Vascular involvement of the liver in Turner's syndrome
Vascular involvement of the liver in Turner's syndrome
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DOI：
10.1002/hep.20026
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发表时间：
2004-01-01
期刊：
影响因子：
13.5
通讯作者：
Valla, D
中科院分区：
文献类型：
--
作者：
Roulot, D;Degott, C;Valla, D
不明原因的肝脏检查异常在特纳综合征患者中很常见。本队列研究旨在阐明这些患者肝脏受累的组织病理学特征、原因和长期结局。对30例肝功能检查结果持续异常的患者进行了8.8 +/- 5.2年的随访。27例患者的肝脏标本可用。10例患者存在明显的结构变化，其中6例为结节性再生性增生，2例为多发性局灶性结节性增生，2例为肝硬化。这些变化经常与闭塞性门静脉病变和主动脉畸形有关。其他17例患者中有15例有轻度至中度门静脉纤维化，9例有炎性浸润，11例有非酒精性脂肪肝。在21例患者中观察到类似于小管硬化性胆管炎的胆管改变（有或无结构改变）。无病毒性、酒精性、自身免疫性或药物性肝损伤。门静脉高压症在4例患者中观察到明显的结构变化，其中包括3例顽固性腹水或复发性静脉曲张出血，其中1例接受移植。无明显结构改变的患者均未发生进展性或失代偿性肝病。没有证据表明雌激素替代疗法会引起肝毒性。总之，特纳综合征肝脏受累的主要原因是血管疾病（可能是先天性的）和非酒精性脂肪肝。在血管疾病患者中，可能会发生需要肝移植的严重肝病。雌激素治疗似乎与发病无关。
Unexplained liver test abnormalities are frequent in patients with Turner's syndrome. This cohort study was performed to clarify the histopathologic features, causes, and long-term outcome of liver involvement in these patients. Thirty patients with persistently abnormal liver test results were followed-up for 8.8 +/- 5.2 years. Liver specimens were available in 27 patients. Marked architectural changes were present in 10 patients, including nodular regenerative hyperplasia in six, multiple focal nodular hyperplasia in two, and cirrhosis in two patients. These changes frequently were associated with obliterative portal venopathy lesions and with aortic malformations. There was mild to moderate portal fibrosis in 15 of the 17 other patients, inflammatory infiltrates in nine patients, and nonalcoholic fatty liver disease in 11 patients. Bile duct alterations resembling small duct sclerosing cholangitis were observed in 21 patients (with or without architectural changes). There was no viral, alcoholic, autoimmune, or drug-induced liver damage. Portal hypertension was observed in four patients with marked architectural changes, including three in whom refractory ascites or recurrent variceal bleeding developed, one of whom underwent transplantation. None of the patients without marked architectural changes experienced progressive or decompensated liver disease. There was no evidence of liver toxicity from estrogen replacement therapy. In conclusion, the main causes of liver involvement in Turner's syndrome are vascular disorders, probably of a congenital origin, and nonalcoholic fatty liver disease. In patients with vascular disorders, severe liver disease requiring liver transplantation may develop. Estrogen therapy does not appear to be pathogenically implicated.