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1
Q

Infective endocarditis criteria

A

Duke (2 major/1M + 2minor/5 minor)
M 1. ECHO vegetations 2. Blood culture (3x)

minor
F fever >38
I immunologic - Osler (fingers), Roth, GN, RF
V vascular - Janeway lesions, splinter hem, conjunctivitis, emboli
E vidence x2 - ECHO, Blood culture not meeting M criteria
R risk factors - IVDU, heart condition/CHD

Empiric antibiotics
-vanco/gentimycin - 4 - 6 weeks total
Micro: Strep viridans, HACEK, staph aureus, enterococcus

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2
Q

Rheumatic fever criteria

A

Jones (2 major, 1 major 2 minor)
+ GAS infection (ASOT, Swab)

S - subcutaneous nodules
P - pancarditis
A - arthritis
C - chorea
E - erythema migrans

F - fever
L - lab ESR, CRP
A - arthralgia
P - PR prolongation

Rx: Pen G IM or PO amoxicillin x 10 days
prophylaxis x 5 years or until 19

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3
Q

Innocent murmurs

A

pulmonary flow murmur newborn - < 5 mo (ULSB chest, axillae)
still’s murmur - 5yo LLSB, vibratory
venous hum - 5 years (jugular) - infraclavicular
pulmonary ejection murmur - 10 years old (ULSB)
carotid bruit - any age (supraclavicular over carotid)

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4
Q

TGA presentation & Rx

A 
may have absent murmurs
ECG - mild RVH or normal
Egg shaped heart (narrow mediastinum)
Normal to increased pul vascular flow
Rx prostaglandin
\+/- atrial septostomy
Arterial switch
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5
Q

Cyanotic Congenital heart disease

CXR findings

A

Dec PBF
TOF - boot shaped
TA - rounded heart/enlarged (may have inc PBF)
EA - wall-wall cardiomegaly

Inc PBF
TGA - narrow mediastinum, egg on string
TAPVR - supracardiac (non obs) = snowman (cardiomegaly)
- infraccardiac = obstructed - N heart
HLHS
- cardiomegaly with inc PBF
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6
Q

TOF presentation and Rx

A

minimal resp distress
SEM ULSB due to RVOTO
no hepatomegaly

boot shaped heart
dry lungs

RVH/RAD

Rx: surgery around 4 - 6 months
complications: Pulm insufficiency, arrhythmias

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7
Q

Long QT genetics

General Rx

A

Jarvell Lange Neison - AR, SNHL, syncope
Romano-ward syndrome - AD, FmHx, syncope
Timothy - webbed fingers/toes

Rx:
beta blockers
no competitive sports. Avoid swim
avoid long QT meds
\+/- ICD, pacemaker
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8
Q

Genetic syndromes predispose leukemia

A 
Fanoni
Shwachman Diamond
SCID
T21
NF1
ataxia telangiectasia
Li Fraumeni
Diamond Blackfan
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9
Q

Sudden risk of death (3)

A

Previous sx of exertional chest discomfort, dizziness or prolonged dyspnea with exercise, syncope or palpitations.

Fam Hx of prolonged QT, Marfans, sudden death, arrhythmias, cardiomyopathy.

Previous recognition of heart murmur or elevated BP.

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10
Q

ASD

A

wide, fixed split s2
grade 2/3 SEM LUSB

dilated RA, RV, PA

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11
Q

Pulsus Paradoxus

A

on inspiration, SBP drops > 10mmHg

ddx
pericarditis
tamponade
asthma
PE
emphysema
hypovolemia
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12
Q 
heart lesions with:
22q11
T21
T13 
T18
Marfan
WIlliams
Noonan
Turner
Alagille
Ehler-Danlos
Holt-Oram
VATER
A 
22q11 - TOF, IAA, TA
T21 - AVSD** >  VSD
T13 - VSD, ASD, PDA, dextrocardia
T18 - VSD, ASD, PDA, PS
Marfan Dilated AO> MVP
William - Supravalular AS/, PA
Noonan - PS, HOCM
Turner - Coarc, AS, bivalve, Ao dissection
Alagille - peipheral PS
Ehler - dil Ao, MVP
Holt-Oram - ASD
VATER  - VSD
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13
Q

Neurocutaneous syndromes

genetic inheritance

A

Tuberous Sclerosis - AD
NF1, NF 2 - AD
Von Hipple Lindau - AD
Herditary Hemorrhagic Telactectasia - AD

