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

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References

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

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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/.