A rare cause of acromegaly: McCune-Albright syndrome

Öz

McCune-Albright syndrome is characterized by polyostatic fibrous dysplasia, brown spots on the skin (café au lait pigmentation) and autonomous endocrine hyperfunction. Early puberty and other endocrinological manifestations, such as acromegaly, gigantism and hypercortisolism are widely observed in the syndrome. Acromegaly is seen in 20% of patients. We report a case of acromegaly accompanied with this syndrome.

Key words: McCune-Albright syndrome; acromegaly; fibrous dysplasia

Anahtar Kelimeler

• McCune-Albright syndrome; acromegaly; fibrous dysplasia

Akromegalinin nadir bir nedeni: McCune-Albright sendromu

Öz

McCune-Albright sendromu, poliostotik fibröz displazi, deride kahverenginde lekelenme (Cafe au lait lekeleri) ve otonomik endokrin hiperfonksiyon ile karakterize bir sendromdur. Bu sendromda yaygın olarak erken puberte ve diğer endokrinolojik manifestasyonlar örneğin akromegali, jigantizm, hiperkortizolizm görülebilir. Akromegali Mc-Cune-Albriht sendromlu hastaların % 20 sinde görülebilir. Biz bu sendroma eşlik eden bir akromegali vakasını sunduk

Anahtar Kelimeler

• McCune-Albright sendromu, fibröz displazi, akromegali

Kaynakça

1. Comite F, ShawkerTH, Loriaux DL, Cutler GB Jr. Ovarian
2. function in girls with McCune Albright syndrome. Pediatr
3. Res 1986;20:859-863.
4. Weinstein LS, Shenker A, Gejman PV, et al. Activating mutation of the stimulatory G protein in McCune Albright syndrome. N Eng J Med 1991;325:1688-1695.
5. Akintoye SO, Chebli C, Booher S, et al. Characterization
6. of gsp-mediated growth hormone excess in the context
7. of McCune-Albright syndrome. J Clin Endocrinol Metab
8. ;87:5104-5112.

9. Sherman SI, Ladenson PW. Octreotide therapy of growth
10. hormone excess in the McCune-Albright syndrome .Journal of Endocrinological Investigation 1992;15:185-190.
11. Congedo V, Celi FS. Thyroid disease in patients with
12. McCune-Albright syndrome. Pediatr Endocrinol Rev 2007;4:429-433.
13. Sargin H, Gozu H, Bircan R, et al. A case of McCune-Albright syndrome associated with Gs alpha mutation in the bone tissue. Endocr J 2006;53:35-44.
14. Salenave S, Boyce AM, Collins MT, Chanson P. Acromegaly
15. and McCune-Albright syndrome. J Clin Endocrinol Metab
16. ;99:1955-1969.
17. Dumitrescu CE, Collins MT. Skeletal Clinical Studies Unit,
18. Craniofacial and Skeletal Diseases Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland, USA. Orphanet 2008; 3:12.
19. Ruggieri P, Sim FH, Bond JR, Unni KK. Malignancies in fibrous dysplasia. Cancer 1994;73:1411-1424.
20. Chanson P, Salenave S, Orcel P. McCune-Albright syndrome in adulthood. Pediatr Endocrinol Rev 2007;4:453-462.