Sturge weber - sporadic
PHACES - sporadic
Ataxia Telangectasia - AR
Inconinentia pigmenti - X linked

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14
Q

abnormal S2

A

S2 - closure of AV and Pv
widely split and fixed S2
ASD
PAPVR

Single S2
PHTN
PA, AS
TGA, TOF (P2 not audible)

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15
Q

Coarctation

classical signs CXR

A

3 sign on CXR
Rib notching (collatoral)
(E on barium)

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16
Q

Coarctation

classical signs CXR

A

3 sign on CXR
Rib notching (collateral)
(E on barium)

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17
Q

Cardiac arrest algorithm

Intervention + doses

A

PEA/asystole
- IV epinephrine 1:10,000 IV 0.01mg/kg q3-5min
- defibrillation shock 2J/kg, next 4kg/kg
up to 10J/kg

VF/VT

  • shock ASAP
  • IV epinephrine
  • IV amiodarone 5mg/kg bolus x 2 times

ETT
age/4 + 4 (uncuffed) size
depth = size x 3

H'T's
Hypovolemia
Hypo/hyperkalemia
Hypothermia
hypoglycemia
hypoxia
Hydrogen (acidosis)
Toxins
Trauma
Thrombosis (pulmonary, coronary)
Tamponade
Tension pneumo
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18
Q

Bradycardia PALS

meds

A

IV epinephrine 0.01mg/kg (1:10,000)

IV atropine 0.02mg/kg x 1 max 0.5mg

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19
Q

SVT PALS

A

IV adenosine 0.1mg/kg rapid bolus + 10ml flush
central line

synchronized shock - if unstable, no adenosine
0.5-1J/kg
increase 2J/kg
sedate if possible

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20
Q

SVT PALS

A

IV adenosine 0.1mg/kg rapid bolus + 10ml flush
central line

synchronized shock - if unstable, no adenosine
0.5-1J/kg
increase 2J/kg
sedate if possible

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21
Q

Torsades de points

A

polymorphous VT
- risk hypoMg and long QT

treatment:
stable - IV MgSo4 50mg/kg
- IV lidocaine 1mg/kg
(no amiodarone due to prolong QT)

unstable - defib shock
2J/kg

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22
Q

Torsades de points

A

polymorphous VT
- risk hypoMg and long QT

treatment:
stable - IV MgSo4 50mg/kg
- IV lidocaine 1mg/kg
(no amiodarone due to prolong QT)

unstable - defib shock
2J/kg

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23
Q

Prolong QT meds

A

antibiotics: macrolides, septra
antifungal: fluconazole, itraco, keto
antidep: TCa, haloperidol, risperidone
antiarrhythmic: amiodarone, procainamide, sotaol
oral hypoglycemics: glyburide
organophosphate
promotility: cisapride

Electrolytes: HypoK, hypoCa, HypoMg (ie lasix)

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24
Q

Brugada syndrome

A

rare condition. C/o palpitations, syncope
normal exam. ECHO normal

ECG: RBB, J point elevation, Concave ST elevation best in V1

No drug including BB helpful
NEED ICD to protect

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25
Q

Galactosemia

A 
CHO metabolism disorder
hepatomegaly, jaundice, FTT
e.coli sepsis
neurocognitive
Cataracts

Dx: RBC GALT
other: +conjugated bili, INR, Hemolytic anemia
+ urine reducing substance

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26
Q

Glycogen storage disorder Not Pompe

A

massive hepatosplenomegaly
short stature
bleeding diathesis

hypoglycemia, lactic acidosis,
dec reponse to glucagon

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27
Q

Methylmalonic acidemia

A 
Organic acidopathies
(other = Proprionic acidemia)

HIGH AG metabolic acidosis, ammonia, LE Urine OA

hepatosplenomegaly, seizures, vomiting, FFT
cardiomyopathy
Rx low protein diet, L carnitine

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28
Q

urea cycle defect

A

ie OTC (x-linked)

Respiratory alkalosis
elevated hyperammonia
OTC: inc urine orotic acid

low protein
sodium benzoate (NH savengers)
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29
Q

Calculate GCS. Kid has abnormal flexion of arms and legs to pain. Eyes open to pain. Incomprehensible sounds.

