[MCQ] Disorders of the Testes and Male Reproductive System- Part 3

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Disorders of the Testes and Male Reproductive System- Part 3

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Question 1

Which of the following is measured to detect presence of testes in prepubertal boys with cryptorchidism ?

A	Inhibin B
B	Müllerian inhibiting substance (MIS)
C	Testosterone
D	All of the above

Question 1 Explanation:

Measurement of MIS, a Sertoli cell product, is estimated to detect the presence of testes in prepubertal boys with cryptorchidism.

Question 2

Normal semen ejaculate volume is ?

A	1 to 3 mL
B	2 to 6 mL
C	5 to 8 mL
D	8 to 10 mL

Question 2 Explanation:

The normal ejaculate volume is 2 - 6 mL & contains sperm counts of >20 million/mL, with a motility of >50% and >15% normal morphology.

Question 3

Puberty in boys before what age is considered precocious ?

A	9 years
B	10 years
C	11 years
D	12 years

Question 3 Explanation:

Puberty in boys before age 9 years is considered precocious. Precocity can be isosexual or heterosexual. Isosexual precocity can be gonadotropin-dependent or gonadotropin-independent.

Question 4

Which of the following is false about central precocious puberty (CPP) ?

A	Gonadotropin-Dependent
B	Less common in boys than in girls
C	Elevated gonadotropin levels
D	None of the above

Question 4 Explanation:

CPP is caused by premature activation of GnRH pulse generator and is characterized by gonadotropin levels that are inappropriately elevated for age. Because pituitary priming has occurred, GnRH elicits LH & FSH responses typical of those seen in puberty or in adults.

Question 5

Which of the following is not a cause of gonadotropin-independent precocious puberty in boys ?

A	hCG-secreting tumors
B	Hyperthyroidism
C	Congenital adrenal hyperplasia
D	McCune-Albright syndrome

Question 5 Explanation:

Gonadotropin-independent causes of precocious puberty in boys are congenital adrenal hyperplasia, hCG -secreting tumor, McCune-Albright syndrome, hypothyroidism, activating LH receptor mutation & exogenous androgens. Androgens from the testis or the adrenal are high.

Question 6

Which of the following disorders is also called testotoxicosis ?

A	McCune-Albright syndrome
B	Familial male-limited precocious puberty
C	Congenital adrenal hyperplasia
D	None of the above

Question 6 Explanation:

Familial male-limited precocious puberty is also called testotoxicosis. It is an autosomal dominant disorder due to activating mutations in LH receptor leading to increased testosterone production. Testosterone levels are elevated and LH is suppressed.

Question 7

Treatment options for familial male-limited precocious puberty include ?

A	Ketoconazole
B	Flutamide
C	Anastrazole
D	All of the above

Question 7 Explanation:

Treatment options for familial male-limited precocious puberty include inhibitors of testosterone synthesis (ketoconazole), androgen receptor antagonists (flutamide) & aromatase inhibitors (anastrazole).

Question 8

Which of the following skin lesion is characteristic of McCune- Albright syndrome ?

A	Erythroderma
B	Café au lait spots
C	Telangiectasia
D	Scarring alopecia

Question 8 Explanation:

Cafe-au-lait spots are characteristic skin lesions and reflect the onset of somatic mutations in melanocytes during embryonic development

Question 9

In McCune-Albright syndrome, mutation occurs in which of the following ?

A	Gsalpha subunit
B	Gsbeta subunit
C	Gsgamma subunit
D	Gsdelta subunit

Question 9 Explanation:

McCune-Albright syndrome is caused by somatic activating mutations in Gs subunit that links G protein-coupled receptors to intracellular signaling pathways. Mutations impair GTP activity of the Gs protein, leading to constitutive activation of adenylyl cyclase and stimulates testosterone production leading to gonadotropin-independent precocious puberty.

Question 10

Which of the following is false about congenital adrenal hyperplasia (CAH) ?

A	Chronic ACTH stimulation
B	Low LH
C	Small testes
D	None of the above

Question 10 Explanation:

Boys with CAH if untreated with adequate glucocorticoid suppression of ACTH can develop premature virilization because of excessive androgen production by adrenal gland. LH is low & testes are small.

Question 11

Breast enlargement in prepubertal boys can result from ?

A	Familial aromatase excess
B	Marijuana smoking
C	Sertoli cell tumors in the testis
D	All of the above

Question 11 Explanation:

Breast enlargement in prepubertal boys can result from familial aromatase excess, estrogenproducing tumors in adrenal gland, Sertoli cell tumors in testis, marijuana smoking, or exogenous estrogens or androgens and germ cell tumors that secrete hCG.

Question 12

In children with gonadotropin-independent precocious puberty, which of the following is not useful ?

