Published online Jan 21, 2019. doi: 10.3748/wjg.v25.i3.378
Peer-review started: December 9, 2018
First decision: December 28, 2018
Revised: January 3, 2019
Accepted: January 9, 2019
Article in press: January 9, 2019
Published online: January 21, 2019
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 (P < 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, P = 0.005) and of ascites (odds ratio = 0.08, 95% confidence interval: 0.01-0.48, P = 0.005). Conversely, WD-associated cirrhosis patients had a higher risk of splenomegaly (odds ratio = 4.15, 95% confidence interval: 1.38-12.45, P = 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.
Core tip: In Asia, especially China, the incidence of Wilson disease (WD) and its complications is much higher in the younger generation compared to Western societies. This article looks beyond the well-characterized, generalized definition of cirrhosis. It addresses an important but simple question: Can the origin of cirrhosis be classified by clinical features, especially in WD-associated cirrhosis? In this manuscript we define specific clinical characteristics of WD-associated cirrhosis in young patients which are very distinct to those of hepatitis-associated cirrhosis in older patients. These important findings may benefit the clinical diagnosis and ultimate treatment of these younger, vulnerable WD patients.