scholarly journals McCune Albright syndrome and bilateral adrenal hyperplasia: the GNAS mutation may only be present in adrenal tissue

HORMONES ◽  
2015 ◽  
Author(s):  
Anna Angelousi ◽  
Filip Fencl ◽  
Fabio Faucz ◽  
Jana Malikova ◽  
Zdenek Sumnik ◽  
...  
Keyword(s):  
Adrenal Hyperplasia ◽  
Mccune Albright Syndrome ◽  
Adrenal Tissue ◽  
Bilateral Adrenal Hyperplasia ◽  
Albright Syndrome ◽  
Gnas Mutation ◽  
Mccune Albright

Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome

2017 ◽  
Vol 88 (3-4) ◽  
pp. 285-290 ◽  
Author(s):  
Tomoyo Itonaga ◽  
Hironori Goto ◽  
Manabu Toujigamori ◽  
Yasuharu Ohno ◽  
Seigo Korematsu ◽  
...  
Keyword(s):  
Cushing Syndrome ◽  
Adrenal Crisis ◽  
Adrenal Hyperplasia ◽  
Unilateral Adrenalectomy ◽  
Mccune Albright Syndrome ◽  
Total Adrenalectomy ◽  
Bilateral Adrenal Hyperplasia ◽  
New Treatment ◽  
Albright Syndrome ◽  
Mccune Albright

Background: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. Objective: A 6-month-old girl presented with café-au-lait spots, short stature, central obesity, a moon face, and hypertension. Endocrinological tests and imaging studies led to the diagnosis of ACTH-independent Cushing syndrome due to bilateral adrenal hyperplasia induced by MAS. “Three-quarters adrenalectomy”, namely right-sided total adrenalectomy and left-sided half adrenalectomy, was carried out. An activating mutation of the GNAS1 gene (p.Arg201Cys) was identified in the adrenal tissues. Since the operation, our patient has been in a state of clinical remission for more than 2 years. Conclusion: Our original surgical intervention, three-quarters adrenalectomy, may be a new treatment option for Cushing syndrome associated with MAS.

Cushing’s syndrome caused by nodular adrenal hyperplasia in children with McCune-Albright syndrome

1999 ◽  
Vol 134 (6) ◽  
pp. 789-792 ◽  
Author(s):  
Jeremy M.W. Kirk ◽  
Caroline E. Brain ◽  
Dennis J. Carson ◽  
John C. Hyde ◽  
David B. Grant
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Mccune Albright Syndrome ◽  
Albright Syndrome ◽  
Mccune Albright

Dental Development in Precocious Puberty

1985 ◽  
Vol 64 (8) ◽  
pp. 1084-1086 ◽  
Author(s):  
M.W. Roberts ◽  
S.H. Li ◽  
F. Comite ◽  
K.D. Hench ◽  
O.H. Pescovitz ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Growth And Development ◽  
Orthodontic Treatment ◽  
Dental Development ◽  
Adrenal Hyperplasia ◽  
Facial Growth ◽  
Mccune Albright Syndrome ◽  
Panoramic Radiographs ◽  
Albright Syndrome ◽  
Mccune Albright

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.

scholarly journals Pathophysiology of Peripheral Precocious Puberty in girls: an overview

2021 ◽  
Vol 1 (4) ◽  
Author(s):  
Konstantina Toutoudaki ◽  
George Paltoglou ◽  
Eleni Paschalidou ◽  
Ermioni Tsrana ◽  
Panagiotis Christopoulos
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Precocious Puberty ◽  
Adrenal Hyperplasia ◽  
Mccune Albright Syndrome ◽  
Albright Syndrome ◽  
Peripheral Precocious Puberty ◽  
Mccune Albright

Precocious puberty can be distinguished in central, or peripheral, based on the presence or absence of the Hypothalamic- Pituitary- Gonadal axis activation. The pathogenesis of peripheral precocious puberty (PPP) is based mainly on excessive estrogenic exposure, either endogenous or exogenous. The congenital causes of PPP include McCune- Albright syndrome (MAS) and Congenital Adrenal Hyperplasia (CAH). The main causes of acquired PPP are oestrogen producing tumours, which are mainly of ovarian or adrenal origin, hypothyroidism and environmental oestrogens or substances with estrogenic function.

Abstract #39: McCune-Albright Syndrome in Conjunction with Acromegaly and Subclinical Hyperthyroidism

2003 ◽  
Vol 9 ◽  
pp. 15-16
Author(s):  
Manju Chandran ◽  
Eric N. Gold ◽  
Roopa Sathyaprakash ◽  
Leonard J. Deftos
Keyword(s):  
Subclinical Hyperthyroidism ◽  
Mccune Albright Syndrome ◽  
Albright Syndrome ◽  
Mccune Albright

Atypical Femoral Fracture in McCune-Albright Syndrome

2020 ◽  
Vol 7 (3) ◽  
pp. 147-152
Author(s):  
Salman Ghaffari ◽  
◽  
Mehran Razavipour ◽  
Parastoo Mohammad Amini ◽  
◽  
...  
Keyword(s):  
Femoral Fracture ◽  
Dynamic Compression ◽  
Plate Fixation ◽  
Medullary Canal ◽  
Mccune Albright Syndrome ◽  
Café Au Lait Spots ◽  
History Of ◽  
Albright Syndrome ◽  
Mccune Albright

McCune-Albright Syndrome (MAS) is characterized by endocrinopathies, café-au-lait spots, and fibrous dysplasia. Bisphosphonates are the most prescribed treatment for reducing the pain but their long-term use has been associated with atypical fractures of cortical bones like femur in patients. We present a 23-year-old girl diagnosed with MAS. She had an atypical mid-shaft left femoral fracture that happened during simple walking. She also had a history of long-term use of alendronate. Because of the narrow medullary canal, we used 14 holes hybrid locking plate for the lateral aspect of the thigh to fix the fracture and 5 holes dynamic compression plate (instead of the intramedullary nail) in the anterior surface to double fix it, reducing the probability of device failure. With double plate fixation and discontinuation of alendronate, the complete union was achieved five months after surgery

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