Alagille Syndrome

Overview

4.5 out of 5 (51 Reviews)

Credits

1.00

Post-Assessment Questions

6

Start Date

1 Sep 2023

Last Review Date

6 Mar 2026

Expiration Date

31 Aug 2026

Estimated Time To Finish

60 Minutes


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Activity Description

Alagille syndrome (ALGS) is a rare, autosomal dominant multisystem genetic disorder most commonly caused by pathogenic variants in JAG1 or NOTCH2. The condition is characterized by chronic cholestasis due to paucity of intrahepatic bile ducts and is frequently associated with cardiac, skeletal, ocular, renal, and vascular abnormalities. Clinical presentation is highly variable, ranging from mild hepatic involvement to severe, life-threatening multisystem disease. Common manifestations include jaundice, pruritus, growth failure, congenital heart disease, characteristic facial features, vertebral anomalies, and ocular findings. Advances in molecular diagnostics have improved diagnostic accuracy, while evolving medical and surgical therapies have expanded management options. Early recognition remains essential to reduce morbidity, guide surveillance for complications, and improve long-term outcomes.

This activity for healthcare professionals is designed to enhance clinician competence in the evaluation and management of ALGS across the lifespan. Participants strengthen their ability to recognize the heterogeneous clinical phenotype, apply genetic and diagnostic testing appropriately, and differentiate ALGS from other causes of pediatric cholestasis. The course reviews evidence-based management strategies, including nutritional support, medical therapies such as ileal bile acid transport inhibitors, and indications for surgical or transplant referral. Emphasis is placed on coordinated care involving hepatology, cardiology, nephrology, genetics, nutrition, and psychosocial support. Effective interprofessional collaboration and clear communication with families are highlighted as critical to optimizing quality of life, supporting informed decision-making, and improving long-term patient outcomes.


Target Audience

This activity has been designed to meet the educational needs of physicians, physician associates, nurses, pharmacists, and nurse practitioners.

Learning Objectives

At the conclusion of this activity, the learner will be better able to:

  • Implement evidence-based medical therapies, including nutritional optimization, pruritus management, and bile acid–modifying treatments, to improve patient outcomes.

  • Assess the characteristic physical findings and multisystem involvement in patients with Alagille syndrome.

  • Identify early clinical and laboratory signs that warrant diagnostic evaluation and referral to specialty care.

  • Develop effective communication strategies to collaborate within the interprofessional healthcare team and support shared decision-making with families regarding prognosis, treatment options, and long-term planning.

Disclosures

StatPearls, LLC requires everyone who influences the content of an educational activity to disclose relevant financial relationships with ineligible companies that have occurred within the past 24 months. Ineligible companies are organizations whose primary business is producing, marketing, selling, re-selling, or distributing healthcare products used by or on patients. All relevant conflict(s) of interest have been mitigated. Hover over contributor names for financial disclosures. Others involved in planning this educational activity have no relevant financial relationships to disclose.

Commercial Support: This activity has received NO commercial support.

Continuing Education Accreditation Information

In support of improving patient care, StatPearls, LLC is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC) to provide continuing education for the healthcare team.

 

Physicians and Physician Associates: StatPearls, LLC designates this enduring material for a maximum of 1.00 AMA PRA Category 1 Credit(s)TM. Physicians and PAs should only claim credit commensurate with the extent of their participation in the activity.

American Board of Internal Medicine: Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 1.00 MOC points in the American Board of Internal Medicine’s (ABIM) Maintenance of Certification (MOC) program. It is the CME activity provider’s responsibility to submit participant completion information to ACCME for the purpose of granting ABIM MOC credit.

American Board of Pathology: Successful completion of this Continuing Certification activity, which includes participation in the evaluation component, enables the participant to earn up to 1.00 Lifelong Learning (CME) credits in the American Board of Pathology’s Continuing Certification Program.

American Board of Pediatrics: Successful completion of this CME activity, which includes participation in the evaluation component, enables the learner to earn up to 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program. It is the CME activity provider’s responsibility to submit learner completion information to ACCME for the purpose of granting ABP MOC credit.

American Board of Surgery: Successful completion of this CME activity, which includes participation in the evaluation component, enables the learner to earn credit toward the CME and Self-Assessment requirement(s) of the American Board of Surgery’s Continuous Certification program. It is the CME activity provider's responsibility to submit learner completion information to ACCME for the purpose of granting ABS credit.

American Board of Thoracic Surgery: Successful completion of this CME activity, which includes participation in the evaluation component, enables the learner to earn credit toward the CME and Self-Assessment in Practice requirements of the American Board of Thoracic Surgery’s Maintenance of Certification program. It is the CME activity provider's responsibility to submit learner completion information to ACCME for the purpose of granting ABTS credit.

Royal College of Physicians and Surgeons of Canada: Through an agreement between the Accreditation Council for Continuing Medical Education and the Royal College of Physicians and Surgeons of Canada, medical practitioners participating in the Royal College MOC Program may record completion of accredited activities registered under the ACCME’s “CME in Support of MOC” program in Section 3 of the Royal College’s MOC Program.

Please consult your professional licensing board for information on the applicability and acceptance of continuing education credit for this activity.

Method of Participation and Credit

  1. Register for the activity.    
  2. Review the target audience, learning objectives, and disclosure information.
  3. Study the educational content of the enduring material.
  4. Choose the best answer to each activity test question. To receive credit and a certificate, you must pass the test questions with a minimum score of 100%.
  5. Complete the post-activity assessment survey.

If you have concerns regarding the CE/CME system, please contact support@statpearls.com.

Disclaimer

This educational activity was planned and produced in accordance with the ACCME Standards for Integrity and Independence in Accredited Continuing Education. Faculty may discuss investigational products or off-label uses of products regulated by the FDA. Readers should verify all information before employing any therapies described in this educational activity.

The information provided for this activity is for continuing education purposes only and is not meant to substitute for the independent medical/clinical judgment of a healthcare provider relative to diagnostic and treatment options of a specific patient’s medical condition. The information presented does not necessarily reflect the views of StatPearls or any commercial supporters of educational activities on statpearls.com. StatPearls expressly disclaims responsibility for any adverse consequences resulting directly or indirectly from information in the course, for undetected error, or through a participant's misunderstanding of the content.

Unapproved Uses of Drugs/Devices: In accordance with FDA requirements, the audience is advised that information presented in this continuing education activity may contain references to unlabeled or unapproved uses of drugs or devices. Please refer to the FDA-approved package insert for each drug/device for full prescribing/utilization information.

Cancellation Policy: Please see the cancellation policy. StatPearls, LLC reserves the right to cancel any course due to unforeseen circumstances.

 

 
 

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Unlimited Physician CME

Stay up to date on the latest medical knowledge with 6344 CME activities. In these online self-assessment activities, read our reference articles and test your knowledge with more than 7563 hours of CME.

Learn About Lifetime CME

Single Activity

Take this single activity

$49 1 activity

6 Month Unlimited Physician CME

Access to all the Unlimited Physician CME activities in all specialties.

$399 per half year per user

1 Year Unlimited Physician CME

Access to all the Unlimited Physician CME activities in all specialties.

$649 per 1 year per user