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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
Galactosemia
``` CHO metabolism disorder hepatomegaly, jaundice, FTT e.coli sepsis neurocognitive Cataracts ``` Dx: RBC GALT other: +conjugated bili, INR, Hemolytic anemia + urine reducing substance
26
Glycogen storage disorder Not Pompe
massive hepatosplenomegaly short stature bleeding diathesis hypoglycemia, lactic acidosis, dec reponse to glucagon
27
Methylmalonic acidemia
``` Organic acidopathies (other = Proprionic acidemia) ``` HIGH AG metabolic acidosis, ammonia, LE Urine OA hepatosplenomegaly, seizures, vomiting, FFT cardiomyopathy Rx low protein diet, L carnitine
28
urea cycle defect
ie OTC (x-linked) Respiratory alkalosis elevated hyperammonia OTC: inc urine orotic acid ``` low protein sodium benzoate (NH savengers) ```
29
Calculate GCS. Kid has abnormal flexion of arms and legs to pain. Eyes open to pain. Incomprehensible sounds.
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
30
Neurofibromatosis inheritance criteria surveillence
``` 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
31
Tuberous Sclerosis inheritance criteria surveillence
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
32
Sturg Weber syndrome inheritance clinical Rx
``` sporadic clinical: nevus flammeous V1/V2 distribution glaucoma CT head - leptomeningeal angiomatosis seizures hemiparalysis developmental delay ```
33
Von Hippel Lindau inheritance clinical risks monitoring
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 ```
34
PHACE syndrome
``` 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
35
Incontinentia pigmenti
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
36
Hypomelantosis of Ito
``` Sporadic disorder Hypopigmented whorls/streaks follow Blashko’s lines Seizures, mental retardation Hemihypertrophy Cardiac - TOF, pulmonary stenosis ```
37
developmental delay | initial w/u
``` CBC, LE, RFT, lytes MRI head microarray if 2 or more anomalies karyotype, MCEP2 (Rett), Fragile X met- low yield unless clinical ```
38
Rett criteria
``` 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
39
Febrile seizure Risk factors for recurrence Risk factors to go onto epilepsy
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 ```
40
C/I to AED
absence/myoclonic seizures - no carbamazepine or phenytoin IEM or developmental delay unknown - valproic acid
41
``` S/E Valproic acid phenytoin carbamazepine lamotrigene vigabatrin ethosuximide topiramate ```
(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
42
breath holding spells age what look like treatment
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
43
empiric AED for 1. absence seizures 2. infantile spasms 3. focal seizures 4. general seizures
1. absence - ethosuximide > VPA 2. infantile - ACTH, vigabatrin 3. focal - carbamazepine, keppra 4. general - VPA, keppra, lamotrigene
44
Migraine headache criteria
``` 5 or more attacks each last 1- 72hrs 2 characteristics -severity -unilateral -Throbbing -Aggravated activity/avoidance 1 association -Nausea/vomiting -Sensitivities ```
45
headache Indications for neuroimaging
``` neurofocal findings worst in AM awakens at night unusual headaches - occipital, thunderclap recent head injury seizures or academic deterioration ```
46
migraine with brainstem aura | basilar
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
47
REd eye reflex ddx
glaucoma cataracts high refractive error retinoblastoma
48
Eye findings with syndromes: 1. T21 2. WAGR 3. Waarenburg 4. NF1 5. Wilsons 6. Williams 7. CHARGE
1. Brushfield spots 2. Anisoria 3. Heterochromiia 4. Lisch nodules 5. Kayser Fleischer 6. Stellate 7 Coloboma
49
Bone tumor Osteosarcoma vs Ewing
``` 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
50
Lupus criteria
``` 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
51
Lupus treatment
hydroxychloroquine - standard therapy +/-corticosteroids if nephritis, hematological crisis, CNS disease - possible azathioprine, MMF, cyclophosphamide
52
Acute rheumatic fever criteria treatment
``` 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
Granulomatosis with polyangitis
+ANCA | URT, LRT, renal
54
Kawasaki disease | bloodwork
``` leukocytosis thrombocytosis anemia (N indices) elevated CRP, ESR LE, mild hyperbili hypoalbuminemia sterile pyuria ```
55
Rickets | Skeletal findings
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
MAS | lab - what is low?
