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33 Cards in this Set
- Front
- Back
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What accelerates onset of dypnea by 15 yrs in alpha 1 protease inhib deficiency?
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Cigarette smoking
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How is alpha1 protease inhib def inherited?
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Autosomal recessive
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What diseases develop/associated w/ alpha 1 prot inhib def?
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Emypsema, asthma, chronic airway inflammation
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When do symptoms occur for pts w/ Alpha 1 prot inhib def?
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3rd-4th decade
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What’s ratio of protease: anti-protease in lung?
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1:1
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What organs involved in alpha1 prot inhib def?
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Lung, liver, skin (panniculitis)
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Which genotype accounts for most of lung disease?
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piZZ
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What does null refer to in A-1PID?
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Complete lack of production of protein, not associated w/ liver disease
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Which genotype associated w/ liver probs?
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ZZ
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What does smoking do to intrapulmonary proteinase inhibitors?
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Causes oxidnt inactivation
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When is it indicated to do replacement therapy for pts w/ A1 PID?
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Only adult pts whose serum concentrations < 0.8 g/dL AND FEV1 b/w 35-65 or annual decline of FEV1 > 100 mL;
-not recommended for liver diz |
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How are 4 forms of pulmonary alveolar proteinosis in kids inherited?
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1. Congenital errors of surfactact
2. Aq’d PAP w/ and w/o anti GMCSF 3. Idiopathic 4. Secondary (infx, malignancy, environmental) |
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What’s the therapy for symptomatic pulmonary alveolar proteinsosis ?
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5. Whole lung lavage
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Whates role of GMCSF in PAP ?
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6. Help in surtactant metabolism;
7. –along w/ ABCA3: |
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How dx PAP?
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-Lung bx confirms dx; presence of anti-GMCSF in BAL and serum;
HRCT: crazy paving; lung fx: restrictive pattern w/ low DLCO |
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What are causes of pulm edema? 4
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Increased microvascular pressure scecondary to CV diz (DAD)
-high altitude pe. -Neurogenic -Lung re-expansion ** others: due to postcapillary pum venous obstruction; altered pulm endothelial cell permability |
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What are sx of pullm ed0ma?
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Tachypnea secondary to hypoxemia and stimulation of J receptors; crackles; bronchodilator responsive rhonchi and wheezes; rtx – due to poor compliance;
-resp failure |
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How tell diff from cardiogenic vs non cardiogenic pulm edema ?
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Cardiogenic: large heart**; inverted pulm blood flow distrib**; peribonchial cuffs; pleural effusion** very common!!;
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What’s driving forces that promote fluid influx from cap bed into interstitial spaces?
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-increased driving pressure (PAOP > 12 and/or greater than pulm artery diast pressure
-increased permeability -both |
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What happens to v/q in pulm edema?
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v/q mismatch; also can cz small airway obstruction , wheezing; impaired lympatic draininage
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What does pulm edema do to pulm vascular resistance ?
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Increases it
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What happens to oncotic pressure and its reslationship w / pulm vasc pressure and its ~ to pulm edema?
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Low oncotic pressure reduces pulmonary vasc pressure at which pulm edema develops
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What does pulm edema do to airway resistance ?
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Increases it
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What does PEEP do to pulm edema?
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Redistributes but doesn’t reduce lung water content in pts w/ pulm edema
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What predisposes a patient to pulm emboli/infarction?
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Virchow’s triad: stasis, endothelial injury; hypercoaguability
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What’s causes of PE in kids?
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Central line -2/3 causes; risk factors: immobility, trauma, surgery, infx, malig; CHF; IBD; estrogen, preng
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What’s supportive tx for pulm edema?
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Reverse hypoxemia; reduce preload (sitting, postivie pressure vent); sedation (decreases preload, afterload); decrease plasma volume (Diureteics)limit salt/water
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What genetic diz predisposes kids to PE?
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Factor V leiden def; prothrombin gene mutations; antithrombin def; protein s/c def; combined defects increased plasma lipprotin
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Child presents w/ tachypnea, cough, crackles, hemopsysis, tacchy; accentuated second heart sound, sudden collapse; what’s dx?
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Pulm embolis; hx/o central venous cath
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Pt w/ pain, swelling, discoloration of affected limb, superior ena cava syndrome: ?
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Sign of DVT
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What’s best dx test do get for detecting PE?
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Spiral ct/helical ct/ angiograpy or V/Q scan
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What dx tests help w/ PE?
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D – dimer; cbc w/ leulocytosis; non specific ST segment and T wave changes;
CXR: atelectasis; pleural effusion |
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What meds are good to give pts to prevent pulm embolism in immobilized pts?
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Low molec weight heparin
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