Article
Neonatal cholestasis in children with Alpha-1-AT deficiency is a risk for earlier severe liver disease with male predominance.
Hepatology communications - 1 Dec 2023
Teckman Jeffrey, Rosenthal Philip, Ignacio Rosalinda V, Spino Cathie, Bass Lee M, Horslen Simon, Wang Kasper, Magee John C, Karpen Saul, Asai Akihiro, Molleston Jean P, Squires Robert H, Kamath Binita M, Guthery Stephen L, Loomes Kathleen M, Shneider Benjamin L, Sokol Ronald J
Abstract excerpt
BACKGROUND: Our objective was to better understand the natural history and disease modifiers of Alpha-1-antitrypsin deficiency (AATD), a common genetic liver disease causing hepatitis and cirrhosis in adults and children. The clinical course is highly variable. Some infants present with neonatal cholestasis, which can resolve spontaneously or progress to cirrhosis; others are well in infancy, only to develop...
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