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 Silva1,2*, Helena Moreira Silva2, Cláudia Melo3, Herculano Rocha4, Margarida Medina4 and Esmeralda Martins1,5 Ermelinda Santos Silva1,2*, Helena Moreira Silva2, Cláudia Melo3, Herculano Rocha4, Margarida Medina4 and Esmeralda Martins1,5
  
  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

  Abstract PDF