Delayed Puberty

Question 1

normal puberty:

1. Withdrawal of ___ inhibition of the ____ GnRH pulse generator
2. Augmented __ and __ secretion in response to ___ GnRH
3. Gradual increase in sex steroid concentrations
4. Appearance of ___ sex __

Answer 1

1. Withdrawal of CNS inhibition of the hypothalamic GnRH pulse generator
2. Augmented LH and FSH secretion in response to pulsatile GnRH
3. Gradual increase in sex steroid concentrations
4. Appearance of secondary sex characteristics

Question 2

outline how the sequence of normal puberty differs between males and females

Answer 2

in males, the height spurt is a bit later, so they grow their testes and pubic hair before they get tall

in females, they often grow taller befroe developing actual fertility, which is why in grade 5 most girls are taller than their boy peers.

Question 3

definition of delayed puberty in girls and boys

Answer 3

in girls, primary amenorrhea at 16, no boobs at 13.

in boys, no secondary sex characteristics by age 14, or if takes over 5 years from onset of puberty to completion

Question 4

hypergonadotropic vs hypogonadotropic hypogonadism

Answer 4

Question 5

Is Kallmans an example of hpogonadotropic or hypergonadotropic hypogonadism

Answer 5

hypogonadotropic hypogonadism

• there is a defect in GNRH production, meaning the problem is at the level of the pituitary

Question 6

isolated gonadotropin deficiencyies resulting in hypogonadotropic hypogonadism

Answer 6

kallmann

prader willi

laurence moon biedl

idiopathic

Question 7

broad causes of hypogonadotropic hypogonadism

Answer 7

1. constitutional growht delay which result in. anormal height kid later on
2. hypopituitariusm (PROP-1)
3. Isolated gonadotropin deficiency (Kallmann, prader willi, laurence moon biedl, idiopathic)
4. functional disordes

Question 8

Anterior Panhypopituitarism

• Case: 17 Year old male with delayed puberty

• Short stature
• Height age 9 yrs

• Low serum T4 with “normal” TSH

• Abnormal ITT
• ACTH deficiency
• GH deficiency

• Subnormal LH response to GNRH stimulation

DX?

Answer 8

• short stature, low T4 and normal TSH– should be HIGH!!
• abnormal ITT; sounds like its more of a pituitary issue, therefore more so a hypogonadotropin hypogonadism issue.
• could be Kallmans, could be prader willi– but the main symptom here is short stature and delayed puberty, not obesity. leaning more toward kallmans.

Question 9

LH, FSH and testosterone levels in Kallman. What other non-repro symptoms are on presentation?

Answer 9

Low KH, low FSH and low testosterone– this is a central issue

• the most important thing you could be missing is a cortisol emergency.
• would also be anosmia, deficiency of olfactory sulcus

Question 10

Prader Willi H4O

Answer 10

H= hyperphadia

H= hypotonia

H= hypomentation

H= hypogonadism

O= obesity

Question 11

LF and FSH levels in someone with ypergonadotropic hypogonadism

Answer 11

HIGH LH and FSH because central is not affected– streak gonads/dysfunctional gonads is the issue

Question 12

Congenital causes of hypergonadotropic hypogonadism

Answer 12

• gonadal dysgenesis
• turner syndrome
• klinefelter syndrome
• anorchia
• disorders of sex steroid biosynthesis

Question 13

would turners lead to primary or secondary ovarian failure

Answer 13

usually primary ovarian failure– streak gonad presecne

Question 14

acquired causes of Hypergonadotropic Hypogonadism

Answer 14

• surgery
• radiation
• systemic chemotherapy
• gonadal torsion
• oophoritis/orchitis

Question 15

Do a ___ stimulation test to assess Leydig cell function in
prepubertal males

Answer 15

HCG stimulation test to assess Leydig cell function in
prepubertal males

Question 16

T/F oral testosterone is often used as an androgen replacement

Answer 16

false. not used because bioavailability isn’t great

Question 17