HY antigen is found on all male cells; however, the only cells that have a receptor for the H-Y antigen are ____?

1.   ?    Located on the genital ridge
2.   ?    In the testis
3.   ?    Sertoli cells
4.   ?    Part of the APUD system

Where is the SRY gene found?

1.   ?    Short arm of X chromosome
2.   ?    Short arm of Y chromosome
3.   ?    Long arm of X chromosome
4.   ?    Long arm of Y chromosome

What cell produces MIS (mullerian inhibiting substance)?

1.   ?    Sertoli cell
2.   ?    Leydig cell
3.   ?    Interstitial cell of Cajal
4.   ?    Spermatogonia

Leydig cells produce which of the following?

1.   ?    Estrogen
2.   ?    17 OH androstenedione
3.   ?    Testosterone
4.   ?    Mullerian inhibiting substance

T or F: Testosterone stimulates the wolffian system to form the vas deferens, seminal vesicles, and epididymis.

1.   ?    True
2.   ?    False

Which substance regulates the development of the external genitalia into penis and scrotum?

1.   ?    Testosterone
2.   ?    Androstenedione
3.   ?    Androgen
4.   ?    Dihydrotestosterone

Which is NOT a mullerian derivative (formed in the absence of HY antigen, and therefore without MIS)?

1.   ?    Ovary
2.   ?    Fallopian tubes
3.   ?    Uterus
4.   ?    Upper 2/3rds of vagina

An absence of testosterone has what effect on wolffian structures?

1.   ?    There is no effect
2.   ?    Regresses
3.   ?    Stimulates

Which typically has an ovotestes?

1.   ?    MGD
2.   ?    True hermaphrodite
3.   ?    Male pseudohermaphrodite
4.   ?    Female pseudohermaphrodite

Which of the three major enzymatic deficiencies found in female PH has a incidence of 1 in 15,000 newborns?

1.   ?    21 hydroxylase deficiency
2.   ?    11 hydroxylase deficiency
3.   ?    3 beta hydroxysteroid dehydrogenase deficiency

T or F: All female PH patients need mineralocorticoid replacement?

1.   ?    True
2.   ?    False

T or F: All female PH patients have normal fertility?

1.   ?    True
2.   ?    False

In female PH, when is clitoral recession and vaginoplasty done?

1.   ?    Neonatal period
2.   ?    3 – 6 months
3.   ?    1 year
4.   ?    Prepubertal

What is the incidence of malignant tumors in the dysgenetic gonad of mixed gonadal dysgenesis?

1.   ?    Less than 5%
2.   ?    10 – 15
3.   ?    50%
4.   ?    75%

What is the most common tumor that develops in the dysgenetic gonad of mixed gonadal dysgenesis?

1.   ?    Dysgerminoma
2.   ?    Seminoma
3.   ?    Gonadoblastoma
4.   ?    Yolk sac tumor

T or F: Surgical reconstruction of MGD (mixed gonadal dysgenesis) patients is essentially same as for female PH?

1.   ?    True
2.   ?    False

T or F: Twenty-five percent of patients with mixed gonadal dysgenesis remain fertile.

1.   ?    True
2.   ?    False

T or F: Since 5-alpha-reductase deficiency is autosomal recessive, carriers have a 12.5% chance of having an affected child.

1.   ?    True
2.   ?    False

Which is NOT one of the three major deficiencies resulting in male pseudohermaphroditism?

1.   ?    Inadequate testosterone production
2.   ?    5 alpha reductase deficiency
3.   ?    Androgen insensitivity
4.   ?    Androgen block syndrome

What is the rarest form of intersex?

1.   ?    Female hermaphroditism
2.   ?    Male hermaphroditism
3.   ?    Mixed gonadal dysgenesis
4.   ?    True hermaphroditism

How is testicular feminization often (incidentally) discovered?

1.   ?    During hernia repair in a male
2.   ?    During hernia repair in a female
3.   ?    During orchiopexy

T or F: In penile agenesis, the testes and scrotum are also absent.

1.   ?    True
2.   ?    False

Which does not usually have an XY karyotype?

1.   ?    MGD
2.   ?    True hermaphrodite
3.   ?    Male pseudohermaphrodite