Pediatrics Flashcards
what is the course/duration of prophylactic antibiotics following rheumatic heart disease?
- 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
what are the cardiac abnormalities associated with Turner’s syndrome?
coarctation of the aorta
bicuspid aortic valve
aortic root dilation -> dissection/rupture
what are the preventive and screening components of routine neonatal care?
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)
what are the indications for newborn echo based on pulse oximetry screening?
<90% in any limb
<95% in both UL and LL
>3% difference in UL and LL
how do you increase the intensity of the HOCM murmur?
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)
what is the most common congenital cardiac abnormality seen in Edwards syndrome (trisomy 18)
ventricular septal defect
holosystolic murmur heard best at the left lower sternal border
what is the congenital cardiac defects seen with DiGeorge syndrome?
conotruncal abnormalities
- tetralogy of fallot
- truncus arteriosus
what is the most common congenital cardiac abnormality in Down’s syndrome?
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)
what is the histopathologic finding of Reye syndrome?
microvesicular steatohepatitis
once AXR shows gasless abdomen, what is the best investigation for malrotation?
upper GI series (barium swallow)
what is the diagnostic test for intussusception?
abdominal ultrasound
S&S = 100% (if performed by experienced sonographer)
what is the difference between B2 (riboflavin) and B3 (niacin) deficiencies?
B2 - angular cheilitis, stomatitis, seborrheic dermatitis, normocytic anaemia
B3 - dermatitis, diarrhoea, delerium
what are the maternal contraindications to breastfeeding?
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
what is the frequency that neonates should be breastfeeding?
what is the rule of thumb for number of wet daipers?
10-20 mins every 2-3 hours (8-12 times per day)
wet daipers = age (days) up until day 7
what are the sinister complications of beckwith weideman syndrome and what screening is indicated?
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
what are the most common consequences of untreated Giardia?
fat malabsorption and lactose intolerance
what is serum sickness-like reaction?
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
which tumour:
long-bone diaphysis and axial skeleton
systemic features, pain and inflammation
codman’s triangle
lamellated periosteal reaction, central lytic lesion
Ewing sarcoma
early metasases
which tumour:
lytic diaphyseal bone lesion
widespread rash
diabetes insipidus
lymphadenopathy
langerhans cell histiocytosis
what is spondylosis?
defect or fracture in the pars interarticularis of low lumbar vertebrae (between inferior and superior articular process)
forward slippage of the vertebral body = spondylolisthesis
thickening of the tunica media of the aortic arch means…
coarctation of the aorta
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?
- symmetric
- normal stature
- no leg length discrepancy
- no lateral trust with walking
by 2 years old
what are the physical exam findings in TOGV?
single heart sound
cyanosis and dyspnoea
‘apical diastolic rumble’
will accompany a holosystolic murmur at the left lower sternal border in large VSD
what are the principle features of Reye syndrome?
fulminant hepatitis (microvesicular fatty infiltration)
encephalopathy
what are the risk factors for jejunal atresia?
in utero exposure to cocaine or vasoactive substances
what is the only infant contra-indication to breastfeeding?
galactosaemia
what must be considered for all meconium ileus?
it is virtually diagnostic for cystic fibrosis
however, only 20% of CF babies present with meconium ileus
in foreign body ingestion, what are the indications for endoscopic removal?
high-risk for perforation - battery, magnet or sharp item
not progressing distally on serial XR
what is the trypical presentation for Meckel’s diverticulum?
infant/toddler with painless PR bleeding, no other abdo pain/diarrhoea/vomiting
+/- anaemia
what is the synovial fluid WBC count diagnostic of septic arthritis?
50,000
what are the features of gaucher syndrome?
most common lysosomal storage disease; accumulation of glucocerebroside in resident macrophages
- HSM
- anaemia, thrombocytopenia
- bone pain
- failure to thrive
atlantoaxial instability is seen in which inherited childhood condition?
