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Part 2 Paediatrics > endocrinology and growth > Flashcards

endocrinology and growth Flashcards

1
Q

Kwashiorkor disease

A

this is usually seen in infants and younger children it is the consequence of protien dificeny and results in hypoalbuminaemia.

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2
Q

Msrasmus

A

consequence of total caloric deprivation

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3
Q

what can untreated hypothyroidism lead to?

A

growth failure

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4
Q

what medical illnesses can cause growth failure?

A

growth hormone deficiency, CF, recurrent vomiting, inflammatory bowel disease, congenital heart disease, coeliac disease.

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5
Q

what is the amount of cm a typical child grows per year between the ages of 4 and 10?

A

4 to 8 cm

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6
Q

what does oestrogen cause in the context of puberty?

A

leads to fusion of the epiphyses which leads to cessation of growth.

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7
Q

what is the typical growth velocity for children reached?

A
  1. 5 years girls

13. 5 years boys

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8
Q

how much will a boy grow during puberty?

A

30 cm

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9
Q

how much will a girl grow during puberty?

A

27.5 cm

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10
Q

what are some examples of genetic conditions that result in short stature?

A

Noonan/ William syndrome

skeletal dysplasia

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11
Q

when should you use a stadiometer?

A

after the age of 2

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12
Q

what is the average length at birth of full term baby?

A

50 cm

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13
Q

what is definition of prematurity?

A

before 37 weeks

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14
Q

What are the factors that influence weight?

A

socioeconomic deprivation, social policy, family history, poverty, calorie dense food, genetics, working patterns, health policy.

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15
Q

when do you weight and measure up to 6 months old?

A

once a month before 6 months,

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16
Q

when do you weight a measure children between the ages of 6 to 12 months?

A

once per two month period

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17
Q

when do you weigh and measure a child over 1 years?

A

every 3 months over the age of one year.

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18
Q

what does insulin like growth factor 1 test for?

A

if this is low then it indicates a growth hormone deficiency, if it is high it can indicate a pituitary adenoma (acromegaly)

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19
Q

what is the formula for calculating expected height?

A

mid parental height = (mum’s height + dad’s height)/2 + 7 cm +/- 2 cm

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20
Q

clinical tip for calculating mid parental height

A

females subtract 7 from MPH and for males add 7

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21
Q

what are the ddx for growth hormone deficiency

A

genetics, pituitary gland, hypothalamic dysfunction, neurosensory dysfunction, receptor defects

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22
Q

what is the growth hormone treatment starting dose?

A

0.025 to .035mg/kg/day

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23
Q

what symptoms will lead to a medical cause of obesity

A

development delay, hypogonadism, attenuated growth velocity, Neuro signs, antipsychotic use, hyperphagia, excess weight gain

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24
Q

ddx genetic syndromes that cause obesity?

A

Prader-Willi
alb rights hereditary osteodystrophy
hypothalamic obesity due to cns damage
leptin deficiency

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25
Q

how long does it take for a girl to attain menarche from the onset of puberty?

A

2 to 2.5 years

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26
Q

What is the median age for girls for the onset of True central puberty?

A

10

27
Q

what is tanner stage 1

A

prepubertal stage

28
Q

what is tanner stage 2

A

breast development: subareolar breast buds
pubic hair: sparse long and slightly straight or slightly curly, along the medial labia
pubertal event: peak height velocity

29
Q

what is Tanner stage 3?

A

breast development: breasts and areolae are enlarged with continuous rounded contour
pubic hair: darker course curled spread over mons pubis
pubertal event: peak height velocity

30
Q

what is tanner stage 4?

A

breast development: areola and nipple form secondary mound above the contour of the breast
pubic hair: adult type but the area covered is smaller and there is no extension to the medial thighs
pubertal event: menarche

31
Q

stage 5 tanner?

A

breast: mature adult stage, nipple projection without secondary mound
pubic hair: adult type and quality, sometimes extending to the medial thighs
pubertal event: menarche

32
Q

the testicular volume during onset of true central puberty?

A

4 mls

33
Q

how long does it take take during normal puberty for the testes to enlarge from 4 mls to 20 to 25 mls?

A

2 to 3 years

34
Q

what is tanner stage 1 boys?

A

pre pubertal

35
Q

Tanner stage 2 boy?

A

genital: enlargement of the testes to 4 mls
pubic hair: sparse long and slightly pigmented straight or curled at the base of the penis
pubertal event: none

36
Q

tender stage 3 in boys?

