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Step 2 Flashcards

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1
Q

medication that can be used for bed wetting

A

desmopressin (ADH analogue)
imipramine (anticholinergic)

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2
Q

if primitive reflexes persist in developing children or return in adulthood, what does this indicate

A

if they persist = problems with development of frontal lobe

if return in adulthood = frontal lobe lesion/tumour

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3
Q

at what percentile indicates macrocephaly or microcephaly

A

> 97th percentile = macrocephaly
< 3rd percentile = microcephaly

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4
Q

describe the expected course of weight in an infant

A

newborns can lose 5-10% birth weight in the first few days but should return to BW by 14 days

infants double their BW by 4-5 months, triple by 1 year and quadruple by 2 years

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5
Q

constitutional growth delay vs familial short syndrome

A

CGD; delayed growth and puberty, positive FH, delayed bone age, will eventually reach normal height

FSS; delayed growth with height < 2nd percentile, positive FH, will remain short height in adulthood, normal bone age, puberty not delayed

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6
Q

describe androgen insensitivity syndrome

A

Androgen insensitivity syndrome (AIS) is a genetic disorder characterized by an X-linked mutation of the androgen receptor leading to a phenotypically female appearance in a 46,XY individual. Affected individuals will have breast development, cryptorchidism, and no pubic hair. Perform a gonadectomy after puberty to prevent testicular cancer.

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7
Q

features of tuberous sclerosis

A

neurocutaneous disorder;
- shagreens patches (type of connective tissue hamartoma on dorsum of body)
- ash leaf spots
- angiolipomas
- subependymal nodules
- giant cell astrocytomas
- seizures
- intellectual disability

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8
Q

most common coloniser in CF < 20 years old

A

staph aureus

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9
Q

role of DNase in cystic fibrosis

A

deoxyribonuclease reduces viscosity of sputum which helps with respiratory symptoms

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10
Q

what is kernicterus and risk factors

A

kernicterus is deposition of bilirubin in the bsal ganglia, pons and cerebellum
risk factors include prematurity, asphyxia and sepsis

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11
Q

what is the APGAR score and its parameters

A

score of 0-2 for each sign and calculated at 1 and 5 minutes after delivery.

appearance; pink, pink with blue, blue/pale
pulse; >100, <100, no pulse
grimmace (reflexes); active, some flexion, flaccid
activity; active, flexion of limbs, absent
respirations; vigorously cries, slow/irregular respirations, absent

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12
Q

conditions which cause impaired bilirubin uptake and secretion from the liver

A

Dubin johnson syndrome
Rotor syndrome

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13
Q

conditions which cause impaired conjugation of bilirubin

A

gilbert syndrome
crigler nijjar syndrome
newborn physiological jaundice

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14
Q

mechanism of newborn physiological jaundice

A

impaired conjugation of bilirubin

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15
Q

mechanism of breast milk jaundice

A

increased enterohepatic circulation

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16
Q

risk factors associated with jejunal atresia

A

cocaine other vasoactive substance exposure

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17
Q

conditions associated with dudenal atresia

A

down syndrome
annula pancreas
malrotation
imperforate anus

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18
Q

conditions associated with tracheosophageal atresia

A

oesophageal atresia
VACTERL (vertebral, anal, cardiac, tracheosophageal, renal, limb defects)

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19
Q

conditions associated with omphalocele

A

beckwith-wideman syndrome
trisomies

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20
Q

what change in amniotic fluid is found with gastroschisis and omphalocele

A

gastroschisis = oligo or polydramnios
omphalocele = polyhydramnios

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21
Q

management for congenital diaphragmatic hernia

A

high-frequency venilation or extracorporeal membrane oxygenation to manage pulmonary hypertension
surgical correction

