Arteriohepatic Dysplasia (Alagille Syndrome) in a Child (Clinical Case)
Keywords:arteriohepatic dysplasia, Alagille syndrome, children.
AbstractThe 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.
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Copyright (c) 2015 Ye.V. Omelchenko, A.S. Senatorova, A.F. Shipko, A.V. Omelchenko-Seliukova, M.N. Yermolaiev, M.M. Yermolaieva, Yu.A. Levchenko
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