Department of Gastroenterology, The First Affiliated Hospital of Guangdong Pharmaceutical University, Guangzhou 510000, Guangdong Province, China.
Department of Gastroenterology, The First Affiliated Hospital of Xi'an Jiao Tong University, Xi'an 710000, Shaanxi Province, China.
World J Gastroenterol. 2019 Jan 21;25(3):378-387. doi: 10.3748/wjg.v25.i3.378.
Cirrhosis is a chronic late stage liver disease associated with hepatitis viruses, alcoholism, and metabolic disorders, such as Wilson disease (WD). There are no clear markers or clinical features that define cirrhosis originating from these disparate origins. We hypothesized that cirrhosis is not one disease and cirrhosis of different etiology may have differential clinical hepatic features.
To delineate the liver features between WD-associated cirrhosis and hepatitis B-associated cirrhosis in the Chinese population.
In this observational study, we reviewed the medical data of consecutive inpatients who had WD-associated cirrhosis or hepatitis B-associated cirrhosis from January 2010 to August 2018, and excluded patients who had carcinoma, severe heart or pulmonary diseases, or other liver diseases. According to the etiology of cirrhosis, patients were divided into two groups: WD-associated cirrhosis group (60 patients) and hepatitis B-associated cirrhosis group (56 patients). The liver fibrosis degree, liver function indices, and portal hypertension features of these patients were compared between the two groups.
No inter-group differences were observed in the diagnostic liver fibrosis markers, however, clinical features clearly defined the origin of cirrhosis. WD-associated cirrhosis patients (16-29 years) had lower levels of alanine transaminase, aspartate transaminase, and bilirubin, lower prothrombin time, lower incidence of hepatic encephalopathy, and lower portal vein diameter ( < 0.05), compared to cirrhosis resulting from hepatitis B in older patients (45-62 years). Importantly, they had decreased risks of progression from Child-Pugh grade A to B (odds ratio = 0.046, 95% confidence interval: 0.006-0.387, = 0.005) and of ascites (odds ratio = 0.08, 95% confidence interval: 0.01-0.48, = 0.005). Conversely, WD-associated cirrhosis patients had a higher risk of splenomegaly (odds ratio = 4.15, 95% confidence interval: 1.38-12.45, = 0.011).
WD-associated cirrhosis presents a higher risk of splenomegaly associated with leukopenia and thrombocytopenia, although revealing milder liver dysfunction and portal hypertension symptoms, which recommends WD patients to be monitored for associated complications.
肝硬化是一种慢性晚期肝脏疾病,与肝炎病毒、酒精中毒和代谢紊乱有关,如威尔逊病(WD)。目前尚无明确的标志物或临床特征可以定义源自这些不同病因的肝硬化。我们假设肝硬化不是一种疾病,不同病因的肝硬化可能具有不同的临床肝脏特征。
描述中国人群中 WD 相关性肝硬化和乙型肝炎相关性肝硬化的肝脏特征。
在这项观察性研究中,我们回顾性分析了 2010 年 1 月至 2018 年 8 月期间连续住院的 WD 相关性肝硬化或乙型肝炎相关性肝硬化患者的临床资料,排除了合并肝癌、严重心肺疾病或其他肝脏疾病的患者。根据肝硬化的病因,将患者分为 WD 相关性肝硬化组(60 例)和乙型肝炎相关性肝硬化组(56 例)。比较两组患者的肝纤维化程度、肝功能指标和门静脉高压特征。
两组诊断性肝纤维化标志物无组间差异,但临床特征明确了肝硬化的病因。与乙型肝炎相关的老年肝硬化患者(45-62 岁)相比,WD 相关性肝硬化患者(16-29 岁)的丙氨酸转氨酶、天冬氨酸转氨酶和胆红素水平较低,凝血酶原时间较短,肝性脑病发生率较低,门静脉直径较小(均<0.05)。重要的是,他们从 Child-Pugh 分级 A 进展为 B 的风险较低(比值比=0.046,95%置信区间:0.006-0.387,=0.005),腹水的风险也较低(比值比=0.08,95%置信区间:0.01-0.48,=0.005)。相反,WD 相关性肝硬化患者脾肿大的风险更高(比值比=4.15,95%置信区间:1.38-12.45,=0.011)。
WD 相关性肝硬化表现为脾肿大相关的白细胞减少和血小板减少的风险较高,尽管肝功能和门静脉高压症状较轻,但建议对 WD 患者进行相关并发症的监测。