Male pseudohermaphroditism is seen in
?
a) 5-a reductase deficiency
b) 21 hydroxylase deficiency
c) 17 hydroxylase deficiency
d) a and c
Correct Answer - D
Ans. is ‘a’ i.e., 5-a reductase deficiency; ‘c’ i.e., 17 hydroxylase
deficiency
Male pseudohermaphroditism
Genotype is XY
External genitalia are female
Causes of Male Pseudohermaphroditism
A. Defect in testicular differentiation
- Deletion of short ann Y chromosome 3. Mixed gonadal
dysgenesis (MGD). - XY pure gonadal dysgenesis. 4. Dysgenetic
male pseudohennaphroditism (DMP).
B. Defect in testicular hormone synthesis - Leydig cell aplasia
- Inborn error of testosterone biosynthesis
i) 17-alpha hydroxyalse deficiency iv) 3 beta-hydroxy
steroid dehydrogenase deficiency.
ii) 17-20 lyase deficiency v) 20-22
desmolase deficiency
iii) 17-ketosteroid reductase deficiency.
C. Defect in mullerian inhibiting hormone action
D. Defect in androgen action
-alpha reductase deficiency 4. Reifenstein
syndrome - Testicular feminization syndrome 5. Undertermined
etiology - Incomplete testicular feminization syndrome
True hermaphroditism
Both ovarian and testicular tissues are present either in the same
(ovotestis) or opposite gonads.