Diagnosis and Management of Precocious Puberty in Atypical Presentations of McCune-Albright Syndrome: A Case Series Review

2012 ◽  
Vol 25 (1) ◽  
pp. e9-e13 ◽  
Author(s):  
Jennifer L. Bercaw-Pratt ◽  
Tracy Patel Moorjani ◽  
Xiomara M. Santos ◽  
Lefkothea Karaviti ◽  
Jennifer E. Dietrich
2018 ◽  
Vol 01 (01) ◽  
pp. 69-74
Author(s):  
Wen Sun ◽  
Yonghong Wang ◽  
Jian Yu ◽  
Min Ji

Introduction: McCune–Albright Syndrome (MAS) is a rare congenital sporadic disorder characterized by fibrous bone dysplasia, café-au-lait pigmented spots on the skin, and non-gonadotropin dependent precocious puberty (PP), which is caused by a postzygotic somatic activating mutation in the GNAS gene encoding the alpha subunit of Gs protein. In our case study, we recorded a girl with the onset of MAS and treated her with Chinese medicinal herbs combined with megestrol acetate. We aim to provide a method for the treatment of children with this rare form of precocious puberty. Case Presention: A 4-year-old girl presented with vaginal bleeding and enlarged breasts. The activating mutation of GNAS was not detected in the patient’s peripheral blood samples, as some had reported. Because of peripheral PP and fibrous dysplasia of the diagnosed bone, the patient was considered as MAS. We chose the Chinese medicinal herbs combined with megestrol acetate for treatment, and the patient was effectively treated. Conclusion: The combination therapy of Chinese medicinal herbs plus megestrol acetate in managing PP in an MAS is one of the useful treatments.


2020 ◽  
Vol 40 (2) ◽  
pp. 134-137
Author(s):  
Subhana Thapa Karki ◽  
Vandana Jain

McCune Albright syndrome (MAS) is a very rare genetic disease characterised by any two of the following three findings: café au lait spots, polyosteotic fibrous dysplasia and endocrine disorders. The clinical presentation of MAS may vary depending on which of the various components of the syndrome predominate. Here, we report one case of MAS presenting with precocious puberty.


1984 ◽  
Vol 18 ◽  
pp. 167A-167A
Author(s):  
Carol M Foster ◽  
Ora H Pescovitz ◽  
Thomas H Shawker ◽  
Judith L Ross ◽  
Gordon B Cutler ◽  
...  

2012 ◽  
Vol 59 (2) ◽  
pp. 111-117 ◽  
Author(s):  
Daniele Tessaris ◽  
Patrizia Matarazzo ◽  
Alessandro Mussa ◽  
Gerdi Tuli ◽  
Francesca Verna ◽  
...  

1985 ◽  
Vol 64 (8) ◽  
pp. 1084-1086 ◽  
Author(s):  
M.W. Roberts ◽  
S.H. Li ◽  
F. Comite ◽  
K.D. Hench ◽  
O.H. Pescovitz ◽  
...  

One hundred and one children with precocious puberty were given an oral examination. Dental root development was assessed using panoramic radiographs. All mandibular canines, pre-molars, and molars which could be visualized without apparent distortion were included. The patients were grouped for analysis according to the etiology of their precocity, e.g., McCune-Albright syndrome, familial male, congenital adrenal hyperplasia, central nervous system lesions, and idiopathic precocious puberty. Dental development was significantly retarded relative to their chronological age in patients with idiopathic precocious puberty. However, no significant abnormal dental development was detected in any of the other groups. Individual oral-facial growth and development remain the primary considerations for timing orthodontic treatment.


2007 ◽  
Vol 92 (6) ◽  
pp. 2100-2106 ◽  
Author(s):  
Penelope Feuillan ◽  
Karim Calis ◽  
Suvimol Hill ◽  
Thomas Shawker ◽  
Pamela Gehron Robey ◽  
...  

PEDIATRICS ◽  
1992 ◽  
Vol 90 (1) ◽  
pp. 102-104
Author(s):  
LAUREN S. FRISCH ◽  
KENNETH C. COPELAND ◽  
PAUL A. BOEPPLE

During the past decade, it has become clear that precocious puberty is a complex disorder with multiple pathophysiologic causes. Although gonadotropin-releasing hormone (GnRH)-dependent mechanisms account for the vast majority of precocious puberty in girls, GnRH-independent processes play an important role in a minority of patients. The recent availability of specific therapies for both GnRH-dependent and GnRH-independent precocious puberty has made it increasingly important for pediatricians to distinguish between these two subsets.1,2 Although most patients with precocious puberty neatly fall into one or the other subset, a significant minority of patients may present a diagnostic challenge even after an extensive evaluation is undertaken.


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