alexa Alpha-1-Antitrypsin Deficiency Presenting as Neonatal Cholestasis: Predictors of Outcome and Effect of Ursodeoxycholic Acid
ISSN: 2167-0889

Journal of Liver
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Research Article

Alpha-1-Antitrypsin Deficiency Presenting as Neonatal Cholestasis: Predictors of Outcome and Effect of Ursodeoxycholic Acid

Ermelinda Santos Silva1,2*, Helena Moreira Silva2, Cláudia Melo3, Herculano Rocha4, Margarida Medina4 and Esmeralda Martins1,5
1Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto, Rua Jorge Viterbo Ferreira, no 228, 4050-313 Porto, Portugal
2Paediatric Gastroenterology Service, Department of Child and Adolescent, Centro Hospitalar do Porto, Largo Abel Salazar, 4099-001 Porto, Portugal
3Paediatric Service, Unidade Hospitalar de Famalicao, Centro Hospitalar do Médio Ave, Rua de Cupertino de Miranda, 4764-958 Vila Nova de Famalicão, Portugal
4Paediatric Service, Department of Child and Adolescent, Centro Hospitalar do Porto; Largo Abel Salazar, 4099-001 Porto, Portugal
5Metabolic Diseases Unit, Paediatric Service, Department of Child and Adolescent, Centro Hospitalar do Porto; Largo Abel Salazar, 4099-001 Porto, Portugal
Corresponding Author : Ermelinda Santos Silva
Serviço de Gastrenterologia Pediátrica
Departamento da Criança e do Adolescente
Centro Hospitalar do Porto
Largo Abel Salazar, 4099-001 Porto, Portugal
Tel: 222 077 500
E-mail: [email protected]
Received July 05, 2015; Accepted July 09, 2015; Published July 20, 2015
Citation: Silva ES, Silva HM, Melo C, Rocha H, Medina M, et al. (2015) Alpha-1- Antitrypsin Deficiency Presenting as Neonatal Cholestasis: Predictors of Outcome and Effect of Ursodeoxycholic Acid. J Liver 4:185. doi:10.4172/2167-0889.1000185
Copyright: © 2015 Silva ES, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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Abstract

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 divided the patients as treated (n=16), and untreated (n=11) with ursodeoxycholic acid. Results: Splenomegaly at admission (P=0.006) and persistent jaundice at 6 months old (P=0.007) were associated with unfavorable outcome. The values of conjugated bilirubin (P=1.000), aspartate aminotransferase (P=1.000), alanine aminotransferase (P=0.371) and gamma-glutamyltransferase (P=0.667) were not significantly different in both groups of outcome. Early treatm e nt with ursodeoxycholic acid was associated with a favorable outcome (P=0.011). Treated patients did not differ significantly from the untreated-ones in biochemical parameters (conjugated bilirubin, aspartate aminotransferase, alanine aminotransferase and gamma-glutamyltransferase), and had significantly lower alpha-1-antitrypsin serum levels (P=0.015). Conclusion: Splenomegaly at admission and persistence of jaundice at 6 months old were predictive for bad prognosis, and early treatment with ursodeoxycholic acid might have interfered positively in the outcome.

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