A

GCS = 7
E4M6V5

E=spont/voice/pain/none
M=spont/touch/withdrawal pain/flex to pain/ext to pain/none
V=oriented/confused/inappropriate/
incomprehensible/none

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30
Q

Neurofibromatosis
inheritance
criteria
surveillence

A 
AD inheritance
CAFE SPO(t) 2/7
C - cafe au lait x6 < 5mm young, >/=15mm puberty
A - axillary freckling
F - neurofibroma (2) or plexifibroma (1)
E - eye: Lisch nodules/iris hamartoma (2)
S - skeletal - sphenoid dysplasia
P - pedigree (1st degree family)
O - optic glioma

annual ophtho exam, BP
follow neurological exam, skeletal exam, derm findings

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31
Q

Tuberous Sclerosis
inheritance
criteria
surveillence

A

AD inheritance
2/11 (major)

Skin (4) Brain (4), lung, heart, kidney
Skin
A - ash leaf x3
S - shagreen patch
A - facial angiofibromas
P  - periungal fibroma
Brain 
eye - retinal hamartomas
subependymal nodules
subependymal giant cell astrocytoma
cortical tuber

Lung - LAM lymphangioleiomyomatosis
heart - rhabdomyoma
kidney - angiomyolipoma

Rx @ ddx MRI, EEG, ophtho
ECG, ECHO, RUS

MRI head, RUS q1-3years
CT chest, PFT adulthood
mental health monitor
Seizures usually < 1 year old

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32
Q

Sturg Weber syndrome
inheritance
clinical
Rx

A 
sporadic
clinical:
nevus flammeous V1/V2 distribution
glaucoma
CT head - leptomeningeal angiomatosis
seizures
hemiparalysis 
developmental delay
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33
Q

Von Hippel Lindau
inheritance
clinical risks
monitoring

A

cerebellar hemangioblastoma
retinal angioma
Pheochromocytoma
renal cell carcinoma

audiology testing
1 - 4 yo: eye, neuro exam annually
5 - 15: urine amphetamines
8year old on - RUS annual 
MRI head/spine16 yo + q2 years
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34
Q

PHACE syndrome

A 
Posterior fossa
Hemangioma
Arterial -(cerebral vascular abnormalities)
Cardiac - coarc Ao
Eye - glaucoma, cataracts

*risk of stroke with head/neck cerebral arterial malformations. Possible coarct/VSD etc

before initiate propranolol –> OPTHO, ECHO + cardiology, MRI head

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35
Q

Incontinentia pigmenti

A

X linked
skin lesion s- hyperpigmented whorls and swirls with Balshko lines. (resolve)

conical deformities of teeth
eye: cataracts, strabismus, retinal changes
Seizures, ID, splastic paralysis

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36
Q

Hypomelantosis of Ito

A 
Sporadic disorder
Hypopigmented whorls/streaks follow Blashko’s lines
Seizures, mental retardation
Hemihypertrophy
Cardiac  - TOF, pulmonary stenosis
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37
Q

developmental delay

initial w/u

A 
CBC, LE, RFT, lytes
MRI head
microarray if 2 or more anomalies
karyotype, MCEP2 (Rett), Fragile X
met- low yield unless clinical
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38
Q

Rett criteria

A 
hand wringing
developmental regression - speech
gait abnormalities
loss of hand skills
seizures
microcephaly (acquired)
-not have abnormal psychomotor retardation in < 6mo and no hx of brain injury, metabolic etc.

genet testing MEP2

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39
Q

Febrile seizure
Risk factors for recurrence
Risk factors to go onto epilepsy

A

RF recurrence
short time from fever to seizures
family history febrile seizure
younger age

RF epilepsy
short time from fever to seizures
family history epilepsy
complex seizure
abnormal development
recurrent febrile seizure
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40
Q

C/I to AED

A

absence/myoclonic seizures
- no carbamazepine or phenytoin

IEM or developmental delay unknown
- valproic acid

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41
Q 
S/E 
Valproic acid
phenytoin
carbamazepine
lamotrigene
vigabatrin
ethosuximide
topiramate
A

(all drowsiness and GI upset)
VPA -thrombocytopenia, hepatotoxicity, pancreatitis
phenytoin - gingival hypertrophy, SJS, ataxia/tremor
carbamazapine - rash, liver toxic, agranulocytosis, SJS
lamotrigene - SJS
vigabatrin - retinopathy
ethosuximide - agranulocytosis
topiramate - kidney stones, glaucoma

