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Uterine Development in Turner Syndrome« Back to Volume 24, Issue 1, May 2008 - Table of Contents Bakalov and associates performed a cross-sectional study evaluating uterine development in 86 women with Turner syndrome (TS), aged 18 to 45 years, who were participating in a comprehensive NIH study. All subjects had a karyotype by G-banding consistent with TS in at least 70% of 50 white blood cells. The women were evaluated by either transabdominal (n=68) and/or by transvaginal (n=20) ultrasonography. Longitudinal and anterior posterior fundal diameters were calculated as well as the maximal transverse uterine diameter. Normative data were used to characterize uterine maturity. Historical and treatment data including pubertal development, age of initiation of hormone replacement therapy, type of estrogen used, years of estrogen use, and history of growth hormone therapy were recorded. In the case of spontaneous menarche, the time interval from menarche to the development of amenorrhea was noted. The mean age of the study population was 31.8 ± 7.3 years. Most subjects (93%) had a karyotype consistent with TS, while 6 (7%) had mosaicism. None had a Y chromosome (intact or abnormal), 15% had spontaneous menarche at age 12.2 ± 1.7 years, but had developed amenorrhea by their late teens. All other subjects (73/86) had started estrogen at an average age of 15.7 ± 4.1 years. Thirty percent (26/86) had also been treated with growth hormone. Almost one quarter (24.4%; 21/86) had a fully developed uterus both in size and shape, while many (44%; 36/86) had a smaller size uterus (transitional) and 31.4% (27/86) had an immature (cylindrical shaped) uterus. Regression analysis demonstrated that uterine size was influenced significantly by age, years of estrogen use, current use of hormone replacement therapy, history of spontaneous menarche and the type of estrogen medication. There was no correlation between age of first exposure to estrogens and the size of the uterus. The degree of uterine maturity was positively associated with years of estrogen use, history of spontaneous menarche, and negatively associated with the lack of current hormone replacement therapy. The authors reviewed recent studies from Germany1 which showed that only mosaic females develop normal uterine size and that karyotype was the only significant predictor of normal uterine development. Findings in the current study were significantly different and 57% of the subjects with a mature uterus had a 45,X karyotype. This may be explained by an average longer duration of estrogen exposure. The authors stated that these findings are encouraging for those women with TS who wish to carry a successful pregnancy. A recent review of women with TS in the US participating in oocyte donation programs found that 69% became pregnant and these pregnancies resulted in the birth of a live infant.2 Editor’s CommentThese authors presented some truly encouraging information for endocrinologists to share with their patients with TS. Indeed hormone replacement therapy is associated with normal uterine development while the age of starting hormone replacement therapy is not a critical factor. Thus those women with TS who wish to participate in oocyte donation programs should be encouraged to do so or may be encouraged to do so with reasonably good assurance that their uterus should be capable of sustaining a normal pregnancy. As the authors noted, their study could have unexpected biases due to its cross-sectional nature. William L. Clarke, MD References - (linked to
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Volume 24 Number 1
May 2008
www.GGHjournal.com
ISSN 1932-9032
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