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Abstract Disorders of sexual development (DSD), formerly termed intersex conditions, are among the most fascinating conditions encountered by the clinician. The ability to diagnose these conditions has advanced rapidly in recent years. It is applied to human beings whose biological sex cannot be determined as either male or female from its outward appearance, and does not necessarily mean that the person has both male and female reproductive organs. The sexual organs are ambiguous and has neither the standard male nor the standard female anatomy. About 1 percent of live births exhibit some degree of sexual ambiguity, between 0.1% and 0.2% of live births are ambiguous enough to become the subject of specialist medical attention There are several possible causes for the various forms and degrees of intersexuality: chromosomal, hormonal and enviromental causes. Logical workup in infants with ambiguous genitalia include:chromosomal analysis, endocrine screening, serum chemistries, androgen-receptor level and 5-alpha reductase type II levels, renal/ bladder ultrasonography, genitography, CT scanning and MRI, exploratory laparotomy/gonadal biopsy, diagnostic laparoscopy/ gonadal biopsy. Medical therapy of intersexuals: depends on the underlying cause such as children with CAH require corticosteroid therapy. Surgical therapy: depends on the part which can be reconstructed, feminizing genitoplasty (vaginoplasty and clitoroplasty) is recommended in virilized females and surgical reconstruction of hypospadias is recommended in undervirilized males. Psychosocial care: important to maintain the normal life for intersexuals. |