A	Ketoconazole
B	Long-acting GnRH analogues
C	Spironolactone
D	Testolactone

Question 12 Explanation:

Long-acting GnRH analogues can be used to suppress gonadotropins in gonadotropin-dependent precocious puberty. Ketoconazole inhibits steroidogenesis, spironolactone is a weak androgen antagonist and testolactone and letrozole are aromatase inhibitor.

Question 13

Puberty is considered delayed in boys if it has not happend by the age of ?

A	14
B	15
C	16
D	18

Question 13 Explanation:

Puberty is delayed in boys if it has not ensued by age 14 years.

Question 14

Which of the following categories of delayed puberty is more common in girls than in boys ?

A	Constitutional delay of growth and puberty
B	Functional hypogonadotropic hypogonadism caused by systemic illness or malnutrition
C	Hypogonadotropic hypogonadism caused by genetic or acquired defects in the hypothalamic-pituitary region
D	Hypergonadotropic hypogonadism secondary to primary gonadal failure

Question 14 Explanation:

Delayed puberty is more common in boys than in girls. But, functional hypogonadotropic hypogonadism is more common in girls than in boys.

Question 15

Which of the following is the most common cause of delayed puberty ?

A	Constitutional delay
B	Functional hypogonadotropic hypogonadism due to systemic illness or malnutrition
C	Hypogonadotropic hypogonadism due to genetic or acquired defects in hypothalamic-pituitary region
D	Hypergonadotropic hypogonadism secondary to primary gonadal failure

Question 16

Constitutional delay in puberty should be suspected when there is ?

A	Positive family history
B	Delayed bone age
C	Short stature
D	All of the above

Question 16 Explanation:

Constitutional delay should be suspected when there is a family history, evidence of delayed bone age & short stature.

Question 17

Familial hypogonadotropic hypogonadism is transmitted most commonly as ?

A	X-linked
B	Autosomal recessive
C	Autosomal dominant
D	Autosomal codominant

Question 17 Explanation:

Testotoxicosis or familial male-limited precocious puberty is an autosomal dominant disorder.

Question 18

What testicular length generally indicates that the child has entered puberty ?

A	> 1.5 cm
B	> 2.0 cm
C	> 2.5 cm
D	> 3.5 cm

Question 18 Explanation:

Testicular size >2.5 cm generally indicates that the child has entered puberty

Question 19

Kallmann syndrome is transmitted as ?

A	X-linked
B	Autosomal recessive
C	Autosomal dominant
D	Autosomal codominant

Question 20

Kallmann syndrome is due to mutation in which gene ?

A	KAL1
B	KAL2
C	GPR54
D	DAX1

Question 21

KAL1 gene encodes which of the following ?

A	Kalmin
B	Anosmin
C	Migrin
D	Neurokinin

Question 21 Explanation:

Kallmann syndrome is an X-linked disorder due to mutations in KAL1 gene, which encodes anosmin, a protein that mediates migration of neural progenitors of the olfactory bulb & GnRH-producing neurons. These individuals have GnRH deficiency and variable combinations of anosmia or hyposmia, renal defects, and neurologic abnormalities including mirror movements.

Question 22

Prader-Willi syndrome is characterized by all except ?

A	Obesity
B	Hypotonic musculature
C	Tall stature
D	Mental retardation

Question 23

Prader-Willi syndrome is characterized by all except ?

A	Hypogonadism
B	Lean and thin constitution
C	Short stature
D	Small hands and feet

Question 23 Explanation:

Prader-Willi syndrome is characterized by obesity, hypotonic musculature, mental retardation, hypogonadism, short stature & small hands & feet. Prader-Willi syndrome is a genomic imprinting disorder.

Question 24

Laurence-Moon syndrome is transmitted as ?

A	X-linked
B	Autosomal recessive
C	Autosomal dominant
D	Autosomal codominant

Question 25

Laurence-Moon syndrome is characterized by all except ?

A	Obesity
B	Hypogonadism
C	Short stature
D	Mental retardation

Question 26

Laurence-Moon syndrome is characterized by all except ?

A	Polydactyly
B	Retinitis pigmentosa
C	Mental retardation
D	Small hands and feet

Question 26 Explanation:

Laurence-Moon syndrome is an autosomal recessive disorder characterized by obesity, hypogonadism, mental retardation, polydactyly & retinitis pigmentosa. Recessive mutations of leptin, or its receptor, cause severe obesity & pubertal arrest because of hypothalamic GnRH deficiency.

