Precocious puberty in McCune-Albright syndrome: a case report

https://doi.org/10.19106/JMedScie/0050032018014

Rianti Puji Lestari(1*), Retno Sutomo(2), Madarina Julia(3)

(1) Department of Child Health, Faculty of Medicine/Dr. Sardjito General Hospital, Universitas Gadjah Mada, Yogyakarta, Indonesia
(2) Department of Child Health, Faculty of Medicine/Dr. Sardjito General Hospital, Universitas Gadjah Mada, Yogyakarta, Indonesia
(3) Department of Child Health, Faculty of Medicine/Dr. Sardjito General Hospital, Universitas Gadjah Mada, Yogyakarta, Indonesia
(*) Corresponding Author

Abstract


McCune-Albright syndrome (MAS) is a rare disease characterized by a triad of fibrous
dysplasia, cafe-au-lait spots and peripheral precocious puberty. We reported a 5-year-
8-month old girl with MAS who has been followed-up for 2 years and 8 months. She
was referred to pediatric endocrinology clinic in our hospital for vaginal bleeding at age
of 2 years 11 months. She had peripheral precocious puberty, i.e. increased estrogen
level associated with very low gonadotropins, and cafe-au-lait spots on her face and was
diagnosed as MAS. The patient was treated with estrogen receptor blocker (tamoxifen).
She had no menses during the 2 years and 8 months of tamoxifen treatment. Her growth
rate and bone maturation were also in normal ranges. However, at the end of tamoxifen
treatment she had an episode of vaginal bleeding so that we had to change to other
treatment modalities.


Keywords


McCune-Albright syndrome - precocious puberty – tamoxifen – genetic disorders - gonadotropins

Full Text:

PDF


References

Collins MT, Singer FR, Eugster E. McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia. Orphanet J of Rare Diseases 2012; 7 (Suppl 1): S4. Collins MT, Sarlis NJ, Merino MJ, Monroe J, Crawford SE, Krakoff JA, et al. Thyroid carcinoma in the McCune-Albright syndrome: the contributory role of Gs alpha activating mutations. J Clin Endocrinol Metab. 2003; 88: 4413-4417. Collins MT. McCune-Albright Syndrome. Obtained from: www.orpha.net/patho/GB/uk-McCune-Albright-Syndrome.pdf Volkl TM, Dorr HG. McCune-Albright syndrome: clinical picture and natural history in children and adolescents. J Pediatr Endocrinol Metab. 2006; 19: 551-559. Mieszczak J, Eugster EA. Treatment of precocius puberty in McCune-Albright syndrome. Ped Endocrinol. 2007; 4: 419-422. Siadati S, Shafigh E. McCune-Albright syndrome: A case report. Iranian Medicine 2010; 13 (3): 245-247. Matarazzo P, Lala R, Andreo M, Einauidi S, Altare F, Viora E, et al. McCune-Albright syndrome: the persistence of autonomous ovarian hyperfunction during adolesance and early adult age. J Pediatr Endocrinol Metab 2006; 19: 607-617. Eugster EA, Rubin SD, Reiter EO, Plourde P, Jou HC, Pescovitz OH, et al. Tamoxifen treatment for precociuos puberty in McCune-Albright syndrome: a multicenter trial. J Pediatr. 2003; 143: 60-6. MorinskyDE, Muntner P. Medication adherence scale versus pharmacy fill rates in senior with hypertension. Am J Manag Care 2009;15(1):59-66. Nunez S, Calis K, Cutler G, Jones J, Feuillan P. Lack of efficicacy of fadrazole in treating precocius puberty in girls with the McCune- Albright syndrome. J Clin Endocrinol Metab. 2003; 88: 5730-5733. Feuillan P, Foster C, Pescovitz O, Hench K, Shawker T, Dwyer A, et al. Treatment of precocious puberty in the McCune - Albright with the aromatase inhibitor testolactone. N Eng J Med. 1986; 315: 1115-1119. Feuillan PP, Jones J, Cutler GB. Long term testolactone therapy for precocious puberty in girls with the McCune-Albright syndrome. J Clin Endocrinol Metab. 1993; 77 (3): 647-651. Feuillan P, Calis P, Hill S, Shawker T, Robey P, Collins M. Letrazole treatment of precocious puberty in girls with the McCune-Albright syndrome: a pilot study. J Clin Endocrinol Metab. 2007; 92: 2100-2106. Estrada A, Boyce AM, Brillante BA, LC Guthrie, Gafni RI, Collins MT. Long-term outcomes of letrozole treatment for precocious puberty in girls with McCune-Albright syndrome. Eur J Endocrinol. 2016; 175(5): 477-483.



DOI: https://doi.org/10.19106/JMedScie/0050032018014

Article Metrics

Abstract views : 838 | views : 1783




Copyright (c) 2018 Rianti Puji Lestari, Retno Sutomo, Madarina Julia

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

View My Stats

 

Creative Commons License
Journal of the Medical Sciences (Berkala Ilmu Kedokteran) by  Universitas Gadjah Mada is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Based on a work at http://jurnal.ugm.ac.id/bik/.