Summary and conclusionsPregnant women receiving daily supplements of 400 IU (10 ,tg) of vitamin D2 from the 12th week of pregnancy had plasma calcium concentrations higher at 24 weeks but similar at delivery to those in control pregnant women who did not receive the supplements. Infants of the women receiving the supplements had higher calcium, lower phosphorus, and similar magnesium concentrations on the sixth day of life and a lower incidence of hypocalcaemia than infants of the control women. Plasma concentrations of 25-hydroxycholecalciferol, which showed a seasonal variation, were higher in mothers and infants in the treated group. Cord-blood calcium, magnesium, phosphorus, and 25-hydroxycholecalciferol concentrations correlated with maternal values at delivery. Breast-fed infants had higher calcium and magnesium and lower phosphorus and 25-hydroxycholecalciferol concentrations than artificially fed infants. A defect of dental enamel was found in a high proportion of infants (many of whom had suffered from hypocalcaemia) born to the control women.These results suggest that vitamin D supplementation during pregnancy would be beneficial for mothers, whose intake from diet and skin synthesis is appreciably less than 500 IU of vitamin D daily.

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We have studied 250 human liver biopsy samples to determine the ontogeny of the microsomal glucose-6-phosphatase (EC 3.1.3.9) system. Human hepatic glucose-6-phosphatase enzyme activity develops at 11 weeks' gestation and slowly increases to approximately 10% of adult activity at term. In the first week after birth, activity rises to adult values. Increases in enzyme activity coincide with increasing concentrations of the glucose-6-phosphatase enzyme protein. The phosphate/pyrophosphate transport protein (T2) of the human hepatic glucose-6-phosphatase complex develops at a different rate from that of the enzyme. Our study shows that the development of rat and human glucose-6-phosphatase activities are completely different. We conclude that deficiencies of the proteins in the microsomal glucose-6-phosphatase complex can be diagnosed with much more certainty perinatally than prenatally.

Three preterm infants born at 26-30 weeks' gestation who died between 103 and 266 days after birth were found to have elevated hepatic glycogen levels. Kinetic analysis of the hepatic microsomal glucose-6-phosphatase system demonstrated that one infant had abnormally low levels of activity of the glucose-6-phosphatase enzyme (partial type 1a glycogen storage disease) and two had deficiencies of T2, a microsomal phosphate/pyrophosphate transport protein (type 1c glycogen storage disease). In all three cases glycogen storage disease was not suspected prior to death even though both hypo- and hyperglycaemic episodes were recorded in the first 15 days after birth indicating that they had somewhat disordered blood glucose regulation. In the infant with low glucose-6-phosphatase enzyme activity, abnormal development of the glucose-6-phosphatase enzyme cannot be ruled out. This is the first description of abnormalities in the glucose-6-phosphatase system in preterm infants.