Rx for Restrictive Lung Disease, PAH, and CF- Sweatman Flashcards
Treatment of Neonatal Respiratory Distress Syndrome?
Administration of antenatal corticosteroids (dexamethasone and betamethasone) to all women at risk of delivery less than 34 weeks
What do all the surfactant drugs end in?
and name them (3)
-actant; Poractant alfa, Calfactant, Beractant –> given to neonats that don’t produce enough surfactant
Treatment of sacroidosis?
Glucocorticoids and Methotrexate
How does methotrexate help with immunosuppression?
Increase in adenosine-mediated immunosuppression
Is Methotrexate front line therapy for sarcoidosis?
No; too many adverse effects
What is the treatment for Idiopathic Pulmonary Fibrosis?
There is none –> don’t use anti-inflammatories
What Rx are used in the treatment of Wegner’s granulomatosis? Which is labeled for this purpose?
Rituximab (labeled; anti-CD20), Azathioprine (DNA/RNA synthesis inhibitor), Cyclophosphamide (alkylating agent), and Glucocorticoids (inflammation and shit)
Which class of Rx are the only drugs used for systemic arterial HTN that are also useful in treating pulmonary arterial hypertension?
Calcium channel blockers
Which calcium channel blockers are used in treatment of pulmonary arterial hypertension?
Diltiazem, Nifedipine ER, Amlodipine
Why is Verapamil not used to treat pulmonary arterial hypertension?
Depresses cardiac function too much
strong negative inotropic effects - more likely to produce symptomatic bradycardia than the others
Name the Prostanoids
Epoprostenol, Iloprost, Trepostinil
Which prostanoid causes hemoptysis?
Iloprost
What is the most common ADE associated will all prostanoids?
Bleeding; patients need to be monitored, especially if on anticoagulants
Administration of prostanoids?
IV (epoprostenol), inhaled (iloprost), SC (trepostinil)
NOT oral
Name the Endothelin-1 Receptor antagonists
Bosentan and Ambrisentan –> both are teratogenic
Block SMC proliferation and pulmonary arterial vasoconstriction
Unique ADE of Bosentan?
Significant liver toxicity; need to monitor LFTs
What medication can you not take Type V phosphodiesterase inhibitors (boner pills) with?
“Do not take Viagra if you are currently taking Nitrates for chest pain”
“if you have an erection lasting for more than 4 hours, you should go to the hospital”
Listen, if I have a hard on for more than 4 hours, it’s my girlfriend that will need to go to the hospital
Do all patients with pulmonary arterial hypertension respond to calcium channel blockers?
No; some even have life threatening circulatory collapse –> use vasodilator challenge before prescribing
Which CF patients can you not use Ivacaftor on?
Those homozygous for F508del;
90% of CF patients have at least one copy of the DeltaF508 mutation
How does Ivacaftor work?
improves chloride transport through mutated channels
What is the effect of combining Ivacaftor and Lumacaftor?
Lumacaftor is a conformation stabilizer drug; end result is delivery of a fully functional protein to the apical surface of the cell and potentiation of that channel’s effect
Pregnancy category of the Endothelin-1 Receptor antagonists?
Bosentan and Ambrisentan
Category X; severely teratogenic
What CF patients does Ivacaftor work on? what deletion?
G551D mutation
what 2 drugs are used for CF?
Ivacaftor and Lumacaftor
What drugs can induce ARDS?
Aspirin, cocaine, opioids, phenothiazines (promethazine), tricyclic antidepressants (amitriptyline)
What si the treatment for Goodpasture’s syndrome?
Plasmapheresis to remove anti-GBM antibodies