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镰状细胞病中的慢性肝脏异常:70例在世患者的临床病理研究

Chronic liver abnormalities in sickle cell disease: a clinicopathological study in 70 living patients.

作者信息

Traina Fabiola, Jorge Stefano Gonçalves, Yamanaka Ademar, de Meirelles Luciana R, Costa Fernando Ferreira, Saad Sara T O

机构信息

Hematology and Hemotherapy Center, State University of Campinas, Campinas, Brazil.

出版信息

Acta Haematol. 2007;118(3):129-35. doi: 10.1159/000107744. Epub 2007 Sep 3.

Abstract

BACKGROUND AND AIM

The present study aimed to evaluate the incidence and etiology of chronic liver abnormalities in 70 living patients with sickle cell disease from the Hematology and Hemotherapy Center of the State University of Campinas.

METHODS

Clinical and laboratory investigations, including liver function tests, serological tests for viral hepatitis and abdominal ultrasound, were performed in all patients. Additionally, liver biopsies were taken from 20 patients.

RESULTS

Sixty-seven (96%) patients had some liver abnormality; these included abnormal liver function tests, viral hepatitis, liver ultrasonographic changes or cholelithiasis. The sickling process was the only explanation for the abnormal liver function tests or liver ultrasonographic changes in 24% of these patients. One or more defined reasons, including viral hepatitis, cholelithiasis, clinical hemosiderosis, alcoholism or diabetes, justified the liver abnormalities in 76% of the patients. Nineteen of the 20 liver biopsies presented some degree of vascular lesion; other histological findings were associated with hemosiderosis, viral hepatitis or cholestasis.

CONCLUSIONS

In patients with sickle cell disease, chronic liver abnormalities are frequent and seem to be a multifactorial phenomenon, depending on overlapping factors such as cholelithiasis, viral damage, iron overload and also the primary disease itself.

摘要

背景与目的

本研究旨在评估坎皮纳斯州立大学血液学与血液治疗中心70例镰状细胞病存活患者慢性肝脏异常的发生率及病因。

方法

对所有患者进行了临床和实验室检查,包括肝功能测试、病毒性肝炎血清学检测及腹部超声检查。此外,对20例患者进行了肝活检。

结果

67例(96%)患者存在某种肝脏异常;这些异常包括肝功能测试异常、病毒性肝炎、肝脏超声改变或胆结石。镰变过程是这些患者中24%肝功能测试异常或肝脏超声改变的唯一原因。一个或多个明确原因,包括病毒性肝炎、胆结石、临床血色素沉着症、酗酒或糖尿病,可解释76%患者的肝脏异常。20例肝活检中有19例出现了一定程度的血管病变;其他组织学发现与血色素沉着症、病毒性肝炎或胆汁淤积有关。

结论

在镰状细胞病患者中,慢性肝脏异常很常见,似乎是一种多因素现象,取决于胆结石、病毒损伤、铁过载以及原发性疾病本身等多种重叠因素。

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