Week1 Lectures Flashcards

1
Q

___ (3) are reabsorbed in the kidney

A

Na+, Cl-, water

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

___ (1) are excreted in the kidney

A

H+

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

___ (2) are reabsorbed and secreted in the kidney

A

K+ and Uric Acid

hint: creatinine + inulin are unchanged

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q
    • porous nephron segment -
  • trancellular transport (K+, H20) -
  • 85% HCO3- abs -
  • site of CAi -
  • site for SGLT2 drugs (diabetes)
A

Proxtimal Tubule

hint: abs carbonate, excretes acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q
    • Passive water movement based on conc gradient -
  • tx site of osmotic diuretic aquaresis after entry (eg mannitol)

segment?

A

Descending limb LOH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q
    • impermeable segment to water -
  • dilutes tubular fluid permb to small solutes (25% Na)

segment?

A

Thin Ascending Limb LOH

hint: diluting segment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

___(enz) restores nephron’s chemical electroneutrality

___(enz) is inh by loop diuretics –> Mg and Ca loss w/ Na, K, Cl

A

ROMK (electroneutrality);

NKCC2 (loops diuretics)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Renal Autocoids (3)

A
  1. Adenosine
  2. Prostaglandins
  3. peptides (ANP/BNP vasoD’s)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Renal Hormones (5)

A
  1. Renin-Ang sys
  2. EPO
  3. Aldosterone (MR rcp Na reabs)
  4. 1,25 OH Vitamin D3 (via I-𝛂-OHase)
  5. ADH (V2 rcp water abs)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

autocoid responsible for:

  • afferent arteriole vasoD
  • glomerular blood flow autoregn
  • ↓Na reabs in LOH;
  • ↓H2O transport in CTs
A

Prostaglandins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Nephron segment: -

  • active NCC co trnsporter -
  • impermb to water -
  • Apical Mg channel -
  • baso Na/Mg transporter
A

DCT I

hint: 25% NaCL reabs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Nephron segment: -

  • active NCC co transporter -
  • apical ENaC channel for Na+ -
  • baso Cl channel -
  • baso Ca/Na + Ca active transporters (PTH reg’d)
A

DCT II hint: 25% NaCL reabs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

____(celltype) in the collecting duct (CD) transports Na, K, H2O; while ____ (celltype) have acid base function in CD (𝛂 for H+ and β for HCO3-)

A

principle cells;

intercalated cells (𝛂 and β)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Name presentation and causative fungus? -

  • hypersensitivity rxn to inftn
  • complicates asthma or CF -
  • dx on exam -
  • treat with itraconazole, sinus surgery , xolar
A

ABPA from Aspergillosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Name presentation and causative fungus? -

  • colonizing fungus ball after cavitary lung disease -
  • air crescent/grape cluster on scan (dx) -
  • tw itraconazole vel surgery
A

Aspergilloma from Aspergillosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Name presentation and causative fungus? -

  • mimics TB air crescent on scan -
  • needle-aspirate lung fluid for histo -
  • ↑risk for alocoholism , COPD, CGD -
  • tw voriconazole + AmphoB
A

CNPA from Aspergillosis hint: unresponsive to antibiotics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Name presentation and causative fungus? -

  • resp distress after prfound PMH of IMNS -
  • halo sign on scan (dx) -
  • needle/biopsy for histo (Acute Angles*)
A

Invasive from Aspergillosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Whats the dx and Tx? -

  • Caused by rhizopus -
  • deadly invasive vasculitis from env mold -
  • non septate right angle* on histo -
  • sinus entry –> brain invasion –> infarction -
  • Risk: unctrl’d T2DM, Fe overload , IMNS
A

Mucormycosis (Rhinocerebral, wound, cutanous, lung/GI) tw amphoB + surgery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Whats the dx and Tx? -

  • ubiq env mold + rare.fatal inftn -
  • seen in HSCT pts -
  • eye, lung, and skin sx -
  • blood culture + histo dx
A

Fusarium (mycotoxicosis, local, disseminated) tw amphoB + voriconazole

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Whats the dx and Tx? -

  • ↓host imm resp -
  • late ppt: meningitis; skin; nodules; pulm sx -
  • dx: biopsy, CSF, crag
A

Cryptococcosis tw Fluctyosine –> AmphoB –> fluconazole

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

how does arteriolar tone affect vascular function curves (esp venous return)

A
    • arterioles hold min vol thf MSFP is not changed -
  • blood trapped upstream via vasoC –> ↓venous blood –> ↓venous P + reutrn at any CVP -
  • vice versa w/vasoD
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

vasc pressure when blood flow ceases?

