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

Zain Majid; Ghazi Abrar; Syed Mudassir Laeeq; Shoaib Ahmed Khan; Hina Ismail; Ghous Bux Soomro; Nasir Mehmood; Abbas Ali Tasneem; Farina Muhammad Hanif; Rajesh Mandhwani; Nasir Hasan Luck

doi:10.5005/jp-journals-10018-1379

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

Euroasian J Hepatogastroenterol. 2022 Jul-Dec;12(2):69-72. doi: 10.5005/jp-journals-10018-1379.

Affiliation

¹ Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation (SIUT), Karachi, Pakistan.

Abstract

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.

Keywords: Child–Turcotte–Pugh score; Dhawan score; Hepatic disease; Kayser-Fleischer rings; Model for End-stage Liver Disease Score; Nazar score; Neurological symptoms; Pakistan; Wilson's disease.