Pediatrics incorrects Flashcards
1
Q
I add incorrects/notes on google drive
A
2
Q
in a newborn with sensorineural hearing loss and systemic findings eg hepatospleenomegaly and growth restriction, what should you suspect?
A
a congenital torch infection.
other causes of sensorineural hearing loss wont have these findings.
learn findings in torch
3
Q
findings of HOCM in infants of diabetic mothers?
why does this occur?
management
A
asymptomatic or respiratory distress, hypotension + xray findings
increased glycogen and fat deposition in myocardial cells
beta blockers-> propanolol
*eventual spontaneous regression by age 1 due to normalization of insulin levels
4
Q
microcephaly and micrograthnia are both seen in what genetic condition
cutis aplasia and micropthalmia is seen in what condition?
A
edwards syndrome - T18
Pataus syndrome - T13
5
Q
Findings in rocky mountain spotted fever?
management?
A
athralgia, headache etc
maculopapular rash -> petechial rash. can include palms and soles
abdominal pain may occur
labs-> thrombocytopenia, elevates aminotransferases
history of living in grassy or wooded area
doxycycline
6
Q
in an infant with a new onset siezure, no fever, normal bloods, imaging
but microcephaly and eczema
what is next best step in diagnosis?
A
serum amino acid analysis -> to find hyperphenalanimea and diagnose phenylketonuria
7
Q
severe coughing in children particularly in setting of respiratory infection can cause subcutaneous emphesema and spontaneous pneumomediastinum. management/next investigation?
A
chest x ray
chest pain, sob =. signs
8
Q
how to detect corneal abrasion
how to detect lens dislocation
distinguishing optic nerve injury from vitreous hemorrhage?
A
+ve fluorescein test
iris tremulousness
both have RAPD. only optic nerve injury has an abnormal red reflex. optic nerve injuries can occur after head trauma
9
Q
a child with mucosal neuromas and tall with thin arms, is at greatest risk of what condition?
what condition does the child have?
A
medullary thyroid carcinoma!!!
tall long arms = marfanoid body habitus. ]
child has Men 2B
note! Men 2A has MTC but not these other features.
MEN1 = primary hypaparathyroidism, no MTC
10
Q
otitis externa treatment?
A
topical fluoroquinolone -> topical ciprofloxacin!! +/- a glucocorticoid.
(ear drops)
11
Q
a neonate with hypothermia, lethargy, leukopenia, and elevated neutrophil bands is concerning of what?
bulging fontanelle and apnoea also make you concerned of?
A
neonatal sepsis
meningitis
*note GBS is most common cause of neonatal sepsis and neonatal meningitis
12
Q
siezures in the setting of acute gastroeneteritis (fever, abdominal pain, bloody diarrhea) should raise suspicion of which particular pathogen?
A
shigella -> a complication
13
Q
name 2 posterior fossa tumours in children that can present with cerebellar dysfunction and increased ICP
A
medulloblastoma
pilocytic astrocytoma
14
Q
differentiate between irittant contact dermatitis in diapers, candida diaper dermatitis, and perianal streptococcus
A
irritant contant -> erythmatous papules/plaques sparing skinfolds. treat with petrolatum/zinc oxide topicals
candida -> beefy red involving skin folds topical nystatin
strep-> sharp red dermacated involving perianal area. oral! beta lactam
15
Q
define macrosomia.
what condition in a fetus predisposes them to macrosomia and thus shoulder dystocia?
A
> /= 4kg newborn
fetal hyperglycemia (as a result of maternal diabetes) -> increased fetal insulin -> macrosomia
15
Q
3 year old from refugee camp, scaling and fissures at corners of mouth (stomatitis), beefy red tongue(glossitis), swollen mucous mumbranes (stomatitis), normocytic anemia and erythematous scaly patches (seborrheic dermatitis) has what deficiency?
A
riboflavin, B2
15
Q
36
A
15
Q
metaphyseal corner fractures are a red flag for __ and should prompt what?
A
child abuse
skeletal survey
16
Q
the knee to chest position for an infant having a tet spell in TOF works by which mechanism?
A
increases systemic vascular resistance
much more than pulmonary vascular resistance
thus, right to left shunting and deoxygenated blood entering aorta is reduced.
17
Q
recurrent pneumonias(sinopulmonary infections) , GI infectons eg with giardia and small tonsils point to what condition?
*immunology coming back up
A
x-linked agammaglobulinemia
GI infections due to lack of IgA
low b cells = absent lymphoid tissue ie tonsils, adenoids
*note, SCID is associated with failure to thrive and therefore unlikely in an infant with normal growth
18
Q
congenital long QT syndrome symptoms?
management
A
syncope
sudden cardiac death
autosomal recessive form -> sensorineural hearing loss
beta blockers
19
Q
failure to thrive and recurrent respiratory symptoms, plus multiple bulky stools a day should raise suspicion of?
A
CF
bulky greasy stools = pancreatic insufficiency
20
Q
neonatal sepsis treatment?
A
ampicillin + gentamicin
21
Q
in a deteriorating child where peripheral venous cannulation has been impossible, next step is?
A
attempt intraosseous cannulation - rapid and requires less skill than central venous access
22
in patients unable to protect airway (somnolent) or hypoxic despite supplemental oxygen, next step in management?
endotracheal intubation with mechanical ventilation
noninvasive positive pressure ventilation cant be used in patient that are somnolent as it does not protect against aspiration
23
in a patient with cafe au lait macules, lisch nodules, what eye condition is often asociated?
optic pathway gliomas
condition = NF1
24
treatment for infantile hemangiomas that are periorbital (increased risk of vision loss) or large/segmental (risk of scarring or ulceration) ?
oral propanolol
25
a child with cyanosis, rhinorrhea, coughing spells and postussive emesis (eg coughing while feeding and apnea ) most likely has?
whooping cough
- risk of siezures also
26
a child with chronic headaches, poor linear growth and pubertal delay raises suspicion of a tumour with what mri findings?
craniopharyngioma
suprasellar mass with calcifications
pituirary stalk compression -> panhypopituitarism, growth failure
27
a pariteal lesion with air fluid levels and hyperdense rim on mri + fever, headache is most likely?
brain abscess
28
tuberous sclerosis mri findings?
subependymal nodules in lateral ventricles
29
what is the biggest risk factor for orbital cellulitis?
bacterial sinusitis
- infections of teeth specifically close to maxillary sinus is also a risk factor but not greatest
30
in a patient with low leukocytes, low platelets, low hemoglobin
bleeding gums, fatigue
short stature, hyper/hypopigmentation patches and absent or hypoplastic thumbs
has what condition?
what is the cause of this condition?
