Pediatrics Flashcards

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1
Q

what is the course/duration of prophylactic antibiotics following rheumatic heart disease?

A
  • uncomplicated: 5 years or until age 21
  • with carditis: 10 years or until age 21
  • carditis and valvular disease: 10 years or until age 40

IM BenPen q4 weeks

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2
Q

what are the cardiac abnormalities associated with Turner’s syndrome?

A

coarctation of the aorta

bicuspid aortic valve

aortic root dilation -> dissection/rupture

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3
Q

what are the preventive and screening components of routine neonatal care?

A

preventive

  • vitamin K IM
  • erythromycin eye ointment (N gonorrhoea)
  • HBV vaccine

screening

  • pre-/post-ductal pulse oximetry
  • genetic/metabolic screen
  • bilirubin
  • hearing screen
  • hypoglycaemia (select populations)
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4
Q

what are the indications for newborn echo based on pulse oximetry screening?

A

<90% in any limb

<95% in both UL and LL

>3% difference in UL and LL

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5
Q

how do you increase the intensity of the HOCM murmur?

A

murmur is due to LVOT

decrease the preload and the obstruction will worsen, murmur intensifies

valsalva, standing abruptly, decrease in systemic vascular resistance (GNT/nitroglycerin)

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6
Q

what is the most common congenital cardiac abnormality seen in Edwards syndrome (trisomy 18)

A

ventricular septal defect

holosystolic murmur heard best at the left lower sternal border

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7
Q

what is the congenital cardiac defects seen with DiGeorge syndrome?

A

conotruncal abnormalities

  • tetralogy of fallot
  • truncus arteriosus
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8
Q

what is the most common congenital cardiac abnormality in Down’s syndrome?

A

complete AV septal defect

failure of endocardial cushion development

fixed splitting of S2 and ejection systolic murmur at left 2nd intercostal space (increased pulmonic valve flow due to L-to-R shunt across the ASD)

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9
Q

what is the histopathologic finding of Reye syndrome?

A

microvesicular steatohepatitis

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10
Q

once AXR shows gasless abdomen, what is the best investigation for malrotation?

A

upper GI series (barium swallow)

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11
Q

what is the diagnostic test for intussusception?

A

abdominal ultrasound

S&S = 100% (if performed by experienced sonographer)

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12
Q

what is the difference between B2 (riboflavin) and B3 (niacin) deficiencies?

A

B2 - angular cheilitis, stomatitis, seborrheic dermatitis, normocytic anaemia

B3 - dermatitis, diarrhoea, delerium

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13
Q

what are the maternal contraindications to breastfeeding?

A

infectious diseases:

  • HIV (where formula is easily available)
  • untreated tuberculosis
  • VZV (during the week of delivery)
  • herpes lesions at the nipple

active drug/alcohol abuse

specific maternal medications

active chemo/radiotherapy

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14
Q

what is the frequency that neonates should be breastfeeding?

what is the rule of thumb for number of wet daipers?

A

10-20 mins every 2-3 hours (8-12 times per day)

wet daipers = age (days) up until day 7

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15
Q

what are the sinister complications of beckwith weideman syndrome and what screening is indicated?

A

postpartum hypoglycaemia - BM monitoring

Wilm’s tumour - abdo ultrasound q3 months until 8 y/o

renal ultrasound annually from 8 y/o-adolescence

hepatoblastoma - alpha-fetoprotein q3 months until 4 y/o

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16
Q

what are the most common consequences of untreated Giardia?

A

fat malabsorption and lactose intolerance

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17
Q

what is serum sickness-like reaction?

A

type III hypersensitivity to beta-lactams or septrin, also seen in acute HBV

fever, urticarial rash, arthralgia

hypocomplimentaemia and elevated inflammatory markers

NOT A TRUE ALLERGY, but avoid the offending agent

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18
Q

which tumour:

long-bone diaphysis and axial skeleton

systemic features, pain and inflammation

codman’s triangle

lamellated periosteal reaction, central lytic lesion

A

Ewing sarcoma

early metasases

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19
Q

which tumour:

lytic diaphyseal bone lesion

widespread rash

diabetes insipidus

lymphadenopathy

A

langerhans cell histiocytosis

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20
Q

what is spondylosis?

A

defect or fracture in the pars interarticularis of low lumbar vertebrae (between inferior and superior articular process)

forward slippage of the vertebral body = spondylolisthesis

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21
Q

thickening of the tunica media of the aortic arch means…

A

coarctation of the aorta

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22
Q

what are the reassuring features to look for in physiologic genu varum (bow-leg)?

at what age do you council parents to expect this to correct?