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42
Q

breath holding spells
age
what look like
treatment

A

6 - 18 months
pallid or cyanotic
precipitated by emotion/surprise/injury

1min, no post ictal
1 ) apnea with cyanosis or
2) limp, pallor, diaphoresis

Reassure
treat Iron deficiency
100% away by age 8

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43
Q

empiric AED for

  1. absence seizures
  2. infantile spasms
  3. focal seizures
  4. general seizures
A
  1. absence - ethosuximide > VPA
  2. infantile - ACTH, vigabatrin
  3. focal - carbamazepine, keppra
  4. general - VPA, keppra, lamotrigene
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44
Q

Migraine headache criteria

A 
5 or more attacks
each last 1- 72hrs
2 characteristics
-severity
-unilateral
-Throbbing
-Aggravated activity/avoidance
1 association
-Nausea/vomiting
-Sensitivities
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45
Q

headache Indications for neuroimaging

A 
neurofocal findings
worst in AM
awakens at night
unusual headaches - occipital, thunderclap
recent head injury
seizures or academic deterioration
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46
Q

migraine with brainstem aura

basilar

A

at least 2 attacks
AURA - visual, auditory, speech reversible, no motor/brainstem
2 characteristics
- dysarthria
-vertigo
-tinnitis
-hypacusis
-diplopia
-ataxia
-decreased LOC
At least 2 of the following 4 characteristics:
1.
At least 1 aura symptom spreads gradually over 5 or more minutes, and/or 2 or more symptoms occur in succession
2.
Each individual aura symptom lasts 5-60 minutes
3.
At least 1 aura symptom is unilateral
4.
The aura is accompanied, or followed within 60 minutes, by headache

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47
Q

REd eye reflex ddx

A

glaucoma
cataracts
high refractive error
retinoblastoma

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48
Q

Eye findings with syndromes:

  1. T21
  2. WAGR
  3. Waarenburg
  4. NF1
  5. Wilsons
  6. Williams
  7. CHARGE
A
  1. Brushfield spots
  2. Anisoria
  3. Heterochromiia
  4. Lisch nodules
  5. Kayser Fleischer
  6. Stellate
    7 Coloboma
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49
Q

Bone tumor

Osteosarcoma vs Ewing

A 
Osteosarcoma
-sunburst,cloud like
metaphysis long bones
-usually solitary lesion
-chemo then surgery (no radiation)

Ewing Sarcoma

  • onion skin, moth like , periosteal reaction
  • diaphysis long bones
  • can be multiple
  • more common < 10 yo
  • chemo, RADIATION
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50
Q

Lupus criteria

A 
4/111 MD SOAP BRAIN
M - malar rash
D - discoid rash
S - serositis
O - oral ulcers
A - ANA
P - photosensitivity
B - blood - pancytopenia
R - renal GN
A - arthritis
I - immunologic ds DNA, anti sm, APL
N - neurologic

other: lab
- normal CRP elev ESR
- low C3, C4
- elevated IgG
- possible RF, Anti Ro, La

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51
Q

Lupus treatment

A

hydroxychloroquine
- standard therapy

+/-corticosteroids if nephritis, hematological crisis, CNS disease
- possible azathioprine, MMF, cyclophosphamide

52
Q

Acute rheumatic fever
criteria
treatment

A 
2M or 1M+2minor
Need GAS evidence
Major
S - subcutaneous nodules
P - pancarditis
A - arthritis
C- chorea
E - erythema marginatum
Minor
F - fever
L - lab ESR, CRP
A - arthralgia
P - PR prolonged
Treatment:
PO amoxicillin x 10days
naproxen/ASA until arthritis resolves
proph IM PenG qmonthly or PO Pen V BID
 x 5 years or until19
prednisone for carditis
PHB for chorea
53
Q

Granulomatosis with polyangitis

A

+ANCA

URT, LRT, renal

54
Q

Kawasaki disease

bloodwork

A 
leukocytosis
thrombocytosis
anemia (N indices)
elevated CRP, ESR
LE, mild hyperbili
hypoalbuminemia
sterile pyuria
55
Q

Rickets

Skeletal findings

A

craniotabes
delayed closure fotntanelles
parietal and frontal bossing
rachitic rosary - enlarged constochondral junction
widening wrist, bowling of distal radiusulna
Harrison sulcus - (muscular pull of diaphragm)

56
Q

MAS

lab - what is low?