Fanconi anemia - most common congenital cause of aplastic anemia
DNA repair defect
31
acute iron poisoning signs
treatment?
how to narrow down choices with lab results?
abdominal pain, diarrhea, hematemesis -> hypovolemic shock may deveolp
xray may show opacities = iron pills specifically show this
defiroxasime chelation therapy
- calculate anion gap -> this + ph = anion gap metabolic acidosis
- lead posinoing causes altered mental status/siezures and hematemesis is not seen
- aspirin you expect tinnitus
32
in nephrotic syndrome due to amylodosis, name an important investigaton
echocardiography - risk of restrictive cardiomyopathy
33
a boy presenting after vomiting and diarrhea (gastroenteritis) with hip pain and decreased range of motion, ultrasound shows small intracapsular fluid collection (small hip effusion)
what is diagnosis and treatment?
what are some other risk factors for this condition?
lab test findings?
transient synovitis
ibuprofen
post infective, posttraumatic eg gymnastic class, idiopathic
WBC, ESR and CRP may be normal or mildly elevated
*joint effusions may be bilateral vs septic arthritis which is always Unilateral. In addition, is septic arthritis, outright refusal to weightbear vs limited in transient synovitis
34
in a patient with localised bony tendernessm erythema and warmth to proximal tibia
in an otherwise well child
what is most likely condition and organism?
osteomyelitis
staph aureus
*osteomyeltis typically occurs in metaphyses of long bones eg tibia, femur
35
biliary cyst symptoms
management?
asymptomatic OR RUQ pain, mass, jaundice
in neonates - jaundice, acholic stools, dark urine
RESECTION! Due to increased risk of malignancy -> cholangiocarcinoma, pancreatic cancer, gallbladder cancer
36
examination findings in a baby with rickets?
x ray findings?
biggest risk factor in infants?
craniotabes - i.eskull bones that are soft and flexible/depress to pressure
radius/ulnar bowing
x ray: metaphyeal widening, metaphsyeal cupping and fraying
exclusive breastfeeding with no vitamin D supplementation!
37
*in conmmon variable immune deficiency, differentiation from b cells into plasma cells is impaired. so b cell count is normal, but all igs are low
*SCID is caused by Tcell impairent and then subsequent b cell dsysfunction, both T and b cells low
*CD40 ligand deficiency = x linked hyper IGM. defective class switching so all Igs low except IgM which is high. normal b cell count x
job syndrome/hyper IgE -> everything normal except elevated igE
X linked agammaglobunemia -> defect in tyrosine kinase -> bone marrow cant make B cells for circulation. low b cells and low all Igs
38
hereditary hemorrhagic telangiectasia causes what neurological condition in children?
symptoms?
CT findings?
stroke -> due to AVM
headache, vomiting, focal neurological deficit, altered mental status
hyperdense intraparenchymal fluid collection
hemorrhagic stroke is always hyperattenuated on CT vs ischemic
39
a neonate that presents with respiratory distress, hypoglycemia, jiteriness, normal chest xray, mother with preclampsia, raised hematocrit, ruddy/plethoric appearance.
what is most likely condition?
name other risk factors
management
neonatal polycythemia. polycythemia = hematocrit >65 % in term neonates
intrauterine hypoxia (maternal diabetes, maternal HTN, smoking). erythrocyte transfusion (delayed cord-clamping, twin to twin transusion), hypo/hyperthyroidism, genetic trisomy conditions
iv fluids, glucose, partial exchange transfusion
*note in TTN - CXR findings include increased pulmonary vascular markings and fluid in the fissures.
40
state etiologies of cerebral palsy and imaging findings
prematurity/LBW = periventricular leukomalacia, IVH
hypoxic ischemic injury = watershed injury, basal ganglia/thalamic lesions
perinatal stroke = vascular distribution
intrauterine infecitons = calcifications
41
how does acute bacterial sinusitis present?
nasal discharge and cough diagnosed as:
- lasting greater than10 days
- OR with severe fever >/= 39 for >/= 3 days
- OR worsening symptoms following initial improvement
viral sinusitis usually resolves by 7-10 days
non typeable hib = most common
strep pneumonia
moxarella catarrhalis
amoxicillin =/ clavulanate
42
10 year old boy, continuous left knee pain. worse at night. no trauma, swelling of leg
x ray = central lytic lesion of distal femur with cortical layering (onion skinning) and 'Moth eaten" appearance and extension into soft tissue
increased leukocyte and increased ESR!
most likely diagnosis?
ewing sarcoma = second most common MALIGNANT pediatric bony tumour after osteosarcoma
other systemic signs like fever and weight loss may be present
*local pain and swelling
* xray features
*white adolescent boys
*common in pelvis and long bones
43
distinguish between the mechanism and clinical features of lactation failure jaundice and breast milk jaundice
lactation failure jaundice:
- insufficient mil intake = delayed stooling -> decreased bilirubin elimination= high unconjugated/indirect bilirubin
- suboptimal breastfeeding (less than every 3 hours)
- signs of dehydration - wet diapers should equal or exceed neonates age in days eg >/=4 on day 4, weight loss
encourage frequent breastfeeding
if this is not sufficient formula feeds and phototherapy may be considered
exchange transfusion is reserved for patients with bilirubin >/= 25 or bilirubin induced neurological dysfunction = change in tone, lethargy or rise in bilirubin despite phototherapy
breast milk jaundice
- beta glucoronidase in brast milk deconjugates intestinal bilirubin and increases its circulation.
