Gynecologic and reproductive outcomes in fibrous dysplasia/McCune-Albright syndrome

Autor:	Andrea Estrada, Alison M. Boyce, Rachel K. Casey, Lori C. Guthrie, Michael T. Collins, Veronica Gomez-Lobo, Diana Ovejero Crespo, Lynette K. Nieman, Cynthia M. Murdock, Beth A Brillante
Jazyk:	angličtina
Rok vydání:	2019
Předmět:	0301 basic medicine Infertility Adult musculoskeletal diseases medicine.medical_specialty Adolescent medicine.medical_treatment Population Puberty Precocious Abnormal uterine bleeding lcsh:Medicine 030105 genetics & heredity Fibrous Dysplasia Polyostotic McCune–Albright syndrome 03 medical and health sciences Young Adult 0302 clinical medicine medicine Precocious puberty Humans Pharmacology (medical) Bone pain education Genetics (clinical) Aged Aged 80 and over Pregnancy education.field_of_study Hysterectomy business.industry Obstetrics Fibrous dysplasia Reproduction lcsh:R General Medicine Middle Aged medicine.disease Estrogen Fertility Gynecology Ovarian cyst Female medicine.symptom business Infertility Female 030217 neurology & neurosurgery Research Article
Zdroj:	Orphanet Journal of Rare Diseases, Vol 14, Iss 1, Pp 1-7 (2019) Orphanet Journal of Rare Diseases
ISSN:	1750-1172
DOI:	10.1186/s13023-019-1057-x
Popis:	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.
Databáze:	OpenAIRE
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