A

Low: ESR, Fibrinogen

57
Q

Drug induced lupus

medications

A

minocycline (tetracyclien)
anticonvulsants
hydralazinne

development of SLE like symptoms (fever, arthralgia, arhtritis, serositis) usually no discoid/malar rash
more insiduious onset
continuous drug > 1mo
similar cytopenia, high ESR

58
Q

catalase positive pathogens
list (4)
(who at risk)

A 
staph aureus
aspergillus
nocardia
serratia marcescens
burkholderia 
salmonella

phagocytes –> CGD

59
Q

Anaphylaxis criteria

A
  1. skin AND one of following
    - resp
    - cardio (BP) or end organ (syncope, collapse)
  2. suspect/KNOWN + 2 or more
    - skin
    - resp
    - BP low or end organ
    - GI symptoms
  3. KNOWn and hypotension
60
Q

Bruton’s agammaglobinemia

xlinked

A

No B cells = no lymphatic = no immunoglobulins
Males, usually 6 - 24mo
recurrent Sinopul, OM, GI, meningitis, sepsis
encapsulated - strep, Hflu
enterovirus meningocephalitis

Lx: absent IgG,A, M,E
Rx IVIG for life

61
Q

leukocyte adhesion defect

A

rare
deficiency in adhesion molecules for phagocytosis
delayed separation of umbilical cord (>4weeks)
NO PUS
staphylococcal infection - dental, ginvigitis, inestinal
Lx: neutrophilia (not function), absent surface adhesion molecules
Rx: BMT, antibiotics

62
Q

Ataxia Telangiectasia

A 
AR in ATM gene (DNA repair)
ataxia--> telanectasias
immunodef - IgA low
(sinopul, encapsulated)
risk malignancy (15% lymphoma)

Supportive only
IVIG
BMT not work

63
Q

Ataxia Telangiectasia

A 
AR in ATM gene (DNA repair)
ataxia--> telanectasias
immunodef - IgA low
(sinopul, encapsulated)
risk malignancy (15% lymphoma)

Supportive only
IVIG
BMT not work

64
Q

Low complement (3)

A

SLE
Post-strep GN
membrano-proliferative GN
liver disease

65
Q

specific antibodies for rheumatic diseases
SLE
Systemic sclerosis
JDM

A

SLE

  • dsDNA, sm, Ro, La
  • histone (drug induced as well)

Sclerosis

  • systemic = Scl70
  • Limited (CREST) = centromere

JDM
- Jo1

66
Q

associations
c-ANCA
p-ANCA

A

C-cytoplasmic CR3
Wegners - granulomatosis with polyangitis

P-perinuclear MPO

  • microscopic polyangitis
  • eosinophlic granulomatosis with polyangitis
  • UC, primary sclerosing cholangiits
  • SLE
67
Q

vasculitis

categories and names

A 
large - Takayasu 
Med - KD, polyarteritis nodosa
small 
1. ANCA associated
- microscopic polyangitis
-eosinophilic granulomatosis polyangitis
-granulomatosis polyangitis (wegner)
2. Immune complex - IgA
variable - Bechet
68
Q

Takayasu

A 
angiographic evidence - aorta/branches
AND
one of 5
- claudication
- limb BP differences
- HTN
- bruit
- acute phase reactant
69
Q

Polyarteritis nodosa

A

medium vessel

histological - necrotizing vasculitis
angiography
AND  1/4
Skin - Erythema Nodosa
myopathy
renal involvement
HTN
peripheral neuropathy

rx: steroid rapid wean

70
Q

Kawasaki disease

counselling

A

recurrence risk 2%
no live vaccines 11mo (after IVIG)
influenza vaccines (not live)
athersclerosis risk - active living

71
Q

Live influenza contraindications

A

severe asthma
(on PO, current wheeze, medically treated wheeze < 7 days, high dose ICS)
2-17 year old on ASA (Reye)
immunodeficiency/Pregnancy

72
Q

HSP IgA vasculitis

management & counseling

A 
supportive
NSAIDs arthritis
prednisone - severe GI 
recurrence 1/3
monitor U/A and BP x 1 year (most settle 1 month)
ESRD 1 - 3%
73
Q

anaphylaxis

Treatment

A

IM epi 1:1000 0.01mg/kg (max 0.5) q 5 - 15mins
steroids, ranitidine, desloratidine, ventolin
IV fluids bolus PRN

if > 3x epi pen and still hypotenisive, IV epi infusion (1:10,000) 0.1 - 10 mcg/kg/min

glucagon(if BB)
Bolus 20 – 30 mcg/kg (maximum 1mg) then infusion 5 – 15mcg/min
monitor 4 -6 hr (biphasic 1 - 72hr)