- adequate breastfeeding and well hydrated
44
In ABO blood group incompatibility, what blood group would mother have?
group O.
anti -a or anti- B
antibodies to child blood group A or B
45
1st step in management of primary amenorrhea in a girl?
pelvic ultrasonography - look for abnormalities of ovaries, uterus etc
-> MRI brain is only indicated in patients with uterus and LOW or inapppropriately elevated FSH
46
when would a urodynamic study be appropriate for a child with eneuresis?
what about imipramine
urodynamic study if bladder dysfunction suspected = daytime incontinence, weak stream, urgency, straining
imipramine = no underlying conditions eg OSA (if osa = polysomnography). and 1st line treatments eg desmopressin and alarm have failed
47
all patients with tracheesophageal fistula and oesophageal atresia should undergo what screening?
echocardiography and renal ultrasonography!
association with VACTERL
- Vertebral
- anal
- cardiac
- tracheoEsophageal fistula
- renal
- limb abnormalities
48
How does an osteoid osteoma present?
x ray findings?
- pain, worse at night
- femur most common
- improves with NSAID use!! - unlike ewings sarcoma in which pain is also worse w activity!
- round, small lucency on x ray!
49
how does an osteosarcoma present?
moth eaten lytic lesions
sunburst pattern!!
chronic localised pain and soft tissue mass
50
a patient with hyperglycemia and anion gap metabolic acidosis with a recent infection most likely has what?
what is most likely decreased in body?
DKA
*if given electrolytes, always calculate anion gap!
total body potassium -> high glucose levels causes osmotic diuresis -> net renal loss of K+ -> however serum K+ may be normal as insulin causes extracellular shift
*in DKA after osmotic diuresis, blood urea nitrogen and creatinine may be acutely elevated due to hypovolemia
*DKA is characterised by increase in circulating fatty acids due to lipolysis
51
gyaecomastia/breast lump in a boy with abnormal features eg rapidly progressing, outside of puberty with an increased beta-hcg. no palpable mass on testes examination
next best step in evaluation?
testicular ultrasound!
high hcg points to a twsticular germ cell tumour (may not be picked up on palpation) (eg seminoma etc)
hcg suppresses testosterone and increases aromatase activity = more estrogen
52
PSGN treatment?
loop diuretics eg furosemide for volume overload
53
most common cause of heavy menstrual bleeding in adolescence is ?
VWD -> impaired platelet adhesion
54
heart failure in Kawasaki disease is due to what aetiology?
lymphocytic myocarditis -> LV dysfunction
daiphoresis during breastfeeding, s3 gallop, pulmonary edema, hepatomegaly
55
minimal change disease pathogenesis?
cytokine induced glomerular injury!
56
differentiate between Ehlers Dahnlos and Marfans syndrome
Ehlers Danhlos
- defective collagen production
- scoliosis, joint laxity, aortic dilation
Marfans syndrome
- defective fibrillin 1 gene
- scoliosis/kyphosis, joint laxity, aortic root dilation (aortic regurg, aneurysm & dissection risk!!)
- unlike Ehlers, also has tall long then extremities, long fingers, upward lens dislocation!!
MarFans. F= fibrillin
57
recurrent episodes of edema in a child, eg face, limbs, genitals. with no other symptoms eg urticaria, pruritus, is what condition?
pathology?
Hereditary angioedema
C1 inhibitor deficiency
58
risk factors for lead poisioning?
if capillary blood test for lead is positive, next step in management?
living in home built before 1978
pica/mouthing behaviours
chipping paint or dust released during renovation
1. measure venous lead level for confirmation and remove from lead containing environment = best next step!!
2. Chelation therapy if lead level >/= 45 -> dimercaptosuccinic acid (succimer) or if at least 70, dimercaprol (British-lewisite) + calcium disodium edetate (EDTA)
AXR only indicated if GI symptoms eg vomiting, constipation, abdominal pain, or suspicion of ingestion
at a later point you will screen for IDA if hemoglobin is low
59
what are the similaritis between marfans syndrome and homocystinuria? how would you differentiate
homocystinuria management?
both have marfanoid body habitus eg skin elasticity, tall etc
homocystinuria only = intelectual disability, thrombosis (stroke in child/MI), downwards lens dislocation, fair complexion, magaloblastic anemia.
management = B6 supplementation + antiplatelets/anticoagulation
marfans only = aortic root dilation, upwards lens dislocation
60
Tuberclous meningitis CSF findings?
onset
high lymphocytes, high protein and low glucose
very gradual onset w weeks/ months of non specific symptoms first in contrast to strep pneumonia <7 days onset + neutrophil predominance
61
in neonatal respiratory distress syndrome, what mechanism drives the hypoxia?
surfactant deficiency -> alveolar/lung collapse -> increased right to left intrapulmonary shunting
62
in paeds, immunology, biochem and repro eg CAH come up
63
most common cause of congenital adrenal hyperplasia?
presentation?
diagnosis?
21 hydroxylase deficiency. it inhibits conversion of progesterone to 11 deoxycorticosterone
aldosterone deficiency
= dehydration -> sunken fontanelle, dry mucous membranes
= salt loss = hyponatremia, low bp, high k+
virilization in females only
increased 17-hydroxyprogesterone = diagnostic
64
in a patient whose urethral meatus extends to the corona of the ventral aspect of the penis (hypospadia), what is the next step in management of patent if circumcision is requested by father?
urology evaluation prior to circumsicion as foreskin may be edded forrepair and traditional circumcision methods may not be safe
renal ultraosund only if other congenital abnormalities eg aniridia indicative of WAGR
Karyotype if sever hypospadia and undescended testes also present -> may be a sexual development disorder
65
boy with tilting his head to right followed by arm twitching, then chewing of lips and picking at shirt while staring ahead. episode lasted 2-3 minutes and patient was confused after
most likely diagnosis?
