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Sex” is gift of nature. What if she betrays?

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Author(s): K. B. Ashok

Journal: International Journal of Collaborative Research on Internal Medicine & Public Health
ISSN 1840-4529

Volume: 3;
Issue: 3;
Start page: 277;
Date: 2011;
Original page

Keywords: Intersex | Pseudo-harmaphodism | sex disorder

ABSTRACT
Introduction: Intersex is a condition of abnormal sexual maturation resulting in abnormal karyotype orprimary or secondary sexual characteristics which can be diagnosed by ambiguous genitalia or abnormalcharacteristics in adolescent. Prevalence of this rare condition is too low (1 in 20,000 live births). It isvery confusing for a doctor to determine the treatment chapter which includes proper diagnostictechnique, sufficient time for interpretation and intervention and informing the patient. But the steps aretoo confusing and controversial. Prescribed case made us highly confused by its characteristics which wasnone of 5-alpha reductage deficiency syndrome, Reifensteine Syndrome, Pseudo-harmaphodism and alsohaving a balance between a male and female which is very rare and unique. As the diagnosis was difficultto define, initiatives were taken to reveal the proper diagnosis for better treatment so that this kind ofcases in future could be managed properly.Case Presentation/Description: 15 years old unmarried girl came to Gynaecology outpatient departmentcomplaining of primary amenorrhoea, gradual swelling of the external genitalia since 12 years of age andhoarseness of voice since last month.Physically she was tall, with male pattern pubic hair, tanner stage 0 sized breast, having cliteromegalyrather micropenis, perineal hypospadius, palpable mass in both labia major and a blunt space resemblingvagina in between.Laboratory investigations revealed very high Testosterone (246ng/dl) and Thyroid Stimulating Hormone(8.09 mcIU/L) but low level of Follicle Stimulating Hormone (13.83IU/L) and Oestrogen (17pg/dl).Cytogenic study showed 46XY/46XX mosaic chromosomal pattern.In laparoscopy there was no internal female genital organ.Biopsy of the labial mass was done after operation which depicted seminiferous tubules lined by sertolicells with no sperm production and interstitial fibrosis.Case was very much mysterious as it was in a balance to support both sexes. After informing the familymembers first, the society had become an obstruction for the doctors to treat with freedom. Thenaccording to the will of the family members and for the sake of the society diagnosis was hidden frompatient and managed by Gonadectomy, Clitorectomy, and Vaginoplasty in same sitting with hormonaltherapy for life long to make her a “girl” again.After 2 months of the treatment patient came with a good respond towards the hormone therapy. Our caseproved that ‘Intersex’ is a mystery of nature and do not always follow the known clinical features.Discussion: Sometimes we treat the patient for sake of society, family etc. and never think about thepatient’s will. This case was not a different in this aspect. Doctors were bound to follow the rules made bysociety without protest. Patient of INTERSEX is one of them who have nothing to do against the Nature’sbetray. More they need physical rehabilitation, ramification, adaptation and a helping hand to come out ofgasping agony inside.
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