Abstract
Congenital osteopetrosis is characterized by severe impairment of bone resorption. Inactivation and/or resistance to parathyroid hormone (PTH) have been suggested as pathogenetic factors. The response to an acute infusion of bovine parathyroid extract (4-8 units/kg) was studied in 3 children with osteopetrosis. Serum calcium, phosphorus and alkaline phosphatase were within normal limits and did not show any consistent change 120 min. after infusion. Plasma cyclic AMP showed a brisk rise with peak levels 4-28 times above the basal values 10 min. after the infusion. The response was most marked at the highest PTH dose level. There was also a prompt increase in urinary cyclic AMP with increments in the range of 100 to 4000 per cent of the basal values when calculated as nmol excreted per min., whereas urinary phosphate excretion was essentially unchanged. The serum levels of 25-hydroxy-vitamin D were subnormal: 28.2 ± 4.4 nmol/1 (normal value 64.4 ± 14.7 nmol/1; range 38.2-98.9 nmol/1). The relative resistance of these patients to the effect mediated by cyclic AMP on calcium and phosphorus metabolism might be related to a relative vitamin D deficiency.
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Aarskog, D., Aksnes, L. Acute response of parathyroid hormone in osteopetrosis. Pediatr Res 13, 79 (1979). https://doi.org/10.1203/00006450-197901000-00061
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DOI: https://doi.org/10.1203/00006450-197901000-00061