Evidence map›Paper›PMID 38829428›Full record

ArticleRadiologie (Heidelberg, Germany)2024

Magnetic resonance imaging features of progressive familial intrahepatic cholestasis type 3.

Nina Xu, Ling Gong, Xiaoxiao Mi, Wenjun Yang, Dong Tang

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In one paragraph

Article in Radiologie (Heidelberg, Germany), 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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1 · What the graph read from it

What it found

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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.

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

5 authors.

Nina XuDepartment of Medical Imaging (Radiology), the Affiliated Hospital of Hangzhou Normal University, Zhejiang, China.
Ling GongDepartment of Infectious Disease (Liver Diseases), the Affiliated Hospital of Hangzhou Normal University, Zhejiang, China.
Xiaoxiao MiInstitute of Translational Medicine, the Affiliated Hospital of Hangzhou Normal University, Zhejiang, China.
Wenjun YangDepartment of Pathology, the Affiliated Hospital of Hangzhou Normal University, Zhejiang, China.
Dong TangDepartment of Medical Imaging (Radiology), the Affiliated Hospital of Hangzhou Normal University, Zhejiang, China. 15258868408@163.com.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

purposeProgressive familial intrahepatic cholestasis type 3 (PFIC-3) is a rare autosomal recessive cholestatic liver disorder. This study aimed to present the clinical and magnetic resonance imaging (MRI) features of three patients with PFIC‑3.

methodsThe study included three patients with cholestasis and pathogenic variants in the ABCB4 gene identified by next-generation sequencing of a targeted-gene panel or by whole-exome sequencing. The clinical, laboratory, histological, molecular, and MRI features of the patients were collected.

resultsThree patients (one male and two females) were enrolled. The age when clinical signs and symptoms were first noted was 21, 14, and 39 years, respectively, and the signs and symptoms included pruritus and splenomegaly (in all three patients). Parenchymatous lace-like fibrosis was associated with periportal hyperintensity and periportal halo sign in three patients. Segmental atrophy was observed in two patients, diffuse atrophy was observed in one patient, and liver surface irregularity caused by regenerating nodules was observed in three patients. Magnetic resonance cholangiopancreatography (MRCP) images showed irregular bile duct changes in three patients, focal hilar bile duct stenosis, and local intrahepatic bile duct dilatation.

conclusionsImaging studies using MRI and MRCP can support the clinical and laboratory results in cases of PFIC‑3 and can also be used as a noninvasive diagnostic option.

Indexed as

Cholestasis, IntrahepaticMagnetic Resonance ImagingAdolescentAdultATP Binding Cassette Transporter, Subfamily BCholangiopancreatography, Magnetic ResonanceFemaleHumansMaleYoung AdultATP Binding Cassette Transporter, Subfamily Bmultidrug resistance protein 3CholestasisLiverMagnetic resonance cholangiopancreatographyMagnetic resonance imagingProgressive familial intrahepatic cholestasis

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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.