Citation Information :
Choudhury N, Quraishi SB, Atiqullah A, Khan MS, Akbar SM. High Prevalence of Wilson's Diseases with Low Prevalence of Kayser–Fleischer Rings among Patients with Cryptogenic Chronic Liver Diseases in Bangladesh. Euroasian J Hepatogastroenterol 2019; 9 (2):67-70.
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.
Asrani SK, Devarbhavi H, Eaton J, et al. Burden of liver diseases in the world. J Hepatol 2019;70(1):151–171. DOI: 10.1016/j.jhep.2018.09.014.
Yang JD, Hainaut P, Gores GJ, et al. A global view of hepatocellular carcinoma: trends, risk, prevention and management. Nat Rev Gastroenterol Hepatol 2019;16(10):589–604. DOI: 10.1038/s41575-019-0186-y.
Nalbantoglu I, Jain D. Cryptogenic cirrhosis: old and new perspectives in the era of molecular and genomic medicine. Semin Diagn Pathol 2019;36(6):389–394. DOI: 10.1053/j.semdp.2019.07.003.
Mercado-Irizarry A, Torres EA. Cryptogenic cirrhosis: current knowledge and future directions. Clin Liver Dis (Hoboken) 2016;7(4):69–72. DOI: 10.1002/cld.539.
Czaja AJ. Cryptogenic chronic hepatitis and its changing guise in adults. Dig Dis Sci 2011;56(12):3421–3438. DOI: 10.1007/s10620-011-1769-9.
Rukunuzzaman M, Karim MB, Rahman MM, et al. Wilson's disease in children with blindness: an atypical presentation. Mymensingh Med J 2013;22(1):176–179.
Karim MB, Rahman MM, Islam MS. Wilson's disease with hepatic presentation in childhood. Mymensingh Med J 2007;16(1):29–32. DOI: 10.3329/mmj.v16i1.244.
Rahman S, Siddiqui NI, Paul GK, et al. A case report on Wilson's disease. Mymensingh Med J 2003;12(2):142–145.
Rukunuzzaman M. Wilson's disease in Bangladeshi children: analysis of 100 cases. Pediatr Gastroenterol Hepatol Nutr 2015;18(2):121–127. DOI: 10.5223/pghn.2015.18.2.121.
Alam ST, Rahman MM, Islam KA, et al. Neurologic manifestations, diagnosis and management of Wilson's disease in children - an update. Mymensingh Med J 2014;23(1):195–203.
Ullah AKMA, Maksud MA, Khan SR, et al. Morning (first) urine copper concentration: a new approach for the diagnosis of Wilson's disease. Biol Trace Elem Res 2019;190(2):283–288. DOI: 10.1007/s12011-018-1547-z.
Ala A, Walker AP, Ashkan K, et al. Wilson's disease. Lancet 2007;369(9559):397–408. DOI: 10.1016/S0140-6736(07)60196-2.
Taly AB, Meenakshi-Sundaram S, Sinha S, et al. Wilson disease: description of 282 patients evaluated over 3 decades. Medicine (Baltimore) 2007;86(2):112–121. DOI: 10.1097/MD.0b013e318045a00e.
Mak LY, Cruz-Ramón V, Chinchilla-López P, et al. Global epidemiology, prevention, and management of hepatocellular carcinoma. Am Soc Clin Oncol Educ Book 2018;38:262–279. DOI: 10.1200/EDBK_200939.
Devarbhavi H, Singh R, Adarsh CK, et al. Factors that predict mortality in children with Wilson disease associated acute liver failure and comparison of Wilson disease specific prognostic indices. J Gastroenterol Hepatol 2014;29(2):380–386. DOI: 10.1111/jgh.12356.
Liu J, Luan J, Zhou X, et al. Epidemiology, diagnosis, and treatment of Wilson's disease. Intractable Rare Dis Res 2017;6(4):249–255. DOI: 10.5582/irdr.2017.01057.
Taly AB, Prashanth LK, Sinha S. Wilson's disease: an Indian perspective. Neurol India 2009;57(5):528–540. DOI: 10.4103/0028-3886.57789.