Arteriohepatic Dysplasia (Alagille Syndrome) in a Child (Clinical Case)
Journal Title: Здоров`я дитини - Year 2015, Vol 2, Issue 62
Abstract
The article presents a clinical case of a child with a rare nosology — Alagille syndrome. Among the causes of neonatal cholestasis, Alagille syndrome is ranked second, it occurs with an incidence of 1 per 70,000 of newborns. This syndrome is characterized by an insufficient number or by a small diameter of intrahepatic bile ducts, which carry bile from the liver. Alagille syndrome includes a combination of at least three of the five main symptoms: chronic cholestasis, cardiovascular defects, abnormalities of the spine, eye defects, typical craniofacial signs. The only definitive therapy with the formation of liver cirrhosis and without gross defects is liver transplantation.
Authors and Affiliations
Ye. V. Omelchenko, M. N. Yermolaiev, A. S. Senatorova, A. F. Shipko, A. V. Omelchenko-Seliukova
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