[Acute hemolytic crisis as the initial manifestation of Wilson disease].
A 25-year-old woman who had delivered a normal child after a normal pregnancy four months ago, was suffering from a common cold. The latter was treated by tetracyclines. A few days later she developed an acute severe hemolysis. Moreover she had signs of hepatic failure which was characterized by a considerably reduced synthetic capacity of the liver but low serum aminotransferase levels and a proximal renal tubular disorder. With a negative Coombs test an autoimmune hemolytic anemia was unlikely. Therefore diagnostic procedures were directed at the cause of the liver injury. An increased urinary excretion of copper, strongly elevated levels of liver tissue copper and the detection of a Kayser-Fleischer ring by slit-lamp examination proved the diagnosis of Wilson's disease-presenting clinically only as severe hemolysis. Ceruloplasmin concentration in serum was in low normal range and not diagnostic.
Rath
H C
HC
Klinik und Poliklinik für Innere Medizin I, Universität Regensburg.
Enger
I M
IM
Rüschoff
J
J
Schölmerich
J
J
Holstege
A
A
D000208:Acute Disease; D000328:Adult; D000743:Anemia, Hemolytic / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D001706:Biopsy; D003300:Copper / Q000097:blood; D003937:Diagnosis, Differential; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008111:Liver Function Tests; D010396:Penicillamine / Q000008:administration & dosage; D011644:Puerperal Disorders / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology
D003300:Copper; D010396:Penicillamine