About this Activity

Alagille syndrome (ALGS) is a rare, life-threatening, multisystem disorder that primarily affects the liver but also involves cardiac, renal, neurological, vascular, and ophthalmic systems.   

With an estimated prevalence of 1 in 30,000 live births, Alagille Syndrome is frequently misdiagnosed, which has significant impacts on the outcomes for these patients. It is perhaps one of the most misunderstood and misdiagnosed of all the rare paediatric liver diseases.

The Global Alagille Alliance (GALA) Study Group was established to examine ALGS progression in a diverse, global cohort of children. Its goals included assessing native liver survival (NLS), identifying early laboratory predictors of long-term outcomes, and evaluating post-transplant survival rates in a large international cohort. In this review, we unpack the high-level findings of the study, and discuss their relevance for the diagnosis and management of the disease.

What You Will Learn

This module intends to provide you with insight into Alagille Syndrome. On completion, you will:
 

1. Gain an understanding of the Rare Paediatric Liver Disease, Alagille Syndrome (ALGS)

2. Gain insight into the impact of the lack of genotypic predictors on disease progression and outcomes

3. Gain insights into the findings of the GALA study and why it matters to clinical practice

4. Gain an understanding of the presentation of Alagille in South Africa
 

This activity is not currently accredited.