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w/c 29/01 Flashcards

1
Q

whats the most common physical health complication in children w downs syndrome?

A

congenital cardiac abnomrmality

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2
Q

what is the most common congenital cardiac abnormality in children with downs syndrome?

A

atrioventricular septal defect

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3
Q

what ix confirms diagnosis of CF

A

chloride sweat test

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4
Q

what ix is done in neonates to screen for CF and whats the result of it

A

neonatal blood spot test, raised immunoreactive tripsinogen is found in CF

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5
Q

what is given for RSV/bronchiolitis prophylaxis?

A

pavilizumab

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6
Q

how do you treat neonatal jaundice that is within 50 micromoles/L of treatment threshold?

A

admit and recheck levels in 18 hrs

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7
Q

what pulse do you check for in an unresponsive child under the age of 1?

A

brachial

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8
Q

what is the first step in asthma management when a child first presents

A

start SABA and very low dose ICS

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8
Q

whats the most common congenital heart defect in those with Turner’s syndrome? what is often associated with?

A

bicuspid aortic valve, associated with coarctation of the aorta

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9
Q

what murmur does ventricular septal defect cause and where is it best auscultated?

A

pansystolic murmur
heard best over the lower left sternal edge

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9
Q

what murmur does atrial septal defect cause, what is heard and where is it best auscultated?

A

ejection systolic murmur
first heart sound normal, wide fixed splitting of second heart sound
heard best over upper left sternal edge

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10
Q

what is seen on otoscopy in acute otitis media?

A

a bulging tympanic membrane

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11
Q

what organism causes acute epiglottits?

A

h.influenzae

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12
Q

at what age can a child provide legal consent to have sex?

A

13

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13
Q

what are the 3 main deficiencies in someone with bone marrow failure?

A

anaemia
neutropenia
thrombocytopenia

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14
Q

what are some RF for DDH?

A

female
first born
breech

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15
Q

describe the course of symptoms in someone presenting with measles?

A
  1. high fever and coryzal sx
  2. conjunctivitis 2-5 days after sx onset
  3. followed by a macropapular rash starting on face behind ears and spreading downwards
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16
Q

what is a pathognomonic for measles?

A

koplik spots

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17
Q

what sign is seen on x ray for acute epiglottitis?

A

thumb or thumbprint (lateral x ray)

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18
Q

what is seen on blood film with G6PD deficiency?

A

heinz bodies and red cell fragments

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19
Q

what medicates trigger acute haemolysis in g6pd deficiency?

A

sulphonylureas
ciprofloxacin
nitrofurantoin

20
Q

what do you need to do if someone has meningitis caused by nisseria meningitidis?

A

notify public health england

21
Q

what are the 3 main live vaccines?

A

MMR
inhaled influenza
varicella

22
Q

when are live vaccines contraindicated?

A

in immunosupression

23
what 4 issues are present in tetralogy of fallot?
1. ventricular septal defect 2. aortic arch overriding the VSD 3. outflow obstruction from right ventricle 4. right ventricular hypertrophy
24
in tetralogy of fallot what determines the degree of cyanosis? why?
the severity of outflow obstruction from the right ventricle, as this determines the magnitude of the shunt and therefore degree of cyanosis
25
what is the definitive diagnostic test for coeliacs disease?
small bowel biopsy
26
what triad is seen on small bowel biopsy in coeliac disease?
subtotal villous atrophy crypt hypertrophy lymphocytic infiltrates
27
whats the first step in paediatric BLS after you notice theres no respiratory effort?
5 rescue breaths
28
whats the mx for localised impetigo?
topical fusidic acid
29
what sign is seen in duchennes muscular dystrophy?
gowers sign: when a patient is supine, they are unable to stand up without walking their hands over their legsa
30
what blood test is done when you suspect duchennes muscular dystrophy?
creatinine
31
describe the pathophysiology of kallmann syndrome
failure of GnRH producing neurons to migrate to the hypothalamus causing hypogonadotrophic hypogonadism
32
what are features of kallmann syndrome
delayed puberty (late menarche, small testes, small breasts, less body hair etc) loss of smell
33
what is the most common causative organism for acute gastroentertitis?
rotavirus
34
describe a typical presentation of henloch schonlein purpura
viral URTI followed by: purpura over legs/buttocks nephritis abdominal pain arthralgia
35
what is the word for when hands/feet peel in kawasaki disease?
desquamation
36
what is the most important ix for someone who has orbital cellulitis?
CT orbit
37
whats the definitive mx of volvulus? what does it entail
ladds procedure, it involves untwisting the volvulus, fixing the gut to the correct position, removing ladds bands and doing an appendicectomy
38
what might you see on mri joint in juvenile idiopathic arthritis?
effusion bone erosion synovial hypertrophy
39
what ix would you do for juvenile idiopathic arthritis?
bloods: FBC, ESR, CRP, ANA antibodies, anti CCP antibodies imaging: MRI
40
how is juvenile idiopathic arthritis managed?
1st line: NSAIDs 2nd line: steroids (oral or intra articular) 3rd line: steroid sparing agents eg methotrexate, TNF alpha inhibitors
41
what is eisenmenegers syndrome?
when a shunt from VSD is reversed as the RV pressure rises above LV pressure, so deoxygenated blood enters the systemic circulation
42
what direction is the shunt in VSD?
left to right
43
what direction is the shunt in eisenmenegers syndrome?
right to left
44
what antidepressant during pregnancy increases risk of VSD?
sertraline
45
what congenital cardiac abnormality is associated with maternal lithium use during pregnancy?
ebsteins anomaly
46
what genetic abnormality causes edwards syndrome?
trisomy 18
47
what are some features of edwards syndrome?
low set ears overlapping digits heart defects eg VSD omphalocele (intestines outside body) microcephaly
48
what is the first line ix for suspected coeliacs disease?
anti Ttg IgA antibody
49
what malignancy is poorly controlled coeliacs associated with?
enteropathy associated t cell lymphoma
50

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