Studies in pseudohypoparathyroidism

Zisman, Elias; Lotz, Myron; Jenkins, Melvin E.; Bartter, Frederic G.

doi:10.1016/0002-9343(69)90048-5

Cited by 43 publications

(3 citation statements)

References 11 publications

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“…It would appear that there are at least two possible explanations to account for the PTE responsiveness of osseous tissue in PHP: either there is a partial defect in the adenyl cyclase system of bone in this syndrome or some but not all of the effects of PTH on skeletal tissue are mediated through the adenyl cyclase system. It has been demonstrated previously that some degree of PTH responsiveness may occur in some patients with PHP but not in others (21)(22)(23)(24)(25)(26). Indeed, some patients develop osteitis fibrosa cystica presumably as a result of secondary increases in circulating parathyroid hormonie (22,27).…”

Section: Introductionmentioning

confidence: 96%

Effects of Dibutyryl Cyclic Adenosine 3′,5′-Monophosphate and Parathyroid Extract on Calcium and Phosphorus Metabolism in Hypoparathyroidism and Pseudohypoparathyroidism

Bell¹,

Avery²,

Sinha³

et al. 1972

J. Clin. Invest.

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A B S T R A C T It has been proposed previously that the metabolic defect in pseudohypoparathyroidism which accounts for parathyroid hormone unresponsiveness is an absence or abnormal form of the adenyl cyclase system in kidney and presumably in bone. To determine whether there is an associated defect in the response mechanism to cyclic adenosine 3',5'-monophosphate (cyclic AMP), the effects of parathyroid extract (PTE), and dibutyryl cyclic AMP were compared in patients with either surgical hypoparathyroidism or pseudohypoparathyroidism. PTE and dibutyryl cyclic AMP both increased serum and urinary calcium, lowered the serum phosphorus, and increased urinary phosphorus in patients with hypoparathyroidism. PTE also increased urinary cyclic AMP in these patients. PTE increased serum and urinary calcium and urinary phosphorus but did not alter serum phosphorus or urinary cyclic AMP in the patients with pseudohypoparathyroidism. Dibutyryl cyclic AMP increased the serum and urinary calcium, lowered the serum phosphorus, and increased urinary phosphorus in all the patients with pseudohypoparathyroidism. The results indicate that (a) dibutyryl cyclic AMP can reproduce the effects of parathyroid hormone on calcium and phosphorus metabolism in man, (b) the response mechanism to cyclic AMP appears to be intact in pseudohypoparathyroidism, and (c) PTE apparently produces some of its characteristic effects on calcium and phosphorus metabolism in pseudohypoparathyroidism in the absence of an increase in urinary cyclic AMP.

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Section: Introductionmentioning

confidence: 96%

Effects of Dibutyryl Cyclic Adenosine 3′,5′-Monophosphate and Parathyroid Extract on Calcium and Phosphorus Metabolism in Hypoparathyroidism and Pseudohypoparathyroidism

Bell¹,

Avery²,

Sinha³

et al. 1972

J. Clin. Invest.

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show abstract

“…In those cases, TSH data was not available and the diagnosis rested on showing hypothyroidism which responded to exogenous TSH. Subsequent reports (Miyai et al 1971, Sato et al 1975, Sachson et al 1972, Zisman et al 1969 have included demonstration of subnormal or normal serum TSH concentrations which did not respond acutely to TRH, though some of these were childhood cases unlike those reviewed by Odell. Such short-term absences of response to TRH do not necessarily prove either TSH deficiency or a pituitary defect, because the TSH response to TRH is reduced or abolished by corticosteroids (Otsuki et al 1973, Re et al 1976 and in the elderly (Wagner et al 1975, Burrows et al 1977, as well as in acromegaly and diabetes (Berthezene et al 1976).…”

Section: Tsh Deficiencymentioning

confidence: 99%

“…The explanation for the familial type (Miyai et al 1971) is obscure. Disorders reported in association with TSH deficiency include Klinefelter's syndrome (10 cases, not detailed: Pousset et al 1974); pseudohypoparathyroidism (Zisman et al 1969, Winnacker et al 1966); Albright's disease (Aubert & Arroyo 1968); and an obscure neuropathy (Grabow & Chon 1968).…”

Section: Tsh Deficiencymentioning

confidence: 99%