Puberty Flashcards

1
Q

Define puberty

A

physiological, morphological and behavioural changes as the gonads switch from infantile to adult forms

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2
Q

Describe the process of puberty

A

inhibition of puberty diminishes
increased FSH and LH secretion
initiation of sex steroid secretion
sexual maturation
adolescent growth spurt

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3
Q

What factors influence timing of puberty?

A

genetic - strong correlation between parents and children/siblings

nutrition - obese children enter puberty earlier, 17% body fat needed for menses, 22% for ovulation

environment - earlier puberty in urban areas, closer to equator, lower altitudes

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4
Q

Requirements for puberty

A

normal capacity for growth
adequate nutrients
normal body composition
emotional wellbeing
good physical health

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5
Q

Secondary sexual characteristics in girls

A

ovarian oestrogens regulate the growth of breast and female genitalia

ovarian and adrenal androgens control pubic and axillary hair

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6
Q

Secondary sexual characteristics in boys

A

testicular androgens:
- external genitalia and pubic hair growth
- enlargement of larynx and laryngeal muscles –> voice deepening

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7
Q

What staging is used to classify puberty?

A

Tanner staging

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8
Q

What stages of puberty are the tanner stages roughly relating to?

A

pre-puberty = Tanner stage 1
early puberty = Tanner stage 2-3
later puberty = Tanner stage 4-5

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9
Q

What signifies the start of puberty in boys and girls?

A

boys = testicular volume 4mls
girls = breast stage 2

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10
Q

Order of male puberty

A

testicular enlargement and pubic hair occur first
growth of penis and growth in height starts after about 12 months

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11
Q

Order of female puberty

A

growth rate increases from onset of puberty
breast and pubic hair development is congruent
menarche occurs late and growth stops shortly thereafter

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12
Q

What is done in an endocrine evaluation of puberty?

A

clinical evaluation
pubertal staging
FSH, LH, testosterone, oestradiol, prolactin, thyroid function, karyotype, androstenedione, DHEAS
GnRH test
bone age xray (dominant hand + wrist)
pelvic ultrasound (girls)

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13
Q

What ages suggest precocious puberty?

A

girls <8y
boys <9y

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14
Q

How is early puberty classified?

A

central or gonadotrophin-dependent:
- usually girls
- usually idiopathic
- CNS lesion may be present

gonadotrophin-independent:
- sex-steroid production independent of FSH/LH

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15
Q

Name some puberty variants

A

premature thelarche
premature pubarche (adrenarche)
isolated premature menarche
adolescent gynaecomastia

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16
Q

Describe premature thelarche

A

isolated breast development - typically very young girls (1-4y)
non-progressive with no growth spurt
remits spontaneously

17
Q

Describe premature pubarche

A

isolated pubic hair development
usually girls 5-7y

18
Q

Describe isolated premature menarche

A

early onset of periods
despite no or minimal pubertal development
exclude hypothyroidism

19
Q

Describe adolescent gynaecomastia

A

common in early to mid puberty in boys, particularly if obese

20
Q

When is central precocious puberty always pathological?

A

in boys
- suggests CNS lesion

21
Q

Causes of true (central) precocious puberty (GnRH dependent)

A

idiopathic precocious puberty

CNS tumours - optic glioma, hypothalamic astrocytoma

CNS disorders - developmental abnormalities, hypothalamic hamartoma, encephalitis, brain abscess, hydrocephalus, myelomeningocele, arachnoid cyst, vascular lesion, cranial irradiation

secondary central precocious puberty

psychosocial eg. adoption from abroad

22
Q

Causes of precocious pseudo-puberty (GnRH-independent)

A

increased androgen secretion - congenital adrenal hyperplasia, virilising neoplasm, leydig cell adenoma, familial male precocious puberty (testotoxicosis)

gonadotrophin secreting tumours

mccune-albright syndrome
ovarian cyst
oestrogen-secreting neoplasm
(hypothyroidism)

23
Q

Describe McCune-Albright syndrome

A

triad of:
- precocious puberty
- large irregular cafe au lait patches
- polyostotic fibrous dysplasia

mosaic expression of G-protein coupled receptor over-activity

spontaneous secretion of various hormones may occur

24
Q

Describe congenital adrenal hyperplasia

A

inborn error of cortisol and/or aldosterone synthesis

mostly 21 alpha hydroxylase deficiency
large amounts of adrenal androgens and testosterone secreted, producing virilisation but with small testes
may go unrecognised in boyd
treatment with hydrocortisone replacement

25
Q

Central precocious puberty treatment

A

GnRH analogues
non-pulsatile stimulation of FSH and LH secretion results in down regulation of responsiveness to GnRH

26
Q

Gonadotrophin-independent precocious puberty

A

treatment of the underlying cause where possible
anti-androgen/androgen receptor blockers/aromatase inhibitors

27
Q

What ages are classed as delayed puberty?

A

> 13y girls
14y boys

28
Q

Causes of delayed puberty

A

constitutional delay in growth and adolescence
illness/malnutrition
failure of hypothalamus/pituitary (hypogonadotrophic hypogonadism)
gonadal failure

29
Q

Describe constitutional delay

A

often familial
more common in males
associated short stature
spontaneous puberty will occur
relative disproportion
reduced final height

30
Q

What illnesses can cause delayed puberty?

A

chronic disease:
- cystic fibrosis
- crohn’s disease
- juvenile arthritis
- steroid therapy

hypothalamic amenorrhoea:
- anorexia nervosa
- athletes/gymnasts
- leptin deficiency

31
Q

What is Kallmann’s syndrome?

A

hypothalamic GnRH deficiency with anosmia

32
Q

Name some causes of hypogonadotrophic hypogonadism

A

Kallmann’s syndrome
Prader-Willi syndrome
Pituitary gonadotrophin deficiency

33
Q

Name some causes of gonadal failure

A

Klinefelter’s syndrome
Turner’s syndrome

34
Q

Normal LH level pre-puberty

35
Q

Klinefelter’s syndrome karyotype

36
Q

Klinefelter’s syndrome features

A

mild developmental delay/autistic features
small, hard testes
gynaecomastia
elevated FSH, LH, low testosterone

37
Q

Constitutional delay treatment

A

sex steroid replacement until puberty well-established