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Endo > puberty > Flashcards

puberty Flashcards

(38 cards)

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1
Q

definition of:
gonardarche
adrenarche
thelarche
menarche
spermache
pubarche

A

gonardarche = gonad activation by LH/FSH
adrenarche = release of androgens (DHEA/DHEAS)
thelarche = breast development
menarche = 1st period
spermache = 1st sperm production
pubarche = first pubic hair

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2
Q

age of onset of puberty determined by?

A
  1. genetics - boys follow MOTHERS, and vice versa
  2. BMI - higher BMI, earlier puberty
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3
Q

what governs adrenarche vs thelarche

A

adrenarche = hair, acne and body odour from ANDROGENS
thelarche from oestradiol (from ovaries)

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4
Q

order of puberty development in boys vs girls

A

girls: breasts > pubes > growth > period
males: balls >4ml > (penile length) > pubes > growth > sperm

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5
Q

which sex hormone is most important for growth?

A

oestrogen –> epiphyseal closure and GH (even in boys, oestrogen > T for this)

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6
Q

most reliable marker of androgens

A

DHEAS

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7
Q

when do GnRH/LH and FSH initially start being produced?

A

1-2y before clinical puberty
released at night, in pulsatile fashion

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8
Q

growth in males vs females

A

males: growth spurt 2y after females, ~13-14y, peak rate ~10cm/year

girls: spurt 0.5y before menarche, usually 11-12, 8.5cm/yr

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9
Q

bone development in males vs females

A

Females = E inhibits apposition + stimulates endocortical formation > narrower medullary cavity

Males = T increases bone size by ↑ apposition and ↑ distance of cortex > thicker cortex

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10
Q

at what age is precocious vs delayed puberty for males vs females?

A

females: <8y, >12y
males: <9y, >14y

all within 2SD of mean

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11
Q

normal testicular volumes

A

4-6yrs = 1ml
(>4ml = first sign of puberty)
10-12yrs =5ml
Adults 15-35mls

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12
Q

menstrual cycle summary

A

follicular phase = D0-13
- FSH stimulates follicles, which produce estradiol
- granulosa’s inhibin -ve FB FSH so it declines afer D5
- estradiol initially inhibits LH, then +ve FB after set point reached

ovulation = D14

luteal phase = D15=18
- corpus luteum > progesterone
- CL degenerates if not fertilised
- lower prog + E > menses

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13
Q

which phase is constant - luteal or follicular?

A

luteal - ovulation always 2 weeks prior to first day of next cycle

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14
Q

when does ovulation occur in relation to LH/oestradiol peak?

A

10-12 hours after LH surge, and 24-36 hours after estradiol peak

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15
Q

when do the pubertal progressions happen in males?

A
  1. first sign = testicular growth (> 4ml) and thinning of scrotum first sign ( 11-12 years)
  2. Pubarche = occurs 6 months after testicular enlargement
  3. Spermache = 2 years post pubarche
  4. Facial hair = 3 years post pubarche
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16
Q

define true precocious puberty, precocious pseudopuberty, incomplete precocious puberty

Study These Flashcards
A

true precocious puberty = central, gonadotropin dependent

precocious pseudopuberty = peripheral, gonadotropin independent

incomplete = partial e.g. premature thelarche / adrenarche / menarche

17
Q

examples of causes of true precocious puberty

Study These Flashcards
A
  1. idiopathic - 80%! almost all girls
  2. hypothalamic hamartoma
  3. ** brain tumour - glioma, germ cell tumour **
  4. prolonged, untreated hypothyroid
18
Q

true precocious puberty vs precocious pseudopuberty

Study These Flashcards
A

true:
- from maturation of HPG axis
- SEQUENTIAL maturation
- isosexual
- more in girls

psedopuberty:
- from excess sex hormones / steroids / ectopic gonadotropin e.g. tumour
- HPG not activated, so not true puberty
- iso/contrasexual
- NON-SEQUENTIAL maturation

19
Q

enlargement of penis without testicular enlargement = what hormone problem?

Study These Flashcards
A

androgen e.g. from tumour

20
Q

key Ix for central precocious puberty?

Study These Flashcards
A

elevated basal LH and/or stimulated LH concentration post GnRH

21
Q

how to treat central precocious puberty? MOA?

Study These Flashcards
A

GnRH agonist = leuprolide, histrelin, goserelin

Physiolgical GnRH is pulsatile, so continious dose > ‘desensitization’/ -ve FB > inhibit endogenous GnRH

22
Q

effects of GnRH agonist treatment in central precocious puberty

Study These Flashcards
A

decreases growth rate > enhanced height
- breasts/balls regress
- public hair slower/regress
- menses cease

23
Q

how to differentiate girls with CPP from those with premature thelarche

Study These Flashcards
A

USS - Increased ovarian and uterine volumes with CPP

24
Q

super rando signs for hypothalamic hamartoma

Study These Flashcards
A

diabetes insipidus, adipsia, hyperthermia, unnatural crying or laughing (gelastic seizures), obesity, cachexia

25
most common genetic mutation found in idiopathic CPP
Loss of function mutations in MKRN3
26
precocious puberty + slow growth =
hypothyroidism!! only cause. also multicystic ovaries
27
pathogenesis of precocious puberty in hypothyroidism
High levels of TSH interact with FSH receptor, but not the LH receptor FSH receptor > estradiol secretion by ovaries LOW LH, FSH
28
why do we need to monitor pubertal status post treatment for peripheral precocious puberty?
6/12 monitoring as peripheral can trigger central e.g. CAH
29
pigmented nipples = what?
high oestrogen levels
30
most common cause of PPP in girls?
large ovarian cyst
31
summary of causes of PPP
1. girls - ovaries (cyst/tumour) 2. boys - testes tumour / germ cell in BOYS only/hepblastoma 3. both: adrenal (tumour, CAH), McCune-Albright!
32
McCune-Albright - key features
- alpha subunit of Gs mutation - classic triad: precocious puberty, cafe au lait macules, polyostotic fibrous dysplasia - spontaneous activation of ACTH, *TSH*, FSH, LH
33
cafe au lait macules in NF vs MAS
MAS: coast of maine, irregular borders, don't cross midline NF: coast of cali, regular borers, cross midline
34
McCune Albright treatment
GnRH independent, so either - girls: anti-oestrogen / azole - boys: as above, or anti-androgens e.g. spiro
35
premature thelarche - key features
<3yo ONLY normal height/bone age/genitalia no Ix unless the above aren't right observe - 10% have true CPP
36
premature pubarche - key features
pubic > axillary SLIGHTLY advanced bone age, still within normal range no other signs of puberty if have any other sign, need to exclude virilising disorders e.g. CAH
37
commonest dx of exclusion for delayed puberty
Constitutional Delay in Growth and Puberty (CDGP) - hallmark is delayed bone age, but height for bone age ok can give short term steroids to kick start things
38
how to treat turners from endo perspective
1. HRT at 12y: estradiol, then prog - NO OCP - HTN risk high in Turner 2. GH both help with height

Endo (8 decks)

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