Biomedical subjects
Publications and source records attributed to H L Teijema.
We describe a girl with neonatal hyperammonaemia due to carbamyl phosphate synthetase I deficiency. Treatment consisted of protein restriction from the second day of life. Sodium benzoate was given for three weeks after birth and again from 7 months of age together with sodium phenylacetate to improve protein tolerance. Growth and development are normal at 15 months of age.
Children with symptomatic hypoglycemia and asymptomatic hypoglycemia-prone children were shown to differ in the degree of ketosis after a 20 h fast. In the latter children the close negative correlation between ketone body levels and glucose levels yielded a regression line against which the former children's data could be compared. Half of the patients were found to be hypoketotic during hypoglycemia. The significance of this hypoketosis in the symptomatology is discussed. The finding of other abnormal responses to fasting, particularly in the patients' nitrogen metabolism, suggests that all these aberrations have a common cause which may be of hepatic origin.
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