A

mean systemic filling pressure (MSFP) hint: ∝ blood vol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

determinants of systolic fx? (3)

A
  • preload
  • afterload
  • contractility
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

COPD, effusion, congestion, or obesity would cause voltage signal to (increase/decrease)

A

decrease (more distant)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Myocardial hypertrophy would cause EKG voltage to (increase/decrease)

A

increase (large muscle)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

____ (chemical class) ↑ intracell cAMP, Ca+, and contractility

A

catecholamines

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Normal ejection fraction (EF)

A

55-65%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q
    • ∆ perfusion to body -
  • ↑ fluid in lungs (congestion) -
  • SOB + pulm edema

characterizes…

A

Left heart failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q
    • ∆ perfusion to lung -
  • ↑ fluid in periphery -
  • ascites + periph edema

characterizes…

A

right heart failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

whats the dx? -

  • ∆ NCC in distale tubule (SLC12A3) -
  • hypoMg* + hypoK + alkalosis + ↑plasma renin + aldo
  • ± low BP -
  • caused by LOOP diuretics
A

Gitelman’s syndrome (low serum Ca+)

hint:

  • Bartter’s + hypoMg
  • dump bicarb –> ↑water into lumen
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

whats the dx? -

  • ∆ NKCC2 symporter in Thick Asc LOH (SLC12A1) -
  • hypoK + alkalosis + ↑plasma renin + aldo -
  • ± low BP
  • caused by THIAZIDE diuretics
A

Bartter’s Syndrome (high urine Ca+)

hint: water (vol) drops –> renin/aldo↑

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

what Rx combo is used for nephron paralysis? (3)

A

CAHi + loop + distal diuretics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

what’s this rare AD dz? and how do you tx? -

  • gain of fx: EnAC channels -
  • clinica; ppt: htn, ↑Na+ reabs, K wasting
A

Liddle’s Syndrome

tw

  • K-sparing diuretics:
  • amiloride (or triamtereneif allergic )
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

2 yrs dapsone + rifampin is tx for ___

A

Tuberculoid Hansen’s (M. Leprae)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Hansen’s disease form chr by:

  • Paucibacillary,
  • vigorous, but ineffective CMI (CD4+, Th1) –> PPD⊕
  • nerve damage (clawed hands + toes, foot drop)
A

Tuberculoid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Hansen’s disease form chr by:

  • multibacillary ,
  • weak CMI (useless Th2 w/ Abs) –> PPD⊖
  • extensive cutaneous sx
A

lepromatous

hint: lionine facies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

____ has no in vitro culture system, slowest growing human pathogen, only affects 5-10% of humans*, prefers 30-37C

A

M. Leprae

common ppt: asymptomatic seroconversion*

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Scrofula in kids indicates ___ inftn; while in adults indicates ____

A

Atypical Mycobacterial inftn; TB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

key histo features for M. Leprae inftn? (2)

A
  1. foam cell mø’s
  2. granulomatous epiths + lymphos

hint: skin smear/punch biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Labs for M. Leprae in the US and Abroad

A

PPD (abroad):

Serology (US):

  • LMIT for tuberculoid
  • phenolic glycolipid-1 for lepromatous
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q
  • health care worker exposed to Mleprae
  • pt with tuberculoid form
  • previous lepromatous form responding to tx

these are indicative of (neg/pos) lepromatin PPD?

A

POSTIVE PPD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q
  • naive pt
  • lepromatous leprosy

these are indicative of (neg/pos) lepromatin PPD?

A

NEGATIVE PPD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Name that drug:

  • Unknwon MOA
  • SEs: N/V; hepatotox; HS rxns
A

Pyrazinamide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Large proteins, cells, and complexes that can be centrifuged* out is a job for ____ (procedure)

A

Apharesis

hint: exchange (replace) vs true pheresis (substract)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Separating plasma contents small enough for a semipermb membrane through diffusion/convection would be a job for ___ (procedure)

A

Dialysis

hint: AEIOU = Acid/base, Electrolytes, Intoxication, Overload of fluid, Uremic Sx

46
Q

types of Dialysis (2) and their subtypes (2 each)

A
  1. Hemodialysis
    • cont CRRT vs intermittent IHD
  2. Peritoneal Dialysis
    • manual vs auto
47
Q

Why is sodium citrate used for dialysis?

