Gynecologic and reproductive outcomes in fibrous dysplasia/McCune-Albright syndrome
Boyce, Alison M. (National Institutes of Health (Estats Units d'Amèrica))
Casey, Rachel K. (Pediatric Specialists of Virginia (Estats Units d'Amèrica))
Ovejero Crespo, Diana (Institut Hospital del Mar d'Investigacions Mèdiques)
Murdock, Cynthia M. (Reproductive Medicine Associates of Connecticut (Estats Units d'Amèrica))
Estrada, Andrea (Children's National Health System (Estats Units d'Amèrica))
Guthrie, Lori C. (National Institutes of Health (Estats Units d'Amèrica))
Brillante, Beth A. (National Institutes of Health (Estats Units d'Amèrica))
Gomez-Lobo, Veronica (MedStar Washington Hospital Center (Estats Units d'Amèrica))
Nieman, Lynette K. (The Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health.)
Collins, Michael T. (National Institutes of Health (Estats Units d'Amèrica))
Universitat Autònoma de Barcelona

Data:	2019
Resum:	Autonomous ovarian activation with recurrent estrogen-producing cysts is a hallmark feature of the rare bone and endocrine disorder fibrous dysplasia/McCune-Albright syndrome. Precocious puberty in girls with McCune-Albright syndrome has been well-described, however long-term effects on gynecologic and reproductive function are unknown. Concerningly, case reports have described poor skeletal outcomes associated with pregnancy in women with fibrous dysplasia. Thirty-nine women with fibrous dysplasia/McCune-Albright syndrome were evaluated as part of a natural history study. Clinical, radiographic, and biochemical data were reviewed. Women were contacted to obtain detailed menstrual and reproductive histories. Abnormal uterine bleeding affected 77% of women (30/39), and was associated with severe anemia requiring blood transfusion in 3 cases. Nine women underwent hysterectomy for management of bleeding, including 67% (6/9) at the unusually young age of less than age 35 years. Infertility affected 43% of women (9/21), including 2 women who developed primary ovarian insufficiency after undergoing surgical treatment of ovarian cysts. Of 25 spontaneous pregnancies in 14 women, 35% (8) were unplanned. Among the 14 pregnancies, pregnancy was associated with no change in bone pain in 7 subjects (53%), increased bone pain in 4 subjects (31%), and decreased bone pain in 2 subjects (15%). No additional skeletal complications were reported during pregnancies. Women with fibrous dysplasia/McCune-Albright syndrome report a high prevalence of gynecologic morbidity and reduced fertility. There is no clear association between pregnancy and poor skeletal outcomes in this population.
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Matèria:	Ovarian cyst ; Abnormal uterine bleeding ; Gynecology ; Fertility ; Estrogen
Publicat a:	Orphanet journal of rare diseases, Vol. 14 (april 2019) , ISSN 1750-1172

DOI: 10.1186/s13023-019-1057-x
PMID: 31036049

7 p, 1.4 MB

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