부산대학교 도서관

Background: 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. Objective: This cross sectional observational study has been designed to compare the serum Zinc level in children suffering from different presentation of WD before started treatment. Methods: This work was carried out at the department of Pediatric Gastroenterology and nutrition, BSMMU, Dhaka between July' 2018 to June2019. Total 27 children diagnosed as WD disease, aged between three to eighteen years were included in this study. The patients of WD were divided into four groups according to their presentation as acute hepatitis, decompensated liver disease, acute liver failure and Wilson disease with neurological manifestation. Informed written consent were obtained from all patients 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. Results: Serum Zinc level was founded low in all Wilson disease patients (43.8 ±19.7 [13-83] µg /dl) compared to normal value (64-124µgm. /dl). Among the patients, Serum Zinc level was significantly lower in those who presented as CLD (18 cases, 38.4±17.4µg/dl) and acute liver failure (4 cases, 33.1±3.7µg/dl) compared to those presented as acute hepatitis (4cases, 71.8±4.3 µg/dl) and p value was 0.001 and <0.001 respectively. Mean serum Zinc level was low in 4 patients suffering from acute liver failure (33.1±3.7µg/dl) but was not significant compared to those (23) who presented as Wilson disease non-ALF (45.7±20.8 µg/dl) (P= 0.013) and as Wilson disease CLD (38.4±17.4µg/dl) (p=.25). Conclusion: Serum Zinc level was lowered in patients of Wilson disease. Serum level of zinc was significantly low in patients presented as CLD and acute liver failure in comparison to patients with acute hepatitis. [ABSTRACT FROM AUTHOR]