Understanding precocious puberty in girls 2012 Flashcards
What is the definition of precocious puberty in girls
< 8years
What is adrenarche when does it begin?
Adrenacrge is the onset of androgen signs of puberty (pubic & axillary hair, greasy hair, acne & adult body odour) - adrenocortical activity.
Age 6-8 years
How many stage to tanner. What is score
Stage 1-5
Breast and pubic hair
What is the start of thelarche?
Tanner stage 2 breast budding, then development of pubic hair (pubarche), then axillary hair.
When does growth spurt occur?
Starts with breast budding, peak height velocity at mid-puberty
How long after therlarche does menarche tend to occur?
2-3 years (average age 13)
What are the 3 main categories of previous puberty?
Central (GnRH dependant)
Peripheral (GnRD indépendant)
Isolated (precious thelarche, pubarche or menarche)
Can also be categoried as isosexual (secondary sexual characteristic appropriates) or heterosexual (sexual characteristics contrary to phenotypic sec)
Can central and peripeheral causes of precocious puberty be isosexual or heterosexual sexual?
Central: Isosexual
Peripheral: Iso or heterosexual
What are causes of central precocious puberty?
Idiopathic
CNS
- Hypothalamic hamartoma
- Tumour
- Congenital: hydrocephalus, arachnid cyst
- Acquired - CND irradiation, post head trauma, post infection, chemotherapy
Secondary to peripheral precocious puberty
Peripheral causes of precocious puberty
Ovarian cause
- estrogen secreting - granulosa cell tumour, function ovarian cyst
- Androgen secreting - Sertoli-leydig cells, arrhenoblastoma (contra sexual)
Adrenal
- CAH (contra sexual)
- Cushing (contra sexual)
- Neplasm - estogen or androgen secreting
Exogenous sex hormones
McCune Albright Syndrome
Severe long lasting hypothyroidism
Variant of normal pubertal development
Premature thelarche
Isolated premature menarche
Premature pubarche/adrenarcge (contrasexual)
What history to ask in evaluation of child with premature sexual development?
o Age onset, sequence, progression pubertal changes
o Family History, timing onset of puberty in mother/siblings
o Neurological Sx
o Exogenous sex steroids exposure in food/drugs/cosmetics
o Social hx – adoption/child abuse
What examination to perform in evaluation of child with premature sexual development?
o Height/weight – plotted age-specific
o BMI
o Pubertal tanner staging
o Neurological examination
o Examination eye – visual fields
o Skin lesions (café au lait)
o Abdo examination
o Examination of external genitalia
o Signs of virilisation
Investigation for premature sexual development
o Serum LH & FSH
o GnRH stimulation test
o Estradiol/testosterone
- Adrenal steroids – 17 OH progesterone, dehydrocepiandrosterone, androstenedione (raised CAH and tumours)
o ACTH stimulation test
o TFTs
o Serum prolocatin
o Urinary steroids
What imaging to consider when investigation precious puberty
● Left wrist X-ray for bone age
● Cranial magnetic resonance imaging/computed tomography (CT)
● CT adrenals (adrenal masses)
● Pelvic ultrasound (size, shape of uterus, endometrial thickness and ovarian morphology)
● Skeletal survey/bone scan (McCune–Albright syndrome)
What impact does acclerated bone maturation and premature epiphyseal fusion have on long term growth
Early cessation of growth, compromised final height
What is the goal of treatment in treating precious puberty?
Half or cause regression in 2ndary sexual characteristic
Prevent early menarche
Retard skeletal maturation and improve fundal height
avoid psychosocial/beahivoural sequelae
Treatment of precious puberty
GnRH analogues (if <6 yr helps increase adult height)
Normally stopped when normal time for puberty to being, mean time to menarche 16 months
What is McCune-Albright Syndrome?
Mutation GNAS1 genet
- Polyostotic fibrous dysplasia (bone cyst)
- Cafe au last spot
- Peripheral precocious puberty
Treatment of McCune-Albringt syndrome
Aromatase inhibitors - testolactone, anastrazole, tetrazole
Ass hyperthyroidism and hyperprolacticaemia
The bone is usually advanced except in which condition
Hypothyroidism
Which hormone level is raised in classical CAH
17-hydroxyprogesterone - 21 hyroxylase deficiency
Which blood test is raised in androgen secreting tumour
DHEAS
What is premature thelarche
Isolated premature breast development (absence of other signs of puberty)
