203 - Puberty Flashcards

1
Q

Puberty is characterised by 4 changes - they are:

A

Secondary sexual characteristics
Acceleration of somatic growth
Advancement of bone maturation
Behavioural + psychological changes

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2
Q

What is the difference between puberty and adolescence?

A
Puberty = physical changes
Adolescence = psychological and nerological
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3
Q

What factors influence the onset of puberty?

A
Genetics - when parents did
Nutritional status
Exercise
Chronic inflammatory conditions
Environment (abusive environment - early start)
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4
Q

When is the normal range of onset of puberty in boys and girls?

A

Girls - 8-13.75

Boys - 9-13.25

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5
Q

What is consonance?

A

The sequence of puberty

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6
Q

What activated + controls puberty?

A

Hypothalamic-pituitary-gonadal axis

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7
Q

What are the 3 consonant stages in male puberty?

A

1) testes growth >4mls
2) Penile growth + pubic hair
3) Growth spurt - late in puberty

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8
Q

What are the 3 consonant stages in female puberty?

A

1) breast development + fat deposition around hips + uterus + vagina increase in size
2) Growth spurt
3) Menarche

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9
Q

Why do boys generally end up taller than girls?

A

Boys have their growth spurt later in puberty (testes vol = 10-12mls), so have 2 more years of childhood growth = 10cm extra.
Boy’s growth spurt is usually 28cm, whereas girls is 25cm.

So boys have 10 + 3 = 13cm extra growth than girls

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10
Q

What stimulates breasts to develop?

A

Begin after 1 yr of pulsatile LH + FSH

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11
Q

What controls development of pubic and auxiliary hair in girls?

A

Adrenal androgens - so not always at same time.

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12
Q

Describe the hypo-pit-gonadal axis

A

Hypothalamus produces GnRH (pulsatile)
This stimulates the anterior pituitary to produce LH and FSH

Girls: LH acts on gonads (ovaries) - oestrogen and progesterone produced, FSH acts on ovaries - Ova produced

Boys - LH + FSH act on gonads. LH acts on leydig cells which produce testosterone

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13
Q

What is Adrenarche?

A

Adrenal hormones begin to be released, eg. DHEA
Cause virilization, pubic and auxiliary hair growth in girls
Can begin 1 yr before puberty

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14
Q

What is gonadarche?

A

Puberty onset controlled by the hypo-pit-gonadal axis

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15
Q

How does growth end?

A

Fusion of the epiphyseal plate of bone

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16
Q

What causes the fusion of the epiphyseal plate?

A

Oestrogen levels (girls have more - stop growth earlier)

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17
Q

Is a delay in puberty more common in boys or girls?

A

10 x more in boys

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18
Q

What defines a delay in puberty in boys?

A

Failure of testicular growth > 4mls by 14yrs

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19
Q

What defines a delay in puberty in girls?

A

Absence of breast development by 13.5yrs

OR when puberty is incomplete - menstruation hasn’t occurred within 3 yrs of breast development

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20
Q

What is the cause of the majority of boys with delayed puberty?

A

Constitutional delay - 80%

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21
Q

What is usually the cause of girls with delayed puberty?

A

Pathology - destruction of axis / lack of gonadal response

22
Q

What investigations are done in delayed puberty?

A
Hormone levels (FSH, LH, Oestrogen, Testosterone, adrenal hormones)
Pelvis US
Bone age
Karyotype
CT/MRI of cranium
23
Q

What do you X ray to asses bone age?

A

L hand and Wrist

24
Q

What treatment can be given to a boy with maturational delay?

A

Give testosterone supplements until natural puberty kicks in

25
Q

What are the two main types of delayed puberty?

A

Primary gonadal failure

Central failure

26
Q

What would the hormone profile look of someone with primary gonadal failure?

A

High FSH, High LH

27
Q

What conditions may cause primary gonadal failure?

A
Turners (XO), Klinefelters (XXY)
Autoimmune 
Enzyme deficiencies
Androgen insufficiency
Chemo/Radiotherapy
Bilateral, unconnected, cryptoorchidysm
28
Q

What is AIS - causes delayed puberty?

