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ESPGHAN Guidelines for PSC in Children

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PF van Rheenen et al. JPGN 2024; DOI: 10.1002/jpn3.12378. Open Access! Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group

Recommendations:

  • In children with suspected or confirmed IBD, screening for liver disease is usually performed at 3 to 6 months intervals and a work‐up for underlying liver disease is most commonly initiated when liver enzymes exceed 2x the upper limit of normal
  • Use MRCP as the radiological modality of choice for diagnosing PSC
  • Consider performing a liver biopsy in children with IBD and suspected PSC in the following circumstances: i) Normal biliary tree at MRCP, ii) raised immunoglobulin G and the presence of liver-specific autoantibodies, or iii) clinical uncertainty before steroid induction therapy for IBD
  • Perform fecal calprotectin screening at least once yearly in children with isolated PSC and/or AIH to select patients for diagnostic endoscopy for suspected inflammatory bowel disease (panel recommends cutoff of >150 indicating need for ileocolonoscopy)
  • Surveillance colonoscopy should be considered in children with PSC–IBD and the following risk factors of colorectal cancer: i) persistent active colonic inflammation, ii) longstanding colitis (≥8 years), or iii)  a family history of colorectal cancer in a first-degree relative <50 years. (The overall risk of colon cancer in those <18 yrs of age is very low)
  • UDCA may be prescribed at doses of 15–20 mg/kg/day. Despite evidence of improvement of liver enzymes, its long-term effect on disease progression has not been demonstrated. Consider a 6-months therapeutic trial of UDCA, either immediately after PSC diagnosis or when spontaneous normalization of GGT does not occur in the first 6 months postdiagnosis. Continue UDCA treatment if there is a meaningful reduction or normalization of GGT or improvement of symptoms
  • Oral vancomycin may be prescribed for a potential improvement in liver biochemistry as well as bowel inflammation. Its long-term effect on disease progression has not been demonstrated
  • In children with PSC–IBD and biochemical, serological, and histological features of AIH, the use of corticosteroids and antimetabolites may suppress immune-mediated hepatitis. In the absence of convincing AIH features, the use of corticosteroids and antimetabolites is not indicated to manage PSC
  • Children with PSC, relevant bile-duct strictures and cholestatic symptoms should be assessed for liver transplantation. When their symptoms are likely to improve following biliary intervention, ERCP can be considered
  • Recommended blood testing for children with PSC: At diagnosis: Autoantibodies (ANA, anti-SMA, anti-LKM-1, anti-LC1, and anti-SLA), Every 3-6 months: ALT, AST, GGT, Albumin, INR, Platelets, CRP. Every 12 months: IgG, AFP, and Fat Soluble vitamins. Consider f/u autoantibodies in those with elevated IgG at f/u lab testing

My take: This is a useful position paper; it does not have a zillion recommendations like some other ESPGHAN positions papers. Given the frequency of liver enzyme elevation in patients with IBD, mild to modest elevations may need to be observed before launching an extensive evaluation (see related blog posts below).

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Optimizing Fluid Resuscitation in Pediatric Acute Pancreatitis

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N Norris et al. J Pediatr 2025; 276: 114329. Liberal Fluid Resuscitation is Associated with Improved Outcomes in Pediatric Acute Pancreatitis

This single-center retrospective study with 227 patients (2013-2023) examined the role of liberal fluid administration/type of fluid administration and outcomes in children with acute pancreatitis. Overall, 100 patients received normal saline (NS) and 41 received lactated ringers (LR). Liberal fluid management was considered to be >1.5x the maintenance.

Key findings:

  • Patients who received liberal fluids were less likely to be admitted or transferred to the intensive care unit compared with those receiving conservative management (OR, 0.32)
  • The liberal NS fluid group with early feeding had the lower rates of moderate/severe manifestations of AP compared with other combinations of diet and fluid orders except the conservative LR group
  • Moderate/severe AP was highest in the conservative NS group (14/37 [38%]), followed by liberal LR (6/31 [19%]), liberal NS (9/63 [14%]); it was lowest in conservative LR group (0/10 [0%]).

In the discussion, the authors note that adult studies have supported a more moderate approach to IVFs (1.5 mL/kg/hr) given the risks of fluid overload in WATERFALL trial.

My take:

  1. It is surprising that so few patients received LR in this study; there has been some evidence that LR is better than NS for AP since 2014 (see blog posts below)
  2. Especially in those receiving NS, more liberal use of IVFs appears beneficial. Reasonable to start at 1.5 x maintenance (as recommended by Dr. Freeman)
  3. Though children generally tolerate liberal IVFs better than adults due to better cardiovascular function, a prospective randomized study is needed to determine which fluid strategy is optimal.
  4. Early enteral feeding is beneficial in most cases
View from the Rialto Bridge, courtesy of Steven Liu who has some amazing pictures

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Key Advances in 2024: An Overview from GutsandGrowth (Part 4)

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This year I had the opportunity to give a lecture to our group that reviewed much of the important advances that happened in 2024. Here are some of the slides (if you have any trouble reading the slides, you can search for the original blog post using author name).

Key Advances in 2024: An Overview from GutsandGrowth (Part 3)

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This year I had the opportunity to give a lecture to our group that reviewed much of the important advances that happened in 2024. Here are some of the slides (if you have any trouble reading the slides, you can search for the original blog post using author name).

Key Advances in 2024: An Overview from GutsandGrowth (Part 2)

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This year I had the opportunity to give a lecture to our group that reviewed much of the important advances that happened in 2024. Here are some of the slides (if you have any trouble reading the slides, you can search for the original blog post using author name).

