Abstract
In order to clarify the complex interrelationship between serum calcium, 1,25-dihydroxyvitamin D (1,25(OH)2D), and parathyroid hormone (PTH), and the urinary excretion of cyclic AMP (cAMP) in response to exogenous PTH in pseudohypoparathyroidism (PHP) and related diseases, we investigated 3 patients with parathyroid disorders before and after treatment with 1α-hydroxyvitamin D3 (1α-OH-D3).
Low plasma 1,25(OH)2D before treatment increased after giving 1α-OH-D3 (0.1 μg/kg/day), where-as high plasma PTH measured by the C-terminal assay (C-PTH) decreased in all 3. No response in urinary cAMP was found before or after treatment in 2 patients with PHP type I, despite the fall of plasma C-PTH. However, in one patient with extremely high plasma C-PTH but normal N-PTH (measured by a homologous radioimmunoassay using 1–34 human PTH), urinary cAMP response to exogenous PTH was increased after treatment with 1α-OH-D3. We suggest that he had pseudopseudohypoparathyroidism (PPHP) with Albright's hereditary osteodystrophy and a partial deficiency of renal 1α-hydroxylase. In this patient secondary hyperparathyroidism is thought to be due to 1,25(OH)2D deficiency, and the decreased responsiveness to exogenous PTH before treatment due to excess PTH occupying renal receptors.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Balachandar V (1975) Pseudohypoparathyroidism with normal serum calcium level. Am J Dis Child 129:1092–1095
Dokoh S (1978) Competitive protein binding assay for 1,25-dihydroxyvitamin D in human plasma. Endocrinol Jap 25:431–436
Drezner MK (1976) 1,25-dihydroxycholecalciferol deficiency: The probable cause of hypocalcemia and metabolic bone disease in pseudohypoparathyroidism. J Clin Endocrinol Metab 42:621–628
Fukase M (1979) Cholecalciferol suppression of PTH secretion from monolayer culture of human parathyroid carcinoma cells. Fourth Workshop on vitamin D, Berlin, p 61 (Abstract)
Forte LR (1976) Renal adenyl cyclose and the interrelationship between parathyroid hormone and vitamin D in the regulation of urinary phosphate and adenosine cyclic 3′,5′-monophosphate excretion. J Clin Invest 57:559–568
Fukushima M (1975) Metabolism of 1α-hydroxyvitamin D3 in perfused rat liver. Biochem Biophys Res Comm 66: 632–638
Haussler MR (1976) The assay of 1α,25-dihydroxyvitamin D3: Physiologic and pathologic modulation of circulating hormone levels. Clin Endocrinol 5:151S-165S
Honma M (1977) An ultrasensitive method for the simultaneous determination of cyclic AMP and GMP in small-volume samples from blood and tissue. Biochem Med 18:257–273
Kind HP (1973) Latent pseudohypoparathyroidism: A clue to the pathogenesis of end-organ refractoriness to parathyroid hormone. Clin Res 21:1059 (Abstract)
Kind HP (1973) A classification of the hypoparathyroid syndrome based on the PTH response test. Pediatr Res 7: 324 (Abstract)
Monn E (1976) Pseudohypoparathyroidism. A difficult diagnosis in early childhood. Acta Paediatr Scand 65:487–493
Nusynowitz ML (1976) The spectrum of the hypoparathyroid states. Medicine 55:105–119
Rodriguez HJ (1974) Pseudohypoparathyroidism type II: Restoration of normal renal responsiveness to parathyroid hormone by calcium administration. J Clin Endocrinol Metab 39:693–701
Seino Y (1978) The plasma levels of 25-hydroxyvitamin D in patients with various liver diseases and the response of 25-hydroxyvitamin D to vitamin D treatment. Acta Paediatr Scand 67:39–42
Seino Y (1980) Treatment of hypophosphatemic vitamin D resistant rickets with massive dose of 1α-hydroxyvitamin D3 during childhood. Arch Dis Child 55:49–53
Seino Y (1980) Plasma 1,25-dihydroxyvitamin D concentrations in cords, newborns, infants and children. Calcif Tiss Int 30:1–3
Seino Y Idiopathic hypoparathyroidism with Albright's hereditary osteodystrophy (in preparation)
Shimotsuji T (1976) A competitive protein binding assay for plasma 25-hydroxyvitamin D3 in normal children. Tohoku J Exp Med 118:233–240
Stögmann W (1975) Pseudohypoparathyroidism: Disappearance of the resistance to parathyroid extract during treatment with vitamin D. Am J Med 59:140–144
Werder EA (1978) Pseudohypoparathyroidism and idiopathic hypoparathyroidism: Relationship between serum calcium and parathyroid hormone levels and urinary cyclic adenosine-3′,5′-monophosphate response to parathyroid extract. J Clin Endocrinol Metab 46:872–879
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Seino, Y., Ishida, M., Yamaoka, K. et al. Secondary hyperparathyroidism with 1,25-dihydroxyvitamin D deficiency and pseudohypoparathyroidism in childhood: Relationship between plasma 1,25-dihydroxyvitamin D and parathyroid hormone levels and urinary cyclic AMP response to exogenous PTH. Eur J Pediatr 135, 267–271 (1981). https://doi.org/10.1007/BF00442101
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00442101