Abstract
Interest of the measure of serum branched-chain aminoacids (BCA) in the diagnosis of hyperinsulinism in infancy.
Fasting values of BCA (valine, leucine and isoleucine) were measured by column chromatography in the sera of 27 normal children aged 2 days to 9 years, 14 children with ketotic hypoglycemia (KH) aged 1 to 7 years, and in 12 sera from 5 infants aged 15 days to 2 years with documented hyperinsulinism. In normal and KH children, each individual BCA and their sum were highly significantly negatively correlated with blood glucose (BG) values ranging between 0.11 and 0.92 g/1 (r = 0.61, p<0.001). In infants with hyperinsulinism BCA concentrations were significantly lower (p < 0.001) than in the other groups without correlation with BG values (ranging from 0.13 to 0.51 g/1), and with plasma insulin concentrations (ranging from 9 to 85 μU/ml). In all the children studied, the sum of BCA was highly significantly correlated with blood beta OH butyrate measured by enzymatic method in the same time (r = 0.75, p < 0.001). The association low BG-low BCA during fast seems characteristic of hyperinsulinic states, and the measurement of fasting BCA in these infants offers a simple way of diagnosis, avoiding the technical difficulties of beta OH butyrate determination.
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Chaussain, J., Georges, P., Gendrel, D. et al. Interest of the measure of serum branched-chain amino-acids (BCA) in the diagnosis of hyperinsulinism in infancy: 13. Pediatr Res 14, 168 (1980). https://doi.org/10.1203/00006450-198002000-00040
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DOI: https://doi.org/10.1203/00006450-198002000-00040