Review Article
Rare Forms of Disorders of Sex Development (DSD) in Patients with Female Phenotype
Kristesashvili J1,2*, Chipashvili M3,4, Jorbenadze T5 and Greydanus DE61Center for Reproductive Medicine, Georgia
2Department of Obstetrics, Gynecology and Reproductology, Tbilisi State University, Georgia
3Zhordania Institute of Human Reproduction, Georgia
4Department of Pharmacology, Tbilisi State University, Georgia
5Department of Pathology, Tbilisi State University, Georgia
6Pediatrics and Human Development, Michigan State University, East Lansing & Kalamazoo, Michigan, USA
- *Corresponding Author:
- Jenara Keristesashvili
Associate Professor, Department of Obstetrics
Gynecology and Reproductology, Tbilisi State University
2 Chiaruli street, Tbilisi, 0159, Georgia
Tel: +995593306294
E-mail: jenarakrist@hotmail.com
Received date: May 26, 2016; Accepted date: June 23, 2016; Published date: June 29, 2016
Citation: Kristesashvili J, Chipashvili M, Jorbenadze T, Greydanus DE (2016) Rare Forms of Disorders of Sex Development (DSD) in Patients with Female Phenotype. J Community Med Health Educ 6:446. doi:10.4172/2161-0711.1000446
Copyright: © 2016 Kristesashvili J, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Abstract
Disorders of Sex development (DSD) belong to uncommon pathologies; in addition, there are especially rare forms, such are ovotesticular disorders (OT), Turner Syndrome and early malignisation of intraabdominal located gonads in the cases of androgen insensitivity syndrome. In this article we present 5 rare cases of DSD in female phenotype patients: 3 cases of ovotesticular DSD with 46,XX and 46,XY karyotypes; 1 familial case of AIS with early malignancy (19-year-old) of intraabdominally-located testicle in older sibs; and a case of spontaneous menstruation in a patient with Turner syndrome and mosaic karyotype 45,X/47,XXX. Rare cases of DSD are connected with diagnostic and management difficulties and so description of each such case and collection of data in this field is very important from a scientific as well as a practical point of view. Determination of prognosis and adequate management of each individual patient are also essential. Study of this issue is especially sensitive in the case of adolescent patients, in order to avoid physiological stress, to reduce health risks, and to improve the quality of their life.