McCune-Albright syndrome: persistence of autonomous ovarian hyperfunction during adolescence and early adult age.
Gonadal hyperfunction is the most frequent endocrine dysfunction in females with McCune-Albright syndrome (MAS). Peripheral precocious puberty is usually the first MAS manifestation in children, characterized by episodes of hypersecretion of estrogens with a consequent reduction in gonadotropin secretion. Little is known about the course of this endocrine disease in adolescence and during young adult life. The aim of this study was to evaluate ovarian function in 10 females with MAS (age 11.4-20.1 years) to detect the persistence of autonomous ovarian hyperfunction throughout and following adolescence, after at least 1 year wash out of any treatment for precocious puberty. LH, FSH, estradiol, prolactin, androgen secretion, ovarian and breast sonography in luteal and follicular phases of some menstrual cycles were evaluated. We demonstrated the persistence of some ovarian autonomy, documented by hyperestrogenism and/or low or absent gonadotropin secretion and/or ovarian cysts.
Matarazzo
Patrizia
P
Department of Pediatric Endocrinology, Regina Margherita Children's Hospital, Turin, Italy. matarpat@libero.it
Lala
Roberto
R
Andreo
Maria
M
Einaudi
Silvia
S
Altare
Franco
F
Viora
Elsa
E
Buzi
Fabio
F
De Luca
Filippo
F
De Sanctis
Vincenzo
V
Rigon
Franco
F
Wasniewska
Malgorzata
M
de Sanctis
Luisa
L
de Sanctis
Carlo
C
D000293:Adolescent; D000328:Adult; D000365:Age Determination by Skeleton; D002648:Child; D002675:Child, Preschool; D004967:Estrogens / Q000378:metabolism*; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D005500:Follow-Up Studies; D006062:Gonadotropins / Q000502:physiology; D006801:Humans; D008597:Menstrual Cycle; D010048:Ovarian Cysts / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D010049:Ovarian Diseases / Q000209:etiology / Q000378:metabolism; D010053:Ovary / Q000000981:diagnostic imaging / Q000503:physiopathology*; D011629:Puberty, Precocious / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome; D014463:Ultrasonography
D004967:Estrogens; D006062:Gonadotropins; D013256:Steroids