Organic acidurias : proceedings of the ninth symposium of the Society for the Study of Inborn Errors of Metabolism / edited by J. Stern and C. Toothill.

  • Society for the Study of Inborn Errors of Metabolism.
Date:
1972
Catalogue details

Licence: Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)

Credit: Organic acidurias : proceedings of the ninth symposium of the Society for the Study of Inborn Errors of Metabolism / edited by J. Stern and C. Toothill. Source: Wellcome Collection.

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    a-KETO ACID ESTIMATION 141 в Fig. 3. Result of the screening method: Excretion of keto acids in different age groups (A-E) and diseases (F, G) in [xmoles per 1 mg per cent creatinine A; Prematures (N = 25) B: Infants, 0-10 days (N = 43) C: Infants, 10 days-2 months (N = 10) D: Infants, 2 months-1 year (N = 40) E: Children, 1-14 years (N = 25) F: PKU and tyrosinosis (N = 11) G: Cystinosis (N = 7) instance 2 months old infants. An obviously significant difference exists between prematures and the group between 2 and 12 months of age. The high value of 72 ± 8 ¿^moles/mg per cent creatinine in phenylketonuria does not surprise. The excretion of three patients with cystinosis when measured on seven succeeding days varied between 38 and 47; 35 and 60; 22 and 28 /xmoles/mg per cent creatinine, respectively, and was of the same order as that of the prematures. Patterns of the keto acid excretion in different metabolic diseases are shown in the Figs. 4-8. At first, we look at the chromatogram of a 10 days old infant. The most intensive spot near the starting point is