Arteriohepatic Dysplasia (Alagille Syndrome) in a Child (Clinical Case)

Authors

  • Ye.V. Omelchenko Kharkiv National Medical University
  • A.S. Senatorova Kharkiv National Medical University
  • A.F. Shipko Kharkiv National Medical University
  • A.V. Omelchenko-Seliukova Kharkiv National Medical University
  • M.N. Yermolaiev Municipal Healthcare Institution «Regional Children’s Clinical Hospital», Kharkiv, Ukraine
  • M.M. Yermolaieva Municipal Healthcare Institution «Regional Children’s Clinical Hospital», Kharkiv, Ukraine
  • Yu.A. Levchenko Municipal Healthcare Institution «Regional Children’s Clinical Hospital», Kharkiv, Ukraine

DOI:

https://doi.org/10.22141/2224-0551.2.1.62.1.2015.83453

Keywords:

arteriohepatic dysplasia, Alagille syndrome, children.

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.

References

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Published

2015-09-01

Issue

Section

Pediatric Gastroenterology