COMBANK Random 3 Flashcards
Which enzyme is responsible for the transport of long-chain fatty acids from the mitochondra, and how will pts with deficiency present?
Carnitine palmitoyltransferase; pts with deficiency will present with episodes of muscle pain, stiffness, and tenderness usually during exercises or fasting d/t rhabdomyolysis
Which disease is a debranching enzyme deficiency that is responsible for the breakdown of glycogen and glucose mobilization from the liver and muscle, and how will pts with this deficiency present?
Cori’s disease
-pts with deficiency will present with fasting hypoglycemia and hepatomegaly d/t accumulation of glycogen in the liver but will have normal lactate levels
What does the enzyme acid maltase do?
degrades glycogen to glucose in lysosomes
Which disease presents with an acid maltase deficiency and presents with early death d/t damaged heart, liver, and muscle tissues?
Pompe’s disease
Which enzyme hydrolyzes glucose-6-phosphate to complete gluconeogenesis and glycogenolysis?
glucose-6-phosphatase
Which disease is deficient in glucose-6-phosphatase and how will pts present?
von Gierke’s disease
-pts will present with severe fasting hypoglycemia, hepatomegaly d/t accumulation of glycogen in the liver, and elevated blood lactate
What does the enzyme phosphorylase do?
It releases glucose-1-phosphate from the terminal a-1,4-glycosidic bonds in glycogenolysis
Which disease is a deficiency in liver glycogen phosphorylase and how will these pts present?
Hers’ disease
-Pts will present with mild hypoglycemia, hyperlipidemia, hepatomegaly, and cirrhosis
What diagnostic support can be given for carnitine palmitoyltransferase deficiency?
elevated serum creatine kinase and elevated myoglobin and myoglobinuria (cola-colored urine)
What is diagnostic for Cori’s disease?
elevated dextrin levels
What is diagnostic for Pompe’s disease?
decreased acid maltase levels
What is diagnostic for von Gierke’s disease?
elevated lactate and uric acid levels
What is diagnostic for Hers’ disease?
hypoglycemia and hyperlipidemia
What are the origins of the long and short head of the biceps femoris muscle?
long head: ischial tuberosity and linea alba
short head: lateral supracondylar line of the femur
What is the innervation of the biceps femoris?
long head: tibial branch of sciatic n. (L5-S3)
short head: common peroneal branch of sciatic n. (L5-S2)
What kind of drug is Zidovudine?
aka Azidothymidine (AZT) - nucleoside reverse transcriptase inhibitor (NRTI) used in HIV: it incorporates itself into the growing viral DNA chain
What kind of drug is Efavirenz?
a non-nucleoside reverse transcriptase inhibitor (NNRTI): it does not incorporate itself into the viral DNA chain
What kind of drug is duloxetine and is it safe to use in pregnancy?
SNRI; yes
What is Busprione indicated for and what pregnancy category is it?
generalized anxiety disorder; Category B
What kind of drug is Fluoxetine and what pregnancy category is it?
SSRI used for depression; Category C
What pregnancy category is phenobarbitol?
Category D - only for emergency situations
What two general disorders can valproic acid treat?
epilepsy or bipolar disorder
What is the indication and MOA of Tamoxifen?
Tamoxifen is a selective estrogen receptor modulator that is used as an estrogen receptor antagonist on breast tissue for the treatment of receptor-positive breast cancer
What is the main side effect of Tamoxifen?
It acts as a partial estrogen agonist on the endometrium, thereby increasing the risk of endometrial hyperplasia, polyps, and cancer
What is the effect of Tamoxifen on bone?
It is an estrogen agonist on bone, so it has a protective effect against osteoporosis
What is the difference between tamosifen and raloxifene?
both are SERMs, but tamoxifen increases the risk of endometrial cancer because it is a partial endometrial agnoist while raloxifene does NOT increase the risk of endometrial cancer because it is an endometrial estrogen antagonist
What is the MOA of Enoxaparin?
