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Santi L, Montanari G, Berardi S, et al. Liver Cirrhosis in a Patient with Sickle Cell Trait (Hb Sβ Thalassemia) without Other Known Causes of Hepatic Disease. Case Rep Gastroenterol. 2009;3(3):275-279doi: 10.1159/000235235.
Santi, L., Montanari, G., Berardi, S., Patti, C., Frigerio, M., Sama, C., Caraceni, P., & Bernardi, M. (2009). Liver Cirrhosis in a Patient with Sickle Cell Trait (Hb Sβ Thalassemia) without Other Known Causes of Hepatic Disease. Case reports in gastroenterology, 3(3), 275-279. https://doi.org/10.1159/000235235
Santi, Luca, et al. "Liver Cirrhosis in a Patient with Sickle Cell Trait (Hb Sβ Thalassemia) without Other Known Causes of Hepatic Disease." Case reports in gastroenterology vol. 3,3 (2009): 275-279. doi: https://doi.org/10.1159/000235235
Santi L, Montanari G, Berardi S, Patti C, Frigerio M, Sama C, Caraceni P, Bernardi M. Liver Cirrhosis in a Patient with Sickle Cell Trait (Hb Sβ Thalassemia) without Other Known Causes of Hepatic Disease. Case Rep Gastroenterol. 2009 Sep 12;3(3):275-279. doi: 10.1159/000235235. PMID: 21103241; PMCID: PMC2988917.
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Case Rep Gastroenterol. 2009 Sep 12;3(3):275-279. doi: 10.1159/000235235.

Liver Cirrhosis in a Patient with Sickle Cell Trait (Hb Sβ Thalassemia) without Other Known Causes of Hepatic Disease.

Case reports in gastroenterology

Luca Santi, Giancarlo Montanari, Sonia Berardi, Corrado Patti, Marta Frigerio, Claudia Sama, Paolo Caraceni, Mauro Bernardi

Affiliations

  1. Department of Clinical Medicine, Alma Mater Studiorum, University of Bologna, Bologna, Italy.

PMID: 21103241 PMCID: PMC2988917 DOI: 10.1159/000235235

Abstract

Liver involvement in patients with sickle cell anemia/trait includes a wide range of alterations, from mild liver function test abnormalities to cirrhosis and acute liver failure. Approximately 15-30% of patients with sickle cell anemia present cirrhosis at autopsy. The pathogenesis of cirrhosis is usually related to chronic hepatitis B or C infection or to iron overload resulting from the many transfusions received by these patients in their lifetime. Thus, cirrhosis has been described almost exclusively in patients with sickle cell anemia, while only mild liver abnormalities have been associated with the sickle cell trait. In the present case study, we describe a young Mediterranean man carrying a sickle cell trait (Hb Sβ(+) thalassemia) who developed liver cirrhosis being negative for hepatitis C and B viruses or for other causes of cirrhosis and not receiving chronic blood transfusions.

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