MZZamanpour MD Differentiation of male and female external genitalia as proceeding from a common embryonic anlage Testosterone acts at 9 to 13 weeks of gestation to virilize ID: 935893
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Slide1
DISORDERS OF SEXUAL DEVELOPMENT
MZ.Zamanpour MD
Slide2Differentiation
of male
and female external genitalia as proceeding from a common embryonic anlage
Testosterone acts
at 9
to 13 weeks of gestation to
virilize
the
bipotential
anlage
.
In the absence
of
testosterone action
, the female phenotype develops
Slide3A diagrammatic scheme of male sex determination
and differentiation.
SRY
is the master gene controlling male sex differentiation, but
there are many other genes and their products
that control
male and female sexual differentiation
.
DHT,
Dihydrotestosterone
;
SRY
,
the gene for the
testis-determining
factor
Slide4The terms
intersex, hermaphroditism,
and pseudohermaphroditism are
not useful
Slide546,XX Disorders of Sexual Development
The terms
intersex, hermaphroditism
,
and
pseudohermaphroditism
are not useful
Slide646,XY Disorders of Sexual Development
Slide7Sex Chromosome and Ovotesticular
Disorders
of Sexual Developmentchromosome DSDTurner syndromeKlinefelter
syndromeMixed gonadal dysgenesis (45,X; 46,XY
)
genitalambiguity
, asymmetric external
genitalia
, and
inguinal hernias
ovotesticular
DSD
Slide8atypical genitalia
ovotesticular
disorder of sexual development
(A)
and
congenital
virilizing
adrenal
hyperplasia
(B-E)
Slide95α
-
Reductase deficiency
Slide10Partial androgen insensitivity
Slide11Partial androgen
insensitivity syndrome at adolescence, male
sex of rearing