focal siezure
motor movements = head tilting,, arm twitching
impairment of awareness may occur (eg staring). this is associated with automatisms eg chewing, picking
66
differentiate between
absence siezure
juvenile myoclonic epilepsy
lennonx-gastaut syndrome
absence siezure = staring spell with or without automatisms. last 10-20 seconds. not asosciated with post-ictal period
juvenile myoclonic epilepsy = in adolescents. jerks immediately on awakening. absence and generalised tonic clonic siezures may also be seen
lennox-gastaut = intellectual disability and several siezures of varying type. slow spike and wave pattern on EEG
67
conjuctivitis, mucosal changes, rash, cervical lymphadenopathy are seen in a certain condition. what other finding is there in distal extremeties in this condition?
edema
68
in a 7 day old infant that presents with ecoli sepsis, vomiting after breastfeeding, jaundice and elevated transaminases, normocytic anemia. what is the most likely diagnosis?
galactosemia -> inability to metabolize galactose to glucose
*excessive galactose can impair leukocyte function and superoxide relase -> increased succeptibility to ecoli
*galactose-1 phosphate acucmulates in RBC, promoting their death -> unconjucated hyperbilirubinemia
69
boy has truamatic eye injury. was prescribed cyclopentolate. patient then develops hallucinations, tachycardia, dilated pupils. management?
physostigmine
patient has anticholinergic toxicity
70
microcephaly (HC<3RD percentile) in a normal child with no developmental delay and normal exam. management?
measure parents heads
benign familial microcephaly
71
where is the location of murmur in VSD?
what signs seen in infants>
left lower sternal base!! - think ventricles at bottom
increased work of breathing, tahcycardia, diaphoresis, pallor, particularly during times of exertion eg feeding
vsds lead to left to right shunting, and as a result, increased pulmonary blood flow and volume overload on left side of heart. signs of high output LV failure described above. signs of right sided HF also seen sometimes eg hepatomegaly.
72
distinguish between non typhoidal salmonella and salmonella typhoid including risk factors for each. which one does vaccine protect against?
non typhoidal = diarrhea(may be bloody in children), abdominal pain, fever, vomitng. bacterial gastroenteritis. inadequate cooking or refrigeration of chickens/egg. diagnosis =stool culture. self limiting
typhoidal = FEVER+ bacteremia, rose spots. hepatosplenomegaly may be seen. diagnosis = blood culture. antibiotics
vaccine is for typhoid. no vaccine for non-typhoidal salmonella
73
in a patient with HSP how is this diagnosed?
clinical -= no additional testing
if incomplete presentation =skin biopsy
74
12 year old with recent diarrheal infection, cant walk anymore, pins and needles sensation in feet and weakness in past few days. absent ankle and patellar reflexes . upper extremeties weak and hyporeflexic. sensation intact.
what structure is primarily impaired in patients condition
peripheral nerve fibres -> GBS
75
newborn baby when initially breastfed began to appear blue around the lips and face but turned pink when removed from the breast and began crying . no murmurs on auscultation. what is the most likely diagnosis?
how is this confirmed
Choanal atresia - failure of posterior nasal passage to cannalize completely
diagnosis:
inability to pass catheter past nasopharynx
CT scan/nasal endoscopy
if unilateral rather than bilateral - presents with chronic nasal discharge
76
how should biphasic anaphylaxis be treated?
with an additional dose of IM adrenaline
if 3 doses fail to resolve symptoms -> IV adrenaline
if hypotenson -> iv crystaloid and trendelenburg positioning
bronchospasm -> give albuterol
77
severe cases of croup (stridor at rest) is treated with what?
Nebulised epinephrine!!! (in addition to dexamethasone used for mild cases)
78
at what age should infants be able to babble and turn to their name?
9 months
if not = audiology evaluation
79
high myopia increases the risk of?
retinal detachment and macular degneration
80
boy presenting with fever, pruritic rash and joint pain and palpable lymph nodes after given penicillin for step throat 9 days ago, what is the most likely cause
Serum sickness like reaction (SSLR) due to penicillin. can also be caused by sulfa drugs.
note* same symptoms as serum sickness (SS) which has a different cause
81
3 year old. 3 episodes of vomiting today and 5 watery stools today (acute onset). Fever of 38. no blood in stool.
what is the most likely diagnosis? management?
viral gastroenteritis
discharge with ORS
additional evaluation only needed if bacterial or parasitic pathogen suspected (bloody diarrhea, diarrhea for more than week, high fevers, particularly >40). *note in a campylobacter infection with mucoid diarrhea abut no blood and no features described above, symptomatic care ie rehydration is sufficient
IV rehydration only neded if signs such as weak or rapid pulse, increased cap refill, reduced skin tugor, decreased urine output
82
what long term complications are expected from a greenstick fracture eg of forearm?
none
limb length discrepancy -> seen with fractures involving growth plate
limited range of supination and pronation -> seen in malunion in severely displaced fractures
83
bronchiolitis is seen in what age group?
symptoms?
CXR findings?
<2 years
cough, crackles, wheezing
CXR: hyperinflation, peribronchial cuffing(bronchial wall haziness due to inflammation), increased interstitial markings
usually clinical diagnosis but x ray may be done to exclude other causes
84
distinguish selective mutism from stranger anxiety
selective mutism = refusal to speak in certain social situations eg home, vs an environment where they feel more comfortable eg friends/family
stranger anxiety -> anxiety and distress encountering unfamiliar people even in presence of parent
85
an 18 month old girl develops IDA due to excessive consumption of cows milk. following a blood transfusion the girl develops:
respiratory distress, HTN, tachycardia and pulmonary edema (bilateral crackles), S3 gallop
managment?
in a toddler (2 years). systolic blood pressure should be expected to be around 105
thus 130/60 will be hypertension
furosemide
S3 gallop indicates HF (JVP distension may also be present). respiratory distress probably due to pulmonary edema.
- anaphylaxis would cause hypotension
- resp support only is used to treat TRALI = resp distress caused by noncardiogenic pulmonary edema. HYPOTENSION is seen in TRALI. signs of circulatory overload not seen (S3 gallop, diffuse crackles)
86
in a patient with acute pharyngitis with no viral symptoms (cough, rihnorrhea, conjuctivitis, oral ulcers), and a negative rapid streptococcal antigen test. what is the next step in management?