A
  • symmetric
  • normal stature
  • no leg length discrepancy
  • no lateral trust with walking

by 2 years old

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23
Q

what are the physical exam findings in TOGV?

A

single heart sound

cyanosis and dyspnoea

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24
Q

‘apical diastolic rumble’

A

will accompany a holosystolic murmur at the left lower sternal border in large VSD

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25
Q

what are the principle features of Reye syndrome?

A

fulminant hepatitis (microvesicular fatty infiltration)

encephalopathy

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26
Q

what are the risk factors for jejunal atresia?

A

in utero exposure to cocaine or vasoactive substances

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27
Q

what is the only infant contra-indication to breastfeeding?

A

galactosaemia

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28
Q

what must be considered for all meconium ileus?

A

it is virtually diagnostic for cystic fibrosis

however, only 20% of CF babies present with meconium ileus

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29
Q

in foreign body ingestion, what are the indications for endoscopic removal?

A

high-risk for perforation - battery, magnet or sharp item

not progressing distally on serial XR

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30
Q

what is the trypical presentation for Meckel’s diverticulum?

A

infant/toddler with painless PR bleeding, no other abdo pain/diarrhoea/vomiting

+/- anaemia

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31
Q

what is the synovial fluid WBC count diagnostic of septic arthritis?

A

50,000

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32
Q

what are the features of gaucher syndrome?

A

most common lysosomal storage disease; accumulation of glucocerebroside in resident macrophages

  • HSM
  • anaemia, thrombocytopenia
  • bone pain
  • failure to thrive
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33
Q

atlantoaxial instability is seen in which inherited childhood condition?

A

Down’s syndrome

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34
Q

what is a typical course of antibiotics for neonatal sepsis?

A

ampicillin and gentamicin

covers: group B Strep; E. Coli; listeria; s. aureus

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35
Q

what are the findings of congenital toxoplasmosis?

A

chorioretinitis, hydrocephalus, ring-enhancing lesions on CT

Rx - pyrimethamine and sulfadiazine

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36
Q

what are the features of congenital syphillus?

A

CN 8 palsy, rash on palms and soles, sniffles, frontal bossing, saddle nose

Rx - penicillin

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37
Q

what are the features of congenital rubella?

A

cateracts, HSM, deafness, PDA, blueberry muffin rash, thrombocytopenia, hyperbilirubinaemia

Rx - supportive

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38
Q

what are the features of congenital CMV?

A

chorioretinitis, hearing loss, periventricular calcifications with microcephaly, petichiae

Rx - ganciclovir if end-organ damage

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39
Q

what are the features of congenital HSV (split by weeks)?

A

week 1 - DIC and shock

week 2 - vesicular rash

week 3 - encephalitis

Rx - acyclovir

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40
Q

what is the most most common bug to colonise CF childrens’ lungs? at what age does pseudomonas become more prevalent?

A

S aureus

around age 20

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41
Q

how do you treat lyme disease in children?

A

do not use doxycycline - slows bone growth in kids <8 years old

amoxicillin/cefuroxime

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42
Q

which bug causes acute rheumatic fever?

A

strep pyogenes (group A strep)

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43
Q

what bugs cause bacterial rhinosinusitis in children?

A
  • haemophillus influenzae*
  • strep pneumo*
  • moraxella catarrhalis*
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44
Q

what is the treatment for enterobius/pin worm?

A

albendazole or pyrantel pamoate

treat patient and all household contacts

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45
Q

what is the respiratory complication of drowning?

A

inhaled water washes out the surfactant and leads to ARDS that can occur at any time up to 72 hours following submersion

patient should be admitted to hospital and observed for this time

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46
Q

what are the risk factors for developing neonatal RDS?

A

prematurity

male sex, maternal diabetes, perinatal asphyxia, c-section without labour

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47
Q

retinal haemorrhage in an infant …

A

shaken baby - NAI

non-contrast CT head, skeletal survey and referral to child protective services

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48
Q

what is the most common supratentoral tumour in children?

A

low-grade pilocytic astrocytoma

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49
Q

what is a neonatal scalp swelling that is firm, does not cross the suture lines, non-tender and without skin discolouration?

A

cephalohaematoma

this is a subperiosteal haemorrhage

bleeding in the periosteum is slow and so will not be evident until a few hours after birth. these do not require active management as they will resolve in weeks to months. The bruise will increase the changes of jaundice as the haematoma breaks down.

