Noncirrhotic Portal Hypertension

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C Jarasvaraparn, et al. J Pediatr Gastroenterol Nutr. 2026;82:751–759. Clinical presentation, management, and outcomes for noncirrhotic portal hypertension in children

Background: Noncirrhotic portal hypertension (NCPH) is a rare condition marked by elevated portal venous pressure in the absence of cirrhosis and is caused by intrahepatic and extrahepatic pathways. NCPH is more common in Eastern countries than in Western countries. In the East, infectious diseases, particularly schistosomiasis in Africa, and poor socioeconomic conditions leading to chronic abdominal infections, are significant contributing factors. On the other hand, Western countries see a higher prevalence of NCPH linked to hypercoagulable disorders and potential genetic predispositions.

Methods: This was a  single-center retrospective study of 63 pediatric patients form 2000-2024.

Key findings:

  • The three most common etiologies included extrahepatic portal vein obstruction (EHPVO) in 27 (42.8%), congenital hepatic fibrosis (CHF) in 17 (27%), and nodular regenerative hyperplasia (NRH) in 10 (15.9%). 6 (9.5%) patients had idiopathic noncirrhotic portal hypertension
  • Most patients presented with incidental splenomegaly (20; 32%) or gastrointestinal (GI) bleeding (20; 32%)
  • Mortality was seen in 5% of children in this series

My take: More than two decades ago, a patient was referred to me with dyspnea as the pulmonologist suspected that this was due to portal hypertension/hepatopulmonary syndrome. Ultimately, after evaluation by a colleague, she was diagnosed with idiopathic noncirrhotic portal hypertension and underwent a successful liver transplantation. This condition is very difficult to diagnose if you are unaware of its existence.

Fatigue in Pediatric Inflammatory Bowel Disease

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MD Stutvoet et al. J Pediatr Gastroenterol Nutr. 2026;82:699–707. Open Access! Fatigue in pediatric inflammatory bowel disease: Explained by transdiagnostic and disease-focused factors

Methods: “The PROactive cohort is a longitudinal study that collects transdiagnostic patient-reported outcomes on fatigue, daily life participation, and psychosocial well-being in children with chronic conditions. It is based at the Wilhelmina Children’s Hospital in the Netherlands.” this study enrolled 127 patients with IBD.

Key findings:

  • Fatigue is highly prevalent in pediatric IBD, with 29% of patients reporting severe fatigue—despite most being in clinical remission
  • 78% of variance in fatigues explained by transdiagnostic lifestyle, psychological and social factors
Fatigue prevalence in pediatric IBD. For reference, fatigue prevalence measured using the same “general fatigue” subscale of the MFS-GCS is also shown for other chronic childhood diseases15 and the general Dutch population.19 

Discussion Points:

  • “Clinical disease activity was significantly associated with fatigue in univariate analysis (p < 0.01). This is consistent with earlier studies showing modest associations between clinical disease activity and fatigue2–4, 33–35 and no association with biochemical markers such as FC, CRP, and anemia.2–4, 11, 35 The discrepancy between clinical and biological markers may reflect the inclusion of functional limitations in the clinical disease activity scores, and their weak correlation with measures of biological disease activity as FC.36… Taken together, the association with clinical disease activity shows that fatigue may be closely linked to perceived disease-related limitations rather than inflammatory activity alone.”
  • “Transdiagnostic factors—including depressive symptoms, poor sleep, and social functioning—were strongly associated with fatigue, consistent with previous findings in pediatric IBD cohorts,3, 4, 33 and other chronic diseases.14, 16…Our results emphasize the complex interactions among psychosocial factors and support a more integrative approach to fatigue assessment. “

My take (borrowed in part from the authors): “While disease activity may initially precipitate fatigue, other factors—such as poor sleep or school pressure—can perpetuate it, even after inflammation resolves.”

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2026 Review: Eosinophilic Gastrointestinal Diseases (EGIDs)

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P Visaggi, ES Dellon. Gastroenterology 2026;170:476–494. Open Access! Epidemiology, Natural History, and Treatment of Eosinophilic Gastrointestinal Diseases

Diagnosis:

  • EoE is diagnosed in the setting of symptoms of esophageal dysfunction, demonstration of at
    least 15 eosinophils per high-power field (eos/hpf) on esophageal biopsy, and exclusion of competing causes of esophageal eosinophilia. At the time of diagnosis, disease severity can be assessed with the Index of Severity for EoE (I-SEE)
  • Non-EoE EGIDs: the diagnostic process is more challenging and pediatric diagnostic guidelines were only published in 2024…biopsy specimen shows pathologically elevated
    levels of GI tract eosinophils, and other conditions that cause GI eosinophilia are excluded…Symptoms are nonspecific, multiple conditions could explain extraesophageal eosinophilia, inflammation can involve muscular and serosal layers rather than mucosal,

Treatment:

  • EoE: PPIs, Swallowed topical corticosteroids, Monoclonal antibodies and food elimination dets
  • Non-EoE EGIDs: “The management of non-EoE EGIDs is challenging due to lack of established end points, validated outcomes, and of successful RCTs leading to approved treatments. Accordingly, management of non-EoE EGIDs is currently based on low-quality evidence.” Main treatments: corticosteroids (prednisone or budesonide), food elimination diets, and monoclonal antibodies (eg. dupilumab). “The use of PPIs, mast cell stabilizers, leukotriene inhibitors, and immunomodulators for non-EoE EGIDs has been reported,
    with variable results.”

