Clinical Characteristics and Comparison of Different Prognostic Scores in Wilson\'s Disease

Abstract

A bstract Aim Wilson's disease (WD) is a rare autosomal recessive disease, that can involve any organ of the body, the main ones being the liver and the brain. These patients can have varied presentations, ranging from having no symptoms to having neurological manifestations to features of chronic liver disease (CLD). Those patients that end up having CLD are prognosticated via the Child–Turcotte–Pugh (CTP) score and the Model for End-stage Liver Disease (MELD) score. However, two specific scores exist for prognostication in patients having WD, namely, the Nazar score and the Dhawan score. However, these are yet to be validated nor has their use been implemented in clinical practice. Materials and methods Our study involved 65 patients with WD, comprising both the pediatric and the adult population. We aimed at evaluating the clinical manifestations the lab parameters and the management of these patients. Furthermore, we tried validating the Nazar and the Dhawan score and later compared them with the CTP and the MELD score, which are well-known prognostic tools in CLD. Results Our patients were subdivided into the pediatric (more than 50%) and the adult group. The most common presenting complaint noted in both groups was abdominal distension. Values of the urine copper and serum ceruloplasmin did not defer between the pediatric and adult patients. Hepatic involvement is frequently seen in the pediatric age-group. Also, CTP class C was chiefly seen in pediatrics 17/33 (51.5%), while CTP class B was in adults 13/32 (40.6%). The mean Nazar score was 3 ± 3, while the mean Dhawan score was 5 ± 4. The main treatment offered for both groups was zinc along with penicillamine. Conclusion Our study showed the Dhawan score was comparable to the CTP and the MELD score in terms of predicting the disease severity of WD in our patient population. How to cite this article Majid Z, Abrar G, Laeeq SM, et al . Clinical Characteristics and Comparison of Different Prognostic Scores in Wilson's Disease. Euroasian J Hepato-Gastroenterol 2022;12(2):69–72.

The history of Wilson disease

“…Additionally, there are several other scores: the revised Nazer King's prognostic score in WD (the New Wilson Index) published by Anil Dhawan et al in 2005 to assist management decisions in patients with acute liver injury, [121] the Unified Wilson's Disease Rating Scale proposed by Anna Czlonkowska et al in 2007 [122] (later evaluated [123] ), and also a Global Assessment Scale from Aggarwal et al [124] While these can be used to score the clinical features in patients with WD, other comparisons of various rating scales in WD have also been published. [125] As with other clinically applicable scores and indices, it is mandatory to review such calculations periodically, as experience with other prognostic instruments has shown that their reliability may need to be scrutinized [126] from time to time.…”