Comparison of Serum Zinc in Children of Wilson Disease and Non-Wilsonian Volunteers in Bangladesh.

Mymensingh medical journal : MMJ Pub Date : 2023-07-01
M S Alam, M B Karim, M W Mazumder, S Begum, M Benzamin, M H Rahman, M M Hassan, M A Rahman, M Mondal, D Saha, S A Biswas
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Abstract

Wilson disease (WD) is an autosomal recessive disorder of copper metabolism with diverse clinical manifestations. Zinc (Zn) has been used for treatment of WD. Recent studies showed low serum zinc level in patients suffering from WD than the normal. This cross-sectional analytical study has been designed to compare the serum zinc level between paediatric patients suffering from WD but yet not started treatment and children who have normal ALT level. This study was carried out at the Department of Pediatric Gastroenterology and Nutrition, BSMMU, Dhaka, Bangladesh from July 2018 to June 2019. Total 51 children were included in this study. Among them 27 were diagnosed case of WD aged between three to eighteen years and 24 children of same ages who were suffering from other than liver disease having normal ALT were included as volunteers. The patients of WD were divided into four groups according to their presentation as acute hepatitis, chronic liver disease (CLD), acute liver failure & neuropsychiatric manifestation. Informed written consent was obtained from all patients and volunteers for participation in this study. Along with other physical findings and laboratory investigations 3 ml of venous blood were collected for estimation of serum zinc level. After estimation of serum zinc level results were analyzed statistically. The difference in serum zinc levels were compared between the groups. Serum zinc level was significantly lower in Wilson disease patients (43.8±19.7μg/dl; range: 13-83) compared to volunteers group (67.8±11.8μg/dl; range: 47-97) p<0.001. Among the diseased group, serum zinc level were significantly lower in 18 CLD (38.4±17.4μg/dl) and in 4 acute liver failure (33.1±3.7μg/dl) compared to 4 acute hepatitis (71.8±4.3μg/dl) (p=0.001) and (p<0.001) respectively. Mean serum zinc level was low in 4 Wilsonian acute liver failure (33.1±3.7μg/dl), which was significant compared to those (23) who presented as Wilson disease non acute liver failure (45.7±20.8μg/dl) (p=0.013). Serum zinc level was significantly lower in Wilson disease children compared to the volunteers. Zinc level was also found significantly low in Wilson disease presented as CLD and acute liver failure in comparison to Wilson disease presented as acute hepatitis.

孟加拉国Wilson病儿童和非Wilson病志愿者血清锌的比较。
威尔逊病(WD)是一种常染色体隐性铜代谢疾病,临床表现多样。锌(Zn)已被用于治疗WD。最近的研究表明,WD患者的血清锌水平比正常人低。本横断面分析研究旨在比较患有WD但尚未开始治疗的儿童患者和ALT水平正常的儿童的血清锌水平。本研究于2018年7月至2019年6月在孟加拉国达卡BSMMU儿科胃肠病学和营养学系进行。本研究共纳入51名儿童。其中年龄在3 ~ 18岁之间诊断为WD的27例,同时选取24例同年龄的非肝脏疾病且ALT正常的儿童作为志愿者。根据临床表现分为急性肝炎、慢性肝病(CLD)、急性肝功能衰竭和神经精神表现四组。参与本研究的所有患者和志愿者均获得知情书面同意。除其他物理检查和实验室检查外,采集静脉血3ml用于估计血清锌水平。测定血清锌水平后,对结果进行统计学分析。比较各组血清锌水平的差异。肝豆状核变性患者血清锌水平显著降低(43.8±19.7μg/dl;范围:13-83),与志愿者组相比(67.8±11.8μg/dl;范围:47-97)p
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