Throat culture -> properly rule out step throat due to risk of acute rheumatic fever.
note if viral symptoms were present, you wont even need rapid antigen test, it would be a clinicial diagnosis
87
cephalohematoma presentation?
firm well demarcated scalp swelling that doesnt cross suture lines -> forceps delivery is risk factor. risk of hyperbilirubinemia
caput succadenum -> boggy, poorly demarcated edema that crosses suture lines
88
subgleal hemorrhage presentation?
fluctuant scalp swelling that expands with time!! and crosses suture lines. can cause life threatening blood loss! overlying skin may be bruised
89
acute mastoiditis is a complication of what?
how does it present?
treatment?
distingusish this from parotitis
otitis media
tenderness and swelling behind ear (mastoid area), displacement of the auricle
tympanic membrane may show evidence of erythema/bulging/perforation or may not be visible
IV antibiotics and drainage
parotitis has both post and pre auricular swelling and typically occurs in the elderly
90
recap blood transfusion reactions
91
in a patient with dark urine, with a urine dipstick that says 3+ blood but red blood cells of only 1-2 what could be the cause?
next investigation?
red blood cells <3 therefore not hematuria. could be myoglobinuria or hemaglobinuria.
in a patient with mechanical heart valve & absence of muscle aches, hemaglobinuria more likely => full blood count, check for evidence of hemolytic anemia due to heart valve
92
SCID treatment?
CVID Treatement?
in which conditions are antiviral and antifungals indicated?
stem cell transplant
intravenous immunoglobulins (also give this in x-linked agammaglobulinemia). note in CVID only the B cell count is normal, i e, variable to Igs which are low.
conditions in which cellular immunity is impaired eg SCID, chronic granulomatous disease
93
baby with meningitis signs. CSF shows elevated wcc and protein. Glucose is NORMAL. baby missed a vaccine at 1 year old. most likely cause
Viral meningitis
probably mumps virus due to missing MMR vaccine
94
3 month old baby with episodes of reflux. normal weight gain, no pain or back arching, what is the management?
what is pathologic reflux? managment?
- reassurance that it is physiolgical and should resolve by 12-18 months.
- upright positioning after feeds, burbing, frequent small volume feeds.
- cholecalciferol/vitamin d supplementation! to all breastfed infants!!
- irritability, poor feeding, weight loss!
- if bottle fed -> thickening feeds, extensive hydrolysed formula
- breastfed -> mother to eliminate cows milk and soy protein from diet
95
newborn presents with hypoxia that is responsive to oxygen supplementation (indicates a pulmonary pathology. if not responsive = cardiac in which you would administer prostaglandin). cardiac examination shows a 1/6 ejection systolic murmur. mother had gestational diabetes.
CXR shows ground glass opacities. next step in management after putting on CPAP?
surfactant administration!!
patient has RDS
*Maternal diabetes is a risk factor
*murmur here = innocent
96
persistent pulmonary hyppertension of newborn symptoms?
management?
differential cyanosis = saturation in right hand> foot
nitric oxide (pulmonary vasodilator)
97
management of patient with a history of acute rhuematic fever?
symptoms of the condition?
penicillin at time of disgnosis
and then penicillin prophylaxis to prevent recurrent infections and progression of heart disease
migratory arthritis, carditis, chorea and erythema marginatum may be present, elevated acute phase proteins
rash here is non purpuric -> contrast HSP
systemic juvenile arthritis in contrast to rhuematic fever, the rash is not migratory! and cant be diagnosed until arthritis is present for >6 weeks
98
toxic metabolic encephalopathy & cerebral edema has different aetiologies. in the case of reyes syndrome, what is it due to?
liver failure -> hyperammonia
not uremia! uremia is acondition that can cause encephalopathy but not cerebral edema
99
elevated PT or PTT or both and being able to identify a cause eg a specific factor deficiency
factor 7 deficiency presents with a prolonged PT!! and all other lab findings are normal!! patient has prolonged bleeding episodes
100
vomiting predominant acute gastroenteritis is caused by what organisms?
management?
b cereus -> rice
s aureus -> raw or prepared foods eg dairy, eggs, meat, potato salad
clinical diagnosis, fluid repletion and reassurance
101
what are some triggers for breath holding spell?
Pallid bhs -> pain or fear from a minor trauma
cyanotic bhs -> crying
pallor with brief LOC
102
in a patient who had a drowning episode rushed to hospital and is seen to have high blood pressure and bradycardia, what is the most likely cause?
increased ICP -> cushings triad = htn, bradycardia, irregular respirations
103
FEVER (myalgias, weight loss) + MURMUR (new or changing) + SPLEENOMEGALY in a child with a history of congenital heart disease
lab evidence of glomerulonephritis + inflammation (raised wcc, anemia of chronic diease)
what do you suspect? next step in management?
infective endocarditis -> echo
103
pancytopenia in the setting of NORMAL cell morphology for all cells points towards?
aplastic anemia
104
pinworms first line treatment?
pyrantel pamoate!!!! or albendazole
105
how does reactive attachment disorder present?
how is disinhibited social engagement disorder different?
childhood abuse/neglect -> emotional and social withdrawal and a lack of response to comfort
lack of boundaries eg sitting in strangers laps
106
manaegement of patient with voiding cystourethogrpahy showing retrogade urine flow and renal ultrasound showing hydronephrois?
prophylactic antibiotic therapy -> child has vesicoureteral reflux
107
in a patient with infections, failure to thrive
lymphopenia
low CD19 and CD3
low immunoglobulins
management?
stem cell transplant.
patient has SCID as T cells and B cells are both low
108
how does respiratory distress syndrome present?
why might a murmur be present in newborn?
what are the cxr findings in RDS,TTN and PPH
cyanosis immediately after birth, as well as grunting flaring and retractions which is not seen in cardiac causes
murmur could represent a physiologically open DA, which only closes from 12 hours after birth
RDS = ground glass opacities, air bronchograms
TNN = bilateral, perihilar streaking, fluid within interlobular fissures
PPH = clear lungs with decreased pulmonary vascularity
109
newborn cyanosis that occurs with feeding but improves with crying evaluation?
