| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
Endocrine and Renal Units, Clinical Laboratories, Meir General Hospital, Kfar Saba, and the Department of Endocrinology and Metabolism (I.M.S.), Sha'are Zedek Medical Center Jerusalem, Israel
Address requests for reprints to: M. S. Shapiro, M.D., Endocrine Unit, Meir General Hospital, Kfar Saba, Israel.
A male with pseudohypoparathyroidism presented with several hormonal abnormalities. He was clinically euthyroid with no palpable goiter. His serum T4, total T3, T3 Sephadex retention, and 131I uptake were normal. However, elevated basal TSH levels and exaggerated TSH responses to TRH which normalized during the administration of thyroid extract suggested reduced thyroidal reserve. Despite these findings, the 131I uptake increased after exogenous TSH, and the T3 level rose after TRH.
Basal testosterone levels and response to hCG were normal. However, gonadotropins were elevated and there was an exaggerated response after LRH treatment. Both LH and FSH levels were suppressed by testosterone propionate. The patient demonstrated intermittent basal hyperprolactinemia and impaired PRL responsiveness after metoclopramide, chlorpromazine, and insulin administration. There was, however, an intact response to TRH. Basal PRL, TSH, and LH levels decreased after the administration of L-dopa and bromocriptine.
Although the precise mechanism underlying these findings is unknown, the elevated basal levels of TSH, LH, and FSH and the exaggerated responses to their respective releasing hormones suggest the presence of a partial degree of end-organ resistance to these pituitary trophic hormones. Together with the resistance to PTH, this may imply a common defect, presumably at a postreceptor level. However, hyporesponsiveness of PRL to metoclopramide and chlorpromazine and normal responsiveness to TRH suggest that an abnormality of dopamine tone also exists in pseudohypoparathyroidism.
* This work was supported by grants (to I.M.S.) from the Israel Ministry of Health, Chief Scientist's Office, The Center for Absorption in Science, The Ministry for Immigrant Absorption, State of Israel.
Received July 3, 1979.
This article has been cited by other articles:
 |
|
 |
|
  A.-S. Balavoine, M. Ladsous, F.-L. Velayoudom, V. Vlaeminck, C. Cardot-Bauters, M. d'Herbomez, and J.-L. Wemeau Hypothyroidism in patients with pseudohypoparathyroidism type Ia: clinical evidence of resistance to TSH and TRH Eur. J. Endocrinol., October 1, 2008; 159(4): 431 - 437. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Plagge, G. Kelsey, and E. L Germain-Lee Physiological functions of the imprinted Gnas locus and its protein variants G{alpha}s and XL{alpha}s in human and mouse J. Endocrinol., February 1, 2008; 196(2): 193 - 214. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. S. Weinstein, S. Yu, D. R. Warner, and J. Liu Endocrine Manifestations of Stimulatory G Protein {alpha}-Subunit Mutations and the Role of Genomic Imprinting Endocr. Rev., October 1, 2001; 22(5): 675 - 705. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Z. Farfel, H. R. Bourne, and T. Iiri The Expanding Spectrum of G Protein Diseases N. Engl. J. Med., April 1, 1999; 340(13): 1012 - 1020. [Full Text] [PDF]
|
|
|
|
 |
|
 Z. FARFEL and E. FRIEDMAN Mental Deficiency in Pseudohypoparathyroidism Type I Is Associated with Ns-Protein Deficiency Ann Intern Med, August 1, 1986; 105(2): 197 - 199. [Abstract] [PDF]
|
|
|
|
 |
|
 R. A. Reeves, J. E. Kiss, M. Meess, G. S. Levey, and A. P. Shapiro Pseudopseudohypoparathyroidism and Pheochromocytoma: Association or Coincidence? Arch Intern Med, August 1, 1983; 143(8): 1619 - 1619. [Abstract] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| Endocrinology | Endocrine Reviews | J. Clin. End. & Metab. |
| Molecular Endocrinology | Recent Prog. Horm. Res. | All Endocrine Journals |