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22
Q

breastfeeding jaundice vs breast milk jaundice

A

both cause jaundice due to increase in intrahepatic circulation
breastfeeding jaundice is fue to inadequate intake and presents first week of life
breast milk jaundice is hypothesized to be due to factor present in breast milk inhibit hepatic enzyme UGT1A1 and peak at 2-3 weeks of life

treatment for breastfeeding jaundice is hydration
treatment for breast milk jaundice usually doesnt require treatment

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23
Q

at what bilirubin level do you start phototherapy and exchange transfusion

A

phototherapy starts > 15
exchange transfusion required when very high > 20

in preterm infants, threshold for phototherapy is lower (10-15)

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24
Q

what imaging modalities can be used to assess the cause of conjugated hyperbilirubinaemia in a neonate

A

USS liver and biliary tree
HIDA scan (hydroxy iminodiacetic acid)

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25
risk factors for neonatal respiratory distress syndrome
c-section maternal diabetes male sex second born of twins
26
feature of NRDS (neonatal respiratory distress syndrome) on CXR
ground glass appearance bronchograms lack of focal opacities
27
feature of transient tachypneoa of newborn on CXR
perihilar streaking in inter-lobular fissures
28
feature of meconium aspiration on CXR
coarse, irregular infiltrates pneumothorax lung hyperexpenasion
29
feature of congenital pneumonia on CXR
non-specific patchy infiltrates
30
complications associated with treatment for NRDS
PDA barotrauma bronchopulmonary dysplasia necrotising enterocolitis intraventricular haemorrhage retinopathy of prematurity
31
pathophysiology of apnea of prematurity
immaturity of central respiratory centers
32
caput seccundum vs cephalhaematoma
caput seccundum: oedema/haematoma of the scalp due to vacuum extraction. Above periosteum and so crosses suture lines. resolves on its own. cephalhaematoma: haematoma below the periosteum commonly occipital or parietal bones and so doesnt cross suture lines. risk of infection and jaundice. usually resolves on its own.
33
neonatal with diffuse fluctuant swelling that shifts with movement and crosses suture lines. neonate develops haemorrhagic shock. ?diagnosis
subgaleal haemorrhage haemorrhage between the periosteum and aponeurosis epicranium due to injury of emissary veins by traction during delivery can coolect 40% of noenates total blood volume resulting in severe volume depletion and haemorrhagic shock
34
most common cause of neonatal hypothyroidism in USA and developing world
USA - thyroid dysgenesis developing world - iodine deficiency
35
coadministration of what nutrients is required in neonatal hypothyroidism
SOY, IRON and CALCIUM due to poor absorption
36
features of congenital hypothyroidism
6 P's pot bellies puffy face protruding tongue protruding umbilicus poor brain development
37
neonate with frequent wakenings, poor suck-swallow coordination, hypertonia, yawning and tremor. ?diagnosis
neonatal abstinence syndrome
38
narrow S2 with increased P2
VSD also; harsh pansystolic murmur at left sternal border, mid-diastolic apical rumble due to increased velocity through mitral valve
39
wide and fixed split S2
ASD also; ejection systolic murmur at left sternal edge, mid-diastolic rumble at left sternal edge due to high velocity through tricuspid valve
40
treatment for PDA
indometacin sugical repair if indometacin fails, CI (such as intraventricular haemorrhage) or > 2 weeks age
41
investigations for PDA
best initial: echo doppler (will show blood flowing into pulmonary artery) ECG (may show LVHV) CXR (may show cardiomegaly with large PDA)
42
conditions associated with PDA
prematurity congenital rubella syndrome
43
condition associated with bicuspid aortic valve and coarctation of aorta
turner syndrome
44
what conditions are associated with coarctation of aorta
bicuspid aortic valve turners syndrome berry aneurysms
45
treatment for coarctation of aorta in infants and older children
infants; initial prostacyclin E1 to maintain PDA then surgical repair older children: balloon angioplasty with or without stent placement
46
