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埃及年轻重型β地中海贫血患者的肝纤维化:与丙型肝炎病毒的关系及螯合治疗的依从性。

Liver fibrosis in young Egyptian beta-thalassemia major patients: relation to hepatitis C virus and compliance with chelation.

机构信息

Department of Pediatric Hematology, Ain Shams University, Cairo 11566, Egypt.

出版信息

Ann Hepatol. 2013 Jan-Feb;12(1):54-61.

Abstract

BACKGROUND

The main causes of liver fibrosis in transfusion-dependent thalassemia major are hepatitis C virus (HCV) infection and hepatic iron overload. The study aimed to assess liver fibrosis in Egyptian adolescents and young adult poly-transfused beta thalassemia patients infected with HCV using liver FibroScan in relation to iron overload and Liver iron concentration (LIC).

MATERIAL AND METHODS

Fifty-one regularly transfused beta thalassemia patients above 12 years old were subjected to measurement of serum alanine transaminase (ALT), serum ferritin (SF), HCV (antibody and RNA), LIC assessed by hepatic R2* and transient elastography (TE) (FibroScan). FibroTest and liver biopsy were done to 25 patients.

RESULTS

Eighty two% of studied thalassemia patients were HCV antibody positive; 21(49%) of them were viremic (HCV RNA positive); median LIC was 12 mg/gm dry weight. There were strong positive correlation between the degree of liver stiffness and Ishak fibrosis score assessed in liver biopsy specimens (P = 0.002) and between FibroScan and FibroTest results (P < 0.001). Patients with HCV viremia showed significantly higher ALT, γ-glutamyl transpeptidase (GGT), SF, LIC and increased liver stiffness compared to patients with no viremia (P = 0.0001, 0.001, 0.012, 0.006 and 0.001) respectively. Liver cirrhosis (TE values > 12.5kPa) was encountered in 23.5% and variable degrees of liver fibrosis (TE values > 6-12.5 kPa) in 35% of studied thalassemic patients.

CONCLUSION

Young beta thalassemia patients with active hepatitis C infection may have hepatic cirrhosis or fibrosis at young age when accompanied with hepatic siderosis. Non invasive Liver FibroScan and Fibro-Test were reliable methods to assess liver fibrosis in young thalassemic-patients.

摘要

背景

导致输血依赖型地中海贫血症患者发生肝纤维化的主要原因是丙型肝炎病毒(HCV)感染和肝铁过载。本研究旨在评估感染 HCV 的埃及青少年和年轻成年β地中海贫血多重输血患者的肝纤维化程度,使用肝脏 FibroScan 评估与铁过载和肝脏铁浓度(LIC)的关系。

材料和方法

对 51 名年龄在 12 岁以上的定期接受输血的β地中海贫血患者进行血清丙氨酸转氨酶(ALT)、血清铁蛋白(SF)、HCV(抗体和 RNA)的测量,使用肝脏 R2* 和瞬态弹性成像(TE)(FibroScan)评估 LIC。对 25 名患者进行 FibroTest 和肝活检。

结果

82%的研究地中海贫血患者 HCV 抗体阳性;其中 21 名(49%)为病毒血症(HCV RNA 阳性);中位 LIC 为 12mg/gm 干重。肝脏硬度与肝活检标本的 Ishak 纤维化评分之间存在很强的正相关(P = 0.002),FibroScan 和 FibroTest 结果之间也存在很强的正相关(P < 0.001)。HCV 病毒血症患者的 ALT、γ-谷氨酰转肽酶(GGT)、SF、LIC 和肝硬度均显著高于无病毒血症患者(P = 0.0001、0.001、0.012、0.006 和 0.001)。研究中 23.5%的地中海贫血患者存在肝硬化(TE 值>12.5kPa),35%的患者存在不同程度的肝纤维化(TE 值>6-12.5kPa)。

结论

患有活动性丙型肝炎感染的年轻β地中海贫血患者可能在年轻时就伴有肝铁过载而出现肝硬化或纤维化。非侵入性肝脏 FibroScan 和 Fibro-Test 是评估年轻地中海贫血患者肝纤维化的可靠方法。

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