The Amino Acid Content of the Blood and Urine in Wilson's Disease

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Abstract
Amino acid distribution in 24-hour specimens of the urine of patients with Wilson''s disease has been investigated by chro-matography on columns of Dowex 50. Of 6 cases examined, 5 exhibited a widespread and massive amino aciduria while 1 case of the disease showed an almost normal excretion of amino acids. The amino acid distribution in the plasma of subjects with Wilson''s disease is qualitatively and quantitatively normal, both in the fasting state and after a protein meal. In contrast to the normal, the level of urinary amino acids in the patients with Wilson''s disease is quite sensitive to the protein intake, so that fasting urine has a very different amino acid composition to that found for the 24-hour specimen. The amino aciduria in Wilson''s disease is a result of a renal lesion. This symptom, however, appears to be a secondary consequence of some other basic disturbance that may well be connected with Cu metabolism.