DEFECTS IN SEX DIFFERENTIATION
1. GONADAL DYSGENESIS:
• It is improper formation of gonads , Neither testis nor ovary develops.
• due to failure of migration of primordial germ cells to genital ridge.
2. HERMAPHRODITISM (INTERSEX):
A )True hermaphrodite:
• In 70% of cases karyotype is 46 XX, Has gonads of both sexes
• there is usually a uterus. External genitalia are ambiguous
B)Pseudohermaphrodite:
Has gonads of one sex & external genitalia of opposite sex.
– Female pseudohermaphrodite:
Cause: Congenital adrenal hyperplasia
(( exposure of female fetus to excessive androgens ))
Features:
• Patient has 46 XX chromosomes
• Has ovaries + masculinization of external genitalia
1. GONADAL DYSGENESIS:
• It is improper formation of gonads , Neither testis nor ovary develops.
• due to failure of migration of primordial germ cells to genital ridge.
2. HERMAPHRODITISM (INTERSEX):
A )True hermaphrodite:
• In 70% of cases karyotype is 46 XX, Has gonads of both sexes
• there is usually a uterus. External genitalia are ambiguous
B)Pseudohermaphrodite:
Has gonads of one sex & external genitalia of opposite sex.
– Female pseudohermaphrodite:
Cause: Congenital adrenal hyperplasia
(( exposure of female fetus to excessive androgens ))
Features:
• Patient has 46 XX chromosomes
• Has ovaries + masculinization of external genitalia
