THE SUCCESSFUL MANAGEMENT OF HEPATOLENTICULAR DEGENERATION WITH PENICILLAMINE: STUDIES ON THREE GENERATIONS OF A FAMILY

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Abstract
A family pedigree is reported in which at least 2 members demonstrate hepatolenticular degeneration, and the total serum Cu and ceruloplasmin levels in 3 generations of the family are presented. Detailed studies were done on one of these patients who had relatively acute neurologic involvement and laboratory evidence of severe functional impairment of the liver. He responded dramatically to penicillamine given over a 3 1/2-year period. DL-penicillamine in doses of 2.4 g given orally in 4 divided doses on alternate days appeared to be optimal in producing cupruresis in this patient. A comparison of the acute cupreretic responses to BAL and penicillamine in 17 previously reported cases of hepatolenticular degeneration is presented.