Precocious Puberty Flashcards

1
Q

What are the ages for precocious puberty for boys and girls?

A

Boys: <9 years old

Girls: <8 years old

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2
Q

If precocious puberty occurs and consonance is followed, where is the problem likely to lie?

A

Hypothalamo-Pituitary Axis problem

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3
Q

With precocious puberty and non-consonance where is the problem likely to lie?

A

Gonadal/Adrenal Problem

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4
Q

What is the commonest cause of precocious puberty following a disconcordant pattern?

A

Congenital Adreanl Hyperplasia due to 21alpha hydroxylase deficiency

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5
Q

What is the hormone profile for CAH due to 21alpha hydroxylase deficiency?

A

Cannot make cortisol so → lots of testosterone → virilisation

Also lack of fight and flight

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6
Q

What is the treatment for CAH due to 21alpha hydroxylase deficiency?

A

Hyrdocortisone replacement therapy

Fludrocortisone (aldosterone replacement)

Adult: Dexamethasone (potent cortisol) → ↓Hyperplasia

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7
Q

Precocious puberty combined with the following suggest:

Both testes enlarged

No enlargment in testes

One testis enlarged

A
  1. Cental cause - exclude tumour
  2. Not central (so low FSH and LH and testosterone)
  3. Testis the problem? (Low FSH/LH but high testosterone)

?Tumour

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8
Q

What is McCune-Albright syndrome?

A

Genetic disorder of bones (polyostotic fibrous dysplasia)

skin pigmentation (Cafe au lait)

precocious puberty (hyperfunction of endocrine system)

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9
Q

What is exaggerated adrenarche?

A

Adrenal androgen secretion → greasy skin, spots, hair, smelly

No increase in breast/testes, no growth spurt, normal genitalia

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10
Q

What would the investigations show for thelarche?

A

Bone age not advanced

Pelvic USS - prepubertal

LHRH test → pronounced FSH response, LH low

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11
Q

What would investigations show for Exaggerated adrenarche?

A

Bone age advanced by 1 year

17OHP normal

Testosterone/Oestrodiol - normal for age

Gonadotrophs normal

DHEAS raised, androstenedione raised

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12
Q

What would investigations show for central precocious puberty?

A

Detectable oestrodiol, LH and FSH

LHRH → LH dominant response

Bone age advanced >2 years

USS - increased ovaries/uterus/follicles/endometrial stripe

MRI pituitary → normal

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13
Q

What is the management of central precocious puberty?

A

GnRH Analogue → inhibit LH/FSH secretion

Stop at age 11-12 → onset of menses 12-18 months after

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14
Q

If untreated, what does peripheral precocious puberty lead to?

A

Menarche

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15
Q

What would the investigations show for peripheral precocious puberty?

A

Detectable Oestrodiol

LH/FSH undetectable

LHRH testing → no response

Bone age advanced >2 years

USS - increased ovaries/uterus/follicles/endometrial stripe

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