My take: This is a useful review with a number of pointers for epidemiology, natural history, evaluation and management of EoE and Non-EoE EGIDs.

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Biliary Atresia Online Screening Tool

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There is a new free screening tool, BiliScreen.org, which helps pediatricians triage patients for followup labs and/or hepatology referral. This site incorporates the recent AAP guidelines (see below) for evaluation.

Screenshots from website:

My take:

  • Overall, this is a quick easy tool which should be helpful & I recommend sharing this website with pediatricians/pediatric health providers
  • A potential improvement to the site would be a section with an explanation of other causes/consequences of cholestasis which could require more urgent evaluation (eg. metabolic diseases, vitamin K deficiency)

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Rashelle Berry: From Tube to Taste

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We had a terrific lecture given to our group by Rashelle Berry. She is a pediatric dietitian specializing in feeding disorders, enteral nutrition, and tube weaning. She practices within the Feeding Program at Children’s Healthcare of Atlanta, partnering closely with a wide variety of disciplines to care for children with significant feeding challenges and GI-related nutrition concerns. My notes below may contain errors in transcription and in omission.

Key Points:

  • Families expectations are often at odds with dealing with tube feeding which makes it more difficult
  • Parents have strong desire to achieve all oral feedings and often look for advice outside of clinical visits
  • Hunger alone is not sufficient to transition off tube feedings
  • Prior to attempts to stop tube feedings, it is important to assess safety and to align feeding patterns to be more physiologic. This includes offering feeds via bolus typically every 3 hours and stopping continuous feedings
  • Hyperosmolar feedings can contribute to GI symptoms
  • Changing formula to improve tolerance can result in quick symptom improvement (1-2 days)
  • Many children with tube feedings are overfed. This can contribute to poor hunger as well as initial weight loss when transitioning off tube feeds
  • Two main options to advance oral feedings: 1. Offer oral feeds prior to tube feeds and reduce tube feedings based on oral intake 2. Plan to reduce tube feedings by a set amount, typically 10-30% and follow to see if oral intake improves
  • Expect some weight loss during transition; if mild weight loss, most often continue to follow closely
In Step 2, these are some of the aspects indicating tolerance

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Comparing Vedolizumab in “Early” and “Late” Crohn’s Disease

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Lancet Gastroenterol Hepatol 2026; 11: 12-21. Vedolizumab in early and late Crohn’s disease (LOVE-CD): a phase 4 open-label cohort study

Methods: Eligible patients were adults aged 18–80 years with moderate to severe Crohn’s disease (Crohn’s Disease Activity Index [CDAI] 220–450, with ulcers at endoscopy). Patients were divided into two groups: those with early Crohn’s disease, n=86 (defined as a diagnosis less than 2 years ago and naive to advanced treatment [naive or only treated with corticosteroids or immunomodulators, or both]); and those with late Crohn’s disease, n=174 (defined as a diagnosis more than 2 years ago and previously treated with corticosteroids, immunomodulators, and anti-TNF agents). The primary endpoint was the proportion of patients with clinical and endoscopic remission (defined as CDAI ≤150 and SES-CD <4) at both week 26 and 52.

Key findings:

  • Clinical and endoscopic remission at both week 26 and 52 was achieved in 27 (31·4%) of 86 patients with early Crohn’s disease versus 15 (8·6%) of 174 patients with late Crohn’s disease (difference 22·8%, 95% CI 12·6–33·7)
  • Serious adverse events occurred in three (3·5%) of 86 patients with early Crohn’s disease versus 46 (26·4%) of 174 patients with late Crohn’s disease and included infections (one [1·2%] vs 13 [7·5%]), surgery (none vs eight [4·6%]), intestinal obstruction (none vs four [2·3%]), exacerbation of Crohn’s disease (one [1·2%] vs six [3·4%]), and malignancy (none vs three [1·7%])
Corticosteroidfree clinical remission at all timepoints

Discussion:

