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LS YEAR 4 Paediatrics > endocrine and growth > Flashcards

endocrine and growth Flashcards

1
Q

what is fetal tyroidixine

A

reverse t3 produced it is a derivative of t3, inacitive
after birth there is a surge in Tsh and causes increase in t3 and t3
This then declines to normal adult range by the end of first week or so
premature babies have low t4 in first few week usually

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2
Q

two causes of hypothyroidism in infancy

A

congenital and juvenile

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3
Q

describe congenital hypothyroidism and its cause

A

a preventable cause of severe learning difficulty

causes:
1. iodine deficency
2. dyshormongenesis -inbhorn error or hormone synthesis -seen increased with consanguinity

  1. maldescent of thyroid ./athyrosis - failure of development, remains as a mass
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4
Q

which is the most common cause

A

maldescent of thyroid ./athyrosis

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5
Q

how do you replace hypothyroidism

A

oral replacement therapy

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6
Q

what is the cause of juvenile hypothyroidism

A

autoimmune thyroiditis

may. be seen with diabetes, turner, downs

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7
Q

symptoms of congenital

A 
failure to thrive
prolonged jaundice
constipation
large tongue 
cold mottled skin
feeding problems 
umbilical hernia 
developmental delay 
goitre 
hoarse cry
coarse facies
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8
Q

symptoms of juvenile hypothyroidism

A 
short stature, growth failure
cold intolerances
dry skin
bradycardia
goitre
loss of third of eyebrow
thin dry hair
cold peripheries
obesity
slow reflexes
delayed puberty
constipation 
slipped upper femoral epiphysis 
puffy eyes
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9
Q

what is phenylketonuria

A

autosomal recessive inheritance

1 in every 10,000-15,000 births

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10
Q

two types of causes

A

deficiency In enzyme phenylalanine hydroylase

deficiency in synthesis or recycling of cofactor needed for the enzyme

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11
Q

presents

A 
development delay at 6-12 months
musty odour- breathe, urine, , skin 
fair hair, blue eyes
eczema or seizures 
recurrent vomiting 
tremors/jerking
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12
Q

what has been implemented in uk

A

Guthrie test which can detect

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13
Q

management

A

restriction of dietary phenylalanine through low protein diet
monitor blood plasma
avoid aspartame

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14
Q

frequency of monitoring

A

<6 months weekly
6m-4 y - fortnightly
4 year and older-monthly

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15
Q

when do you need to be careful

A

in pregnancy can cause damage to foetus

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16
Q

what is short stature

A

children whose height is 2 standard deviations below the mean for the sex and age and ideally ethnic group
may be caused by disease of be a variant of normal growth

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17
Q

what are the two variants of normal growth

A

familial /geneic
delayed( constitutional )
non pathological causes

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18
Q

what are the pathological causes

A 
IBD /coeliac 
Turner syndrome
growth hormone deficiency 
JIA 
undernutrition 
CF
CKD
cancer
His
diabetes, Cushing, hypothyroidism 
syndrome -noonan, turner, silver-russell 
prader willi (also obesity)
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19
Q

tests

A 
rule out causes
coeliac tests 
X-ray-for bone age 
fbc-anaemia
gh tests 
creatine raised in cvd
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20
Q

management

A

reassurance

dependent on cause-somatotrophin replacement

21
Q

red flags for short stature

A

discrepancies in height and weight and parental height mid thingy

22
Q

what are the three key sexual development disorders

A

ambiguous genetalia
delayed puberty
precocious puberty

23
Q

what is precocious puberty

A

when puberty occurs before age of 8 and 9 in female and males

24
Q

what is thelarche and pubarche

A

early breast development

early pubic hair development

25
Q

causes of precocious puberty

A

gondatrophin release
tumour
neurofibroma

management USS, MRI

26
Q

delayed puberty

A

later than 14 and 15 in females and males respectively
more common in males
delayed skeletal maturity
often a fix

girls can be seen ion Turner syndrome

27
Q

ix for delayed puberty

A

karyotyping

thyroid and sex hormones

28
Q

management of delayed puberty

A

reasurrance
can give oxandrolone or low dose testosterone In males
oestradiol in girls

29
Q

key thing about ambigous genitalia

A

do not assign gender
can delay birth paperwork
a medical emergency indeterminate sex

