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Defective Urinary Acidification in Wilson's Disease

Ann Intern Med. 1968;68(4):770-777. doi:10.7326/0003-4819-68-4-770
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Four of 12 patients with treated Wilson's disease 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 three of these patients and was minimal in a fourth. 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.





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