  • “After 52 weeks of open-label treatment, almost 60% of patients with early disease achieved clinical remission and more than 50% were in endoscopic remission. By contrast, deep remission rates at both weeks 26 and 52 were observed in less than 10% of patients with late Crohn’s disease (ie, those with longstanding disease and previous exposure to anti-TNF
    agents).”
  • “Despite the earlier stage at which vedolizumab was initiated, disease severity was comparable betweenthe early and late Crohn’s disease groups, from a clinical (median CDAI 255 vs 259), endoscopic (median SES-CD 9 vs 12), and biochemical perspective (median serum C-reactive protein 9 mg/L vs 8 mg/L).”
  • “A pivotal observation in LOVE-CD was that dose intensification after week 26 (ie, doubling the dose) in patients without an endoscopic response did not lead to higher endoscopic remission rates, despite significantly higher serum vedolizumab concentrations. This
    finding suggests that the dosing schedule that was originally designed and approved is optimal for most patients and saturates the target. Patients who do not respond most likely have other dominant immune pathways that are activated and remain unaffected by
    vedolizumab.”
  • “All three classes of biologics approved for the treatment of Crohn’s disease perform better when initiated early in the disease course.”

My take: For Crohn’s disease (CD), vedolizumab should be mainly used in those without prior biologic therapy. In addition, changes in vedolizumab dosing based on concentrations is much less helpful than it is with anti-TNF agents.

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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.

What’s Changing For the GI Tract in Patients with Cystic Fibrosis Due to Highly Effective Modulator Therapy

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P Saikumar et al. Am J Gastroenterol 2026; 121: 103-111. A Comprehensive Review of Gastrointestinal Manifestations in Cystic Fibrosis in the Era of Highly Effective Modulator Therapy

Thanks to Ben Gold for sharing this article.

Exoocrine Pancreatic Insufficiency (EPI)

  • “Mutlicenter studies, KIWI and ARRIVAL, demonstrated increase in FE-1 (fecal elastse-1) after treatment with ivacaftor, signifying improvement in in pancreatic function”
  • “The PROMISE study, a large 56-center prospective observational study with participants at least 12 years of age…found ETI (Elexacaftor-tezcaftor-ivacaftor) did not result in improvement in pancreatic function…raises the question if age of initiation of CFTR modular therapy has an influence in recovery of pancreatic function.”

Acute Pancreatitis

  • “With the improvement in pancreatic function seen in some patients with CF on HEMT, there have been emerging reports of acute pancreatitis.”

CF Hepatobiliary Involvement

  • “Previously referred to as CF liver disease (CFLD), hepatobiliary complications in CF are now recommended to be classified as CF hepatobiliary involvement (CFHBI) or advanced CFLD”
  • “Vigilant monitoring of transaminase levels and liver function is crucial after CFTR modulator initiation, because drug-induced liver injury (DILI) is a potential concern with elexacaftor/tezacaftor/ivacaftor (ETI) therapy…A disproportionality analysis of Food and Drug Administration adverse event reporting system data from 2019 to 2022 identified 452 reports of DILI associated with ETI use, demonstrating a statistically significant association”
  • “ETI is not recommended for those with Child Pugh Classification C and dose reduction is recommended for Child Pugh classification B in clinically appropriate situation when benefit outweighs the risk”

Nutrition

  • “In addition to macronutrient deficiency, Children with Cystic Fibrosis are at risk of deficiencies in fat-soluble vitamins, minerals, and essential fatty acids (38). With an overt focus on nutrition, PERT, and advancements in CFTR modulators, an opposite trend is now being seen, with a higher incidence of obesity and overweight in this population…[and] associated comorbidities such as coronary heart disease, metabolic dysfunction-associated steatotic liver disease, and metabolic syndrome-including hyperlipidemia and diabetes.”

Eating Disorder

  • “Disordered eating is relatively a new concern in this population. There are controversial data on the prevalence of eating disorder in PwCF” (people with CF).”

GERD

  • “Data indicate that children with CF are 4 times more likely to experience acid gastroesophageal reflux than the general population (59)…RECOVER, a multicenter study of ETI in PwCF displayed decreased GI symptoms, including GERD, after 12 months of treatment (61). Similarly, treatment with IVA was associated with symptomatic relief of GERD at 52 weeks and also showed a decline in extraesophageal reflux in PwCF treated with
  • IVA for 12 months (62).”

Meconium Ileus

  • Recent case reports, “although differing in ETI initiation timing, outcome severity, suggest that prenatal ETI exposure may positively influence MI (69).”

Constipation

  • “There are limited data regarding the effect of ETI on constipation, asthe PROMISE trialshowed only small improvements in Patient Assessment of Constipation-Symptoms score and Patient Assessment of Constipation-Quality of Life”

DIOS

  • “There is paucity of data describing impact of HEMT [highly effective modulator therapry] on DIOS. By correcting the CFTR dysfunction, HEMT may help reset the ion/fluid balance which is considered one of the main factors in pathogenesis of DIOS.”

