This Article Full Text (PDF) Submit a related Letter to the Editor Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by CUTTLER, L. Articles by FROHMAN, L. A. Search for Related Content PubMed PubMed Citation Articles by CUTTLER, L. Articles by FROHMAN, L. A.

Hypersecretion of Growth Hormone and Prolactin in McCune-Albright Syndrome^*

Department of Pediatrics, University of Chicago Chicago, Illinois 60637
The Departments of Medicine, Scott and White Clinic Temple, Texas 76500
Henry Ford Hospital Detroit, Michigan 48202
University of Cincinnati Cincinnati, Ohio 45267

Address all correspondence and requests for reprints to: Dr. Leona Cuttler, Section of Pediatric Endocrinology, Department of Pediatrics, Wyler Children’s Hospital, University of Chicago, 5841 South Maryland Avenue, Box 118, Chicago, Illinois 60637.

Acromegaly and hyperprolactinemia have been reported in association with the McCune-Albright syndrome, but the pathophysiology of the GH and PRL hypersecretion that occurs in patients with this disorder has not been defined. We studied GH and PRL secretory dynamics in three patients with McCune-Albright syndrome and hypersecretion of these hormones. Each patient had excessive linear growth, glucose-nonsuppressible plasma GH concentrations, and GH responsiveness to TRH and GHRH. In response to exogenous GHRH, plasma GH concentrations rose approximately 2-fold in all three patients. Plasma GHRH levels were 20–40 ng/L (normal, <30). Study of the spontaneous GH secretory pattern in two patients indicated nocturnal augmentation of GH release. Bromocriptine therapy failed to reduce plasma GH in all patients; in one patient treatment with octreotide, a long-acting somatostatin analog, partially suppressed plasma GH and insulin-like growth factor I levels.

These results suggest that hypersecretion of GH in the McCune-Albright syndrome is not due to ectopic GHRH production or autonomous somatotroph function. The results are similar to those described in classic acromegaly due to GHsecreting pituitary tumors. However, the lack of radiographic pituitary enlargement, the variable pituitary pathology reported in similar patients, and frequent concordance of GH and PRL excess suggest that the pathogenesis of this disorder may differ fundamentally from other forms of acromegaly or gigantism. The pathophysiology may reflect abnormal hypothalamic regulation and/or an embryological defect in pituitary cellular differentiation and function.

^* Presented in part at The Society for Pediatric Research National Meeting, Washington, D.C., May 1986, and the Southern Section Meeting of the American Federation for Clinical Research, New Orleans, LA, February 1988. This work was supported in part by the Clinical Associate Physician Award USPHS Grant RR-00055, USPHS Grant DK-40221, and Award 5-609 from the March of Dimes Birth Defects Foundation (to L.C.); Scott and White Clinic, Scott and White Memorial Hospital, and the Scott, Sherwood, and Brindley Foundation (JAJ 250-072-0); and USPHS Grant DK-30667 (to L.A.F.).

Received June 17, 1988.

This article has been cited by other articles:

				S. A. Boikos and C. A. Stratakis Molecular genetics of the cAMP-dependent protein kinase pathway and of sporadic pituitary tumorigenesis Hum. Mol. Genet., April 15, 2007; 16(R1): R80 - R87. [Abstract] [Full Text] [PDF]

				N Kalfa, P Philibert, F Audran, A Ecochard, T Hannon, S Lumbroso, and C Sultan Searching for somatic mutations in McCune-Albright syndrome: a comparative study of the peptidic nucleic acid versus the nested PCR method based on 148 DNA samples Eur. J. Endocrinol., December 1, 2006; 155(6): 839 - 843. [Abstract] [Full Text] [PDF]

				F. Galland, P. Kamenicky, H. Affres, Y. Reznik, D. Pontvert, Y. Le Bouc, J. Young, and P. Chanson McCune-Albright Syndrome and Acromegaly: Effects of Hypothalamopituitary Radiotherapy and/or Pegvisomant in Somatostatin Analog-Resistant Patients J. Clin. Endocrinol. Metab., December 1, 2006; 91(12): 4957 - 4961. [Abstract] [Full Text] [PDF]

				S. Lumbroso, F. Paris, and C. Sultan Activating Gs{alpha} Mutations: Analysis of 113 Patients with Signs of McCune-Albright Syndrome--A European Collaborative Study J. Clin. Endocrinol. Metab., May 1, 2004; 89(5): 2107 - 2113. [Abstract] [Full Text] [PDF]

				F Sandrini, L S Kirschner, T Bei, C Farmakidis, J Yasufuku-Takano, K Takano, T R Prezant, S J Marx, W E Farrell, R N Clayton, et al. PRKAR1A, one of the Carney complex genes, and its locus (17q22-24) are rarely altered in pituitary tumours outside the Carney complex J. Med. Genet., December 1, 2002; 39(12): e78 - 78. [Full Text] [PDF]

				K. Obuobie, V. Mullik, C. Jones, R. John, A. E. Rees, J. S. Davies, M. F. Scanlon, and J. H. Lazarus McCune-Albright Syndrome: Growth Hormone Dynamics in Pregnancy J. Clin. Endocrinol. Metab., June 1, 2001; 86(6): 2456 - 2458. [Abstract] [Full Text] [PDF]

				S. D. Pack, L. S. Kirschner, E. Pak, Z. Zhuang, J. A. Carney, and C. A. Stratakis Genetic and Histologic Studies of Somatomammotropic Pituitary Tumors in Patients with the "Complex of Spotty Skin Pigmentation, Myxomas, Endocrine Overactivity and Schwannomas" (Carney Complex) J. Clin. Endocrinol. Metab., October 1, 2000; 85(10): 3860 - 3865. [Abstract] [Full Text]

				F. I. Sharara and L. C. Giudice Role of Gorwth Hormone in Ovarian Physiology and Onest of Puberty Reproductive Sciences, January 1, 1997; 4(1): 2 - 7. [Abstract] [PDF]

				J. F. Sotos Overgrowth Clinical Pediatrics, November 1, 1996; 35(11): 577 - 590. [PDF]

				J. A. Majzoub and R. E. Scully Case 7-1993- A Six-Year-Old Boy with Multiple Bone Lesions, Repeated Fractures, and Sexual Precocity N. Engl. J. Med., February 18, 1993; 328(7): 496 - 502. [Full Text]