Wilson's disease of exclusively hepatic localization. Study of a family

J. Guardia, R. Bacardi, J. M. Martinez Vazquez

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Three cases of juvenile chronic hepatopathy are reported in 3 sisters, 2 of whom died with a clinical picture of subacute hepatitis. In the third sister, the familial association suggested the diagnosis of Wilson's disease which was confirmed by study of the Cu metabolism, its determination in liver tissues and a decrease in the ceruloplasmin level. Treatment with chelating agents seemed to favor the clinical, histological and laboratory picture for 3 mth, showing intense excretion of Cu in the urine, with disappearance of liver deposits and evident histological improvement. Study of the family appears to have uncovered a preclinical case in a homozygous sister. The ease of confusion of the merely hepatic clinical picture with that of chronic juvenile hepatitis is emphasized.
Original languageEnglish
Pages (from-to)463-468
JournalRevista Clinica Espanola
Issue number5
Publication statusPublished - 1 Jan 1973


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