w/c 29/01 Flashcards
whats the most common physical health complication in children w downs syndrome?
congenital cardiac abnomrmality
what is the most common congenital cardiac abnormality in children with downs syndrome?
atrioventricular septal defect
what ix confirms diagnosis of CF
chloride sweat test
what ix is done in neonates to screen for CF and whats the result of it
neonatal blood spot test, raised immunoreactive tripsinogen is found in CF
what is given for RSV/bronchiolitis prophylaxis?
pavilizumab
how do you treat neonatal jaundice that is within 50 micromoles/L of treatment threshold?
admit and recheck levels in 18 hrs
what pulse do you check for in an unresponsive child under the age of 1?
brachial
what is the first step in asthma management when a child first presents
start SABA and very low dose ICS
whats the most common congenital heart defect in those with Turner’s syndrome? what is often associated with?
bicuspid aortic valve, associated with coarctation of the aorta
what murmur does ventricular septal defect cause and where is it best auscultated?
pansystolic murmur
heard best over the lower left sternal edge
what murmur does atrial septal defect cause, what is heard and where is it best auscultated?
ejection systolic murmur
first heart sound normal, wide fixed splitting of second heart sound
heard best over upper left sternal edge
what is seen on otoscopy in acute otitis media?
a bulging tympanic membrane
what organism causes acute epiglottits?
h.influenzae
at what age can a child provide legal consent to have sex?
13
what are the 3 main deficiencies in someone with bone marrow failure?
anaemia
neutropenia
thrombocytopenia
what are some RF for DDH?
female
first born
breech
describe the course of symptoms in someone presenting with measles?
- high fever and coryzal sx
- conjunctivitis 2-5 days after sx onset
- followed by a macropapular rash starting on face behind ears and spreading downwards
what is a pathognomonic for measles?
koplik spots
what sign is seen on x ray for acute epiglottitis?
thumb or thumbprint (lateral x ray)
what is seen on blood film with G6PD deficiency?
heinz bodies and red cell fragments
what medicates trigger acute haemolysis in g6pd deficiency?
sulphonylureas
ciprofloxacin
nitrofurantoin
what do you need to do if someone has meningitis caused by nisseria meningitidis?
notify public health england
what are the 3 main live vaccines?
MMR
inhaled influenza
varicella
when are live vaccines contraindicated?
in immunosupression
what 4 issues are present in tetralogy of fallot?
- ventricular septal defect
- aortic arch overriding the VSD
- outflow obstruction from right ventricle
- right ventricular hypertrophy
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
what is the definitive diagnostic test for coeliacs disease?
small bowel biopsy
what triad is seen on small bowel biopsy in coeliac disease?
subtotal villous atrophy
crypt hypertrophy
lymphocytic infiltrates
whats the first step in paediatric BLS after you notice theres no respiratory effort?
5 rescue breaths
whats the mx for localised impetigo?
topical fusidic acid
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
what blood test is done when you suspect duchennes muscular dystrophy?
creatinine
describe the pathophysiology of kallmann syndrome
failure of GnRH producing neurons to migrate to the hypothalamus causing hypogonadotrophic hypogonadism
what are features of kallmann syndrome
delayed puberty (late menarche, small testes, small breasts, less body hair etc)
loss of smell
what is the most common causative organism for acute gastroentertitis?
rotavirus
describe a typical presentation of henloch schonlein purpura
viral URTI followed by:
purpura over legs/buttocks
nephritis
abdominal pain
arthralgia
what is the word for when hands/feet peel in kawasaki disease?
desquamation
what is the most important ix for someone who has orbital cellulitis?
CT orbit
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
what might you see on mri joint in juvenile idiopathic arthritis?
effusion
bone erosion
synovial hypertrophy
what ix would you do for juvenile idiopathic arthritis?
bloods: FBC, ESR, CRP, ANA antibodies, anti CCP antibodies
imaging: MRI
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
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
what direction is the shunt in VSD?
left to right
what direction is the shunt in eisenmenegers syndrome?
right to left
what antidepressant during pregnancy increases risk of VSD?
sertraline
what congenital cardiac abnormality is associated with maternal lithium use during pregnancy?
ebsteins anomaly
what genetic abnormality causes edwards syndrome?
trisomy 18
what are some features of edwards syndrome?
low set ears
overlapping digits
heart defects eg VSD
omphalocele (intestines outside body)
microcephaly
what is the first line ix for suspected coeliacs disease?
anti Ttg IgA antibody
what malignancy is poorly controlled coeliacs associated with?
enteropathy associated t cell lymphoma
