الفهرس | Only 14 pages are availabe for public view |
Abstract Ambiguous genitalia constitutes one of the most scinating and complex disease processes encountered in nfants and children. Over the past decade, advances in cell biology, genetic and biochemical techniques have provided new into the precise mechanisms responsible for sexual ’differentation and specific intersex disorders. Among the disorders of sexual differentation, the four most causes of ambiguous genitalia are female and male pseudohermaphrodi’tism, true hermaphroditism and mixed gonadal dysgenesis. The birth of a baby with ambiguous genitalia is a social as well as a medical emergency. An almost immediate choice must be made about the sex of rearing which means that the diagnosis must be established with accuracy as rapidly as possible. Any delay may result in death in early infancy from an uncorrected metabolic disorder, the appearance of Lnappr oprate secondary sexual changes at puberty or malignant degeneration of the gonads. An early but inappropriate see choice can also be disastrous and sex l;’eversal aftelr the age of two years will have marked. |