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paeds: child development > Precocious puberty > Flashcards

Precocious puberty Flashcards

1
Q

When is the development of secondary sexual characteristics condisdered outside the normal range in the UK?

A

<8 years in females

<9 years in males

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2
Q

What are 4 recognised patterns of premature sexual development?

A
  1. Precocious puberty
  2. Premature breast development (thelarche)
  3. Premature pubic hair development (pubarche or adrenarche)
  4. Isolated premature menarche
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3
Q

The levels of what 3 hormones help to diagnose and categorise precocious puberty?

A
  1. Pituitary derived gonadotrophins
  2. Follicle-stimulating hormone (FSH)
  3. Luteinizing hormone (LH)
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4
Q

What are the 2 types of precocious puberty?

A
  1. Gonadotrophin-dependent (central, ‘true’ precocious puberty)
  2. Gondadotrophin independent (pseud, ‘false’ precocious puberty
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5
Q

What causes gonadotrophin dependent (central/ ‘true’) precious puberty?

A

Premature activation of the hypothalamic-pituitary-gonadal axis

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6
Q

How is the sequence of pubertal development in gonadotrophin-dependent precocious puberty described?

A

Normal sequence, described as ‘consonant’

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7
Q

What causes gonadotrophin-independent precocious puberty?

A

excess sex steroids outside pituitary gland

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8
Q

How is the sequence of pubertal development in gonadotrophin independent precocious puberty described?

A

Abnormal, ‘dissonant’

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9
Q

What are 5 causes of gonadotrophin-dependent precocious puberty?

A
  1. Idiopathic/familial
  2. Congenital CNS abnormalities e.g. hydrocephalus
  3. Acquired CNS abnormalities e.g. post-irradiation, infection, surgery, brain injury
  4. CNS tumours e.g. craniopharyngioma, neurofibromatosis
  5. Hypothyroidism
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10
Q

What are 4 causes of gonadotrophin-independent precocious puberty?

A
  1. Adrenal disorders: tumours, congenital adrenal hyperplasia
  2. Ovarian: tumour (granulosa cell)
  3. Testicular: tumour (Leydig cell)
  4. Exogenous sex steroids
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11
Q

How can blood tests differentiate between gonadotrophin-dependent and -independent precocious puberty?

A

In gonadotrophin dependent, LH will be raised, as will FSH. LH will be raised more than FSH (source is pituitary, stimulates ovaries/testis/adrenals)

In gonadotrophin independent, FSH and LH will both be low (suppressed by negative feedback - oestrogen and testosterone raised from gonads or exta-gonadal source)

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12
Q

How frequent is gonadotrophin-dependent precocious puberty in girls and why?

A

It is fairly common in girls because the ovaries are very sensitive to secretion of gonadotrophins from the pituitary glands

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13
Q

What is the most common cause of precocious puberty in girls?

A

Gonadotrophin-dependent precocious puberty: idiopathic or familial (i.e. premature onset of normal puberty)

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14
Q

What is the most common gonadotrophin-dependent cause of precocious puberty in girls?

A

Pituitary adenoma

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15
Q

How will puberty develop in precocious puberty caused by pituitary adenoma in girls (gonadotrophin-dependent cause)?

A

Will be consonant, but perhaps rapid

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16
Q

What are 2 of the most common causes of gonadotrophin-independent precocious puberty in girls?

A
  1. Congenital adrenal hyperplasia
  2. Adrenal tumours
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17
Q

What are 4 typical features of the development of gonadotrophin-independent (‘false’) precocious puberty in girls?

A
  1. Pubic and axillary hair
  2. Adult body odour
  3. Acne
  4. Virilisation of genitalia before breast development
18
Q

What is a useful investigation for assessing the progress of precocious puberty in girls?

A

Ultrasound examination of the ovaries and uterus - uterus changes from infantile ‘tubular’ shape to ‘pear’ shape with progression of puberty, endometrial lining can be identified close to menarche

19
Q

What type of precocious puberty is more common in boys and why?

A

Gonadotrophin-independent; testes are relatively insensitive to secretion of gonadotrophins from the pituitary gland

20
Q

What is important to exclude in precocious puberty in males?