Down’s syndrome
what is a typical course of antibiotics for neonatal sepsis?
ampicillin and gentamicin
covers: group B Strep; E. Coli; listeria; s. aureus
what are the findings of congenital toxoplasmosis?
chorioretinitis, hydrocephalus, ring-enhancing lesions on CT
Rx - pyrimethamine and sulfadiazine
what are the features of congenital syphillus?
CN 8 palsy, rash on palms and soles, sniffles, frontal bossing, saddle nose
Rx - penicillin
what are the features of congenital rubella?
cateracts, HSM, deafness, PDA, blueberry muffin rash, thrombocytopenia, hyperbilirubinaemia
Rx - supportive
what are the features of congenital CMV?
chorioretinitis, hearing loss, periventricular calcifications with microcephaly, petichiae
Rx - ganciclovir if end-organ damage
what are the features of congenital HSV (split by weeks)?
week 1 - DIC and shock
week 2 - vesicular rash
week 3 - encephalitis
Rx - acyclovir
what is the most most common bug to colonise CF childrens’ lungs? at what age does pseudomonas become more prevalent?
S aureus
around age 20
how do you treat lyme disease in children?
do not use doxycycline - slows bone growth in kids <8 years old
amoxicillin/cefuroxime
which bug causes acute rheumatic fever?
strep pyogenes (group A strep)
what bugs cause bacterial rhinosinusitis in children?
- haemophillus influenzae*
- strep pneumo*
- moraxella catarrhalis*
what is the treatment for enterobius/pin worm?
albendazole or pyrantel pamoate
treat patient and all household contacts
what is the respiratory complication of drowning?
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
what are the risk factors for developing neonatal RDS?
prematurity
male sex, maternal diabetes, perinatal asphyxia, c-section without labour
retinal haemorrhage in an infant …
shaken baby - NAI
non-contrast CT head, skeletal survey and referral to child protective services
what is the most common supratentoral tumour in children?
low-grade pilocytic astrocytoma
what is a neonatal scalp swelling that is firm, does not cross the suture lines, non-tender and without skin discolouration?
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.
what is a neonatal scalp swelling present immediately at the time of birth that crosses the suture lines?
caput succedaneum
typically at the vertex of the skull or whatever the presenting part was
occurs secondary to instrumental delivery
what are the tumours associated with NF1?
neurofibroma - nerve sheath tumours
optic pathway glioma - regular eye examination for screening and MRI brain for any reported visual disturbance
FMR1 hypermethylation refers to …
fragile X syndrome
accumulation of hypoxanthine and uric acid
early hypotonia followed by hypertonia
dystonia/chorea/spasticity
male sex
Lesch-Nyhan syndrome
- self-mutilation is the give-away
- leads to gouty arthritis and obstructive nephropathy in untreated disease
port wine stain in the trigemminal nerve distributionl, visual disturbance and seizures indicate…
Sturge-Weber syndrome
GNAQ mutation
diagnosis with MR brain
at what age can you no-longer use US brain as an imaging technique?
>6 months
what are the gross motor developmental milestones?
- 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
what are the language developmental milestones?
- first words other than mama/dada
- 10-25 words, identifies body parts
- 2-word phrases, >50 words
- 3-word sentances, 75% intelligible speech
- identifies colours, 100% intelligible
- counts to 10
what are the social developmental milestones?
- 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
what are the fine motor developmental milestones?
- 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
what is the typical duration of Todd’s paralysis?
36 hours
microcephaly, wide anterior fontanelle, phalange dysplasia, cleft lip/palate
what was the teratogen?
foetal hydrantoin syndrome
pes cavus
hypertrophic cardiomyopathy
kyphscoliosis
gait instability
freidrich’s ataxia
trinucleotid repeat sequence in frataxin gene
what does a cephalocaudal-spreading flaccid paralysis indicate?
botulism
what is the inheritance pattern of myotonic dystrophy?
autosomal dominant CTG trinucleotide repeat sequence expansion
what are the risk factors for neonatal intraventricular haemorrhage?
prematurity
very low birth weight
developmental regression at 6-18 months
breathing difficulty
movement disorder
female sex
Rett syndrome
intelectual disability
happy disposition
jerky movements, hypermotor behaviour, hand flapping
Angelman sydnrome
several seizure types of child age 3-5 years
intelectual disabillity
slow, generalised spike pattern on EEG
Lennox-Gestaut syndrome
what is the enzyme deficiency in galactosaemia?
galactose-1-phosphate uridyl transferase
what are the key differences between Neimann-Pick and Tay-Sachs disease?
what are the enzyme deficiencies associated with these?
