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- W2000403037 abstract "Blood acid-base status, serum electrolytes and urine-pH were examined in 64 PKU-patients receiving 3 different low-phenylalanine protein hydrolysates and 2 synthetic amino acid mixtures. The different formulae caused significant differences in acid-base status, K and Cl in blood-and in urine-pH. Metabolic acidosis seemed to be correlated to an excess of anions (Cl−) and a high content of the sulphur containing amino acids. Balance studies were done in two children. In 6 balance periods 4 formulae were given. Formula A had an excess of cations, whereas formula B1 showed an excess of anions. Both were commercially available products. Formulae B2-B3 were modifications of formula B1 with a reduction in the content of Cl, cystine and methionine. We found an increasing metabolic acidosis from A(blood-pH 7.45, serum HCO3 25.8 mval/L) to B3(7. 41, 24.0), B2(7. 39, 19. 3) and B1(7.36, 17.0). Urine-pH dlcreased( A 7.25, B36.22, B1 5.65, B1 5.6) and total H excretion in urine increased ( A 0.3, B3 2.7, B2 3.3, B1 4.4 mEq/Kg/24h). In the formulae B3, B2 and B1 the difference in total H+excretion correlated with the different Cl− and S− content of the diet and the different Cl− and SO4− content of the urine. We propose that formulae should have an excess of cations and that organic sulphur intake should not exceed 1mMS/Kg/24h." @default.
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- W2000403037 date "1974-02-01" @default.
- W2000403037 modified "2023-10-17" @default.
- W2000403037 title "ACID-BASE STATUS IN DIETARY TREATMENT OF PHENYLKETONURIA" @default.
- W2000403037 doi "https://doi.org/10.1203/00006450-197402000-00057" @default.
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