My take: “CFTR modulators have revolutionized the management of CF by dramatically altering the course of the disease and improving patient outcomes.” They are also altering the clinical GI manifestations.

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Misleading Study on Topical Steroids for Eosinophilic Esophagitis -Comparing Four Apples to One Apple

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FB Murray et al. Am J Gastroenterol 2026; 121: 130-139. Loss of Response to Off-Label Swallowed Topical Corticosteroids in Eosinophilic Esophagitis Can be Overcome by a Switch to an Esophageal-Targeted Budesonide Formulation

A quick glance at this study gives the impression that off-label swallowed topical corticosteroids (olSTC) are an inferior treatment to the budesonide orodispersible tablet (BOT) as many patients who had either a non-response to olSTC achieved remission with BOT.

Methods: This study from the Swiss Eosinophilic Esophagitis Cohort Study with 340 patients (mean age 43 years) analyzed prospectively collected data. Twenty-six percent had prior olSTC nonresponse (n=66) , 16% were in remission with prior olSTC (n=44), and 58% were STC-naïve.

Here were the key results according to the authors (Figure 2):

  • Histologic remission (<15 eos/hpf) was achieved in 62% who had not responded to olSTC previously and in 72% who had prior olSTC response (P=0.094)
  • The authors conclude that “our results provide conclusive evidence that off-label STC cannot be translated into corticosteroid-refractory disease per se.”

Here’s the main problem with this study:

  • “Most patients in Switzerland have been treated with a rather low-dose olSTC regimen (</ = to 0.5 mg per day).” Typical BOT dosing is 2 mg per day. Thus, the authors are comparing the use of 4-times the amount of budesonide in the BOT group to the olSTC non-responders.
  • Their discussion states that “most patients with EoE without a response to olSTC are not truly corticosteroid-refractory but respond to an esophageal adjusted topical corticosteroid formulation…In the United States, a BOS [suspension] has been recently approved…Due to similar mechanism of action, it can by hypothesized that patients without prior response to olSTC will respond to BOS in similar rates as shown in our study.” Yet, there is no proof that the formulation made a difference in this study; the more likely explanation is that patients previously who had not responded to olSTC were under-treated.

My take:

  1. In patients with eosinophilic esophagitis who have not responded to topical steroids, it is important to make sure that they were prescribed the right dose and that they are actually taking the medication.
  2. Future studies of esophageal formulations should be compared to off-label STC using the same dose to determine if the formulations confer additional benefits.
  3. Getting the right dose is important for every malady.

Thanks to Ben Gold for sharing this publication with me.

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Reduced Dosing of GLP-1 RAs May Work for Maintenance Treatment of Obesity

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Roni Rabin, NY Times 3/5/26: Most Patients Keep Weight Off With Fewer GLP-1 Shots, Study Finds

An excerpt:

After 36 weeks of follow-up, most of the patients who spaced out their GLP-1 injections kept the weight off and also maintained health benefits like reduced blood pressure and better blood sugar control…

The study was small, only 34 patients in a relatively homogeneous group — mostly white and privately uninsured… the research, published in February in the journal Obesity, provides a potentially appealing new option for patients who are loath to commit to lifelong weekly injections of a costly medication that may not be covered by insurance and that some fear could have unknown side effects…

The patients reduced the frequency only after achieving their desired weight loss and reaching a weight-loss plateau.

My take: In some patients, less frequent dosing of GLP-1s may be effective as maintenance therapy. Whether this strategy could be applied to the daily oral semaglutide is not clear.

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How to Score the Ulcerative Colitis Endoscopic Index of Severity (UCEIS) Properly

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J Blackwell et al. Gastroenterol 2026; 170: 452-455: Open Access! A Normal UCEIS Is Zero: A Score Divided by a Common Language

“The index uses a Likert scale of vascular pattern, bleeding, erosions and ulcers, with a total score intended to reflect severity of disease. When the index was first published, the baseline score for each descriptor was 1, meaning that a normal vascular pattern, no bleeding, and no erosions or ulcers scored 3. However, when the index was subsequently validated, the values attributed to each descriptor were rebased to 0, to improve clinical utility…This means that a completely normal flexible sigmoidosopy scored 0 rather than 3, and the worst activity of UC ever seen by the investigators (compared with a visual analogue scale 0 to 100) was 8 rather than 11…

The UCEIS has since been shown to predict the need for escalation of medical treatment and has recently been incorporated into a validated prognostic clinical index to predict response to intravenous steroids among patients with acute severe UC.10,11 Accurate scoring is therefore essential, with direct implications for clinical decision making.”

My take: The UCEIS is more detailed than the Mayo score. However, I expect that before long artificial intelligence will be able to review images and give a more consistent interpretation of the severity of endoscopic findings than either of these scoring systems.

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