A
  • sodium citrate binds Ca+
  • Ca+ is vital for coagn via intrinsic pathway (F9,10,V and thrombin)
  • ↓ Ca+ –> bleeding risk
48
Q

___ moves solute; ____ moves water; is also known as solvent drag or the movement of water with stuff in it

A
  • diffusion;
  • hydrostatic F (osmosis)
  • convection = solvent drag
49
Q

Why does hereditary hemochromatosis and transfusion lead to iron overload myocardial dz?

A

ventriclular iron deposition –> ∆ metal dep enz systems + O2* injury –> systolic dsfx (∆ pumping)

50
Q

dilated heart and ↑hemosiderin laden mø’s (on Prussian blue stain) is chr histopathology for ___

A

Iron Overload (myocardial dz)

51
Q

these are signs of what tupe of thryoid disfx?

  • nonspecific ischemic hypertrophy
  • tachycardia, palpitations, and cardiomegaly
A

HypERthyroidism

52
Q

these are signs of what type of thryoid disfx?

  • ↓stroke vol –> ↓CO
  • myofibers swelling w/↓ striations + basophilic staining
  • flabby, dilated heart
A

hypOthyroidism

53
Q

what the dx?

  • AD inheritance
  • ∆ desmosomes –> thinned R ventr wall (fatty + fibrotic change)
  • leads to heart failure, vtach, and vfib
A

Arrhythmogenic R Ventricular Cardiomyopathy/Dysplasia

54
Q

Whats the Dx?

  • AD congenital dz
  • spongy ventricular myocardium –> arrythmias + heart failure
  • tx sx + heart tranplasnt
A

Non-compaction cardiomyopathy

55
Q
  1. On histology _____ (dx) shows intersitital lymphocytic infiltrate + focal necrosis –> focal or patchy inflamm/progressive fibrosis. Clinical sx incl dyspnea, palps, precordial discomfort + fever w/± normal/dilated heart
  2. WHat is the most common cause for this dx in US?
A
  1. myocarditis;
  2. viral inftn = most common cause in US

hint: ppt = precordial discomfort + fever

56
Q

on clinical exam, pt complains of dyspnea and excerise intolerance, and appears to have right sided heart signs. On echo myocardium appears thick, w/↑ atrial size and normal ventr size. On EKG, voltage is low with condduction abnormalities (afib + arrhtymia). Biopsy is seen below. Dx?

bonus? what contributes to dyspnea and exercise intolerance?

A

Amyloidosis leading to Restritive Cardiomyopathy (Diastolic dysfx)

bonus: ↑ filling pressure (which –> ↓CO)

57
Q

Least common type of cardiomyopathy. Chr by myoc infiltr –> ↓ ventr compliance –> ↓diastolic ventr filling

A

Restrictive Cardiomyopathy

58
Q

What’s your dx?

  • 100% genetic (sarcomeric prots)
  • ↑ IVS thickness (obstruction) w/ banana shaped LV
  • Myocyte disaaray + replacement fibrosis on histo

bonus: why does sarcomeric change lead to gross heart changes?

A

Hypertrophic cardiomyopathy (diastolic dysfx)

Bonus: sarcomeric dyfx/change –> defective E transfer from mt to sarcomere

59
Q

What does the pt have? how would you dx (2)

  • Sx:
    • Dyspnea on exertion (↑HR and contractility)*
    • chest pain
    • syncope*
  • signs:
    • loud S4
    • systolic ejection murmur ± mitral regurg
    • 2 upstrokes –> ↑↑↑carotid pulse rate
A

Hypertrophic cardiomyopathy

dx by echo + family hx

hint: dyspnea + syncope dt outflow obstruction

60
Q

Explain how systolic ejection murmur changes with:

  1. squatting/lying/hand grip/straight leg raise
  2. standing/Valsalva
  3. where is its best heard?
A
  1. decreases dt ↓outflow obstruction
  2. increases dt ↓ventr filling
  3. best heard @ L lower sternal border (tricuspid posn)
61
Q
  • Most common type of cardiomyopathy
  • 50% cases are idiopathic
  • both ventricles are affected
  • death w/i 5 years
A

Dilated Cardiomyopathy (systolic dysfx)

hint: Toktsubocardiomyopathy (broken heart syndrome)

62
Q

Treatment for Dilated CMP (4) + anticoagn

A
  1. Digoxine (improves contractility)
  2. diuretics (ACE inh)
  3. VasoDs (βBs)
  4. Cardiac Transplant

hint; remove offending agent if possible

63
Q

Clinical features point to what dx?

