Understanding precocious puberty in girls 2012 Flashcards

1
Q

What is the definition of precocious puberty in girls

A

< 8years

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2
Q

What is adrenarche when does it begin?

A

Adrenacrge is the onset of androgen signs of puberty (pubic & axillary hair, greasy hair, acne & adult body odour) - adrenocortical activity.

Age 6-8 years

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3
Q

How many stage to tanner. What is score

A

Stage 1-5
Breast and pubic hair

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4
Q

What is the start of thelarche?

A

Tanner stage 2 breast budding, then development of pubic hair (pubarche), then axillary hair.

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5
Q

When does growth spurt occur?

A

Starts with breast budding, peak height velocity at mid-puberty

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6
Q

How long after therlarche does menarche tend to occur?

A

2-3 years (average age 13)

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7
Q

What are the 3 main categories of previous puberty?

A

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)

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8
Q

Can central and peripeheral causes of precocious puberty be isosexual or heterosexual sexual?

A

Central: Isosexual
Peripheral: Iso or heterosexual

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9
Q

What are causes of central precocious puberty?

A

Idiopathic
CNS
- Hypothalamic hamartoma
- Tumour
- Congenital: hydrocephalus, arachnid cyst
- Acquired - CND irradiation, post head trauma, post infection, chemotherapy

Secondary to peripheral precocious puberty

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10
Q

Peripheral causes of precocious puberty

A

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

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11
Q

Variant of normal pubertal development

A

Premature thelarche
Isolated premature menarche
Premature pubarche/adrenarcge (contrasexual)

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12
Q

What history to ask in evaluation of child with premature sexual development?

A

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

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13
Q

What examination to perform in evaluation of child with premature sexual development?

A

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

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14
Q

Investigation for premature sexual development

A

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

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15
Q

What imaging to consider when investigation precious puberty

A

● 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)

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16
Q

What impact does acclerated bone maturation and premature epiphyseal fusion have on long term growth

A

Early cessation of growth, compromised final height

17
Q

What is the goal of treatment in treating precious puberty?

A

Half or cause regression in 2ndary sexual characteristic
Prevent early menarche
Retard skeletal maturation and improve fundal height
avoid psychosocial/beahivoural sequelae

18
Q

Treatment of precious puberty

A

GnRH analogues (if <6 yr helps increase adult height)
Normally stopped when normal time for puberty to being, mean time to menarche 16 months

19
Q

What is McCune-Albright Syndrome?

A

Mutation GNAS1 genet
- Polyostotic fibrous dysplasia (bone cyst)
- Cafe au last spot
- Peripheral precocious puberty

20
Q

Treatment of McCune-Albringt syndrome

A

Aromatase inhibitors - testolactone, anastrazole, tetrazole

Ass hyperthyroidism and hyperprolacticaemia

21
Q

The bone is usually advanced except in which condition

A

Hypothyroidism

22
Q

Which hormone level is raised in classical CAH

A

17-hydroxyprogesterone - 21 hyroxylase deficiency

23
Q

Which blood test is raised in androgen secreting tumour

A

DHEAS

24
Q

What is premature thelarche

A

Isolated premature breast development (absence of other signs of puberty)