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Alagille syndrome 

Alagille syndrome
Chapter:
Alagille syndrome
Source:
Paediatric Gastroenterology, Hepatology and Nutrition (Oxford Specialist Handbooks in Paediatrics) (1 ed.)
Author(s):

R. Mark Beattie

, Anil Dhawan

, and John W.L. Puntis

DOI:
10.1093/med/9780198569862.003.0050

Genetics 358

Clinical features 358

Differential diagnosis 359

Investigations 359

Management: multidisciplinary approach 360

Prognosis 360

Alagille syndrome is a complex multisystem disorder with a prevalence of 1:40 000–1:100 000, characterized by hepatic, cardiac, renal, and ocular involvement. At least three of the following five major criteria are required to establish the diagnosis: ...

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