Journal of Liver

Ermelinda Santos Silva

Publications

• Research Article
  Alpha-1-Antitrypsin Deficiency Presenting as Neonatal Cholestasis: Predictors of Outcome and Effect of Ursodeoxycholic Acid
  Author(s): Ermelinda Santos Silva, Helena Moreira Silva, Cláudia Melo, Herculano Rocha, Margarida Medina and Esmeralda MartinsErmelinda Santos Silva, Helena Moreira Silva, Cláudia Melo, Herculano Rocha, Margarida Medina and Esmeralda Martins
  
  Background and objective: Alpha-1-antitrypsin deficiency presenting as neonatal cholestasis occurs in a small percentage of affected individuals. The prognosis is variable, from “healing” to liver cirrhosis and/or severe hepatocellular failure, requiring liver transplantation. We researched for predictors of outcome, including the effect of ursodeoxycholic acid. Methods: Retrospective cohort study of 27 cases of neonatal cholestasis due to alpha-1-antitrypsin deficiency, in the period between 1985 and 2013. Inclusion criteria: patients with neonatal cholestasis and ZZ phenotype. Exclusion criteria: presence of other diagnosis or known risk factors for developing neonatal cholestasis. We analyzed several clinical, biochemical, histological and therapeutic variables. Patients were categorized into two groups: favorable outcome (n=18), unfavorable outcome (n=9). We also divid.. View More»
  DOI: 10.4172/2167-0889.1000185

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