Şımşek Halis, Pinar Aslı, Altinbaş Akif, Alp Alparslan, Balaban Yasemin H, Büyükaşik Yahya, Özcebe Osman, Hasçelık Gülşen, Gedıkoğlu Gökhan, Tatar Gonca
Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey.
Turk J Gastroenterol. 2012;23(5):569-73.
BACKGROUND/AIMS: Alpha-1 antitrypsin deficiency causes accumulation of mutant alpha-1 antitrypsin molecules in hepatocytes, and is attributed to severe liver injury even in heterozygous state. However, there is a question as to whether alpha-1 antitrypsin deficiency is only a cause of liver injury or has a worsening effect on the underlying liver disease. We aimed to determine the role of alpha-1 antitrypsin deficiency in the ongoing chronic hepatitic process.
Fifty-four patients with the diagnosis of chronic hepatitis by liver biopsy (36 chronic hepatitis B virus, 8 chronic hepatitis C virus, 7 non-alcoholic steatohepatitis, 2 primary biliary cirrhosis, and 1 autoimmune hepatitis) and 51 age- and sex-matched control subjects chosen from among healthy blood donors were included in the study. Isoelectric focusing for identifying alpha-1 antitrypsin phenotypes was performed in all patients and control subjects, whereas the histopathological examination was done only in patients.
Alpha-1 antitrypsin-deficient variant was absent in patients and controls. The mean serum alpha-1 antitrypsin level was significantly lower in patients (157.4 ± 33 mg/dl) than controls (134.8 ± 30 mg/dl) (p<0.00). Histological activity index and fibrosis grade in the liver were not related to the serum alpha-1 antitrypsin level (p: 0.276 and 0.902, respectively). Additionally, the serum alpha-1 antitrypsin levels among normal variants of alpha-1 antitrypsin did not differ according to the underlying liver diseases (p: 0.928).
This prospective case-control study could not define any additional effect of alpha-1 antitrypsin deficiency on liver histopathology in chronic hepatitis patients.
背景/目的:α1抗胰蛋白酶缺乏症会导致突变的α1抗胰蛋白酶分子在肝细胞中蓄积,即使在杂合状态下也会引发严重肝损伤。然而,α1抗胰蛋白酶缺乏症仅仅是肝损伤的一个病因,还是会使潜在的肝脏疾病恶化,这一问题尚存争议。我们旨在确定α1抗胰蛋白酶缺乏症在慢性肝炎进程中的作用。
本研究纳入了54例经肝活检确诊为慢性肝炎的患者(36例慢性乙型肝炎病毒感染、8例慢性丙型肝炎病毒感染、7例非酒精性脂肪性肝炎、2例原发性胆汁性肝硬化和1例自身免疫性肝炎)以及51名从健康献血者中选取的年龄和性别匹配的对照者。所有患者和对照者均进行了用于鉴定α1抗胰蛋白酶表型的等电聚焦分析,而仅对患者进行了组织病理学检查。
患者和对照者中均未发现α1抗胰蛋白酶缺乏变异体。患者的血清α1抗胰蛋白酶平均水平(157.4±33mg/dl)显著低于对照者(134.8±30mg/dl)(p<0.00)。肝脏的组织学活性指数和纤维化分级与血清α1抗胰蛋白酶水平无关(p值分别为0.276和0.902)。此外,α1抗胰蛋白酶正常变异体中的血清α1抗胰蛋白酶水平在不同潜在肝脏疾病之间并无差异(p值为第0.928)。
这项前瞻性病例对照研究未能明确α1抗胰蛋白酶缺乏症对慢性肝炎患者肝脏组织病理学的任何额外影响。