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Managing Late-Onset Genetic Cholestasis: Drawing on Pediatric PFIC Expertise

Clinical Thought

Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of disorders caused by variations in multiple genes that are critical for bile formation and transport. Typically, PFIC has been identified as a severe condition presenting in young children, and it has been managed by pediatric specialists. However, advances in genetic diagnosis have revealed that PFIC also affects adolescents and adults. In addition, with improvements in PFIC management, more pediatric patients are transitioning to adult care. In this commentary, a specialist in management of pediatric PFIC shares insights relevant to the care of these diseases in adult patients.

Released: October 02, 2026

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Provided by

Provided by Clinical Care Options, LLC dba Decera Clinical Education in partnership with Progressive Familial Intrahepatic Cholestasis (PFIC) Network.

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Supporters

This activity is supported by Ipsen Biopharmaceuticals, Inc

Ipsen Biopharmaceuticals Inc

Partners

Progressive Familial Intrahepatic Cholestasis (PFIC) Network

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Target Audience

This activity is intended for hepatologists, gastroenterologists, nurses, nurse practitioners, physician associates, pharmacists, and other healthcare professionals involved in the management of adult patients with or at risk of PFIC and ALGS.

Learning Objectives

Upon completion of this activity, participants should be able to:

  • Implement a multidisciplinary care plan that integrates long-term disease monitoring, transition of care planning, and family-centered genetic counseling for patients with PFIC and ALGS

Financial Disclosures

Primary Author

James E. Squires, MD, MS: consultant/advisor/speaker: Ipsen, Mirum; data and safety monitoring board: Sanofi.