Disorder of Sex Differentiation and Development: Report of 3205 Cases

The main disorder are due to sex chromosomal aberration comprising 2644(82.5%), cases ranging from 1211 klinefelter followed by 1025 Turner syndrome, 345 triple X, 49 double Y and 14 cases of XX male. The second group consists 116 cases of gonadal dysgenesis bearing 0.042 risk of malignant transformation for XY gonadal dysgenesis. The 3rd group is hormonal defects consisting 29 cases of congenital adrenal hyperplasia followed by 50 cases of androgen insensitivity eight cases of 5α reducture deficiency and 3 cases of persistent Mullerian duct, which one of them is a 27 well grown up man uterine hernia with good fertility, probably the unic cases of such condition that have been reported in the literature sofar there are also 36 cases of true hermaphrodite