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

Main Article Content

Ye.V. Omelchenko
A.S. Senatorova
A.F. Shipko
A.V. Omelchenko-Seliukova
M.N. Yermolaiev
M.M. Yermolaieva
Yu.A. Levchenko


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.

Article Details

How to Cite
Omelchenko, Y., A. Senatorova, A. Shipko, A. Omelchenko-Seliukova, M. Yermolaiev, M. Yermolaieva, and Y. Levchenko. “Arteriohepatic Dysplasia (Alagille Syndrome) in a Child (Clinical Case)”. CHILD`S HEALTH, no., Sept. 2015, pp. 128-32, doi:10.22141/2224-0551.
Pediatric Gastroenterology


Дегтярева А.В. Дифференциальная диагностика и принципы этиопатогенетического лечения заболеваний печени и желчных путей у новорожденных и детей раннего возраста: Автореф. дис… д-ра медицинских наук. — М., 2010. — 40 с.

Fabris L., Cadamuro M., Guido M., Spirli C., Fiorotto R., Colledan M., Torre G., Alberti D., Sonzogni A., Okolicsanyi L., Strazzabosco M. Analysis of Liver Repair Mechanisms in Alagille Syndrome and Biliary Atresia Reveals a Role for Notch Signaling // Am. J. Pathol. — 2007. — 171 (2). — 641-653.

Hirschfield G. Novel perspectives on the pathogenesis of primary biliary cirrhosis and primary sclerosing cholangitis // EALS Postgra­duate course «Cholestatic diseases of the liver and bile ducts». — Berlin, 2011. — P. 3-69.

Kamath B., Munos P., Bab N., Baker A. et al. A longitudinal Study to Identify laboratory predictors of liver disease outcome in Alagille syndrome // J. Pediatr. Gastroenterol. Nutr. — 2010. — 50 (5). — 526-530.

Ling S.C. Сongenital cholestatic syndromes: what happens when children grow up? // Can. J. Gastroenterol. — 2007. — 21 (11). — 743-751.

Rauch R., Hofbeck M., Zweier C., Koch A., Zink S., Trautmann U., Hoyer J., Kaulitz R., Singer H., Rauch A. Comprehensive genotype-phenotype analysis in 230 patients with tetralogy of Fallot // J. Med. Genet. — 2010. — 47. — 321-331.

Most read articles by the same author(s)