Thirteen out of 268 children (less than 18 years old) underwent hepatic transplantation (OLT) for end-stage liver disease (ESLD) associated with arteriohepatic dysplasia (AHD). Seven children are alive and well with normal liver function. Six children died, four within 11 days of the operation and t
Hepatobiliary scintigraphy in arteriohepatic dysplasia (Alagille's syndrome)
โ Scribed by D. A. Summerville; M. Marks; S. T. Treves
- Publisher
- Springer-Verlag
- Year
- 1988
- Tongue
- English
- Weight
- 811 KB
- Volume
- 18
- Category
- Article
- ISSN
- 0301-0449
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We report on a boy with Alagille syndrome. Chromosome analysis on a peripheral blood lymphocyte culture showed a de novo deletion of the short arm of chromosome 20 with a 46,XY,de1(20)(p11.2) chromosome constitution. This is the second report of a del(2Op) in a patient with Alagille syndrome. The po
Arteriohepatic dysplasia (Alagille syndrome) is a congenital cholestatic disease associated with ocular abnormalities. Three Japanese siblings, a 14-year-old girl, an 11year-old boy, and a 9-year-old girl with this syndrome were studied. All three patients showed neonatal jaundice, hepatic dysfuncti