Gynecologic and reproductive outcomes in fibrous dysplasia/McCune-Albright Syndrome
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- dc.contributor.author Boyce, Alison M.
- dc.contributor.author Casey, Rachel K.
- dc.contributor.author Ovejero Crespo, Diana
- dc.contributor.author Murdock, Cynthia M.
- dc.contributor.author Estrada, Andrea
- dc.contributor.author Guthrie, Lori C.
- dc.contributor.author Brillante, Beth A.
- dc.contributor.author Gomez-Lobo, Veronica
- dc.contributor.author Nieman, Lynette K.
- dc.contributor.author Collins, Michael T.
- dc.date.accessioned 2020-03-26T10:32:13Z
- dc.date.available 2020-03-26T10:32:13Z
- dc.date.issued 2019
- dc.description.abstract Background: 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. Methods: 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. Results: 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. Conclusions: 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.
- dc.format.mimetype application/pdf
- dc.identifier.citation Boyce AM, Casey RK, Ovejero Crespo D, Murdock CM, Estrada A, Guthrie LC,et al. Gynecologic and reproductive outcomes in fibrous dysplasia/McCune-Albright Syndrome. Orphanet J Rare Dis. 2019 Apr 29; 14(1):90. DOI: 10.1186/s13023-019-1057-x
- dc.identifier.doi http://dx.doi.org/10.1186/s13023-019-1057-x
- dc.identifier.issn 1750-1172
- dc.identifier.uri http://hdl.handle.net/10230/44032
- dc.language.iso eng
- dc.publisher BioMed Central
- dc.rights This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
- dc.rights.accessRights info:eu-repo/semantics/openAccess
- dc.rights.uri http://creativecommons.org/licenses/by/4.0/
- dc.subject.keyword Abnormal uterine bleeding
- dc.subject.keyword Estrogen
- dc.subject.keyword Fertility
- dc.subject.keyword Gynecology
- dc.subject.keyword Ovarian cyst
- dc.title Gynecologic and reproductive outcomes in fibrous dysplasia/McCune-Albright Syndrome
- dc.type info:eu-repo/semantics/article
- dc.type.version info:eu-repo/semantics/publishedVersion