Neimann-Pick: HSM and hypotonia, sphingomyelinase
Tay-Sachs: hyperreflexia, beta-hexosaminidase A
fair skin, eyes and hair
ectopia lentis
childhood CVA
joint hypermobility and skin laxity
homocystinuria
treat with vitamin B6, B12 and folate; anticoagulation/antiplatelets
what is the treatment of hereditary angioedema?
what is the vasoactive substance that builds up in this condition that leads to oedema?
C1 inhibitor concentrate
bradykinin
what are the Plts and coag screen results in fibrinogen dysfunction?
fibrinogen is the final step in the coagulation cascade
PT prolonged
aPTT prolonged
platelets normal
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?
inhibitor development
immune recognition of the exogenous factor VIII and inactivating antibodies formation
what is the prevention and treatment of neonatal conjunctivitis?
neisseria - topical erythromycin and silver nitrate, Rx IM cefotaxime
chlamydia - maternal screening, PO azithromycin
what is the only pathogen for infective diarrhoea that requires treatment with antibiotics?
Shigella
azithromycin, ceftriaxone or ciprofloxacin are all effective
what are the consequences of SGA (<10% percentile) in neonatal period?
hypoxia, polycythaemia, hypoglycaemia, hypothermia, hypocalcaemia
what are the contraindications to the rotavirus vaccine?
intussusception
anaphylaxis to the ingedients
meckel’s diverticulum (untreated)
SCID
management of haemodynamically stable abnormal uterine bleeding in adolescents?
high-dose oral oestrogen or IV equine oestrogen
what are the indications for surgical management of recurrent acute otitis media?
what is the medical management of acute otitis media?
>3 episode in 6 months/ >4 episodes in 12 months appropriately treated with oral antibiotics
1st line: amox
2nd line: co-amox
what is the antibiotic treatment for acute epiglotitis?
ceftriaxone and vancomycin
suprasellar calcified mass on CT of a child
craniopharyngioma
what are the levels of B cells and immunoglobulins with:
- bruton’s agammagolbulinaemia
- Hyper-IgM syndrome
- IgA deficiency
- CVID
- 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
what are contraindications of DTaP vaccination?
anaphylaxis, encephalopathy or uncontrolled, complicated seizures following prior exposure
a febrile seizure alone is not a contraindication
what are the complications of HSP?
mostly GI
intussusception, GI perforation/bleed
what is the presentation of laryngomalacia?
stridor in an otherwise healthy infant that is exacerbated on lying supine and relieved by lying prone
what do you expect for change in birth weight and height after the first year of life?
weight - triples
height - increase by 50%
what are the cut-offs for phototherapy and exchange transfusion in neonatal jaundice?
phototherapy - 20
ET - 25
what are the sorts of infection you would see with complement deficiency?
recurrent meningitis
what is the appropriate prophylaxis for malaria?
mefloquine or primaquine
craniofacial abnormalities
triphalangeal thumbs
red cell aplasia, macrocytosis
what is the diagnosis? what are these patients at risk for?