Low: ESR, Fibrinogen
57
Drug induced lupus | medications
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
catalase positive pathogens list (4) (who at risk)
``` staph aureus aspergillus nocardia serratia marcescens burkholderia salmonella ``` phagocytes --> CGD
59
Anaphylaxis criteria
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 2. KNOWn and hypotension
60
Bruton's agammaglobinemia | xlinked
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
leukocyte adhesion defect
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
Ataxia Telangiectasia
``` AR in ATM gene (DNA repair) ataxia--> telanectasias immunodef - IgA low (sinopul, encapsulated) risk malignancy (15% lymphoma) ``` Supportive only IVIG BMT not work
63
Ataxia Telangiectasia
``` AR in ATM gene (DNA repair) ataxia--> telanectasias immunodef - IgA low (sinopul, encapsulated) risk malignancy (15% lymphoma) ``` Supportive only IVIG BMT not work
64
Low complement (3)
SLE Post-strep GN membrano-proliferative GN liver disease
65
specific antibodies for rheumatic diseases SLE Systemic sclerosis JDM
SLE - dsDNA, sm, Ro, La - histone (drug induced as well) Sclerosis - systemic = Scl70 - Limited (CREST) = centromere JDM - Jo1
66
associations c-ANCA p-ANCA
C-cytoplasmic CR3 Wegners - granulomatosis with polyangitis P-perinuclear MPO - microscopic polyangitis - eosinophlic granulomatosis with polyangitis - UC, primary sclerosing cholangiits - SLE
67
vasculitis | categories and names
``` 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
Takayasu
``` angiographic evidence - aorta/branches AND one of 5 - claudication - limb BP differences - HTN - bruit - acute phase reactant ```
69
Polyarteritis nodosa
medium vessel ``` histological - necrotizing vasculitis angiography AND 1/4 Skin - Erythema Nodosa myopathy renal involvement HTN peripheral neuropathy ``` rx: steroid rapid wean
70
Kawasaki disease | counselling
recurrence risk 2% no live vaccines 11mo (after IVIG) influenza vaccines (not live) athersclerosis risk - active living
71
Live influenza contraindications
severe asthma (on PO, current wheeze, medically treated wheeze < 7 days, high dose ICS) 2-17 year old on ASA (Reye) immunodeficiency/Pregnancy
72
HSP IgA vasculitis | management & counseling
``` 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
anaphylaxis | Treatment
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
risk factors for biphasic reaction in anaphylaxis
delayed epinephrine more than 1 dose epi severe symptoms at presentaiton
75
Prevention food allergy in high risk ifnant
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
venom allergy Rx - anaphylaxis - generalized cutaneous
Anaphylaxis - needs allergy referral (possible immunotherapy) - EpiPen, medical allert generalized cutaneous if > 16 risk of systemic reaction higher, so refer
77
Drug allergy
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
2 pain syndromes | ddx
``` 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
Causes of uveitis (5) | - systemic and infectious
``` JIA (oligo, poly) ERA SLE KD cat scratch Lyme disease tuberculosis ```
80
complications uveitis (4)
synechae glaucoma cataracts vision loss
81
Reactive arthritis | bacteria (3)
``` campylobacter salmonella shigella yersinia chlamydia, ureaplasma ``` arthritis - after 1 -4 weeks
82
Septic arthritis Kocher criteria microbacteria empiric antibiotics
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
periodic fever PFAPA FMF
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
Raynauds phenomenon | what, causes, rx
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
Sjogen's
``` autoimmune ANA positive AND RF or RO/La keratoconjunctivitis sicca (dry eyes) xerotstomia (dry mouth) Rx: supportive treatment ```
86
systemic scleroderma | 5 systemic involvement
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
Parry Romberg syndrome
Linear scleroderma - involves face below forehead - progressive hemifacial atrophy - can be assoicated with intracranial lesions, seizures, uveitis, dental abnormalities
88
JDM criteria
``` 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
small vessel vasculitis
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
Bechet's disease
variable size vasculitis recurrent ora ulcers 3x/12monwths oral/genital ulcers pathergy skin - Erythema nodosum uveitis
91
IgA vasculitis (HSP)
purpura AND - hematuria - arthritis - abdominal pain rx: supportive, NSAIDS f/u BP, U/A up to 1 year < 5% ESRD recurrence 1/3
92
JIA classification | criteria
> 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
JIA x ray findings (4)
``` accelerated growth accelerated maturation loss cartilage erosion osteoporosis ```
94
JIA complications (4)
``` misaligned joints muscle atorphy growth disturbance delayed motor development leg-length discrepency contractures ```
95
hypoglycemia critical sample (10)
``` 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
Sweat Chloride False negatives False positives
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
3 other ways other than Sweat chloride to confirm diagnosis
serum IRT - immunoreactive tyrpsin nasla potentials gene analysis
