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Current Gastroenterology Reports
Erschienen in: Current Gastroenterology Reports 11/2023

31.08.2023

Treatment of Cholestasis in Infants and Young Children

verfasst von: Nicole Heinz, Jennifer Vittorio

Erschienen in: Current Gastroenterology Reports | Ausgabe 11/2023

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Abstract

Purpose of Review

Cholestasis is characterized by a conjugated hyperbilirubinemia secondary to impaired bile synthesis, transport, or excretion from the liver. It is always pathologic and can be indicative of an underlying hepatobiliary, genetic, or metabolic disorder, several of which require timely diagnosis to ensure proper management and optimal outcomes. This review provides an overview of the evaluation of cholestasis with a focus on current and emerging treatment strategies.

Recent Findings

Increased accessibility of next generation sequencing (NGS) allows for utilization of genetic testing early in the diagnostic process. This may alter the clinical algorithm for diagnosis of cholestatic disorders. An enhanced understanding of the underlying pathophysiology may help guide future development of targeted therapies, such as ileal bile acid transporter (IBAT) inhibitors. These were recently approved for treatment of cholestatic pruritus in patients with Alagille syndrome and Progressive Familial Intrahepatic Cholestasis.

Summary

Current management of cholestasis is aimed at the biochemical consequences of impaired bile flow, including malnutrition, pruritus, and progressive fibrosis. NGS has led to an enhanced understanding of biliary pathology and may guide development of future treatment modalities based on specific gene mutations. Rapid discernment of the underlying etiology is essential as new treatment modalities emerge.
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Metadaten
Titel
Treatment of Cholestasis in Infants and Young Children
verfasst von
Nicole Heinz
Jennifer Vittorio
Publikationsdatum
31.08.2023
Verlag
Springer US
Erschienen in
Current Gastroenterology Reports / Ausgabe 11/2023
Print ISSN: 1522-8037
Elektronische ISSN: 1534-312X
DOI
https://doi.org/10.1007/s11894-023-00891-8

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