Growth And Puberty Flashcards

1
Q

What parts of life have the fasted growth

A

Infancy and puberty

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2
Q

What are the driving factors for growth at each stage of life? Infant , childhood , puberty? Key in each?

A

Infant - NUTRITION, good health & happiness, thyroid hormones
Childhood - GROWTH HORMONE, thyroid hormones, health & happiness, genes
Puberty - SEX HORMONES, growth hormone

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3
Q

Female passage of puberty? First sign onwards…

A

breast development
Pubic hair growth & growth spirt almost immediately after
Menarche - approx 2.5 years after start of puberty and signals end of growth (~5cm remaining)

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4
Q

Males passage through puberty ?

A

1st - testicular enlargement to >4ml
Pubic hair growth
Growth spirt (~18 months after start of puberty)

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5
Q

How can you measure tesicles

A

Orchidometer

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6
Q

What signs are seen in both males and females going through puberty

A

Acne, body odour, axillary hair, mood changes

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7
Q

Define short stature

A

Height 2SD below mean for age and sex

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8
Q

What’s a sensitive way to measure growth failure ?

A

Assessing height velocity

2 accurate measurements 6/12 - 12/12 apart allow HV to be calculated in cm/year

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9
Q

What cohort will have 1/3 remaining short ?

A

IUGR / extreme prem

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10
Q

Estimate genetic height target

A

Parents height / 2
(+7 for boys, -7 for girls)
Range - (+-10cm for boys, +-8cm for girls)

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11
Q

What are the causes of short stature

A
Familial 
Constitutional delay 
Endocrine
Nutritional / chronic illness
Chromosomal disorders / syndromes 
Disproportionate short stature
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12
Q

What causes constitutional delay ? What will the body type be? Other features? How can you induce puberty ?

A

Dieting or extreme exercise (FHx if not)
short body, long legs
Bone age will show moderate delay
Androgens

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13
Q

Usual Sx of endocrine causes of short stature ? Causes?

A

Falling off centiles, short & relatively overweight
Hypothyroidism - usually autoimmune
GH deficiency
Corticosteroid excess

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14
Q

Causes of GH deficiency ? What other feature?

A

Isolated or secondary to pan hypopituitarism (craniopharyngioma, meningitis, trauma)
Bone age markedly delayed

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15
Q

Usual cause of corticosteroid excess? What needs to be looked out for?

A

Iatrogenic

Features of cushings

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16
Q

Usual shape of nutritional cause of short stature? Causes?

A

short & underweight

Coeliac, crohns, cystic fibrosis, chronic renal failure

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17
Q

Chromosomal causes of short stature

A

Downs, tuners, noonan’s & russel-silver syndromes

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18
Q

What causes disproportionate short stature ? Limbs

A

Achrondroplasia, storage disorder

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19
Q

What investigations can be done for short stature and rational? Which diseases cause?

A

X-ray L wrist - bone age (*+++decreased in endocrine, + decreased in constitutional)
FBC - anaemia in coeliac / crohns
U&E’s - increased creatinine in renal failure
TSH- increased in hypothyroidism
CRP - increased in crohns
Antiendomysial / anti globin antibodies - coeliac
Karyotype (females) - 45X0 in turners
MRI - if neuro Sx - craniopharyngioma / Intra cranial tumour

20
Q

Primary causes of tall stature ? What happens in secondary (rare) endocrine causes?

A

Marfan’s (fibrillin) & Klinefelter’s (XXY, small testis)
CAH & precocious puberty cause an initial growth spurt, but early epiphyseal fusion -> usually means eventual height is decreased

21
Q

Define precocious puberty

A
22
Q

How is precocious puberty categorised ?

A

Levels of pituitary gonadotropins
If ^ FSH & LH -> central cause
If decreased FSH & LH -> peripheral cause

23
Q

Most common peripheral cause of precocious puberty ?

A

Adrenal (+gonadal) tumours -> excess sex steroids

24
Q

When would you ?tumour in central cause of precocious puberty?

A

Visual disturbance, CNS abnormality, other signs of raised ICP

25
Q

Management of precocious puberty

A

Detection and treatment of any underlying pathology

GnRH analogues are treatment of choice if physiological (to prevent short stature)

26
Q

What is breast development called?

A

Thelarche

27
Q

Usual person affected by premature Thelarche ? Is it always symmetrical ?

A

6 months - 2 years, female

No

28
Q

How do you differentiate premature Thelarche from PP?

A

Absence of axillary & pubic hair and no growth spurt

29
Q

What needs to be done for premature breast development ?

A

Nothing

It is benign and self limiting

30
Q

What is the growth of pubic hair called?

A

Arenarche

31
Q

When is premature pubic hair growth?

A

Before 8 in girls, 9 in boys

32
Q

What is the common cause of premature pubic hair growth ?

A

Accentuation of the normal maturation of androgen production by the adrenal glands (adrenarche)

33
Q

Who commonly gets premature pubic hair growth ?

A

Asian and Afro Caribbean children

34
Q

Management of premature pubic hair growth ?

A

Usually self limiting

USS ovaries & uterus, and bone age to exclude PP

35
Q

When is puberty classes as delayed ? Who is it more common in?

A

14 in girls, 15 in boys

Boys - *constitutional delay (with FHx in father) *

36
Q

Males with delayed puberty are often….?and have…?

A

Short during childhood

Delayed skeletal maturity on bone age

37
Q

What should be done in girls with delayed puberty ?

A

Karyotype to identify turners (45XO)

Measure sex hormones, TSH, T3,T4

38
Q

None delayed puberty causes of decreased FSH & LH

A
Systemic disease (CF, coeliac, crohns, anorexia nervosa, excess exercise)
Isolated GNrH/GH deficiency or panhypopituitarism (?craniopharyngioma)
39
Q

Causes of increase FSH & LH

A
Chromosomal abnormalities (Klinefelter's 47XXY, turners 45XO)
Acquired gonadal damage (chemo, trauma, testicular torsion)
40
Q

What can cause a disorder of sexual differentiation ? How common

A

Congenital adrenal hyperplasia (1:5000)

41
Q

What makes Congential adrenal hyperplasia more common?

A

Presents from number of autosomal recessive disorders -> if parents related = more common

42
Q

What is cause in >90^ of CAH ? What is it used for? What is also a common feature?

A

Deficiency of 21-hydroxylase (needed for cortisol biosynthesis)
80% cannot produce aldosterone (salt losers)

43
Q

What does cortisol deficiency do to a fetus?

A

Increased ACTH release & adrenal hyperplasia

44
Q

Clinical features of CAH

A

Virilization of female external genitalia with clitoral hyper trophy (+- fusion of labia)

  • salt losing crisis* at 1-3 weeks old in 80% of males (salt losers)
  • Tall stature in remaining 20% (non salt losers) and precocious puberty*
45
Q

Features of salt losing crisis

A

Hypotension, circulatory collapse, floppiness, vommiting & weight loss

46
Q

What can aid CAH diagnosis

A

Markedly raised levels of metabolic precursor 17a-hydroxyprogesterone in blood

47
Q

Management of CAH

A

Females - surgical correction of genitalia malformations
Life long glucocorticoids to suppress ACTH levels and allow normal growth
Mineralcorticoids (fludrocortisone) in salt losers
Growth monitoring