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A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding

BACKGROUND: McCune-Albright syndrome (MAS) is a rare non-inherited disorder characterized by the clinical triad of precocious puberty, cafe-au-lait skin lesions, and fibrous dysplasia of bone. CASE PRESENTATION: We report a girl with MAS, presenting initially with vaginal bleeding at the age of 17 m...

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Autores principales: Rostampour, Noushin, Hashemipour, Mahin, Kelishadi, Roya, Hovsepian, Silva, Hekmatnia, Ali
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Tehran University of Medical Sciences 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3446187/
https://www.ncbi.nlm.nih.gov/pubmed/23056821
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author Rostampour, Noushin
Hashemipour, Mahin
Kelishadi, Roya
Hovsepian, Silva
Hekmatnia, Ali
author_facet Rostampour, Noushin
Hashemipour, Mahin
Kelishadi, Roya
Hovsepian, Silva
Hekmatnia, Ali
author_sort Rostampour, Noushin
collection PubMed
description BACKGROUND: McCune-Albright syndrome (MAS) is a rare non-inherited disorder characterized by the clinical triad of precocious puberty, cafe-au-lait skin lesions, and fibrous dysplasia of bone. CASE PRESENTATION: We report a girl with MAS, presenting initially with vaginal bleeding at the age of 17 months. Ultrasonography revealed unilateral ovarian cysts and ureteral and ovarian enlargement. Considering the clinical and paraclinical findings, the patient diagnosed as a case of gonadotropin-independent precocious puberty was treated with medroxy-progestrone acetate (MPA) for three months. During the follow up, recurrent episodes of bleeding, ovarian activation and cyst formation, as well as breast size development were reported. At the age of 5.5 years, fibrous dysplasia was detected, which in coexistence with precocious puberty confirmed the diagnosis of MAS. The patient had no cafe-au-lait skin macles during follow up. CONCLUSION: Considering that clinical manifestations of MAS appear later in the course of recurrent periods of ovarian activation and cyst formation, a careful clinical observation and follow up of patients is necessary and the diagnosis of MAS must be kept in mind in cases with gonadotropin-independent precocious puberty.
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spelling pubmed-34461872012-10-09 A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding Rostampour, Noushin Hashemipour, Mahin Kelishadi, Roya Hovsepian, Silva Hekmatnia, Ali Iran J Pediatr Case Report BACKGROUND: McCune-Albright syndrome (MAS) is a rare non-inherited disorder characterized by the clinical triad of precocious puberty, cafe-au-lait skin lesions, and fibrous dysplasia of bone. CASE PRESENTATION: We report a girl with MAS, presenting initially with vaginal bleeding at the age of 17 months. Ultrasonography revealed unilateral ovarian cysts and ureteral and ovarian enlargement. Considering the clinical and paraclinical findings, the patient diagnosed as a case of gonadotropin-independent precocious puberty was treated with medroxy-progestrone acetate (MPA) for three months. During the follow up, recurrent episodes of bleeding, ovarian activation and cyst formation, as well as breast size development were reported. At the age of 5.5 years, fibrous dysplasia was detected, which in coexistence with precocious puberty confirmed the diagnosis of MAS. The patient had no cafe-au-lait skin macles during follow up. CONCLUSION: Considering that clinical manifestations of MAS appear later in the course of recurrent periods of ovarian activation and cyst formation, a careful clinical observation and follow up of patients is necessary and the diagnosis of MAS must be kept in mind in cases with gonadotropin-independent precocious puberty. Tehran University of Medical Sciences 2011-09 /pmc/articles/PMC3446187/ /pubmed/23056821 Text en © 2011 Iranian Journal of Pediatrics & Tehran University of Medical Sciences http://creativecommons.org/licenses/by-nc/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0), which allows users to read, copy, distribute and make derivative works for non-commercial purposes from the material, as long as the author of the original work is cited properly.
spellingShingle Case Report
Rostampour, Noushin
Hashemipour, Mahin
Kelishadi, Roya
Hovsepian, Silva
Hekmatnia, Ali
A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding
title A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding
title_full A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding
title_fullStr A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding
title_full_unstemmed A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding
title_short A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding
title_sort case of atypical mccune-albright syndrome with vaginal bleeding
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3446187/
https://www.ncbi.nlm.nih.gov/pubmed/23056821
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