1Department of Forensic Medicine & Toxicology, S.P. Medical College, Bikaner Rajasthan.
2Department of Obst & Gyn, S.P. Medical College, Bikaner Rajasthan.
3Department of Obst & Gyn., J.L.N. Medical College, Ajmer, Rajasthan.
A 19 year old Phenotype female with absent uterus, ovaries, pubic, auxiliary hair, primary amenorrhea and chromatin negative buccal smear is presented. This patient is representative of the androgen insensitivity syndrome.
According to highest estimates (Fausto – sterling et al, 2000) perhaps 1% of live births exhibit some degree of sexual ambiguity and that between 0.1% and 0.2% of live birth are ambiguous enough to become the subject of specialist medical attention, including surgery to disguise their sexual ambiguity, Other sources (Leonard sax, 2002) create a narrower definition of “ True “ intersexual conditions and estimate the incidence as far lower, at approximately 0.018%. The estimated incidence of testicular feminization syndrome varies from 1 in 20,000 (Prader 1957) male births to 1 in 62,400 (Jagiello 1962). This wide variation may be due to the reluctance of some authors to include the incomplete forms in their series.
Isolated cases of malepseudohermaphroditism have been reported during the last 150 years (Ashley 1962) “but it is only since the work of Morris in 1953 that the condition has been recognized as an important clinical entity. He coined the term testicular feminization syndrome.” More recently it has been designated under the term androgen insensitivity syndrome (Timmreck 2003).
Male Pseudo Hermaphrodite, Intersex, Testicular Feminization Syndrome
