Wilson's Disease: Laboratory Evaluation and Clinical Presentation of Iraqi Sample

Autor: Omar Qahtan Yaseen, Mohammed Qais Al-Ani, Yasin Hamad Majeed
Rok vydání: 2019
Předmět:
Zdroj: Indian Journal of Public Health Research & Development. 10:606
ISSN: 0976-5506
0976-0245
DOI: 10.5958/0976-5506.2019.01342.1
Popis: Wilson's disease (WD) is an inherited defect in copper metabolism that causes accumulation of copper in various body organs. It is treatable if it is diagnosed promptly and treated consistently. In this study, the mode of hepatic presentation constituted (57.14%), The neurological presenting problems in this study was(28.57%). Routine biochemistry testing was recorded for all study patients, So Mean values of serum level of ALT, AST, ALP and bilirubin in patients with WD were 136.83 ± 34.770 U/L, 111.86 ± 32.343 U/L, 174.51 ± 25.897 U/L and 6.629 ± 1.6063 mg/dl respectively which differed significantly from their counter parts in asymptomatic group that were (26.36 ± 3.370 U/L, 31.53 ± 1.645 U/L, 76.04 ± 2.698 U/L and 0.780 ±.0510 mg/dl respectively), Mean values of serum level of copper 145.29 ± 24.427 μg/dl despite that many cases exceeded the normal range (70–155 μg/dl), this average does not differ significantly from that of Asymptomatic (their families) with a Mean values 74.40 ± 3.145 μg/dl. On the other hand, mean serum levels of ceruloplasmin in patients 9.20 ± 2.745 mg/dl compared with 73.60 ± 2.258 mg/dl in Asymptomatic (their families) significant difference (Table3). The serum AST to urine cu mg/24 hrs Positive correlation (the other variable has a tendency to also increase)(0.951**), total bilirubin Positive correlation(0.795*), S.CU mg/dl Positive correlation lower (0.691). In this study Majority, part of Wilson disease patients had hepatic presentations. the patients were with below 20 years age of onset. The majority part of Wilson disease patients of positive consanguineous parents. A significant association exists between the serum AST, billirubin, 24 hr-urine copper. Serum cooper and 24-hr urine copper levels were significantly lower.
Databáze: OpenAIRE