Abstract
Summary
The serum level of free phenylalanine and phenyllactic acid was determined in 18 patients suffering from phenylpyruvic oligophrenia. No significant amounts of the hydroxy acid were found. The concentration range of the amino acid was between 19 and 38 mg per 100 ml of serum with larger inter- than intra-individual variations. No correlation exists between the degree of mental deficiency and the concentration of phenylalanine in serum. Phenylalanine concentration in the spinal fluid varied from 6.1 to 8.2 mg/100 cc.
It is suggested that although the increased amount of phenylalanine is caused by the metabolic error, its blood plasma level is determined by the ability of the kidney tubuli to reabsorb the amino acid.
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