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Cystinuria

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      Abstract

      The excretion of cystine, lysine, ornithine and arginine in the urine is increased in cystinuria, and cystine stones form in the urinary tract. The present state of knowledge of the disease is reviewed with particular reference to its hereditary basis, to the abnormalities of amino acid transport which have been demonstrated in the kidney and small intestine, and to the clinical and therapeutic problems which it poses. The use of d-penicillamine and of N-acetyl-d-penicillamine for treatment is discussed and the clinical side effects of these compounds are summarized.
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