  • L and R sided CHF sx
  • S3, S4 + murmurs*
  • Cardiomegaly (balloon~ on CXR)
  • sudden death*

bonus: Explain the reasons behind the *

A

Dilated Cardiomyopathy

bonus:

  • mitral/tricuspid insuffc –> murmurs
  • cardiomegaly –> ↑thrmobotic risk
64
Q

These are etiologies for what cardiomyopathy?

  • Alcohol abuse,
  • wet Beriberi,
  • Coxsackie B viral myocarditis,
  • chronic Cocaine use,
  • Chagas disease,
  • Doxorubicin
A

Dilated Cardiomyopathy

hint: ABCCCD

65
Q

what leads to sudden death in young athletes?

A

ventricular arrythmia –> syncope –> suddetn death dt Hypertrophic Obstructive Cardiomyopathy

66
Q

These are etiologies for what cardiomyopathy?

  • Postradiation fibrosis,
  • Löffler endocarditis (hypereosinophicic syndrome)
  • Endocardial fibroelastosis in kids (thick fibroelastic tissue in endocardium)
  • Amyloidosis
  • Sarcoidosis
  • Hemochromatosis (although dilated cardiomyopathy is more common)

Puppy LEASH*

A

Restrictive Cardiomyopthaty

hint: Puppy LEASH* is restrictive

67
Q

6 stages of plaque formation

A
  1. endoth cell damage
  2. monocyte/lø adhesion (ox LDL + leukotriene chemotaxis)
  3. mø maturation
  4. lipid uptake by mø (foam cells)
  5. Sm mucle cell migration (media –> intima)
  6. advance lesion
68
Q

Whats your dx?

  • mostly asympto but AD
  • mitral valve ballooning during systole –> mitral insuffc –> incidental mid systolic click + murmur
  • emboli
  • mixoid change –> ∆ conductionsystem –> sudden death
A

Mitral Valve Prolapse (MVP)

hint: floppy valves in 3-5% of Americans; Marfan + Ehlers Danlos syndrome assc

69
Q

Whats your dx?

  • degenerative change in the elderly
  • stenotic leaflets + valve ring regurgitation
A

Mitral Valve annular calification

70
Q

Whats your dx?

  • Cardiac sx of seratonin sydnrome
  • R heart lesions (subendo gray yellow plaques)
A

Carcinoid Valve Dz

71
Q

Click and murmur become ___ (softer/louder) w/ squatting in mitral valve prolapse bc?

A

softer bc ↑ systemic resistance –> ↓ventr emptying

72
Q

Complications of MVP include (3)

A
  1. infectious endocarditis
  2. arrythmia
  3. severe mitral regurg

hint: valve replacement

73
Q

The difference bwn acute and subacute endocarditis (virulence, valve, predispn, eg bug)

A

Subacute

Acute

  • Low virulence + small vegetations
  • Mitral valve involvmt
  • Previously dmg valves infted
  • E.g. strep viridans
  • High virulence
  • Tricuspid valve (why?)
  • Normal valves infted
  • E.g. staph aureus

triscupid valve involvement dt to R heart drainage

74
Q

Name the assc etiology:

  1. strep viridans (subacute)
  2. staph aureus
  3. staph epidermidis
  4. strep bovis
A
  1. chronic RHD, mitral valve prolapse
  2. IV drug abuse
  3. prosthetic valves
  4. colorectal carcinoma –> endocarditis
75
Q

Name the HACEK organisms and key assc with endocarditis

A
  1. Haemophilus
  2. Actinobacillus
  3. Cardiobacterium
  4. EIkenella
  5. Kingella

assc with NEGATIVE blood cultures

76
Q

Clinical features of bact endocarditis (4)?

A
  1. Fever (bactermia)
  2. Murmur (∆flow)
  3. veg emboli –> skin involvemnt: Janeway lession (palms and soles); Osler nodes (ouch); splinter hemorrhages; roth spots (retina)
  4. Anemia of chronic dz (microcytic + inflamm)
77
Q

what’s the dx?

  • small, sterile vegetations dt SLE
  • undersurface of mitral > tricuspid valves
  • ANA changes (eosinophilic necrotic debris) + mitral regurgitation
A

Libman-Sacks/Lupus Endocarditis

78
Q

What’s the dx?

