Turner sendromlu bir gebenin sağlıklı bir şekilde doğurtulması: Olgu Sunumu
Turner sendromu X kromozomunun kısmi veya tam yokluğuna bağlı olarak görülen boy kısalığı, gecikmiş puberte ve primer amenore ile karakterize bir sendromdur. Bu hastalarda ergenlik öncesi over yetmezliği gelişmesi infertiliteye neden olmaktadır. Yardımcı üreme teknikleri ile başarılı gebelik sonuçları bildirilmesine karşın bu kadınlarda gebeliğe bağlı gelişen ölümcül komplikasyonlar önem arz etmektedir. Turner sendromu gebelik için kısmi kontraendikasyon teşkil etmekle birlikte, mevcut kardiyovasküler anomaliler kesin kontraendikasyon olarak belirtilmektedir. Bu yazıda, gebeliği başarılı
Successful delivery in a patient with Turner syndrome: Case Report
Turner syndrome, resulting from the partial or complete loss of an X chromosome, is characterized by short stature, delayed puberty and primary amenorrhea. Before puberty, infertility is caused by the development of premature ovarian failure in these patients. Although successful pregnancies have been reported with the aid of assisted reproduction, women with this syndrome have lethal complications relating to the pregnancy. Turner syndrome is a relative contraindication for pregnancy however; it is an absolute contraindication for pregnancy in a patient with a documented cardiac anomaly. In this article, 31-year-old pregnant patient with Turner syndrome who had undergone successful termination of pregnancy is presented.
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- 1. Sybert VP, McCauley E. Turners syndrome. N Engl J Med. 2004; 351 (12):12271238.
- 2. Hassa H, Aydın Y. [Premature ovarian failure and fertility: Review] Turkiye Klinikleri J Gynecol Obst-Special Topics 2012;5(2):57-64
- 3. Hovatta O. Pregnancies in women with Turners syndrome. Ann Med 1999; 31(2):10610.
- 4. Practice Committee of American Society For Reproductive Medicine. Increased maternal cardiovascular mortality associated with pregnancy in women with Turner syndrome. Fertil Steril 2005;83(4):1074-5.
- 5. Pasquino AM, Passeri F, Pucarelli I, Segni M, Municchi G. Spontaneous pubertal development in Turners syndrome. Italian Study Group for Turners Syndrome. J Clin Endocrinol Metab. 1997;82(6):1810-3.
- 6. Birkebaek N, Crüger D, Hansen J, Nielsen J, Bruun-Petersen G. Fertility and pregnancy outcome in Danish women with Turner syndrome. Clinical Genetics. 2002;61(1):359.
- 7. Borgstrom B, Hreinsson J, Rasmussen C, Sheikhi M, Fried G, Fridstrom M. Fertility preservation in girls with Turner syndrome: prognostic signs of the presence of ovarian follicles. J Clin Endocrinol Metab 2009; 94(1):7480.
- 8. Alves C, Silva SF. Spontaneous procreation in Turner syndrome: report of two pregnancies in the same patient. Syst Biol Reprod Med. 2012;58(2):113-5.
- 9. Gotzsche CO, Krag-Olsen B, Nielsen J, Sorensen KE, Kristensen BO. Preva- lence of cardiovascular malformations and association with karyotypes in Turners syndrome. Arch Dis Child 1994; 71(5):4336.
- 10. Schoemaker MJ, Swerdlow AJ, Higgins CD, Wright AF, Jacobs PA, United Kingdom Clinical Cytogenetics Group. Mortality in women with Turner syndrome in Great Britain: a national cohort study. J Clin Endocrinol Metab 2008; 93(12):473542.
- 11. Bondy CA. Aortic dissection in Turner syndrome. Curr Opin Cardiol 2008; 23(6): 51926.
- 12. Hagman A, Loft A, Wennerholm UB, Pinborg A, Bergh C, Aittomäki K, Nygren KG, Bente Romundstad L, Hazekamp J, SöderströmAnttila V. Obstetric and neonatal outcome after oocyte donation in 106 women with Turner syndrome: a Nordic cohort study. Hum Reprod. 2013;28(6):1598-609
- 13. Boissonnas CC, Davy C, Bornes M, Arnaout L, Meune C, Tsatsatris V, et al. Careful cardiovascular screening and follow-up of women with Turner syndrome before and during pregnancy is necessary to prevent maternal mortality. Fertil Steril 2009; 91(3):929.e57.
- 14. Matura L, Ho V, Rosing D, Bondy C. Aortic Dilatation and dissection in Turner syndrome. Circulation 2007; 116(15):166370.
- 15. Practice Committee of American Society For Reproductive Medicine. Increased maternal cardiovascular mortality associated with pregnancy in women with Turner syndrome. Fertil Steril 2012; 97(2):2824.
- 16. Gawlik A, Gawlik T, Januszek-Trzciakowska A, Patel H, Malecka-Tendera E. Incidence and dynamics of thyroid dysfunction and thyroid autoimmunity in girls with Turners syndrome: a longterm follow-up study. Horm Res Paediatr. 2011; 76(5): 314-20.
- 17. Roulot D. Liver involvement in Turner syndrome. Liver Int. 2013; 33(1): 24-30.