The development of pseudohypoparathyroidism. Involvement of progressively increasing serum parathyroid hormone concentrations, increased 1,25-dihydroxyvitamin D concentrations, and 'migratory' subcutaneous calcifications
Tsang, R.C.; Venkataraman, P.; Ho, M.; Steichen, J.J.; Whitsett, J.; Greer, F.
American Journal of Diseases of Children 138(7): 654-658
1984
ISSN/ISBN: 0002-922X PMID: 6328970 Document Number: 228907
The hormonal changes in the development of pseudohypoparathyroidism (PSH) have not been previously reported. The male sibling of a child with PSH was studied for 2.5 yr. At 1 yr of age he had generalized s.c. calcifications that subsequently migrated over his body. At 3 yr of age and over a 6-mo. period, serum Ca levels fell; serum P, parathyroid hormone (PTH), and 1,25-dihydroxyvitamin D (1,25-[OH]2D) concentrations increased. There was no calcemic, phosphaturic, or urinary cAMP response to PTH. The concentration of serum PTH was suppressed by infusion of Ca and doubled with edetic acid infusion, indicating that the parathyroids were sensitive to changes in Ca levels. Thus, increasing PTH and increased 1,25-(OH)2D concentrations occur in the development of PSH. Migratory skin calcifications may occur. Increasing the serum PTH level reflects increasing compensatory parathyroid production to overcome a progressive PTH receptor defect and serves, with increased 1,25-(OH)2D concentrations, to prevent severe falls in serum Ca concentrations in the early stage of the disease.