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Cases of Confounding Cholestasis: Navigating the Diagnostic and Therapeutic Challenges of PFICs

Slideset

Delayed recognition of progressive familial intrahepatic cholestasis (PFIC) can contribute to ongoing symptoms, disease progression, and missed opportunities for appropriate management. This downloadable slideset reviews key considerations for diagnosing PFIC, selecting and interpreting genetic testing, and individualizing treatment decisions based on disease characteristics and patient needs. Additional content highlights management of cholestatic pruritus, the evolving role of IBAT inhibitors, and strategies for lifelong monitoring, surveillance, and multidisciplinary care.

Released: November 04, 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:

  • Assess for late-onset and adult-presenting genetic cholestatic disorders in patients with suspected mimics or established diagnoses that warrant reevaluation

  • Apply an evidence-informed diagnostic framework that incorporates systematic use of genetic testing to evaluate subtype-specific presentations in patients with suspected PFIC or ALGS across the lifespan

  • Devise individualized, evidence-informed treatment and symptom management strategies for adults with PFIC and Alagille syndrome

  • 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 Alagille syndrome

Additional Disclosures

Generative artificial intelligence (AI) tools were used for generating clinical case(s) or scenario(s) and drafting assessment question(s) and/or rationale(s). All AI-assisted content underwent human review, editing, and validation by qualified faculty and accredited education staff to ensure scientific accuracy, balance, and compliance with the ACCME Standards for Integrity and Independence in Accredited Continuing Education.