Liver Involvement in White Patients With Sickle-cell Disease

Samperi, Piera; Consalvo, C.; Romano, Vittorio; Gelardi, Salvatore; Bella, Domenico Di; Schilirò, Gino

doi:10.1001/archpedi.1996.02170360067011

Cited by 7 publications

(2 citation statements)

References 12 publications

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“…73 In an Italian study of patients with sickle cell anemia, sickle cell trait and sickle ␤ thalassemia, only 2 of 142 patients had significantly abnormal liver function tests, with only marginal abnormalities being seen in the rest. 74 Three-to 4-fold elevations were seen in the ALT in these 2 patients, with serum bilirubin levels between 10 to 14 mg/dL. Both patients were positive for HBsAg and for HCV antibody, and the abnormalities in their liver function tests may be explained on this basis.…”

Section: Sickle Cell Hepatopathy In White Populationsmentioning

confidence: 80%

Sickle cell hepatopathy

2001

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Section: Sickle Cell Hepatopathy In White Populationsmentioning

confidence: 80%

Sickle cell hepatopathy

2001

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“…In contrast, patients with microdrepanocytosis rarely show hepatic involvement, the severity of which appears to be mild, particularly in the white Mediterranean population [7]. An Italian study in patients with sickle cell anemia, sickle cell trait, and sickle β thalassemia documented that only 2 of 142 patients had significantly abnormal liver function tests, with marginal abnormalities being seen in the remainder [8]; moreover, both patients were positive for HBsAg and HCV antibody, so that their liver disease could be related to the viral infection rather than to the hematological disorder [8]. …”

Section: Discussionmentioning

confidence: 99%

Liver Cirrhosis in a Patient with Sickle Cell Trait (Hb Sβ<sup>+</sup> Thalassemia) without Other Known Causes of Hepatic Disease

Santi

Montanari

Berardi

et al. 2009

Case Rep Gastroenterol

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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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