today we will discuss a very important topic of the disorders of the sex development and that is the survive syndrome let us talk about its epidemiology severe syndrome is a rare disorder where the incidence in general population of about 1 in 80 000 and what is the etiology of the silver Central survive syndrome is caused by the mutation in Assad YG on the Y chromosome and it inhibits the differentiation of the embryonic goodies into testes so here you can see the Y chromosome and the defective assignment Gene let us talk about the karyotype of sewire syndrome the patience of Soviet syndrome or X Y females with the phenotype or female and the can you type is 46xy let us talk about the clinical features of severe syndrome the clinical features include tall Stitcher the internal genitalia include small uterus and the fallopian tubes when we talk about the external genitalia these patients have female external genitalia with the vagina and large clitoris and what about the grenades these patients have got the absent ovaries or even if the ovaries are present these are functionalists or the street ovaries that is why the children syndrome is considered to be an example of pure grenadyl distress or the complete konital dystenesis so what is the presentation of the turn of the Savar syndrome uh in short words we would like to say that patients of cervoir syndrome presents with primary amenorrhea and the absence secondary sexual characteristics so without the diagnostic tests or the investigations done in the levels are normal in these patients and when estrogen is done that is found to be decreased let us talk about its treatment very important to know that the treatment of survived syndrome is done by hormone replacement therapy and in the long term these sort of patients need gonadectomy and why gonidectomy is done in these patients it's because when the streak ovaries are left in situ they carry a very high risk of Bonito blastoma so there is no point of keeping those grenades which are basically functionless so the best surgical option adopted in these patient is the surgical removal of these grenades so that brings us to the end of our presentation it was a kind of short video related to survival syndrome let me complete it with these golden words patience persistence and perseverance are essential for achieving any goal personal or professional success is the result of working the process that is necessary for the time that is required thank you so much wish you all the best in every step of your life Allah Hafiz