Atypical McCune-Albright syndrome associated with growth hormone-prolactin pituitary adenoma: natural history, long-term follow-up, and SMS 201-995--bromocriptine combined treatment results.

1992 ◽  
Vol 75 (4) ◽  
pp. 1166-1169
Author(s):  
N Cremonini ◽  
E Graziano ◽  
V Chiarini ◽  
A Sforza ◽  
G A Zampa
Keyword(s):  
Growth Hormone ◽  
Pituitary Adenoma ◽  
Combined Treatment ◽  
Treatment Results ◽  
Mccune Albright Syndrome ◽  
Long Term Follow Up ◽  
Albright Syndrome ◽  
Mccune Albright

Long-Term Follow-Up of a Patient With McCune-Albright Syndrome by Whole-Body Bone Scan and SPECT

2004 ◽  
Vol 29 (11) ◽  
pp. 712 ◽  
Author(s):  
Manabu Fujii ◽  
Shigeru Kosuda ◽  
Motoyuki Jitsu ◽  
Daisuke Maeda ◽  
Shoichi Kusano ◽  
...  
Keyword(s):  
Bone Scan ◽  
Whole Body ◽  
Mccune Albright Syndrome ◽  
Long Term Follow Up ◽  
Albright Syndrome ◽  
Body Bone ◽  
Mccune Albright

Long-Term Follow-Up of a Case of Cheek Hyperpigmentation Associated with McCune-Albright Syndrome Treated with Q-Switched Ruby Laser

2011 ◽  
Vol 37 (2) ◽  
pp. 263-266 ◽  
Author(s):  
TOSHIYUKI OZAWA ◽  
CHIHARU TATEISHI ◽  
MAKIKO SHIRAKAWA ◽  
EMI MURAKAMI ◽  
MASAMITSU ISHII ◽  
...  
Keyword(s):  
Ruby Laser ◽  
Mccune Albright Syndrome ◽  
Long Term Follow Up ◽  
Albright Syndrome ◽  
Mccune Albright

McCune-Albright syndrome. Long-term follow-up

JAMA ◽  
1986 ◽  
Vol 256 (21) ◽  
pp. 2980-2984 ◽  
Author(s):  
P. A. Lee
Keyword(s):  
Mccune Albright Syndrome ◽  
Long Term Follow Up ◽  
Albright Syndrome ◽  
Mccune Albright

Pituitary adenoma in McCune-Albright syndrome: Follow-up information

1976 ◽  
Vol 89 (1) ◽  
pp. 159 ◽  
Author(s):  
Elmer S. Lightner ◽  
Robert Penny ◽  
S. Douglas Frasier
Keyword(s):  
Pituitary Adenoma ◽  
Mccune Albright Syndrome ◽  
Albright Syndrome ◽  
Mccune Albright

scholarly journals Regulation of spermatogenesis in McCune–Albright syndrome: lessons from a 15-year follow-up.

2008 ◽  
Vol 158 (6) ◽  
pp. 921-927 ◽  
Author(s):  
Filippo De Luca ◽  
Valérie Mitchell ◽  
Malgorzata Wasniewska ◽  
Teresa Arrigo ◽  
Maria Francesca Messina ◽  
...  
Keyword(s):  
Gene Encoding ◽  
Α Subunit ◽  
Mccune Albright Syndrome ◽  
Sexual Precocity ◽  
Albright Syndrome ◽  
Histology And Immunohistochemistry ◽  
Shed Light ◽  
Mccune Albright

ContextMcCune–Albright syndrome (MAS) is a disorder caused by a post-zygotic gain-of-function mutation in the gene encoding the Gs-α protein. Sexual precocity, common in girls, has been reported in only 15% of boys, and little is known on the long-term evolution of MAS in males.ObjectiveIn a boy with MAS, we studied spermatogenesis, testis histology, and immunohistochemistry with the aim to shed light on seminiferous tubule activity.DesignA boy who presented at the age of 2.9 years with sexual precocity, monolateral macroorchidism, increased testosterone levels, and suppressed gonadotropins was followed up until the age of 18.ResultsThroughout follow-up testicular asymmetry persisted and gonadotropin and testosterone pattern did not change. At the age of 18, inhibin B was undetectable while α-immunoreactive inhibin was within normal range. Anti-Mullerian hormone level was slightly subnormal. Sperm cells were 3 900 000 per ejaculate. Histology of both testes showed spermatogonia, spermatocytes, and, in some tubes, matured spermatozoa. Sertoli cells were markedly stained with anti-inhibin α-subunit antibody in both the testes. There was no immunostaining of Sertoli, Leydig, or germ cells with anti-βA or anti-βB antibody. MAS R201H mutation was identified in both the testes.ConclusionThe 15-year follow-up in this boy with MAS demonstrated that autonomous testicular activation and gonadotropin suppression persisted over time. This provides an interesting model of active spermatogenesis despite long-term FSH suppression. It also suggests that FSH is needed for the full expression of the inhibin βB-subunit gene, an expression previously reported in the germ and Leydig cells of normal adult subjects.

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

Fibrous dysplasia in McCune Albright syndrome; treatment and follow up

2015 ◽  
Author(s):  
Damla Gokeen ◽  
Samim Ozen ◽  
Nurhan Ozcan ◽  
Sukran Darcan
Keyword(s):  
Fibrous Dysplasia ◽  
Mccune Albright Syndrome ◽  
Albright Syndrome ◽  
Mccune Albright
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