  • sterile thrombotic vegs on mitral valve –> regurg
  • hypercoagb state/underlying adenocarcinoma/marantic endocarditis
A

Non bact thrombotic endocarditis (NBTE)

79
Q

Triad for chronic RHD

A
  1. Leaflet thickening (mitral fish mouth)
  2. Commissural fusion (aortic valve involement)
  3. thickened cordae tendinae
80
Q

What’s the dx?

  • Dyspnea on exertion, slowly ↑ with age
  • Fainting spell + vision loss in one eye (aortic valve sx)
  • Blood tinged sputum (pulm edema + hemosiderin cells)
  • history of acute rheumatic illnesses (firbotic changes)
A

Chronic RHD

81
Q

WHats the Dx with this type of pancarditis? assign each sx to a layer :

    • mitral verrucae + regurg
    • Ashoff bodiess + Anitschkow cells
    • friction rub and chest pain
A

ACUTE rheumatic Fever

  1. endocarditis - mitral verrucae + regurg
  2. myocarditis (sudden death) - Ashoff bodiess + Anitschkow cells
  3. pericarditis - friction rub and chest pain
82
Q

what does this dx point to?

  • ↑ASO/Anti-DNase B titers
  • minor criteria: fever and ↑ESR
  • major criteria: J❤NES (what does this stand for?)
A

ACUTE rheumatic fever

hint: J❤NES

  • Joints (polyarth)
  • ❤ heart (pancarditis)
  • Nodules on skin
  • Erythema marginatum (trunk + limbs)
  • Syndenham Chorea
83
Q

why does strept throat (Pharyngitis) cause ARF?

A

bact M protein in Aβ-hemolytic strep mimics human tissue (Ab cross reactivity)

hint: S. pyogenes + anti streptolysin O titer

84
Q
A

Check Valve

85
Q

Whats your dx?

  • Chronic wear + tear (age > 60)
  • Hx of Healed RHD or congential biscupid valve –> fibrosis
  • A fib and LV hypertrophy on ECG
  • Cardiomegaly + calcification @ aortic valve on CXR
A

Calcified aortic stenosis

86
Q

the ∆F508 mutation is class ___ (2). Describe the problems in each class.

A
  • Class II - no traffic –> need to correct prot folding
  • Class VI - less stable –> need to promote statbility
87
Q

5 steps in CF pathophys:

A
  1. CFTR gene defect
  2. ∆ ion tranpsort
  3. ↓airway surface liq
  4. ∆ mucciliary clearance
  5. inftn + inflamm + mucus obstruction
88
Q

explain why dosage reqs are much higher with short interval/cont admin (3)?

how would you dose tobramycin or Ceftazidime?

A

high/short dosage rationale:

  1. ↑metabs
  2. ↑excretion
  3. ↑vol of distribution

IV Tobramycine: 14 mg/kg/day

Ceftazidime: cont infusion of 200 mg/kg/day (10-14 g)

89
Q

THe most common pathogen in CF pts is ____. Why are outcomes so bad?

A

Pseudomonas

  1. Polymicrobial Biofilms
  2. Co-dependancy
  3. ∆ virulence
90
Q

2 non TB atypical mycobacterials that affect IMNS CF pts

A
  1. M. Avium (MAC)
  2. M. abscessus/M. chelonae
91
Q

what is the dx? explain the asterisks

  1. ↓NaCl alkalosis
  2. infertility*
  3. manutrition + vit defc
  4. pancreatitis + diabetes*
  5. steatorrhea and billiary cirrhosis*
  6. neonatal jaundice + meconium plug
A

Cystic Fibrosis

* hint: ø vas deferens in males; pancreatic burnout of islet cells –> diabetes; mucus obstruction in biliary tree –> fat mal-abs

92
Q

Whats the dx?

  • onset of dyspnea/difficulty breathing hours within exposure to welding (self limited)
  • fever, chills, cough, wheezing,chest pain (normal CXR)
  • metallic taste in mouth
A

Metal Fume Fever

hint: zinc/cadmium oxide + teflon

93
Q

Large Vessel Vasculitis Dzs (2)

A
  1. Takayasu Arteritis
  2. Giant Cell Artertitis
94
Q

Medium Vessel Vasculitis Dzs (2)

A
  1. POlyarteritis Nodosa
  2. Kawasaki Dz
95
Q

ANCA-assc Small Vessel Vasculitis Dzs (3)

A
  1. MIcroscopic Poyangiitis
  2. GPA (Wegener’s)
  3. Churg Strauss (Eosionophil GPA)
96
Q

IMmune COmplex Small Vessel Vasculitis

A
  1. Cryoglobulemic Vasc
  2. Henoch-Schōnlein (IgA Vasc)
  3. AntiC1q Vasc (hypocomplementemic Urticarial Vasc)
  4. Anti-GBM Dz
97
Q

Immune complex mediated Vasc is assc with what 2 dz?