Key Advances in 2024: An Overview from GutsandGrowth (Part 1)

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This year I had the opportunity to give a lecture to our group that reviewed much of the important advances that happened in 2024. Here are some of the slides (if you have any trouble reading the slides, you can search for the original blog post using author name).

Fecal Microbiota Transplantation for Severe Constipation in Children

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Methods: The efficacy of retrograde colonic enema (RCE) with fecal microbiota transplantation (FMT) was studied in a randomized, double-blind, controlled trial with 110 children. The initial cohort recruited was 576 patients; however, 466 were excluded for not meeting inclusion criteria. All participants received a daily RCE, followed by a 4-week FMT treatment (twice a week) and a 12-week follow-up period. 

Key findings:

  • At the end of the follow-up period, 22 patients (40.0%) in the FMT with RCE group and 10 patients (18.2%) in the placebo with RCE group had ≥ 3 spontaneous bowel movements per week

There was a low response to RCE alone which the authors attributed in part to the severity of constipation in the cohort. It is unclear the degree of compliance with the treatment protocol which was done in the home setting. There was a prior open-label study with NJ FMT which improved constipation in half of participants.

My take: Modulating the microbiome can have beneficial effects on stool frequency. This can be through diet and possibly FMT in severe cases of constipation. The availability of capsules could make this type of therapy easier but perhaps less palatable. Even if FMT proves to be a useful treatment, the optimal treatment regimen is not clear.

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Weird Xray and Successful Treatment for Water Bead Ingestion

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C Hoffman et al. J Pediatr Gastroenterol Nutr. 2024;78:448–449. Open Access! Orbeez ingestion: Successful medical management of1000 absorbent bead ingestion in a 3‐year‐old patient

An excerpt:

“There is no consensus regarding optimal management in instances of Orbeez™ ingestion. However, a review of reported cases from 2021 indicated that of 43 cases of bowel obstruction secondary to water bead ingestions, two patients required endoscopic removal and the remaining 41 required surgical enterotomy or intestinal resection. We report a case of successful medical management following ingestion of over 1000 water beads…The patient was placed on a clear liquid diet and received a mineral oil enema with minimal passage of beads. She was then started on sennosides and erythromycin ethylsuccinate to help stimulate expulsion. Stools were closely monitored and revealed passage of greater than 1000 beads over the next 24 h.”

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Is Breastfeeding Linked to IBD Risk in Offspring?

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M Agrawal et al. Clin Gastroenterol Hepatol 2024; 22: 2459-2467. Open Access! Breastfeeding Duration Is Not Associated With Offspring Inflammatory Bowel Disease Risk in Three Population-Based Birth Cohorts

The authors utilized  prospectively collected data from 3 population-based birth cohorts (Danish National Birth Cohort, Norwegian Mother, Father, and Child Cohort, and All Babies in Southeast Sweden). This collectively included nearly 170,000 offspring.

Key findings:

  • During median follow-up of 16.3–22.3 years, between 1996 and 2021, 543 offspring were diagnosed with IBD
  • In each country, there was no association between exclusive breastfeeding duration and offspring IBD risk

Discussion:

“In contrast to majority of case-control studies, both cohort studies reported null association between breastfeeding, treated as a binary exposure (any versus no breastfeeding) or by duration, and offspring IBD risk. Similarly, 2 nested case-control studies, leveraging prospectively collected data on early life exposures as part of the population-based Jerusalem Perinatal Study and 2 United Kingdom birth cohorts (the 1946 National Survey of Health & Development and the 1958 National Child Development Study) reported null associations between breastfeeding and IBD risk.22,23 Data from these studies, which are more rigorous in methodology compared with case-control studies, are consistent with findings from our analyses.”

My take: While this study has some limitations inherent in observational data, this study with prospectively-collected data indicates that breastfeeding did not modulate the risk of developing IBD.

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La Fortuna, Costa Rica

Botox for Pediatric Gastrointestinal Disorders

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M Homan et al. J Pediatr Gastroenterol Nutr. 2024;79:1096–1105. Open Access! Drugs in focus: Botulinum toxin in the therapy of gastrointestinal disorders in children (Review)

Botox has been used for the following:

  • Achalasia
  • Cricopharyngeal achalasia
  • Retrograde cricopharyngeal dysfunction
  • Delayed gastric emptying
  • Anal achalasia
  • Constipation after Hirschsprung disease surgery
  • Selected functional constipation
  • Chronic anal fissure

With regard to achalasia, “Botox can be considered only in patients in whom rapid weight gain is important to improve surgical outcomes.”

With regard to gastroparesis/delayed gastric emptying in children, the authors note the following:

‘A recent retrospective study from the Mayo Clinic analysed the response to intrapyloric Botox in children (n = 20) with gastroparesis and concluded that intrapyloric Botox injection in children is safe and can provide temporary relief for patients with refractory upper gastrointestinal symptoms with and without gastroparesis.43 [This was] a meta-analysis, including six studies, 160 patients, which showed that 68% of patients responded to intrapyloric Botox irrespective of the presence of gastroparesis, while among patients diagnosed with gastroparesis the therapeutic response was 66%.43 These results suggest that intrapyloric Botox can be effective not only in children with gastroparesis but also in children with refractory functional upper gastrointestinal symptoms.’ In their conclusion, the authors note “Intrapyloric Botox injection is increasingly used for the treatment of gastroparesis but evidence supporting its use in children is still scarce.”

My take: Overall, this is a helpful review.

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