reminder that thiopurines are still a reasonable option. This would have been a good opportunity for a commentary in JPGN to add some context to this article regarding the role of these agents.

AGA guidelines for moderate-to-severe ulcerative colitis: “In adult outpatients with moderate to severe UC in remission, AGA makes no recommendation in favor of or against using biologic monotherapy or tofacitinib rather than thiopurine monotherapy for maintenance of remission.”

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Ospedaletti, Italy
Calanques, France