pass a catheter through the nares
choanal atresia
110
in a child, cough, sudden onset respiratory distress, unilateral hyperinflation on xray and mediastinal shift with decreased breath sounds and expiratory wheezing is concerning for?
management?
foreign body aspiration
rigid bronchoscopy
*differentiate form pneumothorax = lung collapse not hyperinflation and reduced lung markings
111
18 month old with meningism signs and a petechial rash, what is the most likely cause
Neisseria meningitiidis
<1month usually listeria
<3 months usually GBS
>1 year, strep pneumonia is most common cause but Neisseria meningitidis is also cause (was answer in question stem)
112
the presence of gamma tetramers on hemoglobin analysis is consistent with what condition? what specific rbc morphology is seen and why?
why is erythrocyte count raised?
alpha thalessemia
target cells due to loss of rbc volume as lack of hemoglobin
abnormal rbcs -> hemolysis -> increaed reticulocytes
113
lab values in a patient with turners syndrome and thus primary amenorrhea?
low estrogen
low testosterone -> ovaries cant make it
high FSH and LH due to lack of negative feedback from eostrogen
turners causes premature ovarian insufficiency NOT pcos
114
*rhD positive mothers do not produce antibodies
115
child that used to struggle to eat, now stealing other childrens food , sees a speech therapist as speaks with 1-2 words, short height, poor muscle tone, surgery done years ago for cryptorchidism
prader willi syndrome - classic triad of hypotonia, hyperphagia, obesity
genetic test -> loss of expression on paternal copy of chromosome 15
116
late onset GBS can present with meningitis, bacteremia and a focal infection like cellulitis!
117
causes and risk factors for acute unilateral cervical lymphadenitis
staph and strep = most common -> pus formation occurs
anerobic bacteria eg prevotella -> poor dental hygeine/ periodontal disease
francisella tularenesis = history of contact with infected animal eg rabbi t
mycobacterium avium = chronic, non tender, violaceous
bartonella henslae = chronic, at site of cat scratch
118
5 year old girl with down syndrome presenting with gait changes (ataxic gait), vertebrobasilar symptoms eg imbalance/dizziness (eg tripping whilst walking), urinary/fecal incontinence and UMN signs such as hypereflexia and positive babinski most likely has what?
atlanto-axial instability -> symptoms due to compression of the spinal cord
119
night blindness, peripheral vision loss
retinal vessel attenuation (narrowing) and pale optic disc are all features of?
Retinitis pigmentosa
*pigment deposition = late finding and might not be seen
120
* in hydrocephalus, developmental delay can occur due to axonal damage in brain due to raised ICP
121
8 year old with chorea, emotional lability, obsessive compulsive disorders, milk maid grip what is this condition? what is the pathology?
syndehma chorea
molecular mimicry!!!! = (step antibodies cross reacting with basal ganglia )
this is NOT basal ganglia atrophy because this occurs in huntingtons disease, a disease with adult onset, characterised by chorea + dementia +pyschiatric features
122
given a picture of a beefy red diaper rash that involves the skins folds. treatment?
topical antifungal - patient has candida dermatitis
(petrolatum and zinc oxide would be used irritant contact dermatitis)
(amoxicillin is used in perianal group A strep disease)
123
patient with history of tetralogy of fallot which was repaired now presents with a decrescendo Diastolic! murmur at the left sternal border in the 3rd intercostal space at 30 years old. most likely cause?
pulmonic regurgitation = common complication of TOF repair, repair of pulmonary stenosis
*tricuspid regurg is a systolic NOT diastolic murmur
124
which of the following is most likely associated with tubero sclerosis?
1. cardiac rhabdomyoma
2. optic nerve glioma
3. phaeochromocytoma
4. vestibular shwannoma
5. wilms tumor (nephroblastoma)
cardiac rhabdomyoma! -> all patients suspected must have an echo!
renal association = angiomyolipoma
125
when there is a personal or family history of drug abuse, or parent prefers a drug without abuse potential, what do yo prescibe for ADHD?
Atomoxetine!
avoid stimulants (methylphenidate, amphetamines) which are normally 1st line
126
first step in evaluating a patient with speech problems even without a history of ear infections or abnormality on otoscopy is?
hearing test
127
baby teething, nasal congestion, fevers
despite mother giving acetaminophen and topical anesthetic she had to babys teeth, baby still fussy and presents with cyanoiss, 85% sats which are refractory to O2 supplementation
what is the most likely cause? most likely laboratory finding on ABG sampling?
treatment?
methemoglobinemia. exposed to topical/local anaesthetic!!! (also seen with other oxidising agents eg dapsone, nitrites)
normal PaO2!!, saturaiton gap between ABG and pulse oximetry
this is because ABG analyses only unbound arterial oxygen
methylene blue. "give meth to meth"
another example = baby with methemoglobinemia after circumcision and lidocaine application
cyanosis respiratory depression (seizures and death may also occur)
128
treatment for a newborn when mother has chickenpox? (varicella exposure)
Varicella immunoglobulins!. give passive vaccination as active vaccination with Varicella vaccine is contraindicated in children <1
same goes for pregnant and immunocompromised peoples!
in contrast, immunocompetent = vaccine within 5 days of exposure!!!. After 5 days, in a healthy person with no symptoms, vaccine no longer recommended as PEP but still recommended to prevent future infection
acyclovir is only used to treat neonates that develop signs of disease!
129
newborn girl, not moving legs, and not responding to heel prick test (ie no sensation) DTR +1 in legs, hemangioma over midline lumbosacral region
what is the most likely diagnosis?
spinal dysraphism!! AKA spina bifida
specifically a closed one = spina bifida occulta!!
common dermatological findings = dimple, hair tuft, hemangioma, lipoma, nevus
*some also present with tethered cord syndrome = LMN signs seen! as in patient = weakness, loss of sensation, hyporeflexia. abnormal gait, urinary dribbling and back pain may also occur
answer would not be spinal cord infarction as that would occur in adults and have UMN signs
130
maneuvers that decrease LV volume, worsen LVOT obstruction in HCOM and increases the intensity of the murmur. name two such maneuvres
Valsalva and standing from supine
131
Cholesteatoma presentation?
