Peads - General Flashcards

1
Q

what is kawasaki disease?

A
systemic, medium sized vessel vasculitis
affects children (usually under 5)
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2
Q

who is kawasaki more common in?

A

boys
asian children
under 5

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3
Q

possible complication of kawasaki?

A

coronary artery aneurysm

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4
Q

key clinical features suspicious of kawasaki?

A
persistent high fever for >5 days
widespread erythematous macupopapular rash
desquamation on palms and soles of feet
strawberry tongue
cracked lips
cervical lymphadenopathy
bilateral conjunctivitis
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5
Q

investigations in suspected kawasaki?

A

FBC (shows anaemia, leukocytosis and thrombocytosis)
LFTs (can show low albumin and high liver enzymes)
raised inflammatory markers

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6
Q

what are the 3 phases of kawasaki disease?

A

acute
subacute
convalescent stage

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7
Q

what happens in acute phase of kawasaki?

A

child is unwell with fever, rash and lymphadenopathy

lasts 1-2 weeks

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8
Q

what happens in sub-acute phase of kawasaki?

A

acute symptoms settle
desquamation and arthralgia start
risk of coronary artery aneurysms in this phase
lasts 2-4 weeks

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9
Q

what happens in convalescent stage of kawasaki?

A

remaining symptoms settle
blood tests slowly return to normal
coronary artery aneurysms (if present) regress
lasts 2-4 weeks

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10
Q

how is kawasaki disease managed?

A
high dose aspirin (thrombosis risk)
IV immunoglobulin (reduce risk of coronary artery aneurysm)
follow up with ECHOs
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11
Q

kawasaki disease is one of the only reasons to use aspirin in kids, why?

A

aspirin causes risk of reye’s syndrome in kids

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12
Q

what is reye’s syndrome?

A

rare condition causing swelling in liver and brain

usually affects kids/teenagers recovering from viral infection (most commonly flu/chickenpox)

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13
Q

what is congenital adrenal hyperplasia?

A

group of rare genetic disorders caused by genetic mutations causing defects in enzymes involved in the conversion of cholesterol to mineralocorticoids, glucocorticoids and sex steroids (all products of adrenals)

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14
Q

how does adrenal hyperplasia occur in CAH?

A

defects in enzymes causes low cortisol production
results in overproduction of ACTH to try and regulate this low cortisol
ACTH acts on steroid producing cells causing adrenal hyperplasia

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15
Q

how is CAH inherited?

A

autosomal recessive

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16
Q

what are the 3 types of CAH (enzymes affected)?

A

21 hydroxylase deficiency
11-beta hydroxylase deficiency
17-beta hydroxylase deficiency

17
Q

most common form of CAH?

A

21 hydroxylase

due to CYP21A2 mutation

18
Q

presentation of 21 CAH?

A

virilisation in females
precocious puberty in males
salt wasting crisis in first 1-3 weeks of life

19
Q

presentation of 11 CAH?

A

virilisation in females
precocious puberty in males
hypertension
hypokalaemia

20
Q

presentation of 17 CAH?

A

virilisation in females
inter-sex in boys
hypertension

21
Q

classical presentation of CAH in males (generally)?

A

struggle to gain weight
adrenal insuffciency
addisons like biochemical pattern
salt wasting

22
Q

classical presentation of CAH in females (generally)?

A

ambiguous genitalia

salt wasting

23
Q

how is CAH managed in children?

A

replace glucocorticoids and mineralocorticoids

surgery to correct

24
Q

how is CAH managed in adults?

A

treat infertility

control androgen excess

25
Q

when is jaundice normal in the neonate?

A

can be normal if occuring >24hrs after birth up to 14 days

26
Q

when is jaundice abnormal in a newborn?

A

occurring <24 hrs after birth

jaundice occurring after 14 days or persisting after day 14 after birth

27
Q

causes of jaundice in first 24 hrs of life?

A

rhesus haemolytic disease
ABO haemolytic disease
hereditary spherocytosis
glucose-6-phosphodehydrogenase

28
Q

in which babies is physiological (normal) jaundice often seen (between 1-14 days)?

A

breastfed babies

29
Q

what tests are done if jaundice still present after 14 days?

A
conjugated and unconjugated bilirubin
direct antiglobulin test (coomb's test)
TFTs
FBC and blood film
urine for MC&S and reducing sugars
U&Es and LFTs
30
Q

causes of prolonged jaundice (>14 days)?

A
biliary atresia
hypothyroid
galactosaemia
UTI
breast milk jaundice
congenital infections (eg CMV, toxoplasmosis)
31
Q

what does coomb’s test show if positive?

A

tests for antibodies directed against RBC

indicates autoimmune haemolytic anaemia if positive

32
Q

what does a high conjugated bilirubin indicate as a cause for prolonged jaundice?

A

biliary atresia

33
Q

tests for jaundice <24 hrs?

A

blood film

  • blister cells indicates G6PD deficiency
  • spherocytes indicates spherocytosis
  • coombs test may show autoimmune haemolytic anaemia
  • osmotic fragility test (shows how fragile RBCs are)