Puberty Disorders

Question 1

Timing of puberty in girls and boys

Answer 1

girls: 8-13 y.o
boys: 9-14 y.o

Question 2

Precocious puberty in girls (age)

Answer 2

<8 y.o

Question 3

Precocious puberty in boys (age)

Answer 3

<9 y.o

Question 4

Delayed puberty in girls (age)

Answer 4

> 13 y.o.

Question 5

Delayed puberty in boys (age)

Answer 5

> 14 y.o.

Question 6

Causes of central/GnRH dependent precocious puberty EXCEPT

a. Hypothalamic hamartoma
b. Glioma
c. Head trauma
d. Infections
e. McCune Albright Syndrome

Answer 6

E

Question 7

Causes of central/GnRH dependent precocious puberty EXCEPT

a. Hydrocephalus
b. Ectopic hCG secretion
c. Severe primary hypothyroidism
d. Idiopathic

Answer 7

B

Question 8

True of Central precocious puberty

a. More common in males
b. idiopathic origin is most common
c. Both

Answer 8

B

Question 9

Cause of precocious puberty more common in females, with activating mutation of gene coding Gs alpha protein. Autonomous hormone production involves gonads, thyroid, pituitary gland, parathyroid gland.

What is the triad of this disease?

Answer 9

Fibrous dysplasia
Peripheral precocious puberty
Cafe au lait spots

Question 10

For girls presenting with signs of estrogen excess, the following tests would be helpful EXCEPT

a. FSH, LH, Estradiol, TSH
b. Pelvic ultrasound
c. 17-OHP
d. MRI/CT Cranial
e. bone age

Answer 10

C

Question 11

For girls presenting with signs of virilization, you would order the following EXCEPT

a. FSH, LH, estradiol
b. DHEAS
c. Testosterone
d. 17-OHP
e. NOTA

Answer 11

E

Question 12

For boys presenting with precocious puberty, you would order the ff EXCEPT

a. FSH, LH
b. hCG
c. 17-OHP, DHEA sulfate
d. x-ray of left hand and wrist
e. NOTA

Answer 12

E

Question 13

Management of Central Precocious puberty except

a. GnRH antagonist
b. GnRH analogue
c. pituitary dessensitization
d. downregulation of HPG axis
e. A and C

Answer 13

A

Question 14

True about management of Peripheral precocious puberty EXCEPT

a. testolactone is an aromatase inhibitor
b. Tamoxifen is an anti-estrogen
c. Child should be treated with tamoxifen/testolactone
d. NOTA

Answer 14

C

Question 15

Definition of delayed puberty for girls/boys

Answer 15

girls: no signs by age 13
boys: by age 14

Question 16

Types of delayed puberty (3)

Answer 16

Constitutional delay of growth and maturation
Hypogonadotropic Hypogonadism
Hypergonadotropic Hypogonadism

Question 17

Hypogonadotropic hypogonadism is seen in the ff EXCEPT

a. CNS neoplasms
b. Isolated gonadotropin deficiency
c. Anorexia Nervosa
d. Turner Syndrome

Answer 17

D

Question 18

Cause of delayed puberty characterized by neonatal and infantile hypotonia, developmental delay, mental retardation, learning problems, narrow face, almond shaped eyes, small appearing mouth, thin upper lip, hyperphagia, hypogonadism

Answer 18

Prader Willi Syndrome

Question 19

Micropenis
Undescended Testes
Anosmia/Hyposmia

Answer 19

Kallman’s Syndrome

Question 20

Cause of Hypergonadotropic Hypogonadism EXCEPT

a. XX and XY gonadal dysgenesis
b. Autoimmune oophoritis
c. Radiation and/or chemo
d. PCOS
e. Noonan
f. NOTA

Answer 20

F

Question 21

Mental development: retarded
Short stature
Cardiac: Pulmonary valvular stenosis, hypertrophic cardiomyopaty, ASD
Karyotype: Normal

Answer 21

Noonan Syndrome