Otoscopy findings?
New onset hearing loss despite antibiotics or chronic ear drainage besides antibiotics
Otoscopy - granulation and skin debris, retraction pockets of the tympanic membrane may also be seen
132
Patient with a history of sickle cell
Groin pain/ thigh pain weight bearing which has now progressed to at rest
Pain on hip abduction and internal rotation
Normal wcc and crp
History of viral illness
Most likely diagnosis?
Osteonecrosis/ avascular necrosis of femoral head
Patients complain of groin thigh or buttock pain
Can also occur at humeral head
133
How to differentiate central precocious puberty from peripheral PP?
Next step in evaluation of central PP?
Management?
Central = high LH, advanced bone age
MRI of brain to rule out tumour
GnRH agonist if no tumor identified to prevent premature fusion of growth plates
134
A patient with pneumonia which has not improved and now has a localised pleural effusion has what?
Medication needs to be changed from amoxicillin to?
Empyema
Ceftriaxone and vancomycin!! to target the most common organisms = strep pneuma and staph including MRSA
135
In a 3 day old boy, with Petechia, prolonged PT and slightly prolonged PTT. Normal platelet count. Diagnosis?
- vitamin K deficiency - presents in newborns in first 2-7 days of life - in newborns that did not receive intramuscular Vit k - easy bruising, mucosal bleeding, GI bleeding, intracranial hemorrhage. PTT prolonged if severe
- in conditions with impaired platelet function, PT and PTT normal, platelet count normal
-in VWD, factor 8 and VW factor deficient. Prolonged PTT only
136
Describe the two ways in which a disseminated gonorrheal infection may present?
Purulent monoarthritis
Migrating Athralgia, pustular rash, tenosynovitis
137
JIA presentation?
Arthritis for at least 6 weeks
Fever for at least 2 weeks
Hepatospleenomegaly and lymphadenopathy common
Pink rash common
Quotidian fevers = spiking once daily
138
Management for atopic dermatitis complicated by impetigo?
Topical mupirocin!!
Or oral antibiotics
139
Short stature, normal growth velocity (eg following the 2nd percentile line on growth chart), delayed puberty and delayed bone age are all features of?
Constitutional growth delay - adult height typical normal
Contrast this to growth hormone deficiency where linear growth velocity is slowed so declining growth percentiles crossing two major percentiles eg 50 and 25
140
Premature neonate with acute neurological symptoms (apnea, hypotonia, lethargy ie decreased movements, seizures) and rapidly enlarging head circumference, temp 37.1, Apgar scores 8 and 9, most likely has?
Next step in diagnosis?
IVH
Hemorrhage can also cause bulging fontanelle, bradycardia
Cranial ultrasound
Apgar scores normal so would point away from HIE
A normal temp = 36.1 to 37.2!!! - points way from meningitis
Inborn errors of metabolism cause seizures but not expanding head circumference
141
Neonate with refractory jaundice and spleenomegaly. Anemia and elevated MCHC. Mothers blood group O+ and baby A+. Direct Coombs test is negative. What is the most likely diagnosis?
Defect of red blood cell membrane - hereditary spherocytosis
Direct Coombs test would be positive in ABO incompatibility/ antibody mediated hemolysis!!
142
Playfully appearing 10 month old girl, fever 38. No Coryzal symptoms. maculopapular rash starting on face and then spreading to extremities
Small tender lymph nodes - suboccipital, posterior auricular, posterior cervical. Patchy erythema of small palate. Most likely diagnosis?
Rubella!!! = low grade fever, rash spreading from head, lymphadenopathy, forchheimer spots (erythematous papules on soft palate)
Measles in contrast which spreads the same way but coalesces and darkens after a few days and patients are ill appearing with high fevers up to 40!!! And coryzal symptoms and cough
In scarlet fever - rash begins Along skin folds (axillae, groin) and spreads to extreme toes
Erythema infectiously = no lymoahadenopathy or palatial lesions. Rash is lacy
143
respiratory distress syndrome pathology?
surfactant deficiency -> increased alveolar surface tension
144
newborn at 3 weeks of age presenting with erythematous papules and pustules limited to the face and scalp has?
management?
neonatal cephalic pustolosis
daily cleansing with soap and water
145
Signs of pathological causes of constipation
Poor weight gain or linear growth
Sacral abnormalities eg hair tuft
Delayed passage of meconium
Blood mixed IN stool
Narrow ribbon stools
146
Differentiate between droplet precautions and airborne precautions
Airborne = small particles that stay in air for long time. Negative pressure rooms, respiratory masks - = TB, varicella, sars, measles
Droplet = larger particles, not suspended for long time in air. Surgical masks = Neisse Ria meningitidis, Hib, mycoplasma pneumonia, influenza, adenovirus
Contact = C. difficile, ecoli, RSv , scabies
147
A premature infant requiring continuous oxygen supplementation for 28 or more days after delivery has what condition? Classic x ray finding?
Bronchopulmonary dysplasia
Diffuse hazy infiltrates on CXR, low or normal lung volumes
In severe cases =
148
Suspected fibroadenoma management?
Observation and repeat examination at 6 weeks ie after completion of full menstrual cycle. If mass has enlarged then ultrasound evaluation required
149
Bow leg is physiological up until what age?
Age 2
If legs are bowed and no other signs and symptoms, rickets unlikely
150
A girl with short stature, ie greater than or equal to 2 standard deviations below the mean or less than or equal to the 2nd percentile. She also has short metacarpals a high arched palate and malocclusion of teeth and strabismus. What is the most likely diagnosis? Management of height?
Turners syndrome
Recombinant growth hormone
151
Thymus silhouette In patients under age 3 is normal. How does it appear in cxr? When would you be worried
Appears as sail sign
Worried if opacities in this location in adults - could be lymphoma or germ cell Tumour
152
Vision is tested at every well child visit. In addition, at what age is formal vision testing recommended?
