Yilmaz Guldal, Sari Sinan, Egritas Odul, Dalgic Buket, Akyol Gulen
Gazi University, Faculty of Medicine, Department of Pathology, Ankara, Turkey.
Pediatr Dev Pathol. 2012 Mar-Apr;15(2):107-13. doi: 10.2350/11-04-1017-OA.1. Epub 2011 Dec 8.
Hepatoportal sclerosis (HPS) is a syndrome of obscure etiology, and is one of the causes of noncirrhotic portal hypertension (PH). We aimed to investigate this heterogeneous group of patients whose presentation showed cholestatic features, histopathologically. Between 1999 and 2009, 12 children diagnosed with HPS were retrospectively evaluated. HPS was diagnosed with evidence of PH, noncirrhotic liver biopsy with typical histopathologic findings, and exclusion of other possible causes of PH. The data was obtained from pathology reports and microscopic slides. In histopathological re-evaluation fibrosis state, aberrant portal vessels, portal tract dilation and inflammation, ductular reaction, regenerative nodular hyperplasia, acinar transformation, presence of bile pigment, and cholangitis were noted. Serum alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl, alkaline phosphatase, bilirubin, and albumin levels, presentation patterns, and radiologic findings were assessed. Familial relationship degrees were also investigated. Twelve patients (9 boys, 3 girls; 3-180 months) were re-evaluated. Two pairs of the patients were siblings. Parents of 7 patients were consanguine. The most common presenting symptom was abdominal distension. Histopathologically, all patients had hepatoportal sclerosis/intimal fibrous thickening of portal vein and periportal fibrosis, acinar transformation, and regenerative nodules not surrounded by fibrous septae. Eight patients had vascular aberrations, 7 had ductular reaction, 1 showed mild cholangitis, and 1 had canalicular bile pigment. We conclude that genetic predisposition might be a possible factor for HPS development in Turkish patients and it should be kept in mind that cholestatic features noticed in histopathological evaluation may represent a variant group in the spectrum of HPS.
肝门脉硬化症（HPS）是一种病因不明的综合征，是非肝硬化性门静脉高压（PH）的病因之一。我们旨在从组织病理学角度研究这一临床表现具有胆汁淤积特征的异质性患者群体。1999年至2009年期间，对12例诊断为HPS的儿童进行了回顾性评估。HPS的诊断依据为存在PH证据、具有典型组织病理学表现的非肝硬化肝活检结果，以及排除其他可能的PH病因。数据来自病理报告和显微镜玻片。在组织病理学重新评估中，记录了纤维化状态、异常门静脉血管、门静脉扩张和炎症、小胆管反应、再生结节增生、腺泡转化、胆色素存在情况以及胆管炎。评估了血清丙氨酸氨基转移酶、天冬氨酸氨基转移酶、γ-谷氨酰转肽酶、碱性磷酸酶、胆红素和白蛋白水平、临床表现模式以及影像学检查结果。还调查了家族关系程度。对12例患者（9例男孩，3例女孩；年龄3 - 180个月）进行了重新评估。其中有两对患者是兄弟姐妹。7例患者的父母为近亲结婚。最常见的临床表现为腹胀。组织病理学检查显示，所有患者均有肝门脉硬化/门静脉内膜纤维增厚及门周纤维化、腺泡转化，以及无纤维间隔包绕的再生结节。8例患者存在血管异常，7例有小胆管反应，1例表现为轻度胆管炎，1例有胆小管胆色素。我们得出结论，遗传易感性可能是土耳其患者发生HPS的一个潜在因素，并且应牢记在组织病理学评估中发现的胆汁淤积特征可能代表HPS谱系中的一个变异组。