It is a LMWH that binds antithrombin III and then inhibits factor Xa to prevent clot formation
Which is the safest anticoagulant to use in pregnancy?
enoxaparin - no monitoring required
What is the most common cause of inherited thrombophilia in whites?
Factor V Leiden
-increases risk of thrombosis by making factor Va resistant to the first cleavage of APC
What is the MOA indication of Argatroban?
selectively inhibits thrombin to suppress clotting factors and prevent platelet aggregation
-can be used in individuals with heparin-induced thrombocytopenia
What is the MOA of heparin and why does it need to be monitored in pregnant pts?
heparin binds to antithrombin III and most be monitored in pts bc it has a narrow therapeutic window
What is the MOA and indication of Rivaroxaban?
factor Xa inhibitor that inhibits platelet activation by selectively blocking the active site of factor Xa; indicated for non-valvular afib and DVT/PE porphylaxis
What is the antidote for Rivaroxaban-induced hemorrhage?
no specific antidote, but prothrombin complex concentrate, activated protqhrombin complex concentrate, or recombinant factor VIIa may reverse some effects of hemorrhage
What is the MOA of warfarin?
antagonizes the vitamin-K dependent carboxylation of clotting factors II, VII, IX, and X
What is the hallmark of aortic regurgitation?
high-pitched decrescendo diastolic murmur
-Sx include weakness, severe dyspnea, and wide pulse pressure?
Why does aortic regurgitation present with wide pulse pressure?
d/t insufficiency of aorta, blood flows backward from aorta to LV after systole, decreasing diastolic pressure and widening the pulse pressure
Which murmur presents with crescendo/decrescendo systolic murmur that radiates to the neck?
aortic stenosis
Which murmur presents with apical crescendo/decrescendo systolic murmur characterized by mid-systolic clicks?
mitral valve prolapse
What is the Tx of aortic regurgitation and why?
afterload reducers, i.e. hydralazine and ACEi –> creates less myocardial stress and optimizes LV function
-vasodilators improve systolic function and reduce afterload
In what setting do you use positive inotropes?
in the setting of heart failure
What are systemic side effects of corynebacterium diptheriae?
myocarditis and peripheral neuropathy
What is the term for an allergic response that becomes apparent within minutes of exposure in a sensitized individual?
atopy
What is the difference between mucus viscosity in CF pts vs. asthma pts?
both form mucus plugs in airways, but CF has very viscous mucus while asthma has mucus of normal viscosity
Which asthma drug prevents airway inflammation by inhibiting mast cell degranulation of histamine, leukotrienes, and slow-reacting substances of anaphylaxis?
Cromolyn
Which drug is a muscarinic antagonist that causes bronchodilation for COPD pts?
ipratropium
Which asthma drugs are leukotriene receptor antagonists prescribed for persistent asthma?
Zarfilukast and montelukast
Which asthma drug prevents leukotriene synthesis by inhibiting 5-lipoxygenase and is used as maintenance Tx for persistent asthma?
Zileuton
What is the pathogenesis of asthma-induced asthma?
Aspirin inhibits the COX pathway (which converts arachidonic acid to PGs, prostacyclin, and thromboxane A2) which shifts metabolism of AA to leukotriene synthesis which leads to excess leukotriene production in the lungs resulting in bronchoconstriction and wheezing
What are examples of seronegative spondyloarthropathies?
conditions that present as joint pain in the absence of rheumatoid factor: psoriatic arthritis, ankylosing spondylitis, IBD, and Reiter syndrome
What are common Sx of psoriasis?
silver scaling plaue, nail pitting, salmon patches, and oncholysis - distal separation of nail plate from inflamed underlying bed
What is HLA-DR4 associated with?
RA and DM 1
What is the input and output of the medial geniculate nucleus?
input: superior olive and inferior colliculus of the tectum
output: auditory cortex of the temporal lobe
