High Prevalence of Wilson's Diseases with Low Prevalence of Kayser-Fleischer Rings among Patients with Cryptogenic Chronic Liver Diseases in Bangladesh.

Abstract

Background: Chronic liver disease (CLD) is common in Bangladesh; however, a major bulk remains as cryptogenic CLD as they remain devoid of known pathological agents leading to have a check of Kayser-Fleischer (K-F) rings for possible Wilson's disease (WD) and many of these patients develop complications such as cirrhosis of liver and hepatocellular carcinoma. However, there remains considerable proportions of CLD patients with undefined etiology (cryptogenic CLD) and these patients cannot be provided effective therapy based on etiological factors. Here, the proportion of WD among cryptogenic CLD patients in Bangladesh has been evaluated to improve the management of CLD and reduce complications.

Materials and methods: A total of 941 patients with cryptogenic CLD [negative for hepatitis viruses, alcohol, nonalcoholic fatty liver disease (NAFLD), drug, and autoimmunity] were enrolled in the study. To assess if they have been suffering from WD, the levels of copper in 24-hour urine were evaluated. Definitive WD was diagnosed when 24-hour urinary copper output was >100 μg and strongly indicative WD patients excreted >40 μg of copper in 24 hours.

Results: Out of 941 patients with cryptogenic CLD, 212 patients were diagnosed as definitive WD and 239 patients as strongly indicative WD on the basis of 24-hours copper excretion. The age distribution ranging of the patients varied from 1 year to 90 years. There was a male predominance. Considerable numbers of WD patients had previous history of jaundice. Kayser-Fleischer rings were mostly uncommon and detected in five patients with WD only.

Discussion: Wilson's disease is not a rare entity in Bangladesh; rather, it seems to be fairly common among CLD patients. A country-wide epidemiological survey should be conducted for diagnosis of WD in Bangladesh to provide a proper management strategy for these huge numbers of WD patients. In fact, most of the WD patients are unaware of their diagnosis and the general physicians are equally unaware of diagnosis and management of WD.

How to cite this article: Choudhury N, Quraishi SB, Atiqullah AKM, et al. High Prevalence of Wilson's Diseases with Low Prevalence of Kayser-Fleischer Rings among Patients with Cryptogenic Chronic Liver Diseases in Bangladesh. Euroasian J Hepato-Gastroenterol 2019;9(2):67-70.