Clinical, Laboratorial and Evolutionary Aspects of Pediatric Patients With Liver Disease Due to Alpha 1-Antitrypsin Deficiency
Mariana Pena COSTA,
Alexandre Rodrigues FERREIRA,
Adriana Teixeira RODRIGUES
et al.
Abstract:Background: Alpha 1-antitrypsin deficiency (AATD) is a hereditary codominant autosomal disease. This liver disease ranges from asymptomatic cases to terminal illness, which makes early recognition and diagnosis challenging. It is the main cause of pediatric liver transplantation after biliary atresia. Objective: To describe the clinical characteristics, as well as those of histologic and laboratory tests, phenotypic and/or genetic evaluation and evolution of a cohort of pediatric patients with AATD. Methods:… Show more
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