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ALAG, ALaG axiolabiogingival
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alagille syndrome <syndrome> Hypoplasia of the hepatic ducts, congenital pulmonary artery stenosis, facial abnormalities, and other congenital malformations, particularly skeletal. It is often presented as jaundice during the neonatal period. It is an autosomal recessive disease generally manifesting during childhood. "arteriohepatic" refers to the pulmonary artery and the intrahepatic bile ducts, not to the hepatic artery.
(12 Dec 1998)
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  • Alagille Syndrome - »õâ An autosomal dominant MUTATION involving CHROMOSOME 20. It is characterized by the almost normal LIVER that has few or no intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). Other extrahepatic malformations include those in the heart, the eyes, the vertebral column, and the facies. Major clinical features include JAUNDICE, and congenital heart disease with peripheral PULMONARY STENOSIS.
    Synonyms : Alagille-Watson Syndrome, Cholestasis with Peripheral Pulmonary Stenosis, Hepatic Ductular Hypoplasia, Syndromatic, Alagille Watson Syndrome, Arteriohepatic Dysplasias, Dysplasias, Arteriohepatic, Syndrome, Alagille, Syndrome, Alagille-Watson
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Alagille s. an autosomal dominant syndrome of neonatal jaundice, cholestasis with peripheral pulmonic stenosis, and occasionally septal defects or patent ductus arteriosus, due to paucity or absence of intrahepatic bile ducts; it is characterized by unusual facies and ocular, vertebral, and nervous system abnormalities.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
Alagille syndrome see under syndrome.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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