74
Q

risk factors for biphasic reaction in anaphylaxis

A

delayed epinephrine
more than 1 dose epi
severe symptoms at presentaiton

75
Q

Prevention food allergy in high risk ifnant

A

first degree with atopy

  • exclusive BF 6mo
  • not delay introduction of food
  • can try hydrolyzed formula
  • regular introduction of newly introduced food to maintain tolerance
76
Q

venom allergy Rx

  • anaphylaxis
  • generalized cutaneous
A

Anaphylaxis

  • needs allergy referral (possible immunotherapy)
  • EpiPen, medical allert

generalized cutaneous
if > 16 risk of systemic reaction higher, so refer

77
Q

Drug allergy

A

2% with proven penicillin allergy will reaction with cephalosporin
Options:
1. give alternative drug
2. give cephalosporin via graded challenge
3. desensitize to cephalosporin

78
Q

2 pain syndromes

ddx

A 
Fibromyalgia
- pain at least 3 areas x 3 months
(without underlying cause, N bloodwork)
- more than 5 of 18 tender points
minor: 3/10
- sleep, fatigue, headaches, anxiety
- IBS, subjective tissue swelling, pain

complex regional pain
regional pain and 2 from each category
1. neurogenic
- burning/allodynia/cold hyperalgesia/parasthesia
2. autonomic
- cyanosis, mottling, hyperhidrosis, cool, edema

79
Q

Causes of uveitis (5)

- systemic and infectious

A 
JIA (oligo, poly)
ERA
SLE
KD
cat scratch
Lyme disease
tuberculosis
80
Q

complications uveitis (4)

A

synechae
glaucoma
cataracts
vision loss

81
Q

Reactive arthritis

bacteria (3)

A 
campylobacter
salmonella
shigella
yersinia
chlamydia, 
ureaplasma

arthritis - after 1 -4 weeks

82
Q

Septic arthritis
Kocher criteria
microbacteria
empiric antibiotics

A

Kocher 3/4 = 93% 2/4 = 40%

  1. non weight bear
  2. ESR > 40
  3. fever
  4. WBC > 12

staph aureus, GAS, (strep pneumo)
neisseria - teenagers
salmonella - SSD

Rx
IV nafcillin or ancef or clinda or vanco
total 3 weeks min (switch PO when improve)

83
Q

periodic fever
PFAPA
FMF

A

periodic fever= 3 episodes in 6months. at least 7 days apart
PFAPA
= periodic fever aphthout stomatitis, pharyngitis, adenitis
- inheritance unknown
- regular periodicity ~ q21 days, 7 days duration
- fever
- < 5 years old. self limited (resolves 5 years)
- throat cultures negative
Rx: single dose corticosteroids

FMF
AR, < 20 yo
fever 1 -3 days. Variable frequency
serositis
skin - erysipelas like rash
mono arthritis
well between episodes
Rx; colchicine (prophy)
prevent amylodosis
84
Q

Raynauds phenomenon

what, causes, rx

A

vascular spasm leading to triphasic color
white (ischemia)
blue (cyanosis)
red (erythema due to perfusion)

primary
secondary - SLE, scleoderma, JDM etc
(risk to autoimmune - ANA, nail bed)

rx: avoid triggers
nifidepine (peripheral vasodilator)
IV prostaglandin

85
Q

Sjogen’s

A 
autoimmune 
ANA positive AND RF or RO/La
keratoconjunctivitis sicca (dry eyes)
xerotstomia (dry mouth)
Rx: supportive treatment
86
Q

systemic scleroderma

5 systemic involvement

A

skin - sclerodactyly, calcium deposits, telangiectasia
lung - PHTN, ILD
MSK - polyarthritis, milld weakness
heart - pericarditis, arrhythmias
Gi - SEVERE GERD, bacterial overgrowth, malabsoprtion
GU - renal HTN, proteinuria

rx> MTX for active diases

87
Q

Parry Romberg syndrome

A

Linear scleroderma - involves face below forehead

  • progressive hemifacial atrophy
  • can be assoicated with intracranial lesions, seizures, uveitis, dental abnormalities
88
Q

JDM criteria

A 
proximal bilateral weakness
heliotrope rash, gottron's papules
lab: CK AST, LDH, aldolase
EMG: denervation and myopahty
Bx: necrosis and inflammation

Lx: ANA, anto Jo1
Rx: induce corticosteroids, MTX.
50% chronic course

89
Q

small vessel vasculitis

A

GPA - c ANCA

  • upper, lower resp tract
  • GU - HTN involvement
  • significant morbidity 11% dialysis

