Defective Urinary Acidification in Wilson's Disease

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Abstract
Studies of urinary acidifying capacity and of certain other aspects of renal function were performed in 12 patients with Wilson''s disease. Seven of these patients had clinically manifest hepatolenticular degeneration and the other 5 had asymptomatic Wilson''s disease. Ten of the patients had been treated with "decop-perizing" regimens, including D-penicillamine. Four of the twelve patients had subnormal urinary acidifying capacity and one of them had bilateral medullary nephrocalcinosis characteristic of distal renal tubular acidosis. Hyperchloremic acidosis was not present in 3 of these patients and was minimal in a 4th one. None of them had urinary symptoms, osteomalacia or hypokalemia. One, who had the Fanconi syndrome before treatment with D-penicillamine, manifested marked improvement in his proximal renal tubular abnormalities after which his urinary acidification defect first appeared. It appears that this abnormality may be an inherent feature of Wilson''s disease.