A
  1. Rheumatoid vasc
  2. Lupus Vasc
98
Q

Whats the dx?

  1. early, self limited childhood dz
  2. risk of renal involvement ↑with age
  3. purpura on buttocks and LE + abdominal pain (GI bleeds) + hematuria+ arthritis
A

HSP

Henoch-Schōnlein (IgA Vasc)

hint: small vessel; tx with steroids if severe; abdominal pain (GI bleeds); IgA nepropathy → hematuria (glomerular/mesangium bleeding)

99
Q

ANCA assc small vessl vasculitis dz with extravasc granulomas (2)?

A
  1. Wegener’s Granulomatosis (cANCA; PR3)
  2. Churg Strauss (pANCA; MPO)
100
Q

ANCA ass small vessl vasculitis dz withasthma and eosionophia (1)?

A

Churg Strauss

hint: pANCA + MPO; also has extravasc granulomas

101
Q

Small vessel ANCA vasculitiis with neither extravasc granulomas/eosinophilia

A

Micro Polyangiitis

hint: pANCA or cANCA

102
Q

Whats the dx?

  1. eosino-med necrotizing granulomas (lung, heart, GI*)
  2. pANCA ∝ severity
  3. progressive, steroid dep asthma + peripheral eoinophilia
A

Churg Strauss

103
Q

Whats the Dx? How would you treat?

  • necrotizing vasulities w/o granulomas (lung + kidney)
  • ø nasopharyngeal involvment
  • ⊕ pANCA (perinucear)
A
  1. Microscopic Polyangiitis
  2. tw: cyclophosphamide + steroids

hint: relapse is common

104
Q
A
105
Q

what’s the Dx? How would you tx?

  • Necrotizing granulomas + vasculitis (nasopharynx, lungs, kidneys)
  • sinusitis, hemoptysis, hematuria
  • ⊕cANCA (cytoplasmic)
  • 82% mortality @ 1 yr
A

GPA (granulomatous Polyangiitis; Wegener’s Granulomatosis)

tw:

  1. Cycophosph/Rituximab
  2. steroids (prednisone)
  3. bactrim prophx (for S.aureus)
106
Q

subglottic stenosis is usually assc with what dx?

A

GPA

107
Q

what’s the dx? How do you tx?

  • Gran vasc in women > 50
  • signs + sx: as ∆vision, headache w/jaw claudn, flu like sx , arthr-/my-algia + ↑ESR
  • vasc biopsy: segnantal lesions w/ giant cells + intimal fibrosis
A
  1. Giant Cell/Temporal Vasculitis
  2. tw: steroids otw blindness!

hint: segmental lesions → false⊖ biopsy; major branches of carotid artery → blindness (opthalmic), HA/jaw (temporal); polymalgia rheumatica –> arthr-/my-algia + ↑ESR

108
Q

(2) what’s the dx? How do you tx?

  • Gran vasc in Asian women < 50 (classically)
  • signs + sx: ∆vision + neuro, weak/absent pulses in UEs + ↑ESR
A
  1. Takayasu Arteritis
  2. tw CCSs

hint: branches of the aortapulseless” dz

109
Q

(2) whats the dx? how would you treat?

  • fibrinoid necr vasculities that spares the lung
  • HbsAg⊕ w/ pears on string renal arterial
  • young adults with htn, mesenteric melena, CNS + sx
A
  1. Polyarteritis Nodosum
  2. tw with CCSs + cyclophosph
110
Q

what’s the dx? how would you tx?

  • Asian kids < 4
  • erythema on palms and soles
  • nonspecf fever + conjunctivitis
  • coronary arteries at risk for 1)thrombosis + MI and 2)aneurysm
A
  1. Kawasaki Dz
  2. tw ASA + IVIG; otw self-limited

hint: the only case that you’d give ASA to a child with fever (usually causes Rye Syndrome)

111
Q

(2) What’s the Dx? How would you tx?

  • necro vasulitis in digits
  • sx: ulcerative, gangrenous → autoamputation of fingers + toes
  • Raynaud’s
  • smokers*
A
  1. Buerger Dz
  2. tw smoking cessation

hint: vasospasm → Raynaud’s

112
Q
A