[Whipple's triad as a clinical manifestation of hepatolenticular degeneration].
Hepatolenticular degeneration (Wilson's disease) is a rare condition characterised by a defect in biliary excretion of copper resulting in excessive copper accumulation and toxicity. To the most frequent symptoms of this disorder belong liver, neurological or psychiatric disturbances, although other less common clinical features may sometimes be present. Since the clinical presentation of the disease is highly heterogeneous, it may mimic the symptoms of many various disorders. Diagnosis of the condition depends primarily on clinical features, biochemical parameters and the presence of the Kayser-Fleischer ring. Early detection and treatment protect patients from devastating organ damage. We describe an atypical case of Wilson's disease in a 23-year-old woman, whose clinical presentation suggested the presence of an insulin-secreting tumour. After the diagnosis was established and zinc sulphate treatment implemented, her clinical status improved remarkably. The presented case suggests that hepatolenticular degeneration should be taken into consideration in a differential diagnosis of hypoglycaemia of an unknown origin.
Krysiak
Robert
R
Klinika Chorób Wewngtrznych i Farmakologii Klinicznej, Katedra Farmakologii, Slaska Akademia Medyczna, Katowice. r.krysiak@poczta.onet.pl
Okopień
Bogusław
B
D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis*; D006801:Humans; D007003:Hypoglycemia / Q000097:blood / Q000175:diagnosis*; D007340:Insulinoma / Q000175:diagnosis*; D010190:Pancreatic Neoplasms / Q000175:diagnosis*