Article Text

DETERMINATION OF LIVER ENZYMES, SERUM CERULOPLASMIN AND URINE COPPER IN PARENTS OF CHILDREN WITH WILSON’S DISEASE
  1. S M Dehghani1,
  2. M Haghighat1,
  3. M H Imanieh1
  1. 1Department of Pediatrics, Shiraz University of Medical Sciences, Nemazee Hospital, Shiraz, Iran
  2. 2Gastroenterohepatology Research Center, Shiraz University of Medical Sciences, Nemazee Hospital, Shiraz, Iran

Abstract

Background Wilson’s disease is an autosomal recessive disorder of copper metabolism leading to liver disease and neuropsychiatric deterioration. These patients have low serum ceruloplasmin levels and high urinary copper excretion. The aim of this study was to determine the level of liver enzymes, serum ceruloplasmin and urine copper in parents of children with Wilson’s disease.

Methods We performed this prospective study on parents of Wilsonian children between February 2005 and May 2007. In this study liver enzymes, serum ceruloplasmin and 24-hour urine copper were evaluated in all parents. Serum ceruloplasmin and 24-hour urine copper content were determined by radial immunodiffusion and flame atomic absorption spectroscopy, respectively.

Results There were 31 parents (16 female, and 15 male) with mean age of 39.7±8.2 years. Only one case had abnormal liver enzymes. Six cases (19.4%) had low serum ceruloplasmin and 14 (45.2%) had high urine copper levels (mean, 110.3±35.8 µg/day).

Conclusion We conclude that a significant number of parents of children with Wilson’s disease have high urine copper levels.

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