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50
Q

what is a neonatal scalp swelling present immediately at the time of birth that crosses the suture lines?

A

caput succedaneum

typically at the vertex of the skull or whatever the presenting part was

occurs secondary to instrumental delivery

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51
Q

what are the tumours associated with NF1?

A

neurofibroma - nerve sheath tumours

optic pathway glioma - regular eye examination for screening and MRI brain for any reported visual disturbance

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52
Q

FMR1 hypermethylation refers to …

A

fragile X syndrome

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53
Q

accumulation of hypoxanthine and uric acid

early hypotonia followed by hypertonia

dystonia/chorea/spasticity

male sex

A

Lesch-Nyhan syndrome

  • self-mutilation is the give-away
  • leads to gouty arthritis and obstructive nephropathy in untreated disease
54
Q

port wine stain in the trigemminal nerve distributionl, visual disturbance and seizures indicate…

A

Sturge-Weber syndrome

GNAQ mutation

diagnosis with MR brain

55
Q

at what age can you no-longer use US brain as an imaging technique?

A

>6 months

56
Q

what are the gross motor developmental milestones?

A
  • 12/12 stands well, throws a ball, first steps independently
  • 18/12 runs, kicks a ball
  • 2 years walks up and down stairs with 2 feet on each step, jumps
  • 3 years stairs like adult, rides tricycle
  • 4 years balances and hops on 1 foot
  • 5 years skips and catches a ball with 2 hands
57
Q

what are the language developmental milestones?

A
  1. first words other than mama/dada
  2. 10-25 words, identifies body parts
  3. 2-word phrases, >50 words
  4. 3-word sentances, 75% intelligible speech
  5. identifies colours, 100% intelligible
  6. counts to 10
58
Q

what are the social developmental milestones?

A
  • separation anxiety, follows 1-step commands with gestures
  • understands ‘mine’, pretend play
  • 2-step commands, parallel play, begins toilet training
  • knows age/gender, imaginitive play
  • co-operative play
  • has friends, completes toilet training
59
Q

what are the fine motor developmental milestones?

A
  • pincer grasp
  • 2-4 block towers, removes clothing
  • 6 block tower, copies a line
  • uses utensils, copies a circle
  • copies a cross
  • copies a triangle, shoe laces, bathes and dresses independently, prints letters of alphabet
60
Q

what is the typical duration of Todd’s paralysis?

A

36 hours

61
Q

microcephaly, wide anterior fontanelle, phalange dysplasia, cleft lip/palate

what was the teratogen?

A

foetal hydrantoin syndrome

62
Q

pes cavus

hypertrophic cardiomyopathy

kyphscoliosis

gait instability

A

freidrich’s ataxia

trinucleotid repeat sequence in frataxin gene

63
Q

what does a cephalocaudal-spreading flaccid paralysis indicate?

A

botulism

64
Q

what is the inheritance pattern of myotonic dystrophy?

A

autosomal dominant CTG trinucleotide repeat sequence expansion

65
Q

what are the risk factors for neonatal intraventricular haemorrhage?

A

prematurity

very low birth weight

66
Q

developmental regression at 6-18 months

breathing difficulty

movement disorder

female sex

A

Rett syndrome

67
Q

intelectual disability

happy disposition

jerky movements, hypermotor behaviour, hand flapping

A

Angelman sydnrome

68
Q

several seizure types of child age 3-5 years

intelectual disabillity

slow, generalised spike pattern on EEG

A

Lennox-Gestaut syndrome

69
Q

what is the enzyme deficiency in galactosaemia?

A

galactose-1-phosphate uridyl transferase

70
Q

what are the key differences between Neimann-Pick and Tay-Sachs disease?

what are the enzyme deficiencies associated with these?

A

Neimann-Pick: HSM and hypotonia, sphingomyelinase

Tay-Sachs: hyperreflexia, beta-hexosaminidase A

71
Q

fair skin, eyes and hair

ectopia lentis

childhood CVA

joint hypermobility and skin laxity

A

homocystinuria

treat with vitamin B6, B12 and folate; anticoagulation/antiplatelets

72
Q

what is the treatment of hereditary angioedema?

what is the vasoactive substance that builds up in this condition that leads to oedema?

A

C1 inhibitor concentrate

bradykinin

73
Q

what are the Plts and coag screen results in fibrinogen dysfunction?

A

fibrinogen is the final step in the coagulation cascade

PT prolonged

aPTT prolonged

platelets normal

74
Q

a haemophilia A (factor VIII), infusion-dependant patient develops a bleed that does not resolve with increasing administration of recombinant factor VIII… what is the diagnosis?

