Evidence map›Paper›PMID 41245040›Full record

ArticleJPGN reports2025

Treatment of intractable pruritus with maralixibat in patients with Alagille syndrome before and after reversal of biliary diversion.

Chiamaka Nwachukwu, David LeVine, Ryan Himes, John Seal, Bryanna Domenick, Elizabeth B Rand, Tamir Diamond

Abstract read
In one paragraph

Article in JPGN reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors.

Chiamaka NwachukwuTulane University School of Medicine New Orleans Louisiana USA.
David LeVineLouisiana State University Shreveport Louisiana USA.
Ryan HimesDivision of Gastroenterology, Hepatology and Nutrition, Ochsner Children's Hospital New Orleans Louisiana USA.
John SealDivision of Transplant Surgery, Ochsner Health New Orleans Louisiana USA.
Bryanna DomenickDivision of Gastroenterology, Hepatology and Nutrition at Children's Hospital of Philadelphia Philadelphia Pennsylvania USA.
Elizabeth B RandDivision of Gastroenterology, Hepatology and Nutrition at Children's Hospital of Philadelphia Philadelphia Pennsylvania USA.
Tamir DiamondDivision of Gastroenterology, Hepatology and Nutrition at Children's Hospital of Philadelphia Philadelphia Pennsylvania USA.ORCID https://orcid.org/0000-0002-1904-5351

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Alagille syndrome (ALGS) is a rare, cholestatic, multisystemic disorder characterized by bile duct paucity. Cholestatic pruritus is a common, and often severe, symptom of ALGS and is the leading cause of liver transplantation. The treatment of cholestatic pruritus is challenging and involves medical and surgical options, such as surgical biliary diversion (SBD) for refractory cases. However, SBD is associated with medical/lifestyle challenges. Maralixibat, an ileal bile acid transporter inhibitor, is a recently approved treatment for cholestatic pruritus in patients with ALGS and is used as part of standard of care. We present cases of two patients with ALGS who initiated treatment with maralixibat: one before, with continuation after, reversal of SBD, and one after SBD reversal. In both cases, treatment with maralixibat was well-tolerated and demonstrated marked improvements in cholestatic pruritus. This suggests that maralixibat is a pharmacological alternative for patients who would like to pursue reversal of SBD.

Indexed as

cholestasisileal bile acid transporter inhibitorsrare pediatric liver diseaseserum bile acids

Identifiers

PMID41245040
PMCPMC12611582

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.