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A pilot study and a subsequent double-blind study have been performed on 7 previously untreated elderly phenylketonuria patients in order to evaluate the effect of phenylalanine-poor diet. The effect of the treatment was evaluated by the observation of 6 psychiatric symptoms according to a 5-grade scale. Good and/or rather good effect of the treatment was noticed in 3 of the patients, while 2 showed only a slight effect during the pilot study. In the subsequent double-blind study on 5 patients, no effect was seen of the treatment with phenylalanine-poor diet. The necessity of double-blind studies in investigations of this kind is discussed.

The influence of dietary protein intake on the urinary amino acid excretion, the plasma amino acid concentrations and the renal clearance of amino acids was studied in 14 children fed a low (LPD), a normal (NPD), and a high protein diet (HPD). A significant decrease in the quantitative urinary excretion of most amino acids was observed when the children were fed LPD instead of NPD, while no changes in the quantitative urinary amino acid excretion were seen when NPD and HPD were compared. No significant decrease in the plasma amino acid concentrations was seen when the protein intake was changed from NPD to LPD, while a significant increase in several amino acids was seen when HPD replaced NPD. The renal clearance of amino acids when on LPD, NPD and HPD, were compared in 11 children. On LPD, the renal clearances of many amino acids were low when compared with NPD but there were no changes in the renal clearances when HPD was given instead of NPD. The quantitative urinary amino acid excretions and plasma amino acid concentrations at a normal protein intake are presented as well.

A significantly lower frequency of atopies was found in diabetic children and their relatives compared to a control group of non-diabetic individuals.

An extraneous ninhydrin-positive spot on high-voltage paper electrophoresis (HVPE) was observed in urine samples collected from children receiving paracetamol medication. Interference by this derivative has not previously been reported. This extraneous spot could simulate an abnormal peptiduria.

The plasma insulin level and glucose tolerance were studied In five patients with homocystinuria. Two of them showed a marked hyperinsulinemia and a concomitant pathologic glucose tolerance, while the remaining three showed a normal glucose tolerance, two of them, however, had plasma insulin values in the upper level of the normal range. These preliminary results support the earlier hypothesis that the pancreatic islets β-cells are particularly sensitive to an imbalance in the sulfhydryl levels of the organism since homocystinurics have an increased plasma methionine level due to an inborn error of the methionine metabolism.

SINCE the discovery of genetically controlled slow and fast transferrin variants in human beings1,2, data on the frequencies of the different transferrin phenotypes have been collected from various populations. It was noted that slow variants seemed to occur mainly in Negroes and Australian Aborigines and fast variants in White people.