Department of Medicine and Molecular Science, Gunma University Graduate School of Medicine, Japan.
J Clin Pathol. 2009 Nov;62(11):1043-5. doi: 10.1136/jcp.2009.070680. Epub 2009 Aug 30.
Non-alcoholic steatohepatitis (NASH) is characterised by steatosis, liver cell injuries, the presence of a mixed inflammatory lobular infiltrate, and variable degrees of fibrosis. Werner syndrome (WS) is a rare autosomal recessive disease characterised by the premature onset of multiple age-related disorders. Central obesity and insulin resistance are common symptoms of both NASH and WS. Three cases were studied to evaluate the association between WS and NASH. NASH was diagnosed by liver biopsies and imaging studies following the exclusion of alcohol consumption, viral disease or autoimmune liver disease. Liver histology was compatible with NASH in all cases. Liver dysfunction, hyperlipidaemia, insulin resistance and regional increase of intra-abdominal fat even though the body mass indices were all normal or low, were observed. Metabolic disorders due to WS may complicate and cause NASH. Hence, the observed clinical association between WS and NASH suggests that patients with WS should also be screened for NASH.
非酒精性脂肪性肝炎(NASH)的特征是脂肪变性、肝细胞损伤、混合性炎症小叶浸润以及不同程度的纤维化。 Werner 综合征(WS)是一种罕见的常染色体隐性遗传病,其特征是多种与年龄相关的疾病过早发生。中心性肥胖和胰岛素抵抗是 NASH 和 WS 的常见症状。本研究分析了 WS 和 NASH 之间的相关性。NASH 通过排除饮酒、病毒性疾病或自身免疫性肝病后进行肝活检和影像学研究来诊断。所有病例的肝组织学均符合 NASH。观察到肝功能障碍、高脂血症、胰岛素抵抗和区域腹内脂肪增加,尽管体重指数正常或较低。WS 引起的代谢紊乱可能会使 NASH 复杂化和加重。因此,WS 与 NASH 之间的临床关联表明,WS 患者也应筛查 NASH。