Evidence map›Paper›PMID 36556142›Full record

ArticleJournal of clinical medicine2022

Ileal Bile Acid Transporter Blockers for Cholestatic Liver Disease in Pediatric Patients with Alagille Syndrome: A Systematic Review and Meta-Analysis.

Hafiza Sidra Tul Muntaha, Mubashar Munir, Syeda Haleema Sajid, Zouina Sarfraz, Azza Sarfraz, Karla Robles-Velasco, Muzna Sarfraz, Miguel Felix, Ivan Cherrez-Ojeda

Open access · goldAbstract read
In one paragraph

Article in Journal of clinical medicine, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed, 1 pooled it
2.0field-weighted citation impact, top 13% of its field
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

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

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

5 citing papers in PubMed, 1 synthesis or guideline pooled it, 9 citations in OpenAlex.

  1. Pooled it
  2. Trial
  3. Review
  4. ATP-binding Cassette Transporter Defects and Their Roles in Hepatic Diseases.Journal of clinical and translational hepatology · 2026
    Review
  5. Review
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

9 authors at 6 institutions in 3 countries.

Hafiza Sidra Tul MuntahaDepartment of Research, Services Institute of Medical Sciences, Lahore 54000, Pakistan.
Mubashar MunirDepartment of Research, King Edward Medical University, Lahore 54000, Pakistan.
Syeda Haleema SajidDepartment of Research, Services Institute of Medical Sciences, Lahore 54000, Pakistan.
Zouina SarfrazDepartment of Research and Publications, Fatima Jinnah Medical University, Lahore 54000, Pakistan.ORCID 0000-0002-5132-7455
Azza SarfrazDepartment of Pediatrics and Child Health, The Aga Khan University, Karachi 74800, Pakistan.
Karla Robles-VelascoDepartment of Allergy and Pulmonology, Universidad Espíritu Santo, Samborondón 092301, Ecuador.ORCID 0000-0001-7780-1015
Muzna SarfrazDepartment of Research, King Edward Medical University, Lahore 54000, Pakistan.
Miguel FelixDepartment of Medicine, New York City Health + Hospitals, Lincoln, NY 10034, USA.ORCID 0000-0002-2243-3646
Ivan Cherrez-OjedaDepartment of Allergy and Pulmonology, Universidad Espíritu Santo, Samborondón 092301, Ecuador.ORCID 0000-0002-1610-239X
King Edward Medical University · PKServices Institute of Medical Sciences · PKUniversidad de Especialidades Espíritu Santo · ECAga Khan University · PKFatima Jinnah Medical University · PKNew York City Health and Hospitals Corporation · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Alagille syndrome (ALGS) is a rare, debilitating inheritable disease that is associated with refractory pruritus due to chronic cholestasis. The following systemic review and meta-analysis presents the latest evidence for ileal bile acid transport (IBAT) blockers in AGLS patients in order to improve their efficacy. This study adhered to PRISMA 2020 Statement guidelines. A systematic search of PubMed/MEDLINE, Web of Science, Scopus, and the Cochrane library was conducted from inception until 23 October 2022. A combination of the following keywords was used: Alagille syndrome, therapeutics, treatment, therapy. Meta-analytical outcomes included effect directions of end-line changes in serum bile acids (sBAs), Itch Scale scores (ItchRO), Multidimensional Fatigue Scale scores, pediatric quality of life (QL), alanine aminotransferase (ALT), and total bilirubin. A total of 94 patients across four trials were enrolled and received maralixibat, odevixibat, or a placebo. There was a significant reduction in ItchRO scores by 1.8 points, as well as in sBAs by 75.8 μmol/L. Both the Multidimensional Fatigue Scale and Pediatric QL scale were also improved by 11.4 and 8.3 points, respectively. However, ALT levels were raised by 40 U/L. The efficacy of IBAT inhibitors across current trials was noted. Future trials may focus on the optimization of dosing regimens, considering gastrointestinal side effects and drug-induced ALT elevation in AGLS patients.

Indexed as

Alagille syndromechronic cholestasismaralixibatodevixibatrefractory pruritusserum bile acids

Identifiers

PMID36556142
PMCPMC9784790
OpenAlexW4312209963

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

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.