4 years
153
In children under 4, avoid grapes, raw vegetables eg carrots, peanuts, hot dogs, hard candy, basically foods that have the potential to choke
154
Transition to forward facing car seat should not be done before age 2 ie 24 months
155
Distinguish ADHD from conduct disorder and ODD
In adhd a patient may grab the doctors stethoscope so hyperactive
However CD involves extreme misconduct ie harming others or animals!!, property destruction so fire setting and vandalization, lying, stealing, skipping school
ODD = willful disobedience and spite vs disobedience due to distraction in ADHd
156
Child with uti, and persistent fevers despite treatment management?
Ultrasound of bladder and kidneys!
Broaden antibiotics. Avoid ct due to uneccessary radiation exposure
157
Name 5 infectious complications of eczema
1. Impetigo = staph or strep. Honey crusted lesions. Papules
2. Eczema herpeticum = HSV = vesicles, crusting, lymphadenopathy
3. Molluscum contagiosum
4. Tinea Corporis =
158
OCD 1st line?
SSRI = fluoxetine
Or CBT
159
When you can’t tell if it is hereditary spherocytosis or G6PD deficiency, a family history of spleenectomies makes which more likely?
Hereditary spherocytosis- which is treated w folic acid, transfusions and splenectomy for severe anemia
G6PD is treated by avoidance of triggers
159
Most common condition causing IVH?
Prematurity
160
Septic arthritis in child management
Vancomycin - strep aureus main cause
If unresolving = ceftriaxone for gram negative cover
161
Distinguish between primary dysmenorrhea and endometriosis
Primary dysmenorrhea = pain a few days before start of menstruation and resolves a few days after start. Normal physical examination
Endometriosis = pain few days prior AND throughout period. Uterosacral ligament tenderness, cul de sac nodularity and endometrioma on examination
162
What tumour of the head and neck is seen in tuberous sclerosis?
Subepyndymal giant cell Tumour!! Cardiac rhabdomyoma and renal angiomyolipoma also seen
NF1= optic nerve glioma
Neurofibromatosis 2 = vestibular shwannoma
163
18 month old with loss of speech , wanting to be soon fed rather than feed herself (loss of purposeful, hand movements) , stereotypical, movements (eg pulling at hair) and lurching gait (gait abnormality), siezures is concerning for? Diagnosis via?
Rett Syndrome
DNA analysis
164
swinging child by arm -> crying and refusal to use arm -> arm held extended downward with forearm pronated and avoiding using it
several bruises at various stages of healing on anterior lower legs bilaterally
what does patient have?
managament?
what d
Nursemaids elbow/radial head subluxation
hyperpronate forearm or supinate+flexion!!!
bruise locations = common for kids
165
17 year old boy, ankle pain ,
then petechial rash on legs and top of feet
then dull ache in scrotum
anemia
sexually active with 1 partner
what is the most likely diagnosis?
IgA vasculitis (HSP)
triad =
1. petechie on LOWER extremeties
2. athralgia
3. abdominal pain (or occult GI bleed which can result in anemia or intussecption)
scrotal pain and swelling common in boys!!! (localised vasculitis)
scrotal edema not seen in syphilis, and chlamydia doesnt have petechial rash
166
small VSD vs large VSD presentation? managment?
small = asymptomatic except holosytolic murmur at left lower sternal edge.
spontaneous closure
large = resp distress, poor feeding and growth. CXR. = cardiomegaly, increased pulmonary markings
diuretics + surgery
167
risk factors for curve progression in adolsecent idiopathic scoliosis?
female sex
age <12
premenarchal status!!
skeletal immaturity
initial severe curvature (cobbs angle > 25)
168
normal serum complement levels in IGaA nephropathy helps distinguish from PSGN with reduced serum complement
169
4 month old boy with occasional episodes of bilious vomiting, refusal to feed
history of omphalocele repaired
normal bowel sounds and rectal exam shows normal stools
most likely diagnosis?
investigation?
management
intermittent volvulus (partial obstruction)
upper GI series
Ladds procedure
170
toddler with a unilateral, foul smelling, purulent nasal discharge (may be bloody)
most likely diagnosis?
nasal foreign body
Acute bacterial sinusitis = bilateral, fever, cough!!
171
distinguish between the findings in transient synovitis and septic arthritis which both cause hip pain and limp
if a child presents with features concerning for septic arthritis, next step in management?
transient synovitis = well appearing, afebrile or low grade fever, able to weight bear/ ambulate, normal or mildly elevated wbc, ESR, CRP
septic athritis = Ill appearing, febrile, not able to weight bear, moderately elevated labs
bilateral hip ultrasound!!! to distinguish cause
unilateral effusion -> arthrocentesis to eveluate for septic arthritis
bilateral effusions -> can occur in transient synovitis
172
patients with ITP are only treated if what?
management?
bleeding!!! (or if platelets less than 30,000 in adults)
glucocorticoids, anti-D, IVIG
173
microcephaly, wide anterior fontanelle, cleft palate, hypoplasia of distal phalanges in newborn mostlikely due to use of what in mother?
phentoyin!! (fetal hyndantoin syndrome) = exposure to epileptic eg carbamezapine, valproate also
- also cause neural tube defects, and cardiac defects
- lowest epileptic dose for mothers + folic acid
- syphilis by contrast presents with rhinitis and maculopapular rash
174
the most common causes o microcytic anemia in children are iron deficiency anemia and thalessemias. which one has a normal RDW and which one an increased RDW?
normal RDW = thalessemias!!!
increased RDW = iron deficiency
*note alpha and beta thalessemia mino/trait are both often asymptomatic but routine blood tests will pick up anemia
175
How to distinguish pyromania from conduct disorder
conduct disorder - fire setting done to cause damage not relieve tension or out of fascination. also must be a history of aggression to others and animals, theft.
pyromania = eg playing with a lighter when hes stressed (done to relive tension). always wanted to be a firefighter (fascination with fire)
