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Endo > puberty > Flashcards

puberty Flashcards

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

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

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

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?

A

androgen e.g. from tumour

20
Q

key Ix for central precocious puberty?

A

elevated basal LH and/or stimulated LH concentration post GnRH

21
Q

how to treat central precocious puberty? MOA?

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

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

A

USS - Increased ovarian and uterine volumes with CPP

24
Q

super rando signs for hypothalamic hamartoma

A

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

25
Q

most common genetic mutation found in idiopathic CPP

A

Loss of function mutations in MKRN3

26
Q

precocious puberty + slow growth =

A

hypothyroidism!! only cause. also multicystic ovaries

27
Q

pathogenesis of precocious puberty in hypothyroidism

A

High levels of TSH interact with FSH receptor, but not the LH receptor
FSH receptor > estradiol secretion by ovaries
LOW LH, FSH

28
Q

why do we need to monitor pubertal status post treatment for peripheral precocious puberty?

A

6/12 monitoring as peripheral can trigger central e.g. CAH

29
Q

pigmented nipples = what?

A

high oestrogen levels

30
Q

most common cause of PPP in girls?

A

large ovarian cyst

31
Q

summary of causes of PPP

A
  1. girls - ovaries (cyst/tumour)
  2. boys - testes tumour / germ cell in BOYS only/hepblastoma
  3. both: adrenal (tumour, CAH), McCune-Albright!
32
Q

McCune-Albright - key features

A
  • alpha subunit of Gs mutation
  • classic triad: precocious puberty, cafe au lait macules, polyostotic fibrous dysplasia
  • spontaneous activation of ACTH, TSH, FSH, LH
33
Q

cafe au lait macules in NF vs MAS

A

MAS: coast of maine, irregular borders, don’t cross midline

NF: coast of cali, regular borers, cross midline

34
Q

McCune Albright treatment

A

GnRH independent, so either
- girls: anti-oestrogen / azole
- boys: as above, or anti-androgens e.g. spiro

35
Q

premature thelarche - key features

A

<3yo ONLY
normal height/bone age/genitalia
no Ix unless the above aren’t right
observe - 10% have true CPP

36
Q

premature pubarche - key features

A

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
Q

commonest dx of exclusion for delayed puberty

A

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
Q

how to treat turners from endo perspective

A
  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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