Puberty Disorders Flashcards
Timing of puberty in girls and boys
girls: 8-13 y.o
boys: 9-14 y.o
Precocious puberty in girls (age)
<8 y.o
Precocious puberty in boys (age)
<9 y.o
Delayed puberty in girls (age)
> 13 y.o.
Delayed puberty in boys (age)
> 14 y.o.
Causes of central/GnRH dependent precocious puberty EXCEPT
a. Hypothalamic hamartoma
b. Glioma
c. Head trauma
d. Infections
e. McCune Albright Syndrome
E
Causes of central/GnRH dependent precocious puberty EXCEPT
a. Hydrocephalus
b. Ectopic hCG secretion
c. Severe primary hypothyroidism
d. Idiopathic
B
True of Central precocious puberty
a. More common in males
b. idiopathic origin is most common
c. Both
B
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?
Fibrous dysplasia
Peripheral precocious puberty
Cafe au lait spots
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
C
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
E
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
E
Management of Central Precocious puberty except
a. GnRH antagonist
b. GnRH analogue
c. pituitary dessensitization
d. downregulation of HPG axis
e. A and C
A
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
C
Definition of delayed puberty for girls/boys
girls: no signs by age 13
boys: by age 14
Types of delayed puberty (3)
Constitutional delay of growth and maturation
Hypogonadotropic Hypogonadism
Hypergonadotropic Hypogonadism
Hypogonadotropic hypogonadism is seen in the ff EXCEPT
a. CNS neoplasms
b. Isolated gonadotropin deficiency
c. Anorexia Nervosa
d. Turner Syndrome
D
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
Prader Willi Syndrome
Micropenis
Undescended Testes
Anosmia/Hyposmia
Kallman’s Syndrome
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
F
Mental development: retarded
Short stature
Cardiac: Pulmonary valvular stenosis, hypertrophic cardiomyopaty, ASD
Karyotype: Normal
Noonan Syndrome