EGA

  • eosinophilia
  • lung - parasinus, pulmonary infiltrates
  • heart - MI, pericarditis
    dx: lung biopsy

MPA

  • pulmonary-renal
  • RPGN, HTN, pul hemorrhage, palpable purpura
90
Q

Bechet’s disease

A

variable size vasculitis
recurrent ora ulcers 3x/12monwths

oral/genital ulcers
pathergy
skin - Erythema nodosum
uveitis

91
Q

IgA vasculitis (HSP)

A

purpura AND

  • hematuria
  • arthritis
  • abdominal pain

rx: supportive, NSAIDS
f/u BP, U/A up to 1 year
< 5% ESRD
recurrence 1/3

92
Q

JIA classification

criteria

A

> 6 weeks, < 16 years onset

  1. Oligo 4 joints or less (ANA + uvelitis risk)
    - persistent
    - extended - > 4 joints in 6mo
  2. Poly 5 or more joints +/- RF
  3. SJIA - fever 2 weeks + arthritis + 1/4
    (HSM, LN, evevesant rash, serositits)
  4. ERA: entheritis and arthritis OR either with 2/5: (SI joint pain, Fmhx, boy >6yo, HLAB27, sym uveitis)
  5. psoriasis: arthritis + 1
    (Fmhx, dactylitis, nail pits)
93
Q

JIA x ray findings (4)

A 
accelerated growth
accelerated maturation
loss cartilage
erosion
osteoporosis
94
Q

JIA complications (4)

A 
misaligned joints
muscle atorphy
growth disturbance
delayed motor development
leg-length discrepency
contractures
95
Q

hypoglycemia critical sample (10)

A 
hormone:
Cortisol, IGF1 , insulin, glucos
Ketone/FAOD:
Betahydroxurlate or urine ketone,  
FFA, acylcarnitine profile
IEM:  Lactate,  Urine aa, oo, serum aa
Other:
C peptide
LE, ammonia
N, K
toxicology
96
Q

Sweat Chloride
False negatives
False positives

A

False negative

  • hypoalbumin
  • edema
  • poor technique
False positive
-malnutrition
adrenal insufficiency
glycogen storage disase
hypothyroidism
Nephrogenic DI
eczema

unsuccessful

  • premature
  • low weight
  • poor technique
97
Q

3 other ways other than Sweat chloride to confirm diagnosis

A

serum IRT - immunoreactive tyrpsin
nasla potentials
gene analysis

98
Q

Chronic wet cough

DDx (5)

A 
CF
primary cilliiary dyskinesia
immuno deficiency
bronchiectasis
missed foreign body
chronic infections - TF
asthma
99
Q

ARDS criteria

A

lung compliance

  1. acute severe resp distress
  2. bilateral infiltrates
  3. not by cardiac failure/causes
  4. PF ratio - PaO2/FiO2 < 200
100
Q

CF related organisms

A 
staph aureus
pseudomonas aeruginosa
burkholdreia cepacia
(H.influenazae
Aspergillus)
101
Q

DM1 targets

A

age < 6 : 6- 10; Hgb A1C 8
age < 12: 4 - 10: Hgb A1c 7.5
Age > 13: 4 - 7: Hgb A1c 7

102
Q

DM1 monitoring

A 
>12yo, + 5yr dx - ACR annual
>15 yo + 5yr dx - ophtho annual
@12, 17 yo - dyslipidemia
BP 2x/year
TSH, T4 - @ ddx, q2yrs
clinical - celiac, addison's
103
Q

Hypoglycemia DM1 management

A 
CHO 10-15g (~100ml juice)
recheck 15 mins
SC/IM mini glucagon 10mcg/year age (home)
Hospital Dextrose bolus
< 20kg = 0.5mg IM
>20kg -1mg IM
104
Q

Risk factors cerebral edema DKA

A 
age < 5 years old
new onset diabetes
bicarbonate
IV insulin bolus
IV insulin within 1hr of fluids
rapid fluid boluses
failure of Na to rise during treatment
105
Q

who to screen for DM2?

