Precocious Puberty
<8yo in girls
<9yo in boys
true aka central = GnRH/gonadotrophin dependent eg GnRH pulse turned on early
pseudo aka peripheral/incomplete = gonadotrophin independent
NB long standing pseudoprecocity can lead to true precocious puberty
F>M
F usually idopathic
M usually central
Good, Im, I've washed
I've been asked
You must be (comment on voice: high pitch, mature female or deep)
Position Pain
Exposure
Equip
Well
Growth + TANNER (compare with parents)
Skin:
Arm span
LS
Palms together for asymmetry
Spine
Hands
Axillary
Head
Chest
Tanner
Galactorrhea (hypothyroid, hypothal/pit tumour)
Abdomen
Gonads
CNS
Ix
LH, FSH; morning gonadal steroids: testosterone, oestradiol.
GnRH stimulation test (LH and FSH responses)
DHEAS (high in adrenal tumours)
hCG (various tumours).
TFTs
Left hand and wrist Xray (Bone age)
US pelvis, testes, adrenals
MRI brain (hypothalamic hamartoma, pinealoma, hydrocephalus, third ventricular cysts)
Rx
Leuprorelin (GnRH analogue) for central precocious puberty (masks the pulses that have initiated) until puberty appropriate
<9yo in boys
true aka central = GnRH/gonadotrophin dependent eg GnRH pulse turned on early
pseudo aka peripheral/incomplete = gonadotrophin independent
NB long standing pseudoprecocity can lead to true precocious puberty
F>M
F usually idopathic
M usually central
Good, Im, I've washed
I've been asked
You must be (comment on voice: high pitch, mature female or deep)
Position Pain
Exposure
Equip
Well
Growth + TANNER (compare with parents)
- tall: early precocity, McC–A (central or peripheral prec), NF1 (central prec)
- increased velocity (central)
- short: long standing precocity, NF1 (central), Russell–Silver (central), Cushing's (premature pubarche), hypothyroidism
- overweight: Cushing's (premature pubarche), hypothyroidism (central)
- macrocephaly: hydrocephalus or intracranial tumours
- microcephaly: head injury or in cerebral palsy
- arm span should be -ve prepuberty, if +ve may have already had a growth spurt
- US:LS short legs in hypothyroid, short body in scoliosis (NF1)
- Compare TANNER with parents timing
- habitus: prep v male v female. muscle, fat, hair distribution, acne, odour, gynocomastia
- scoliosis (NF)
- Moon: Cushing’s syndrome,
- NF1
- Coarse: Hypothyroid
- Russell–Silver syndrome, McC–A syndrome
Skin:
- Cafe au lait: NF1 (coast of california), Russell Silver, McAS (bigger/more irregular aka coast of Maine)
- hyperpigmentation (CAH)
- Xanthoderma (yellow skin) Carotenemia from hypothyroidism
- acne
- dry/cool: hypothyroidism
Arm span
LS
Palms together for asymmetry
Spine
- Scoliosis (NF1)
- Spinal dysraphism
Hands
- clinodactyly (Russell-silver)
- palmar crease pigmentation (CAH)
- cool dry, slow pulse (hypothyroid)
- warm sweating, collapsing pulse (hyperthroid)
Axillary
- hair, odour
- freckling (NF1)
Head
- Shunt
- Scar
- Hair: dry (hypothyroid), greasy (CAH)
- Proptosis: hyperthyroidism (MAS)
- Ophthalmoplegia: hyperthyroidism (MAS), IC tumour, hydrocephalus (parinauds paralysis of upwards gaze)
- Lisch nodules: NF1
- Argyll Robertson pupils DONT react to light but react to accomodation
- Cataracts (Cushing's)
- Fields: bitemporal (pituitary tumour), homonymous (IC tumour)
- Papiloedema
- hyperpigmentation of lips and oral mucosa (Peutz–Jeghers syndrome > tumour predisposition including liver and gonads)
- goitre (hypo/perthyroidism)
Chest
Tanner
Galactorrhea (hypothyroid, hypothal/pit tumour)
Abdomen
- hepatomegaly and bruit (hepatoblastoma > hCG)
- Adrenal mass
- Ovarian mass
Gonads
- testicle size (<3ml or small grape = prepubertal)
- Penile length (with cotton tip then measure)
CNS
- lower limb and gait (IC tumour, hydrocephalus, head injury)
- delayed ankle jerk relaxation (hypothyroid)
Ix
LH, FSH; morning gonadal steroids: testosterone, oestradiol.
GnRH stimulation test (LH and FSH responses)
DHEAS (high in adrenal tumours)
hCG (various tumours).
TFTs
Left hand and wrist Xray (Bone age)
- normal: premature adrenache, premature thearche, testosterone abuse
- advanced: central, adrenal, ovarian, McAS
US pelvis, testes, adrenals
MRI brain (hypothalamic hamartoma, pinealoma, hydrocephalus, third ventricular cysts)
Rx
Leuprorelin (GnRH analogue) for central precocious puberty (masks the pulses that have initiated) until puberty appropriate