A

genital development: continued testicular and scrotal enlargement with penile growth
pubic hair: darker course and more curled spread sparsely over the pubis
pubertal event: peak height velocity spermarche

37
Q

What is the tanner stage 4 in boys

A

genital: continued testicular, scrotal and penile growth with enlargement of the glans
pubic hair: adult type but area covered is smaller and there is no extension to the medial thighs or the lines alba
pubertal event: peak height velocity, spermarche, facial hair voice change

38
Q

tanner stage 5 boys

A

genital: mature males
pubic hair: adult
pubertal event: none

39
Q

what is mini puberty?

A

active between about 1 week an peaks between 1-3 months of age

40
Q

what is mini puberty helpful for?

A

pituitary deficiency

DSD disorder

41
Q

What is precocious puberty defined as?

A

the onset of breast tissue before the age of 8 and the in boys a testicular volume of 4 mls before the age of 9

42
Q

what is central precocious puberty?

A

brain and gonaotropin mediated

43
Q

what is peripheral precocious puberty?

A

exogenous sex steroids production by the ovaries, testes, adrenals or tumours or other or administration of exogenous sex steroids.

44
Q

What is the cause of congenital adrenal hyperplasia?

A

the most common deficiency is 21 hydroxyls deficiency. This causes a defect in the production of cortisol. The precursor to cortisol builds up leads to excess androgens. The adrenals then become bully and females will be born with virilisation.

45
Q

What are the cause of delayed puberty?

A

consitutional delay in growth and puberty
familial delayed puberty
delayed puberty due to medical condtions: CF, chronic renal disease, inflammatory bowel disease, Juvenile idiopathic arthritis.

46
Q

when is puberty considered delayed in girls?

A

no breast development by 13.5

47
Q

What is primary amenorrhoea defined as?

A

no onset of Menes by 15

48
Q

What is the most common cause of delayed puberty?

A

constitutional delay and family delay

49
Q

what are other more rarer causes of delayed puberty?

A

medical conditions (nutritional failure- IBD) cancer, radiotherapy and chemotherapy

50
Q

what are some of the SE of chemotherapy agents in prepubertal children?

A

hypogonadotropic hypogonadism and primary ovarian failure.

51
Q

what are some genetic conditions that are associated with delay in puberty?

A

Turner syndrome, kallman syndrome

52
Q

what is the workup for a girl with delayed puberty?

A

basal osetrodiol, LH, FSH, tester one, karyotype, bone X-ray, ovarian and uterine ultrasound to assess pubertal status.
Karyotype
LHRH testing to assess HPG axis, MRI brain, anti-mullerian hormone (to assess ovarian reserve) further androgens, urinary steroid profile, laparoscopy and assessment of other pituitary functions can be left to the endocrinologist.

53
Q

what is the underlying cause of delayed puberty is not treatable in girls what is the treatment?

A

exogenous oestrogen increased slowly over 6 months then progesterone added to the mix.

54
Q

what I the definition of delayed puberty in males?

A

not having reached a testicular volume of 4 mls by the age of 14.

55
Q

What’s the causes of delayed puberty in boys?

A

constitutional delay, familial delay and history of chronic disease
testicular torsion or orchitis can cause primary testicular failure. previous orchidopexy can also cause failure.
genetic causes such as Kaman syndrome and klinfelters syndrome

56
Q

what biochemical tests should you be doing in a boy with pubertal delay?

A

basal early morning testosterone, dihydrotestosterone, androstenedione, LH, FSH, bone xray.
HCG stimulation test, LHRH testing, karyotyping, urinary steroid profile, tesicualr US, MRI brain laparoscopy etc.

57
Q

what is the treatment for pubertal delay in men?

A

exogenous testosterone

58
Q

what test would you do in a girl with precocious puberty?

A

oestradiol, androstenedione, DHEAS, testerone, 17OHP, bone age

59
Q

what is premature adrenarche? biochemistry

A

this is characterised by increased levels of DHEAS and androstenedione.

60
Q

premature adrenarche symptoms

A

axillary hair may be associated with pubarche it may be isolation. There is no virilisation. This does not require any further management.

61
Q

precocious puberty in a girl with breast budding?

A

bone age, pelvic US, blood test, MRI brain.

62
Q

what are the blood tests that are need to in a child with precocious puberty girl?

A

LHRH, FSH, LH, oestradiol, DHEAS, androstenedione, testosterone, 17OHP

63
Q

how would you diagnose idiopathic central precocious puberty?

A

MRI: looking for brain tumour
abdominal U/S: tumours
high LHRH, FSH, high oestradiol, elevated testosterone

64
Q

what is the treatment for idiopathic CPP?

A

GnRH agonist therapy

Part 2 Paediatrics (8 decks)

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