A

inhibitor development

immune recognition of the exogenous factor VIII and inactivating antibodies formation

75
Q

what is the prevention and treatment of neonatal conjunctivitis?

A

neisseria - topical erythromycin and silver nitrate, Rx IM cefotaxime

chlamydia - maternal screening, PO azithromycin

76
Q

what is the only pathogen for infective diarrhoea that requires treatment with antibiotics?

A

Shigella

azithromycin, ceftriaxone or ciprofloxacin are all effective

77
Q

what are the consequences of SGA (<10% percentile) in neonatal period?

A

hypoxia, polycythaemia, hypoglycaemia, hypothermia, hypocalcaemia

78
Q
A
79
Q

what are the contraindications to the rotavirus vaccine?

A

intussusception

anaphylaxis to the ingedients

meckel’s diverticulum (untreated)

SCID

80
Q

management of haemodynamically stable abnormal uterine bleeding in adolescents?

A

high-dose oral oestrogen or IV equine oestrogen

81
Q

what are the indications for surgical management of recurrent acute otitis media?

what is the medical management of acute otitis media?

A

>3 episode in 6 months/ >4 episodes in 12 months appropriately treated with oral antibiotics

1st line: amox

2nd line: co-amox

82
Q

what is the antibiotic treatment for acute epiglotitis?

A

ceftriaxone and vancomycin

83
Q

suprasellar calcified mass on CT of a child

A

craniopharyngioma

84
Q

what are the levels of B cells and immunoglobulins with:

  • bruton’s agammagolbulinaemia
  • Hyper-IgM syndrome
  • IgA deficiency
  • CVID
A
  • BXA - low B cells, all Ig low
  • hyper-IgM - normal B cells, low IgG/IgA, high IgM
  • IgA - normal B cells, low IgA
  • CVID - normal B cells, all Ig low
85
Q

what are contraindications of DTaP vaccination?

A

anaphylaxis, encephalopathy or uncontrolled, complicated seizures following prior exposure

a febrile seizure alone is not a contraindication

86
Q

what are the complications of HSP?

A

mostly GI

intussusception, GI perforation/bleed

87
Q

what is the presentation of laryngomalacia?

A

stridor in an otherwise healthy infant that is exacerbated on lying supine and relieved by lying prone

88
Q

what do you expect for change in birth weight and height after the first year of life?

A

weight - triples

height - increase by 50%

89
Q

what are the cut-offs for phototherapy and exchange transfusion in neonatal jaundice?

A

phototherapy - 20

ET - 25

90
Q

what are the sorts of infection you would see with complement deficiency?

A

recurrent meningitis

91
Q

what is the appropriate prophylaxis for malaria?

A

mefloquine or primaquine

92
Q
A
93
Q

craniofacial abnormalities

triphalangeal thumbs

red cell aplasia, macrocytosis

what is the diagnosis? what are these patients at risk for?

A

Diamond-Blackfan syndrome

increased risk of malignany

94
Q

what are the diagnostic criteria for acute bacterial rhinosinusitis?

A

perisistant symptoms >10 days

severe onset (fever >39.0 + drainage) >3 days

worsening symptoms following initial improvement

95
Q

what is the test for hereditary spherocytosis?

A

eosin-5-maleimide binding

96
Q

what is the management of squint (strabismus)?

A

patch over the healthy eye (occlusion therapy)

cytoplegic eye drops (penalization therapy)

eye glasses to correct any refractive error

97
Q

what is the treatment of UTI in children?

A

third-generation cephalosporins

i.e. cefixime

98
Q

cyanide-nitroprusside test…

A

identifies cystine in the urine

can be a screening test for cystinuria

these patients will have hexagonal crystals in the urine and recurrent kidney stones during childhood

99
Q

limited upward gaze

non-reactive to light, reactive to accomodation

upper eyelid retraction

A

Parinaud syndrome (dorsal midbrain) from pineal gland mass

100
Q

how do you manage neonatal fracutre of the clavicle?

A

reassurance and education on gentle handling

pinning the elbow at 90deg in long sleve top to reduce pain

101
Q

when would you expect both testes to have descended by and at what age should surgical consult for orchiopexy be saught?

A

should have come down by 6 months

refer to surgeon at 4 months

102
Q

what is the underlying defect in Wiskott-Aldrich syndrome?

A

defect in cytoskeleton remodelling of leukocytes and platelets

WAS gene mutation, X-linked recessive

103
Q

what is the medical treatment for Tourette syndrome?

