Endocrine Flashcards
What percentage of kids experience pubic hair growth as first sign of puberty?
20%
When do GnRH neurons migrate to hypothalamus
6-9 wga
When does FSH/LH secretion start in the fetus
12 weeks
When do fetal levels of FSH/LH peak
20-24 weeks
What happens to FSH/LH after delivery
GnRH pulsatility starts with FSH/LH rising with FSH > in females (peaks 12-18 months)
LH > males (peaks 3-6 months)
**Then there is change in central inhibition of pulsatile GnRH and high sensitivity to low levels of gonadal steroid feedback –> diphasic pattern of secretion
How does adrenarche start and when?
Age 6
Increased in responsiveness of adrenal glands to ACTH causing shift in sex steroid production
Decreased 3B HSD activity + increased activity in P450 Cyp 17
Shunts towards DHEA/DHEAS
Genes associated with early puberty
LIN28B, FTO, NEGR1
Shift in body composition associated with puberty
% body fat
16-23%
Leptin and puberty
Made by adipocytes and acts on CNS to regulate eating/energy balance
Leptin knockout mice have normal sexual development but are anovulatory
What is precocious puberty
Pubertal development occurring more than 2.5 SD earlier than the norm
Types of precocious puberty
GnRH Dependent: early activity of HPG axis
-constitutional (RUNS IN FAMILY) /idiopathic (does NOT RUN in family & onset is usually younger)
-CNS pathology
-Hypothyroidism
GnRH Independent:
-Sex steroid production independent of HPG axis
-Ovarian tumors
-McCune Albright
-Adrenal disease
-Exposure to estrogens
Hypothalamic hamartoma
Heterotopic neuronal masses of GnRH neurons that function as ectopic GnRH pulse generator
PP in first few years of life
Craniopharyngioma
Neuroepithelial CNS tumor
Disrupts normal pre-pubertal HP axis inhibition
Hypothyroidism and GnRH dependent PP
Delayed bone age (Thyroid hormones have permissive activity directly on bone and GH)
High TSH stimulates FSH-R and causes high prolactin and galactorrhea
Gonadoblastoma
complex tumor with a mix of gonadal elements
90% associated with abnormal karyotype, gonadal dysgenesis, or Y chromosome
Premature adrenarche
pubic hair < 8 yo
consider checking bone age to make sure there isn’t precocious puberty too (~20% have both)
Most significant outcome of precocious puberty
short stature because of premature epiphyseal closure
GnRH stimulation test
Check basal LH
leuprolide 20 ug/kg subcu
if LH > 5 IU/L suggests HPG axis activation
if LH is normal/low eg < 5 then likely to be GnRH independent
When to treat precocious puberty
-Sexual maturity advances to next stage in 3-6 months
-growth velocity > 6 cm/year
-bone age is advanced by > 1 year
-predicted adult height is < target range or decreased on serial measurements
Treatment for GnRH dependent precocious puberty
lupron
monitor symptoms and E2 (<10 pg/mL) at 3-6 month intervals
Delayed puberty
Absent or incomplete sexual maturation by age at which 95% of kids of same sex started pubertal development
Short stature
height 2 or more standard deviations below mean height for children of same sex/age
-Dx: familial/constitutional, chromosomal (downs/turners), idiopathic
Treatment of short stature
consider GH if height > 2.25 SD below mean