Arteriohepatic Dysplasia (Alagille's Syndrome) with Keratoderma
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چکیده
منابع مشابه
Hepatobiliary scan in Alagille syndrome; arteriohepatic dysplasia.
Arteriohepatic dysplasia or congenital paucity of interlobular bile ducts - Alagille Syndrome, is a well defined syndrome characterized by five major features, including chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects, peripheral pulmonary artery hypoplasia or stenosis and facial dysmorphy. The disease is very rare. Only three cases have been reported in Greece...
متن کاملLiver transplantation for arteriohepatic dysplasia (Alagille's syndrome).
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 the other two at 4 and 10 months after the OLT. Vascular complications with associated septicemia ...
متن کاملArteriohepatic dysplasia (Alagille's syndrome): a common cause of conjugated hyperbilirubinemia.
Syndromatic paucity of interlobular bile ducts is a common cause of conjugated hyperbilirubinemia in children. The clinical presentation is not always obvious. Therefore, the liver biopsy may be a useful diagnostic tool in the definition of this entity. The hepatic and biliary morphology of five children with arteriohepatic dysplasia (Allagille' syndrome) is described. Prior to diagnosis, four ...
متن کاملArteriohepatic dysplasia: familial pulmonary arterial stenosis with neonatal liver disease.
Watson, G. H., and Miller, V. (1973). Archives of Disease in Childhood, 48, 459. Arteriohepatic dysplasia: familial pulmonary arterial stenosis with neonatal liver disease. A new syndrome is described of which the salient features are (1) congenital hypoplasia and stenoses of the pulmonary arteries, sometimes with associated cardiovascular malformations; (2) neonatal liver disease, commonly wit...
متن کاملScintigraphic progress of the liver in a patient with Alagille syndrome (arteriohepatic dysplasia).
We encountered a 9-year-old Japanese girl with Alagille syndrome. Her scintigraphic examinations of the liver were performed at the ages of 16 months and 9 years. 99mTc-PMT, a hepatobiliary imaging agent, was distributed homogeneously in the liver at the younger age, but unevenly produced an area of focally increased uptake in the medial segment of the liver surrounded by peripheral atrophy at ...
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ژورنال
عنوان ژورنال: Journal of the Royal Society of Medicine
سال: 1989
ISSN: 0141-0768,1758-1095
DOI: 10.1177/014107688908200517