A

Androgen insensitivity syndrome
- genital tissue insensitive to androgens - so despite testes forming and producing androgens, they aren’t detected, so the genitals develop as female.
X-linked recessive
1/20,000

29
Q

What treatment can be given for AIS : androgen insensitivity syndrome?

A

Counselling (genetically man, phenotypically female, blind ending vagina)
Gonadectomy
Vaginal dilators?
HRT to stop osteoporosis

30
Q

What hormone profile would you expect to see in a central failure delayed puberty?

A

Low FSH, Low LH

31
Q

What may cause a central failure delayed puberty?

A
Constitutional
Eating disorders
Excessive exercise
Hypothyroid
Intracranial tumour
Pan hypopituitarism
GH/GnRH deficiency
PCOS
Chronic illness
32
Q

What is precocious puberty?

A

Onset of secondary sexual characteristics early

33
Q

Under what age is it considered precocious puberty?

A

Girls - under 8 or menarche under 9

Boys - under 9

34
Q

What impact does precocious puberty have?

A

Reduced height
Fertility + menopause age - ovarian reserve?
Psychological issues

35
Q

What symptoms might you see in precocious puberty?

A

Sex steroids cause - greasy skin/hair, mood changes, deep voice, growth spurt, pubic hair in boys

The aetiology might be evident - tumour cause - headaches, vomiting, visual issues?

36
Q

What are the 2 types of precocious puberty?

A

Central/true/gonadotrophin dependant

Peripheral/gonadotrophin independant

37
Q

What are the features of central precocious puberty?

A

puberty is consonant but early, hypothal is activated

more common in girls

38
Q

What can cause central precocious puberty?

A
idiopathic
secondary to tumours
cerebral palsy
hydrocephelus
trauma
sex abuse
adoption
39
Q

What are the features on peripheral precocious puberty?

A

May be non consonant

Sexual characteristics due to abnormal secretion of sex steroids - independent of hypo-pit-gonadal control

40
Q

What causes are there for peripheral precocious puberty?

A
  • Genetic - McCune Albright synd., testotoxicosis
  • Precocious breast development - Thelarche, hypothyroidism, ovarian cysts
  • Virilisation - Adrenarche, CAH, Cushings
  • Gonadotrophin releasing tumour
  • Exogenous steroids
41
Q

What is McCune Albright syndrome?

A

A rare hereditary condition, triad of effects:

  • Polyostotic fibrous dysplasia.
  • Café au lait skin pigmentation.
  • Autonomous endocrine hyperfunction -> precocious puberty, hyperthyroid..
42
Q

What is Thelarche?

A

Isolated breast development, in absence of other symptoms (no growth spurt or sexual precocity)

43
Q

Do you need to investigate Thelarche? What is the treatment?

A

No - can be normal

No treatment, just follow up.

44
Q

What would an LHRH test show in Thelarche?

A

Pronounced FSH, Low LH

45
Q

What is exaggerated adrenarche?

A

‘adrendal puberty’ - adrenal androgens secreted early

Age 6-8

46
Q

What does exaggerated adrenarche cause?

A

Early pubic + auxillary hair, body odour, mild acne

47
Q

What investigations could you do in exaggerated adrenarche?

A

Hormone levels - would show:

- High DHEAs + High Androstenedione + high adrenal hormones

48
Q

What investigations might you do for a child with precocious puberty?

A
Auxology (measurements)
Parental heights
Hormone levels
TFT
Bone age
Pelvic U/S in girls
49
Q

What hormone profile would you expect in central precocious puberty?

A

High LH, FSH and Oestradiol

LHRH response - LH dominant

50
Q

What hormone profile would you expect in peripheral precocious puberty?

A

No LH or FSH
High oestradiol
No LHRH response

51
Q

In any precocious puberty, what would you find when measuring bone age and on pelvic US (girls)?

A

Bone age advanced by around 2 yrs
Increase in ovary and uterus size
Endometrial stripe present

52
Q

What is the management of a central precocious puberty?

A

GnRH analogue - binds for longer than normal GnRH, which causes a -ve feedback which inhibits LH/FSH

Take until 11/12, then periods begin a year later