Daily rhythmic changes in hepatic phenylalanine hydroxylase activity: role of dietary phenylalanine.
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Biomedical subjects
Publications and source records attributed to S Castells.
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The response of plasma insulin concentration to an oral glucose tolerance test (OGTT) and to the maximum stimulatory effect obtained with administration of glucose, glucagon and tolbutamide was studied in 24 siblings of diabetic children and in ten obese children. Five siblings of patients with diabetes sound to have chemical diabetes had hyperinsulinism during the OGTT. Serum insulin concentrations during the maximum stimulation of the beta cells in the children with chemical diabetes, although diminished at 15 minutes, were considered not significantly different from controls. Obese children had hyperinsulinism during the OGTT and the maximum stimulation of the beta cell. The data suggest that hyperinsulinism may precede or accompany carbohydrate intolerance in siblings of diabetic children.
UNLABELLED: Bone density in growth hormone (GH) deficient children is decreased more than expected for delayed skeletal maturation. Previous studies suggest GH enhances mineral retention and deposition in bone. Seven GH deficient prepubertal children were studied during 2 years of GH therapy to assess the effect on bone density and plasma osteocalcin. Bone density (radiographic photodensitometry) of the phalanges (cortical and trabecular bone) was expressed as the standard deviation score (SDS) of the mean for sex, bone age and chronological age. Relative osteopenia, less pronounced for bone density/bone age (BD/BA) than bone density/chronological age (BD/CA), improved significantly during GH therapy. After 12 months there was increase over pretreatment levels, significant for BD/CA (-1.65 +/- 0.46 vs -1.15 +/- 0.64; mean +/- SD: p = 0.002), but less pronounced for BD/BA. After 24 months increase in both measurements continued, reaching significance also for BD/BA (Pre: -1.02 +/- 0.55 vs -0.41 +/- 0.29; p = 0.011). Plasma osteocalcin levels were low before GH therapy (11.6 +/- 9.9 ng/ml; n = 7; vs control 24.4 +/- 12.5 ng/ml; n = 21; p < 0.05), rose significantly after one week (31.2 +/- 10.5 ng/ml; p < 0.001), with continued upward trend to plateau between 2-6 months, with elevated levels persisting during 2 years of GH therapy. CONCLUSION: The early and sustained rise in plasma osteocalcin and subsequent increase in bone density with continued gain over 24 months of the study suggests that GH therapy in GH deficient children has a significant prolonged effect on bone formation and mineralization in addition to stimulating linear growth.
We describe a 5 years and nine months old boy who presented with facial features, vertebral anomalies and dwarfism consistent with Robinow syndrome. Investigations revealed growth hormone (GH) deficiency to be the cause of his dwarfism. We reviewed data on four other patients with Robinow syndrome from the Genentech National Cooperative Growth Study (NCGS). Results of GH testing on three out of four were available and showed GH deficiency. Recombinant human GH therapy in our patient and the three patients from the NCGS resulted in a significant increase in the growth rate per year. The cause of dwarfism in children with Robinow syndrome has hitherto not been studied. We propose its association with GH deficiency and that treatment with rhGH can result in a significant increase in the growth rate of these children.
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