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Introduction: Wilson disease is a hereditary disorder of Copper metabolism which can present with hepatic, neurologic or psychiatric symptoms and in rare cases as a hemolytic disturbance. Free Copper can result in red blood cell damage and hemolysis which is a rare feature of the disease affecting less than 10% of patients. In this condition the liver is usually involved and liver transplantation can be life saving. This article is a case report of acute hemolytic crisis as the initial manifestation of Wilson disease. Case: An 8 years old girl was admitted in pediatric ward of Golestan hospital because of abdominal pain, icterus, anemia and tea color urine. Hepatitis or Glucose 6 Phosphate Dehydrogenase deficiency was the first diagnosis. Because of unresponsiveness to transfusion, Wilson disease was considered and the diagnosis was established with the presence of Kayser-Fleischer ring. Conclusion: Chronic or acute hemolytic anemia is a rare or unusually presentation of Wilson disease. In any child especially older than 5 years with liver disease or hemolytic anemia, Wilson disease should be considered and appropriate diagnostic tests performed

Keywords: Wilson disease, hemolysis, Kayser-Fleischer ring, Copper

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