Diamond-Blackfan syndrome
increased risk of malignany
what are the diagnostic criteria for acute bacterial rhinosinusitis?
perisistant symptoms >10 days
severe onset (fever >39.0 + drainage) >3 days
worsening symptoms following initial improvement
what is the test for hereditary spherocytosis?
eosin-5-maleimide binding
what is the management of squint (strabismus)?
patch over the healthy eye (occlusion therapy)
cytoplegic eye drops (penalization therapy)
eye glasses to correct any refractive error
what is the treatment of UTI in children?
third-generation cephalosporins
i.e. cefixime
cyanide-nitroprusside test…
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
limited upward gaze
non-reactive to light, reactive to accomodation
upper eyelid retraction
Parinaud syndrome (dorsal midbrain) from pineal gland mass
how do you manage neonatal fracutre of the clavicle?
reassurance and education on gentle handling
pinning the elbow at 90deg in long sleve top to reduce pain
when would you expect both testes to have descended by and at what age should surgical consult for orchiopexy be saught?
should have come down by 6 months
refer to surgeon at 4 months
what is the underlying defect in Wiskott-Aldrich syndrome?
defect in cytoskeleton remodelling of leukocytes and platelets
WAS gene mutation, X-linked recessive
what is the medical treatment for Tourette syndrome?
1st line - alpha2-blockers (clonidine, guanfacine)
2nd line (more effective) - antidopaminergic agents
- tetrabenazine (dopamine depleter)
- antipsychotics
what are the considerations for renal biopsy in children with suspected minimal change disease?
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
at what age do you consider intervention for the management of bed wetting?
what are the first lines?
older than 5 years
eneuresis alarm and desmopressin
what happens to platelets during sickle cell splenic sequestration crisis?
thrombocytopenia
platelets get trapped in the spleen as well
what are the most common pathogens for otitis externa?
- pseudomonas aeruginosa*
- staphylococcus aureus*
when do you start formal visual acuity testing in children?
typically 4 years old, but can be 3 if the child is co-operative
what part of the brain does medulloblastoma affect?
cerebellar vermis
less likely to affect the cerebellar hemispheres
leads to truncal ataxia and obstructive hydrocephalus
autonomous endocrine dysfunction
recurrent fractures
cafe-au-lait spots
McCune Albright Syndrome
GNAS activating mutation leading to overproduction of pituitary hormones
abdominal mass unilateral
periorbital ecchymoses
jerky eye movements
neuroblastoma with ocular metastases
opsoclonus
what medications should be avoided in G6PD deficiency?
dapsone
nitrofurantoin
primaquine
rasburicase
what is the congenital infection that can present with neurologic symptoms in the absence of intracranial calcifications?
what will you find?
herpes simplex virus
temporal lobe changes with haemorrhage
how do you treat otitis externa?
topical fluroquinolone to cover for pseudomonas
ciprofloxacin +/- wick for delivery
what are the findings with PDA?
loud S2
continuous machinery murmur at L 2nd intercostal space
bounding pulses
how old until you have to surgically close the PDA?
6-8 months
which are the congenital heart diseases that are PDA-dependent?
hypoplastic L heart
TOGA
Tetralogy of Falot
auscultation findings in TOF?
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
is hypoplastic left heart syndrome a cyanotic heart disease?
no
there is no LV or pulse; the infant is grey/pale/mottled rather than cyanotic
pulseless
describe juvenile myoclonic epilepsy
a progression of seizures
- absence around age 10
- myoclonic partial seizures around 14
- generalised tonic-clonic after 15
describe lennox-gestault syndrome
mutliple recurrent seizures of different types before age 7 with associated mental retardation
what is the difference between anterior and posterior vesicular lesions on the buccal mucosa?
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
recurrent respiratory tract infections are associated with which congenital heart defect?
patent ductus arteriosus
what is the antibiotic treatment of UTI in children under the age of 2?
1-2 weeks of 3rd generation cephalosporin
cefTazidime, cefTriaxone, cefoTaxime
neuroblastoma
pathogenesis, affected tissues, features, lab findings
- 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
brushfield spots
white spots in the iris
down’s syndrome
IgA nephropathy and PSGN - prognosis?
IgA - 40-50% will slowly progress to ESRD, no treatment for the disease
30% resolve spontaneously
PSGN - completely resolve with supportive care
which posterior fossa tumours in children are more likely to affect the
vermis
cerebellar hemispheres
vermis - medulloblastoma
hemispheres - pilocytic astrocytoma