98
Chronic wet cough | DDx (5)
``` CF primary cilliiary dyskinesia immuno deficiency bronchiectasis missed foreign body chronic infections - TF asthma ```
99
ARDS criteria
lung compliance 1. acute severe resp distress 2. bilateral infiltrates 3. not by cardiac failure/causes 4. PF ratio - PaO2/FiO2 < 200
100
CF related organisms
``` staph aureus pseudomonas aeruginosa burkholdreia cepacia (H.influenazae Aspergillus) ```
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DM1 targets
age < 6 : 6- 10; Hgb A1C 8 age < 12: 4 - 10: Hgb A1c 7.5 Age > 13: 4 - 7: Hgb A1c 7
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DM1 monitoring
``` >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 ```
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Hypoglycemia DM1 management
``` 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 ```
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Risk factors cerebral edema DKA
``` 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 ```
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who to screen for DM2? | 5
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 ```
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DM2 managment
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
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clinically significant #'s
2 or more long bones by 10yo 3 or more by age 19 any vertebral compression # DEXA scan --> Z scores in kids!
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Definition of precocious puberty delayed puberty
Male (normal 9 - 14) < 9 yo > 15yo Female (normal 8 - 13) < 8 year old > 14 year old >16 no menses
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``` side effects (4) antithyroid med ```
methimazole/prophylthiouracil - agranulocytosis (white) - hepatotoxicity (yellow) - rash/serum sickness like (red) - teratogen - hypothyroid (non permanent)
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Oxygen index
OI = MAP x FiOW/PaO2 x 100 Generally OI > 40 indicates need for ECMO
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Findings Resp distress syndrome CXR
ground glass air bronchograms hyperinflation
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B12 deficiency manifestations (5)
``` macrocytic anemia hypersegmented neutrophils parasthesia ataxia seizures dementia deprression fatigue glossitis ```
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vitamin C deficiency | 5 manifestations
``` hypertrophic gums/gingival disease easy bleeding petechiae roasry pseudoparalysis poor wound healing perifollicular hemorrhages ```
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pellagra what is it? | manifestations
niacin (vitamin B3) deficiency - stable food dependent on usualy corn/Kwashi patients - photosensitive, dermatitis like picture - dementia - diarrhea
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FTT investigations (10)
``` 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 ```
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zinc deficiency
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 ```
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Celiac disease | manifestations
``` classic - FFT, diarrhea, abdomianl distension, pain, vomiting, cachexia dermatitis herpetiformis refractory iron def anemia short stature seizure with occipital calcification dental enamel hypoplasia osteoporosis ```
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Celiac disease testing (3)
``` ttG and IgA EMA - anti endomysium AGA - anti glaidin CBC, iron studies albumin ```
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Celiac disease | associated syndromes
``` T21 Turners Williams DM1 autoimmune thyroiditis selective IgA def 1st degree celiac FmHx ```
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Celiac disease histology findings | name 4 other ddx
villous atopy ``` CMPA viral infections EoE Crohn's immunodeficiency malnutrition ```
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Contraindications to air enema/contrast enema for intussusception
peritonitis persistent hypotension free air/pneumoperitoneium
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IBD | skin manifestations
``` pyoderma gangenosum erythema nodosum crohn's metatatic disease perianal fistulas dental - pyostomatis vegetans ```
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IBD life threatening complications
GI hemorrhage toxic megacolon GI obstruction GI perforation
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differences between Crohn's vs UC
``` 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
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Functional constipation
1 month duration, 2 or more: - < 3 BM in toilet - unable to flush - retentive behaviour - large caliber rectum - fecal soiling - painful or hard BM
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Functional diarrhea
``` 4 weeks onset 6 mo - 5 years occur waking ours painless >/=3 unformed stools no FTT if adequate itnake ```

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