20. Physiology of puberty Flashcards

1
Q

Disorders and syndromes of puberty

A

Disorders of Puberty
Premature Puberty
Delayed Puberty

Syndromes
Klinefelter Syndrome
Turner Syndrome

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2
Q

Definition of puberty

A

Definition:
The stage of physical maturation in which an individual becomes physiologically capable of pro-creation (sexual reproduction)

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3
Q

Physical changes in puberty

A

Physical changes:

  • Growth spurt
  • Secondary sex characteristics
  • Menarche/spermatogenesis
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4
Q

Physical changes in girls

A

GROWTH SPURT 8 - 14 yrs
6 - 10 cm / yr peak
2.5 yrs duration

BREAST GROWTH 8 - 13 yrs ( 11 yrs )
(Thelarche) Usually the first sign

PUBIC HAIR 9 - 13 yrs
(Pubarche) 1st Pubertal Sign in 25%

AXILLARY HAIR 9.5 - 15 yrs
(Adrenarche) May follow menarche

MENSTRUATION 10 - 16 yrs ( 13 yrs )
(Menarche)

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5
Q

Signs of puberty in boys

A

In boys, first sign of puberty is testicular size - testicular volume of 4 is about start of puberty
Growth spurt later in boys than girls, can grow 12-14cm in a year.
Axillary or pubic hair is not true puberty until see testicular volume of 4ml or above.

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6
Q

Endocrine changes in puberty - prepuberty

A

H-P-G synchrony – established in fetal life
Until puberty – neural mechanisms suppress GnRH release
At 6-9 yrs – pulsatile nocturnal GnRH release

Mini-puberty in babies - HPG axis becomes synchronous, gets ready for later maturation
Need LHRH test

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7
Q

How does FSH and LH change in puberty and what are the conseuqences

A

Pulsatile GnRH release (nocturnal) every 90-120 min - 6 to 9 y
increase in GnRH leads to increase in FSH and increase in LH
Ovaries/testes become sensitized to the effects of FSH and LH
Final phase: development of positive/negative feedback mechanism

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8
Q

What results in hair growth at puberty?

A

ACTH stimulates the adrenals – Pubic & Axillary hair

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9
Q

What is the role of the testes?

A

Production of gametes (sperms)
Sertoli cells under FSH control
Production of androgens (Testosterone)
Leydig cells under LH control
95% from testes, 5% from adrenals
Testosterone in blood – converted to DHT in the target organs
Testosterone is converted to oestrogen which acts on the long bones resulting in bone growth

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10
Q

The ovarian cycle

A

Follicular phase
Initially E rises (FSH) with LH surge in mid cycle
Ovulation occurs

Luteal phase
Negative feedback after ovulation
No further ovulation in the same cycle

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11
Q

Age of menarche

A

Related to general health, genetic and nutritional factors
Mean age is falling at a rate of 4 months per decade
- Mean age in 1840 = 16.5 y; 1990 = 12.8 y
One in 8 girls now reaches menarche while at primary school
Body weight and % fat is also important
- Mean weight at menarche is 47.8kg
- 16-24% fat
- Athletes, patients with anorexia – late onset

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12
Q

What is adrenarche?

A

Adrenal androgens – responsible for axillary and pubic hair

ACTH stimulates zona reticularis of adrenal cortex

DHEAS & Androstenedione:
Girls – starts by 6, adequate levels by 8
Boys – starts by 8, adequate levels by 10

Test with urine steroid profile test

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13
Q

Chronological order of puberty in girls

A
Growth spurt
Breast development
Pubic hair
Axillary hair
Menarche
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14
Q

Chronological order of puberty in boys

A
Testicular volume
Penile length
Pubic hair
Growth spurt
Axillary / Facial hair
Deep voice
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15
Q

When is puberty considered early or precocious?

A

Girls – under 8 yrs

Boys – under 9 yrs

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16
Q

When is puberty considered delayed?

A

Girls – over 14 yrs
Boys – over 14 yrs

But if there is no breast budding at all by 13 then investigate for treatment because it means androgens aren’t working

17
Q

Precocious puberty

A

Early or Premature puberty
Presence of true pubertal features at an young & inappropriate age

Central or True precocious puberty
Gonadotrophin dependent
Peripheral or Pseudo-precocious puberty
Gonadotrophin independent

Normal variants
Premature Thelarche
Premature Adrenarche

18
Q

Central or true precocious puberty

A

Gonadotrophin dependent

19
Q

Peripheral or pseudo-precocious puberty

A

gonadotrophin independent

20
Q

Concerns raised by early onset

A

Possible underlying sinister cause
- Boys – upto 80%
Emotional & pyscho-social upheaval at an inappropriately young age
Early cessation of growth leading to decreased final adult height

21
Q

Central precocious puberty

A

Long acting LHRH analog therapy

  • Sustained supra-physiological LHRH levels
  • Paradoxical cessation of gonadotrophin release
  • Stops further pubertal progression

Pubertal progression resumes when treatment stopped (at 10-12 yrs)

22
Q

Normal variants

A

Premature thelarche
Isolated breast development
Usually seen in children <2-3 yrs of age

Premature adrenarche
Isolated pubic hair development
Caution: first sign of puberty in some
And . . .

23
Q

Pseudo-precocious puberty in males

A

Iso-sexual or masculinising
CAH, Adrenal/Leydig cell tumor

Hetero-sexual or feminising
Adrenal

24
Q

Pseudo-precocious puberty in females

A

Iso-sexual or feminising
MAS, Ovarian/Adrenal

Hetero-sexual or masculinising
CAH, Ovarian/Adrenal

25
Delayed puberty
Absence of true pubertal onset at an appropriate age Not necessarily lack of periods in a girl X-files – very important Normal variant - Constitutional growth & pubertal delay
26
Concerns raised by delay
Possible sinister underlying cause Fear that puberty will never occur Emotional and psychosocial upset of immaturity, specially when associated with short stature Long term sequelae: Reduced bone mineralization
27
Klinefelter syndrome
``` 1 in 1000 male infants 47 XXY / Multiple X Behavioural problems Androgen deficiency Azoospermia / Infertility (Micro genitalia  Sex change) Lifelong testosterone replacement therapy ```
28
Turner syndrome
1 in 2000 live female births Triad - Short stature, streak gonads, primary amenorrhoea Dysmorphic features – Webbing of neck, cubitus valgus Coarctation of aorta, horse shoe kidneys Early clue - Lymphedema Surprisingly normal !!! Turner Mosaic
29
Managing turner syndrome
``` Exclude co-existing congenital anomalies Growth Hormone therapy Pubertal induction + ongoing HRT Active monitoring to detect co-morbidities Assisted conception ```
30
Normal variant of delayed puberty
``` Constitutional delayed growth & puberty More common in boys Small & Short in school days Late onset of puberty Bone age delayed slightly ``` Family history – often present Normal adult height Pubertal induction – sometimes necessary