A

Pathological cause e.g. intracranial tumour, liver tumour (rarely), adrenal pathology (tumour/congenital adrenal hyerplasia), gonadal tumour

21
Q

What are 3 key things to look for when examining the testes in males with precocious puberty and what do they indicate?

A
  1. Bilateral enlargement of testes with testicular volumes >4ml: gonadotrophin-dependent, e.g. due to intracranial tumour, rarely by secretion of beta-human chorionic gonadotropin from liver tumour
  2. Prepubertal testes: gonadotrophin-independent cause e.g. adrenal pathology such as tumour or congenital adrenal hyperplasia
  3. Unilateral enlarged testis: gonadal tumours
22
Q

What are the 2 possible causes of bilateral enlargement of the testes >4ml in precocious puberty in boys?

A
  1. Intracranial tumour
  2. Secretion of beta-human chorionic gonadotropin from a liver tumour
23
Q

What is the best way to investigate tumours in the hypothalamic region in precocious puberty (gonadotrophin-dependent)?

A

Cranial MRI scan

24
Q

Compared with girls, how frequently is gonadotrophin-dependent precocious puberty pathological in males?

A

Much more often has a pathological cause

25
Q

What are the 3 key aspects of the management of precocious puberty?

A
  1. Detection and treatment of underlying pathology e.g. MRI scan to identify intracranial tumour (particularly in males)
  2. Reducing rate of skeletal maturation, assessed by bone age. Early growth spurt may result in early cessation of growth and a reduction in adult height
  3. Addressing psychological/behavioural difficulties associated with early progression through puberty
26
Q

What decision must be made with a girl who is going through puberty early?

A

Deciding whether to treat; if treatment required to delay onset of mencharche, gonadotrophin-releasing hormone analogues are treatment of choice

27
Q

What is the medication of choice when treating a girl going through normal puberty early, to delay onset of menarche?

A

Gonadotrophin-releasing hormone analogues

28
Q

What is the management of precocious puberty specific to gonadotrophin-independent causes?

A
  • Source of excess sex steroids needs to be identified
  • Inhibitors of androgen or oestrogen production or action e.g. medroxyprogesterone acetate, cyproterone acetate, testolactone, ketoconazole may be used
29
Q

Between what age does thelarche aka premature breast deveolpment usually affect females?

A

between 6 months and 2 years of age

30
Q

What is the nature of the premature breast development (thelarche) that may occur in infant girls?

A

May be asymmetrical and fluctuate in size, rarely progressing beyond stage 3 of puberty

31
Q

How is premature breast development (thelarche) differentiated from gonadotrophin-dependent preococious puberty?

A

Absence of axillary and pubic hair and of a significant growth spurt

32
Q

What is the management of premature breast development (thelarche)?

A

It is nonprogressive and self-limiting, so investigations not usually required

33
Q

How is bone age determined?

A

Looking at appearance of bones on x-ray

34
Q

What is premature pubarche (adrenarche)?

A

Occurs when pubic hair develops before 8 years of age in females and before 9 years in males, but with no other signs of sexual development

35
Q

What is the most common cause of premature pubarche (adrenarche)?

A

Accentuation of normal maturation of androgen production by the adrenal gland between the age of 6 and 8 years

36
Q

In which ethnic groups is premature pubarche more common?

A

Asian and Black children

37
Q

What features may sometimes be associated with premature pubarche?

A

Slight increase in growth rate and bone age (by 12-15 months)

38
Q

What is the usual course of premature pubarche?

A

Usually self-limiting

39
Q

What would a more aggressive course of virilisation in combination with premature pubarche (rapid growth rate, higher bone age) suggest?

A

Congenital adrenal hyperplasia or adrenal tumour

40
Q

What are 3 investigations that can be in performed premature pubarche, to help differentiate it from nonclassical congenital adrenal hyperplasia or adrenal tumour?

A
  1. Urinary steroid profile
  2. Evaluating levels of androgens in the blood
  3. Measuring bone age
41
Q

What are girls who develop premature pubarche at increased risk of developing?

A

Polycystic ovarian syndrome in later life

paeds: child development (8 decks)

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