Journal of Clinical Endocrinology & Metabolism, Vol 71, 1596-1601, Copyright © 1990 by Endocrine Society

ARTICLES

Thyroid abnormalities in the McCune-Albright syndrome: ultrasonography and hormonal studies

PP Feuillan, T Shawker, SR Rose, J Jones, RK Jeevanram and BC Nisula
Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892.

Hyperthyroidism and goiter have been reported frequently in association with the McCune-Albright syndrome (MAS). To assess the prevalence and extent of thyroid abnormalities in girls with MAS, we studied 19 patients [mean age, 6.6 +/- 1 (+/- SE) yr; mean bone age, 9.5 +/- 1 yr] and 18 normal control girls (mean age, 10.3 +/- 0.5 yr). All patients appeared euthyroid when examined; 1 was taking antithyroid medication. Ultrasonography revealed thyroid abnormalities in 7 patients, including generalized inhomogeneity, small (2-4 mm) and large (greater than 10 mm) hypoechoic regions, and echogenic nodule-like regions. Repeat ultrasonography after intervals of 9-18 months showed enlargement of large hypoechoic regions in 2 patients. In the patients with abnormal ultrasound findings, serum TSH was uniformly low or suppressed both at baseline and after administration of 7 micrograms/kg TRH. The mean serum T3 level in this group was significantly higher than that in controls (2.9 +/- 0.2 vs. 2.3 +/- 0.1 nmol/L; P less than 0.05), whereas mean serum T4, free T4, and T4-binding globulin levels did not differ from those of controls. In the remaining 11 patients, thyroid ultrasonography was normal, and the serum levels of T3, T4, free T4, and TSH were normal. Bioassay showed no detectable thyroid-stimulating activity in the plasma of the MAS patients with suppressed TSH levels. None of the patients became overtly thyrotoxic over 3-6 yr of observation, and their serum iodothyronine levels remained stable. We conclude that thyroid dysfunction is common in girls with MAS, but that it may be clinically occult and not rapidly progressive. The thyroid dysfunction, like that of the ovaries, is associated with structural abnormalities in the gland itself, together with suppressed levels of the respective stimulating hormones.

This article has been cited by other articles:

				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]

				N. Wettschureck and S. Offermanns Mammalian G Proteins and Their Cell Type Specific Functions Physiol Rev, October 1, 2005; 85(4): 1159 - 1204. [Abstract] [Full Text] [PDF]

				S. J. Marx and W. F. Simonds Hereditary Hormone Excess: Genes, Molecular Pathways, and Syndromes Endocr. Rev., August 1, 2005; 26(5): 615 - 661. [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]

				M. T. Collins, N. J. Sarlis, M. J. Merino, J. Monroe, S. E. Crawford, J. A. Krakoff, L. C. Guthrie, S. Bonat, P. G. Robey, and A. Shenker Thyroid Carcinoma in the McCune-Albright Syndrome: Contributory Role of Activating Gs{alpha} Mutations J. Clin. Endocrinol. Metab., September 1, 2003; 88(9): 4413 - 4417. [Abstract] [Full Text] [PDF]

				L. S. WEINSTEIN, M. CHEN, and J. LIU Gs{alpha} Mutations and Imprinting Defects in Human Disease Ann. N.Y. Acad. Sci., June 1, 2002; 968(1): 173 - 197. [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]

				A. G. DOUFAS and G. MASTORAKOS The Hypothalamic-Pituitary-Thyroid Axis and the Female Reproductive System Ann. N.Y. Acad. Sci., April 1, 2000; 900(1): 65 - 76. [Abstract] [Full Text] [PDF]

				M. Ivan, M. Ludgate, V. Gire, J. A. Bond, and D. Wynford-Thomas An Amphotropic Retroviral Vector Expressing a Mutant gsp Oncogene: Effects on Human Thyroid Cells in Vitro J. Clin. Endocrinol. Metab., August 1, 1997; 82(8): 2702 - 2709. [Abstract] [Full Text] [PDF]

				C. A. Stratakis, N. A. Courcoutsakis, A. Abati, A. Filie, J. L. Doppman, J. A. Carney, and T. Shawker Thyroid Gland Abnormalities in Patients with the Syndrome of Spotty Skin Pigmentation, Myxomas, Endocrine Overactivity, and Schwannomas (Carney Complex) J. Clin. Endocrinol. Metab., July 1, 1997; 82(7): 2037 - 2043. [Abstract] [Full Text] [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]