risk factor for transposition of great arteries
diabetic mother digeorge syndrome
47
CXR appearance of transpotition of great arteries
egg on string appearance
48
best initial treatment for transposition of great arteries
prostaglandin E1 to maintain PDA to allow growth for a more successful surgical outcome, a balloon atrial septostomy is performed to create a shunt that is not reliant on PDA and prostaglandin infusion
49
best definitive treatment for transposition of great arteries
surgical correction
50
risk factors for tetralogy of fallot
downs syndrome di george syndrome
51
whats the first step in the surgical correction of tetralogy of fallot
modified Blalock-Thomas-Taussig shunt
52
lab finding in pyloric stenosis
hypochloraemia hypokalaemic metabolic alkalosis
53
best initial treatment and deficinitive treatment for pyloric stenosis
best initial: keep patient nill by mouth, IV hydration deficinitive: pyloromyotomy
54
pathophysiology of malrotation with volvulous
congenital malrotation fothe midgut can result in abnormal positioning of the small intestine and the formation of Ladd's bands (fibrous bands) which predispose to obstruction and volvulous with constriction of blood flow
55
definitive treatment for malrotation and volvulous
Ladds procedure
56
best initial treatment malrotation and volvulous
NG tube and decompression, IV fluids
57
what is the rule of 2's with meckel diverticulum
2 inches long found within 2 feet of the illeoceacal valve 2% of population 2x more common in males 2 tissue types (pancreatic and gatsric)
58
best initial and definitive diagnostic modality for hirshprungs
initial: abdominal XR definitive: rectal biopsy
59
investigation of choice for suspected vesicoureteric reflux
VCUG (voiding cytstourethrogram)
60
treatment for cryptochordism in prepuberty vs post puberty
prepubertal = orchiopexy (fixes testes into scrotum) post-puberty = orchiectomy vs close observation to minimise risk of cancer if found at birth, surgery should be performed asap after 4 months age, ideally before 1 year
61
benefits of orchiectomy vs orchiopexy for cryptochidism
orchiectomy (removal of testes) eliminates risk of cancer whereas orchiopexy is fixing the testes into the scrotum, it reduces infertility but lowers (not eliminates) risk of cancer
62
what immunisation should be given with caution in patients with egg allergy
MMR yellow fever
63
why are children < 6 years most susceptible to lead poisoning
due to immature blood brain barrier
64
presentation of lead poisoning
peripheral neuropathy (wrist and foot drop) cognitive impairment colicky abdominal pain constipation headache hyperactivity or apathy anorexia
65
describe treatment for lead poisoning depending on blood levels
< 5: family education and remove source of lead 5-14: retest in 1-3 months 15-44: retest in 1-4 weeks 45-69: retest in 48 hours, start chelation therapy (oral succimer) >70: urgent treatment with chelation therapy and admission, retest in 24 hours
66
conditions associated with multiple cafe au lait spots
neurofibromatosis McCune albright syndrome
67
what is the next step if you suspect tracheosophageal fistula in a newborn
place an NG tube if it doesnt pass 10-15cm then TEF is present
68
fixed drug eruption vs exanthematous drug rash
both occur due to medications exanthematous causes generalised maculopapular rash fixed drug euption causes one or several red patches and the rash wouldnt be generalised.
69
what initial investigation should be carried out in an infant with suspected digeorge syndrome
echo
70
from what maternal and baby blood groups would ABO incompatability be a cause of jaundice
if mother is 0 and baby is A, B or AB
71
gold standard to diagnose intestinal marotation
upper gastrointestinal studies
72
in suspected shaken baby syndrome, what imaging should be done first
MRI spine to check for cervical injury
73
what is the treatment infants who show signs of bilirubin encephalopathy (kernicterus) ?
high pitched cry, hypertonicity, lethargy, poor feeding and arching exchange transfusion
74
patch of hair loss with pustules and flaking of the scalp
tinea capitis. remnants of infected hairs that have broken at the scalp line (“black-dot” hairs)

Paediatrics (10 decks)

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