explain what familial hypercholesterolemia is. name 3 S&S.
it is a common hereditary deficit in LDL tissue receptors, this causes low tissue LDL and persistent high serum LDL. this increases risk of atherosclerosis and alternate deposition of cholesterol.
high serum LDL, atherosclerosis, xanthomas
what would we check for BW in patients with hypercholesterolemia?
we would check for complete PLT count, especially in patients on anticoagulants/platelets
4 causes of coagulation deficits
thrombocytopenia = bone marrow deficit, DIC, cancer
vitamin K deficiency = lack of coagulation factors
liver disease = infection, cirrhosis, drug-induced
inherited disorders
3 coagulation disorders
von Willebrand disease
Hemophilia A
Factor V deficiency
explain what von Willebrand disease is and how it causes disease; name 2 drugs and what their function is
von Willebrand disease is autosomal recessive/ dominant; a deficiency of vWF causes bleeding.
TX: desmopressin (synthetic), vasopressin (biologic) = stimulates vWF endothelial cell synthesis and secretion
explain what Hemophilia A is and who is more likely to get it. determine 2 TX and how long it is treated for.
it is a factor VIII deficiency that causes bleeding. it is an X-linked recessive disorder (more likely in men)
TX: synthetic factor VIII and FFP; these are taken for life d/t t1/2
how many types does factor V deficiency have? explain what each cause
factor V deficiency has 2 types: acquired and inherited
acquired: factor V antibodies (immune)
inherited: Owren’s disease = autosomal recessive (lack of clotting factor V)
what are 2 common things patients with coagulation deficiencies should avoid?
should avoid contact sports and restrictive diets
provide an example of a hypercoagulation disorder. explain how hypercoagulation occurs and what it puts patients at risk of.
Factor V Leiden = autosomal (incomplete dominance)
anti-coagulation substance, protein C cannot bind to anti-coagulate as need, leads to a risk of hypercoagulation.
patients are at risk for DVT and PE
4 thrombus risk factors caused by tissue injury
inflammation, surgery, atherosclerosis, infection
1 thrombus risk factor caused by high viscosity
dehydration (low plasma)
2 thrombus risk factors caused by venous stasis
immobility and arrhythmias
2 thrombus risk factors caused by smoking
cytotoxicity and ROS
2 thrombus risk factors caused by drugs
HIT and birth control pills
how do birth control pills pose a thrombus risk?
high estrogen causes increased hepatic coagulation factor synthesis and decreases clot lysis
what is the most common site of hypercoagulation?
DVT
3 S&S of DVT, how is it diagnosed? determine ER TX and longer term TX (2)
S&S: affected site edema, pain, erythema distal to clot
DX: ultrasound
ER TX: heparin
LT TX: LWMH SC, Apixaban PO
what is the most common cause of PE? determine 4 S&S of PE, how it is DX, 2 ER TX and 2 longer term TX
DVT = most common cause
S&S: chest pain, SOB, low SpO2, compensatory mechanisms
DX: CT scan
ER TX: thrombolytics + heparin
longer term TX: LWMH SC, Apixaban PO
3 ways PE can be prevented post surgery
early mobilization post surgery, compression stockings, and heparin + LWMH TX pre/during/post surgery
3 risk factors of pulmonary HTN
chronic small pulmonary embolisms, COPD, heart failure
5 S&S of pulmonary HTN
cough, SOB, lethargy, low SpO2, signs of right ventricular dysfunction (peripheral edema, JVD)
ER TX + 1 drug for pulmonary HTN + 2 TX
ER TX: phosphodiesterase inhibitors = drug: Nipride (nitroprusside)
TX: decrease preload (diuretics); prevention of cor pulmonale (RHF)
describe the MOA of phosphodiesterase inhibitors, describe its effects on PDE3 and PDE5
inhibit phosphodiesterase (PDE), increasing cyclic monophosphate activity
PDE3 inhibition = increased contractility in myocardial cells
PDE5 = vasodilation in endothelial cells
name 2 PDE3 selective drugs, name 3 PDE5 selective drugs. describe 2 things they TX.
PDE3 selective (-none): Milrinone, Amrinone
PDE5 selective (-afil): sildenafil (Viagra), vardenafil, tadalafil
TX: pulmonary HTN + acute HF