30
Q

what can cause it

A 
secondary to
-excessive androgens most common 
-congenital adrenal hyperplasia 
inadequate androgen action 
gondatrophin insufficiency 
true hermaphroditism
31
Q

what can gondatrophin insufficiency be seen in

A

Prada willi syndrome

32
Q

tests that can be done

A

bp
blood sugars
karyotyping
pelvis uSS

33
Q

what is congenital adrenal hyperplasia

four biochemical features

A

80% lack aldosterone production therefore reduce cortisol production causes acth increase and increase in adrenal androgens

low NA increase K
metabolic acodisosis and hypoglycemia

34
Q

symptoms and signs of congenital adrenal hyperplasia

management

A 
- clitoral hypertrophy and labia fusion 
enlarged pension
tall stature
precocious pubarche
salt crisis-  weight loss, vomiting flopping and circulatory collapse)

glucorticosteriods and mineraocortiods (salt)

35
Q

what is diabetes mellitus

A

inadequate insulin production mechanism dependent on type
most common childhood -type 1 - < 5 years of age, destruction of beta cells in pancreas leading to insulin deficiency
other types :
type 2
mature onset
cf-pancreas insufficiency
induced through steroid use

36
Q

symptoms

diagnosis

A 
polyuria
polydypsia 
tired 
weight loss 
diagnosis of blood sugar >11.1 or fasting >7
37
Q

what else can be used to monitor

other things to rule out

A 
hb1ac better reflection of 2-3 months control 
urinalysis
blood gas 
coeliac screen 
tft
islet of langerham cell antibodies
38
Q

consequences of poor diabetic control

A 
ketoacidosis 
recurrent hypoglycemia
poor growth
hyperglycemia
lipodystrophy if same injection site used
39
Q

hypoglycaemia

A 
when blood sugars dip inadequate carb intake 
feel hungry
pale sweating tremors
make be drowsy
collapse
coma 
tachycardia
100ml sugary drink
 need rapid carbs 20g or dextrose 2 tablets
40
Q

what is Dka?

A 
may be triggered by infection or poor compliance
thirst 
polyuria 
vomiting
abdo pain
acetone smell on breathe
high blood glucose level 
ketones in blood and urine
metabolic acidosis - <7.25 needs iv insulin 
urea raised 
signs of dehydration 
kussmaul breathingg
hypovolemic shock
recurrent dka major difficulty with self management -pschological and medical support may be needed
41
Q

management

A 
on-going
screening and monitor for complications
dietary advice
monitoring sdvice
education
diet and exercise
education on injection technqiue
lease with sxhools
dealing with emergencies

poor control can lead to renal and visual impairment neuropathy and atherosclerosis

42
Q

hypoglycaemia management

A

glucose 10-20g and then snack repat
if unconscious consider 1m glucagon 200mcg
then dextrose

43
Q

what is failure to thrive?

A

significant interruptionn in the expected rate of growth compared with other children of a similar age and sex during early childhood

44
Q

weight faltering causes

A 
- cf
coeliac disease
endocrine dysfunction 
GORD
chronic illness 
genetic syndrome 
immunodeficiency 
iUGR 
environmentall/psychological

consider other causes - pain, cleft palate, oromotor dysfunction, neuro disorders e.g. cerebral palsy
reflex

45
Q

investigations

A

bloods, tft, u and e, stools for malabsorption
coeliac antibiodies
karotyping for genetic abnormalities associated with short stature
examinations for other signs
growth charts

46
Q

things to consider in history

A 
-infections in utero
maternal smokin and alcohol
placental insufficiency 
complications during labour 
interaction with child of parents-signs of neglect or abuse 
does the child look healthy?
any signs of turner or downs 
normal tone
seem alert?
height weight head circumstance 
red book
look for other signs 
signs of dehydration
47
Q

non medical causes of FTT

A

pyschosocial -difficulties at home, disturbed attachment, post natal depression, neglect
-consider family intervention social services
constitution-small parents

48
Q

management of FTT

A 
address causes 
diatary- 3 meals and 2 snacks a day
add more fatty foods
avoid fruiti juices 
limit milk intake
avoid distractions
praise when food eaten 
never force-feed 
regular meal times with family all eating together
49
Q

when to refer for FTT

A

not responsive to measures in place
paediatrician
consider social work if social problems
psychologist- if food refusal and anxious and stressful meal times

LS YEAR 4 Paediatrics (24 decks)

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