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See all quizzes of Disorders of the Testes and Male Reproductive System at here:

Disorders of the Testes and Male Reproductive System- Part 1| Disorders of the Testes and Male Reproductive System- Part 2|Disorders of the Testes and Male Reproductive System- Part 3|Disorders of the Testes and Male Reproductive System- Part 4|Disorders of the Testes and Male Reproductive System- Part 5|Disorders of the Testes and Male Reproductive System- Part 6

1.Which of the following is measured to detect presence of testes in
prepubertal boys with cryptorchidism ?
A. Inhibin B
B. Müllerian inhibiting substance (MIS)
C. Testosterone
D. All of the above

2. Normal semen ejaculate volume is ?
A. 1 to 3 mL
B. 2 to 6 mL
C. 5 to 8 mL
D. 8 to 10 mL

3. Puberty in boys before what age is considered precocious ?
A. 9 years
B. 10 years
C. 11 years
D. 12 years

4. Which of the following is false about central precocious puberty
(CPP) ?
A. Gonadotropin-Dependent
B. Less common in boys than in girls
C. Elevated gonadotropin levels
D. None of the above

5. Which of the following is not a cause of gonadotropin-independent
precocious puberty in boys ?
A. hCG-secreting tumors
B. Hyperthyroidism
C. Congenital adrenal hyperplasia
D. McCune-Albright syndrome

6. Which of the following disorders is also called testotoxicosis ?
A. McCune-Albright syndrome
B. Familial male-limited precocious puberty
C. Congenital adrenal hyperplasia
D. None of the above

7. Treatment options for familial male-limited precocious puberty
include ?
A. Ketoconazole
B. Flutamide
C. Anastrazole
D. All of the above

8. Which of the following skin lesion is characteristic of McCune-
Albright syndrome ?
A. Erythroderma
B. Café au lait spots
C. Telangiectasia
D. Scarring alopecia

9.In McCune-Albright syndrome, mutation occurs in which of the
following ?
A. Gsalpha subunit
B. Gsbeta subunit
C. Gsgamma subunit
D. Gsdelta subunit

10. Which of the following is false about congenital adrenal hyperplasia
(CAH) ?
A. Chronic ACTH stimulation
B. Low LH
C. Small testes
D. None of the above

11. Breast enlargement in prepubertal boys can result from ?
A. Familial aromatase excess
B. Marijuana smoking
C. Sertoli cell tumors in the testis
D. All of the above

12. In children with gonadotropin-independent precocious puberty,
which of the following is not useful ?
A. Ketoconazole
B. Long-acting GnRH analogues
C. Spironolactone
D. Testolactone

13. Puberty is considered delayed in boys if it has not happend by the
age of ?
A. 14
B. 15
C. 16
D. 18

14. Which of the following categories of delayed puberty is more
common in girls than in boys ?
A. Constitutional delay of growth and puberty
B. Functional hypogonadotropic hypogonadism caused by
systemic illness or malnutrition
C. Hypogonadotropic hypogonadism caused by genetic or
acquired defects in the hypothalamic-pituitary region
D. Hypergonadotropic hypogonadism secondary to primary
gonadal failure
Delayed puberty is more common in boys than in girls. But, functional hypogonadotropic
hypogonadism is more common in girls than in boys.
15. Which of the following is the most common cause of delayed
puberty ?
A. Constitutional delay
B. Functional hypogonadotropic hypogonadism due to systemic
illness or malnutrition
C. Hypogonadotropic hypogonadism due to genetic or acquired
defects in hypothalamic-pituitary region
D. Hypergonadotropic hypogonadism secondary to primary
gonadal failure
16. Constitutional delay in puberty should be suspected when there
is ?
A. Positive family history
B. Delayed bone age
C. Short stature
D. All of the above

17. Familial hypogonadotropic hypogonadism is transmitted most
commonly as ?
A. X-linked
B. Autosomal recessive
C. Autosomal dominant
D. Autosomal codominant

18. What testicular length generally indicates that the child has entered
puberty ?
A. > 1.5 cm
B. > 2.0 cm
C. > 2.5 cm
D. > 3.5 cm

19. Kallmann syndrome is transmitted as ?
A. X-linked
B. Autosomal recessive
C. Autosomal dominant
D. Autosomal codominant
20. Kallmann syndrome is due to mutation in which gene ?
A. KAL1
B. KAL2
C. GPR54
D. DAX1
21. KAL1 gene encodes which of the following ?
A. Kalmin
B. Anosmin
C. Migrin
D. Neurokinin

22. Prader-Willi syndrome is characterized by all except ?
A. Obesity
B. Hypotonic musculature
C. Tall stature
D. Mental retardation
23. Prader-Willi syndrome is characterized by all except ?
A. Hypogonadism
B. Lean and thin constitution
C. Short stature
D. Small hands and feet

24. Laurence-Moon syndrome is transmitted as ?
A. X-linked
B. Autosomal recessive
C. Autosomal dominant
D. Autosomal codominant
25. Laurence-Moon syndrome is characterized by all except ?
A. Obesity
B. Hypogonadism
C. Short stature
D. Mental retardation
26. Laurence-Moon syndrome is characterized by all except ?
A. Polydactyly
B. Retinitis pigmentosa
C. Mental retardation
D. Small hands and feet

Disorders of the Testes and Male Reproductive System

[MCQ] Disorders of the Testes and Male Reproductive System- Part 3

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Disorders of the Testes and Male Reproductive System- Part 3

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