Abstract
Studies on skeletal muscle composition were performed in one child with primary renaltubular acidosis and in two children with the Fanconi syndrome. A low concentration of intracellular potassium was found in each of them. A decreased renal capacity to conserve potassium was demonstrated by adequate challenges (acetazoleamide, nonreabsorbable anion load). The composition of the muscle cell with regard to several intermediates of the glycolytic pathway and citric acid cycle, as well as the related enzyme activities, was also very abnormal in comparison with values obtained in normal subjects. The possible significance of these observations is discussed. Studies on skeletal muscle composition were performed in one child with primary renaltubular acidosis and in two children with the Fanconi syndrome. A low concentration of intracellular potassium was found in each of them. A decreased renal capacity to conserve potassium was demonstrated by adequate challenges (acetazoleamide, nonreabsorbable anion load). The composition of the muscle cell with regard to several intermediates of the glycolytic pathway and citric acid cycle, as well as the related enzyme activities, was also very abnormal in comparison with values obtained in normal subjects. The possible significance of these observations is discussed. ErratumThe Journal of PediatricsVol. 69Issue 3Preview Full-Text PDF
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