A

1st line - alpha2-blockers (clonidine, guanfacine)

2nd line (more effective) - antidopaminergic agents

  • tetrabenazine (dopamine depleter)
  • antipsychotics
104
Q

what are the considerations for renal biopsy in children with suspected minimal change disease?

A

no need for renal biopsy in children <10 years old

just ensure that it is isolated proteinuria without creatinine rise or haematuria

Bx unlikely to change management and is invasive

105
Q

at what age do you consider intervention for the management of bed wetting?

what are the first lines?

A

older than 5 years

eneuresis alarm and desmopressin

106
Q

what happens to platelets during sickle cell splenic sequestration crisis?

A

thrombocytopenia

platelets get trapped in the spleen as well

107
Q

what are the most common pathogens for otitis externa?

A
  • pseudomonas aeruginosa*
  • staphylococcus aureus*
108
Q

when do you start formal visual acuity testing in children?

A

typically 4 years old, but can be 3 if the child is co-operative

109
Q

what part of the brain does medulloblastoma affect?

A

cerebellar vermis

less likely to affect the cerebellar hemispheres

leads to truncal ataxia and obstructive hydrocephalus

110
Q

autonomous endocrine dysfunction

recurrent fractures

cafe-au-lait spots

A

McCune Albright Syndrome

GNAS activating mutation leading to overproduction of pituitary hormones

111
Q

abdominal mass unilateral

periorbital ecchymoses

jerky eye movements

A

neuroblastoma with ocular metastases

opsoclonus

112
Q

what medications should be avoided in G6PD deficiency?

A

dapsone

nitrofurantoin

primaquine

rasburicase

113
Q

what is the congenital infection that can present with neurologic symptoms in the absence of intracranial calcifications?

what will you find?

A

herpes simplex virus

temporal lobe changes with haemorrhage

114
Q
A
115
Q
A
116
Q

how do you treat otitis externa?

A

topical fluroquinolone to cover for pseudomonas

ciprofloxacin +/- wick for delivery

117
Q

what are the findings with PDA?

A

loud S2

continuous machinery murmur at L 2nd intercostal space

bounding pulses

118
Q

how old until you have to surgically close the PDA?

A

6-8 months

119
Q

which are the congenital heart diseases that are PDA-dependent?

A

hypoplastic L heart

TOGA

Tetralogy of Falot

120
Q
A
121
Q

auscultation findings in TOF?

A

ejection systolic murmur in left upper strenal border (pulmonary stenosis/RVOT obstruction)

single S2 - only the aortic valve will be snapping shut as the pulmonic valve doesn’t open properly

122
Q

is hypoplastic left heart syndrome a cyanotic heart disease?

A

no

there is no LV or pulse; the infant is grey/pale/mottled rather than cyanotic

pulseless

123
Q

describe juvenile myoclonic epilepsy

A

a progression of seizures

  • absence around age 10
  • myoclonic partial seizures around 14
  • generalised tonic-clonic after 15
124
Q

describe lennox-gestault syndrome

A

mutliple recurrent seizures of different types before age 7 with associated mental retardation

125
Q

what is the difference between anterior and posterior vesicular lesions on the buccal mucosa?

A

antieror - likely herpes

posterior - likely herpangia caused by coxsackie A virus

herpangia is a benign condition treated with supportive care that will resolve typically within a week

126
Q

recurrent respiratory tract infections are associated with which congenital heart defect?

A

patent ductus arteriosus

127
Q

what is the antibiotic treatment of UTI in children under the age of 2?

A

1-2 weeks of 3rd generation cephalosporin

cefTazidime, cefTriaxone, cefoTaxime

128
Q

neuroblastoma

pathogenesis, affected tissues, features, lab findings

A
  • N-myc oncogene
  • adrenal medulla/sympathetic chain
  • features
    • abdominal mass +/- calcifications
    • bone pain
    • periorbital darkening/’bruises’
    • horner syndrome
    • opsoclonus/myoclonus syndrome
  • elevated catecholamine metabolites, small round blue cells on histology
129
Q

brushfield spots

A

white spots in the iris

down’s syndrome

130
Q

IgA nephropathy and PSGN - prognosis?

A

IgA - 40-50% will slowly progress to ESRD, no treatment for the disease
30% resolve spontaneously

PSGN - completely resolve with supportive care

131
Q

which posterior fossa tumours in children are more likely to affect the

vermis

cerebellar hemispheres

A

vermis - medulloblastoma

hemispheres - pilocytic astrocytoma