5

A

screen q 2 yrs with fasting glucose
if +obese, OGTT might be more sensitive
>/= 3risk factors pre puberty
>/=2 risk factors puberty

family history or exposure in utero
insulin resistance (acanthosis nigrans, HTN, PCOS, dysl)
BMI >/= 95%
aboriginal, Hispanic
antipsychotic atypical meds
106
Q

DM2 managment

A

target HbA1c < 7
metformin - if fail lifestyle for 3-6month
insulin (start immediate if Hgb A1c >9)

  1. lifestyle change - eating, exercise, sedentary lifestyle
  2. screen comorbidity
    @ dx and yearly
    - ACR, Ophtho, dyslipidemia, AST (NAFLD)
    - BP 2x/year
    - clinical neuropathy, PCOS
107
Q

clinically significant #’s

A

2 or more long bones by 10yo
3 or more by age 19
any vertebral compression #

DEXA scan –> Z scores in kids!

108
Q

Definition of
precocious puberty
delayed puberty

A

Male (normal 9 - 14)
< 9 yo
> 15yo

Female (normal 8 - 13)
< 8 year old
> 14 year old
>16 no menses

109
Q 
side effects (4)
antithyroid med
A

methimazole/prophylthiouracil

  • agranulocytosis (white)
  • hepatotoxicity (yellow)
  • rash/serum sickness like (red)
  • teratogen
  • hypothyroid (non permanent)
110
Q

Oxygen index

A

OI = MAP x FiOW/PaO2 x 100

Generally OI > 40 indicates need for ECMO

111
Q

Findings Resp distress syndrome CXR

A

ground glass
air bronchograms
hyperinflation

112
Q

B12 deficiency manifestations (5)

A 
macrocytic anemia
hypersegmented neutrophils
parasthesia
ataxia
seizures
dementia
deprression
fatigue
glossitis
113
Q

vitamin C deficiency

5 manifestations

A 
hypertrophic gums/gingival disease
easy bleeding
petechiae
roasry
pseudoparalysis
poor wound healing
perifollicular hemorrhages
114
Q

pellagra what is it?

manifestations

A

niacin (vitamin B3) deficiency
- stable food dependent on usualy corn/Kwashi patients

  • photosensitive, dermatitis like picture
  • dementia
  • diarrhea
115
Q

FTT investigations (10)

A 
CBC, diff
iron studies
IgA, TTG
LE, renal function
electrolytes, extended
U/A
CRP, ESR
TSH
albumin, protein
serum immunoglobulins
2nd step
sweat chloride
stool elastase
vitamin levels
bone age
116
Q

zinc deficiency

A

acrodermatitis enteropathica
- AR disease, zinc malabsoprtion
rx zinc for life

oerporal, acral, perinala regions with vsiculobullous, ecematous patches
- glottitis
growth retardation
superinfection
poor wound healing
117
Q

Celiac disease

manifestations

A 
classic - FFT, diarrhea, abdomianl distension, pain, vomiting, cachexia
dermatitis herpetiformis
refractory iron def anemia
short stature
seizure with occipital calcification
dental enamel hypoplasia
osteoporosis
118
Q

Celiac disease testing (3)

A 
ttG and IgA
EMA - anti endomysium
AGA - anti glaidin 
CBC, iron studies
albumin
119
Q

Celiac disease

associated syndromes

A 
T21
Turners
Williams
DM1
autoimmune thyroiditis
selective IgA def
1st degree celiac FmHx
120
Q

Celiac disease histology findings

name 4 other ddx

A

villous atopy

CMPA
viral infections
EoE
Crohn's
immunodeficiency
malnutrition
121
Q

Contraindications to air enema/contrast enema for intussusception

A

peritonitis
persistent hypotension
free air/pneumoperitoneium

122
Q

IBD

skin manifestations

A 
pyoderma gangenosum
erythema nodosum
crohn's metatatic disease
perianal fistulas
dental - pyostomatis vegetans
123
Q

IBD life threatening complications

A

GI hemorrhage
toxic megacolon
GI obstruction
GI perforation

124
Q

differences between Crohn’s vs UC

A 
Crohn
- transmural, skipped lesions, oral to anus
- cobblestone 
- less likely bloody diarrhea
perianal/oral disease
more likely nephrolithasis and choleithiasis
-strictures, obstruction
- more Growth impairment

UC

  • higher risk PSC
  • more common than Crohn
  • more likley to present< 10 yo
125
Q

Functional constipation

A

1 month duration, 2 or more:

  • < 3 BM in toilet
  • unable to flush
  • retentive behaviour
  • large caliber rectum
  • fecal soiling
  • painful or hard BM
126
Q

Functional diarrhea

A 
4 weeks
onset 6 mo - 5 years
occur waking ours
painless >/=3 unformed stools
no FTT if adequate itnake

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