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Transfus Clin Biol ; 16(1): 39-42, 2009 Mar.
Article in French | MEDLINE | ID: mdl-19329346

ABSTRACT

INTRODUCTION: The liver and central nervous system are the usual targets of Wilson's disease, an inherited disorder of copper metabolism. Severe hemolytic anemia is an unusual complication of Wilson's disease. EXEGESIS: We report a case of Wilson's disease revealed by acute intravascular repeating hemolytic anemia associated with liver failure. The initially negative etiologic investigation was directed by occurred of liver failure. The genetic study allowed to discover an other similar case. The evolution was favourable under treatment with zinc sulfate and penicillamine. DISCUSSION: Diagnosis of Wilson's disease must be considered in case of acute hemolytic anemia associated with liver failure in young adults.


Subject(s)
Anemia, Hemolytic/etiology , Hepatolenticular Degeneration/diagnosis , Acute Disease , Arrhythmias, Cardiac/etiology , Chelating Agents/therapeutic use , Chelation Therapy , Child , Consanguinity , Copper , Dyspnea/etiology , Female , Hepatolenticular Degeneration/complications , Hepatolenticular Degeneration/drug therapy , Hepatolenticular Degeneration/genetics , Humans , Jaundice, Obstructive/etiology , Liver Failure/etiology , Male , Penicillamine/therapeutic use , Recurrence , Young Adult , Zinc Sulfate/therapeutic use
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