Clinical Profile and HLA Typing of Autoimmune Hepatitis From Pakistan.
Clinical Profile and HLA Typing of Autoimmune Hepatitis From Pakistan.
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DOI：
10.5812/hepatmon.13598
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发表时间：
2013
影响因子：
0.6
通讯作者：
Zafar MN
中科院分区：
文献类型：
--
作者：
Hassan N;Siddiqui AR;Abbas Z;Hassan SM;Soomro GB;Mubarak M;Anis S;Muzaffar R;Zafar MN
人类白细胞抗原（HLA）分型在自身免疫性肝炎（AIH）已在不同的人群和种族群体进行了调查，但没有这样的数据来自巴基斯坦。本研究的目的是评估自身免疫性肝炎（AIH）的临床特征，并通过HLA分型确定相关抗原和等位基因。本文回顾了近10年来诊断和治疗的58例AIH患者。诊断基于国际AIH组标准。41例患者接受肝活检。对44例患者和912名对照者进行了HLA分型，用血清学方法检测HLA A和B，用序列特异性引物PCR技术检测DR等位基因。58例中，35例为女性（60.3%）。中位年龄为14.5岁（范围4-70岁），AIH评分为14（10-22）。36例（62.0%）为1型AIH，10例（17.2%）为2型AIH，其余12例血清学阴性，活检证实AIH。肝硬化49例（84.4%）。24例（41.4%）患者在就诊时有腹水。在41名接受肝活检的患者中，32名患者患有晚期III期和IV期疾病，20名患者患有严重的炎症。15例患者有其他相关的自身免疫性疾病，1例发展为肝细胞癌。HLA A2（P = 0.036），HLA A9（23）（P = 0.018），HLA A10（25）（P = 0.000），HLA A19（33）（P = 0.000），HLA B15（63）（P = 0.007），HLA B40（61）（P = 0.002），HLA DR 6（P = 0.001）及其亚型HLA-DRB 1 *13（P = 0.032）和HLA-DRB 1 *14（P = 0.017）在AIH中的分布明显高于对照组，差异有统计学意义。在我们地区，AIH表现为主要影响儿童和青少年的晚期疾病。在我们的AIH患者中，HLA DR 6沿着其它等位基因和抗原存在遗传关联。
Human leukocyte antigen (HLA) typing in autoimmune hepatitis (AIH) has been investigated in different populations and ethnic groups, but no such data is available from Pakistan. The aim of this study was to evaluate the clinical profile of autoimmune hepatitis (AIH), and determine the associated antigens and alleles by performing HLA typing. A total of 58 patients, diagnosed and treated as AIH in the last 10 years were reviewed. Diagnosis was based on International AIH Group criteria. Forty one patients underwent liver biopsy. HLA typing was performed in 44 patients and 912 controls by serological method for HLA A and B, and by PCR technique using sequence specific primers for DR alleles. Of 58 cases, 35 were females (60.3%). The median age was 14.5 (range 4-70 years), and AIH score was 14 (10-22). Thirty-six (62.0%) patients had type 1 AIH, 10 (17.2%) type 2, and the remaining 12 were seronegative with biopsy proven AIH. Forty-nine patients (84.4%) had cirrhosis. Twenty-four (41.4%) patients had ascites at the time of presentation. Among 41 patients who underwent liver biopsy, thirty-two had advance stages III and IV disease, and twenty had severe grade of inflammation. Fifteen patients had other associated autoimmune diseases and one developed hepatocellular carcinoma. HLA A2 (P = 0.036), HLA A9 (23) (P = 0.018), HLA A10 (25) (P = 0.000), HLA A19 (33) (P = 0.000), HLA B15 (63) (P = 0.007), HLA B40 (61) ( P = 0.002), HLA DR6 (P = 0.001) with its subtypes HLA-DRB1*13 (P = 0.032) and HLA-DRB1*14 (p = 0.017) were more prevalent in AIH with statistical significance than controls. AIH in our region presents with advanced disease affecting predominantly children and adolescents. There is a genetic association of HLA DR6 along with other alleles and antigens in our patients with AIH.
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影响因子：
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作者：
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作者：
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作者：
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