Puberty Flashcards

1
Q

In boys, what indicates the start of puberty?

A

Testes larger than 3mL in volume or >2.5cm in diameter.

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2
Q

In girls, what indicates the start of puberty?

A

Breasts noted or palpable and enlargement of areola.

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3
Q

What is the effect of oestrogen on the breasts?

A

Causes:

  • Ductal proliferation
  • Site specific adipose deposition
  • Enlargement of the areola and nipple
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4
Q

Differences between prepubertal and pubertal-adult uterus

A

Prepubertal Uterus

  • Tubular shape
  • 2-3cm
  • 0.4-1.6ml
  • Endometrium single layer of cuboidal cells

Pubertal - Adult

  • Pear shaped
  • 5-8cm
  • 3-15ml
  • Endometrium increased thickness
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5
Q

Differences between prepubertal and pubertal-adult ovaries

A

Prepubertal Ovaries

  • 0.2-1.6ml
  • Non-functional

Pubertal Ovaries

  • 2.8-15ml
  • Multicystic
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6
Q

What is adrenarche? What does it cause?

A

Maturation process of the adrenal gland observed in humans
Development of androgen producing zona reticularis
Peaks around 20 years of age
Increased levels of DHEA and DHEA-S which are precursors of androgen hormones
We see advanced bone age, axillary hair, oily skin, mild acne and body odour
More pronounced in obese. children.

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7
Q

Describe the HPG axis

A
  1. Hypothalamus releases GnRH
  2. Anterior pituitary releases LH, FSH
  3. Gonads ovaries - oestrogen
  4. Gonads testes - testosterone
  5. Sex hormone action in body
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8
Q

What is idiopathic central precocious puberty? Is it more common in males or females?

A

Precocious puberty in girls is defined by the development of sexual characters before the age of 8 years.
Precocious breast development is usually due to the premature activation of the hypothalamo-pituitary-ovarian axis, defining central precocious puberty
80% of CPP are females

It is RARE for males to go into CPP, if they do, we need to RULE OUT brain tumours - males presenting usually indicates something sinister.

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9
Q

What is precocious pseudopuberty and how do we treat?

A
  • Looks like normal puberty with gonadal development and puberty but the GnRH is not activated
  • The most common thing is adrenal sex hormone excess or secreting tumours of the gonad, brain, retroperitoneum, mediastinum and liver secreting hCG which has similar function as LH and FSH which stimulates the gonads and leads to sex hormone action
    Treat = GnRH super agonist to suppress pulsatility of GnRH secretion
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10
Q

Is delayed puberty more common in males or females?

A

More common in males
Cause for concern if females present with delayed puberty.
Occurs in about 3% of children

  • Hypogonadotrophic hypogonadism: Sexual infantilism related to gonadotrophin deficiency
  • Hypergonadotrophic hypogonadism: Primary gonadal problems (testes or ovaries)
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11
Q

What can an X-ray of the left hand show us?

A

Delayed bone age in GH deficiency or Advanced bone age in precocious puberty.
Early puberty can also cause short stature as bones mature quicker.

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12
Q

What is the difference between hypogonadotrophic hypogonadism and hypergonadotrophic hypogonadism?

A

Hypogonadotropic hypogonadism:

  • Problem with hypothalamus or pituitary where there is underactivation of gonads through reduction in hypothalamus and pituitary
  • This is a symptom, not a diagnosis so there can be many causes - tumours etc

Hypergonadotropic hypogonadism:
- Where the gonads are not functioning properly so the hypothalamus (GnRH) and pituitary (LH, FSH) are over acting to produce response

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13
Q

What is the diagnostic dilemma in delayed puberty?

A

No gold standard to differentiate between central hypogonadotropic hypogonadism and constitutional delay in growth and puberty so we usually need to wait and diagnosis can take some time.

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14
Q

What is constitutional delay in growth and puberty?

A

Children experience delayed puberty compared with peers
More common in boys
Likely to be short for their age with history of normal growth
Delays in bone. maturation and delay in adrenarche
Frequently there is a family history of puberty delays too

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15
Q

Cause for hypergonadotrophic hypogonadism in males and females?

A
Females = turners syndrome
Males = Klinefelter's syndrome
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16
Q

Explain Klinefelters syndrome & it’s effects

A
  • Primary hypogonadism
  • High LH and FSH but low testosterone or oestrogen = diagnosis of primary gonadal failure and gonad doesn’t work
  • Pretty common but underdiagnosed
  • Typical finding = don’t have a testicular volume of more than 5ml, some never go into puberty or some go through some puberty and stop
  • Don’t have testes larger than 5ml
  • Approx 1 in 1,000 males
  • Reduced secondary sexual hair
  • Osteoporosis
  • Tall stature
  • Reduced IQ in 40% (not necessarily learning difficulties)
  • 20 fold increased risk of breast cancer compared to other men
17
Q

Explain Turner’s syndrome and it’s effects

A
  • 1 in 2,000 females
  • Ablation of gene on X chromosome
  • Relatively common
  • Hypergonadotropic hypogonadism
  • Need to rule out in every girl with unexplained short stature
  • At birth, oedema of dorsa of hands, feet, loose skinfolds at nape of neck, high arched palate
  • Webbing of neck
  • Low posterior hairline
  • Small mandible
  • Prominent ears
  • Broad chest
  • Hyperconvex fingernails
  • Cubitis valgus
  • Cardiovascular malformations - heart echo needed and risk for problems with aortic arch
  • Renal malformations (horseshoe kidney)
  • Recurrent otitis media
  • Short stature
18
Q

What hormone replacement therapy do we use for women?

A
  • Ethinyloestradiol or oestrogen tablets
  • Start low dose and increase dose to provide time for pubertal growth and gradual breast development
  • Several incremental steps over 2 years until full adult replacement dose achieved
  • Once full replacement achieved, progesterone should be added
19
Q

What hormone replacement therapy do we use for men?

A
  • Testosterone enanthate, IM injection most common method of pubertal induction and maintenance
  • Increasing use of transdermal testosterone (gel applied once a day)
  • Several incremental steps of 2 years until full adult replacement dose achieved