MIX Flashcards
Von Gierke Disease (type I) mutated enzyme
glucose-6-phosphatatse
Pompe disease (type II) mutated enzyme
lysosomal alpha-1,4-glucosidase
Cori Disease (type III) mutated enzyme
debranching enzyme (alpha-1,6-glucosidase)
McArdle disease (type V) mutated enzyme
skeletal muscle glycogen phosphorylase
Fabry disease mutated enzyme
alpha-galactosidease
Gaucher disease mutated enzyme
glucocerebrosidase
Niemann-Pick mutated enzyme
sphingomyelinase
Tay-Sachs mutated enzyme
hexosaminidase A
Krabbe disease mutated enzyme
galactrocerebrosidase
metachromic leukodystrophy mutated enzyme
arylsulfatase A
Hurler syndrome mutated enzyme
alpha-L-iduronidase
Hunter syndrome mutated enzyme
iduronate sulfatase
_1 (alpha1) receptor action
increase vascular smooth muscle contraction, increase pupillary dilator muscle contraction (mydriasis), increase intestinal and bladder sphincter muscle contraction
_2 (alpha2)receptor action
decrease sympathetic outflow, decrease insulin reslease, decrease lipolysis, increase platelet aggregation, decrease aqueous humor production
_1 (beta1) receptor action
increase heart rate, increase contractility, increase renin release, increase lipolysis
_2 (beta2) receptor action
vasodilation, bronchodilation, increase lipolysis, increase insulin release, decrease uterine tone (tocolysis), ciliary muscle relaxation, increase aqueous humor production
M1 receptor action
CNS, enteric nervous system
M2 receptor action
decrease heart rate and contractility of atria
M3 receptor action
increase exocrine gland secretions (lacrimal, salivary, gastric acid), increase peristalsis of gut, increase bladder contraction, bronchoconstriction, increase pupillary sphincter muscle contraction (miosis), ciliary muscle contraction (accomodation)
D1 receptor action
relaxes renal vascular smooth muscle
D2 receptor action
modulates transmitter release, especially in the brain
H1 receptor action
increases nasal and bronchial mucus production, increase vascular permeability, contraction of bronchioles, pruritus, pain
H2 receptor action
increase gastric acid secretion
V1 receptor action
increase vascular smooth muscle contraction
V2 receptor action
increased H2O permeability and reabsorption in collecting tubules of the kidney
bethanechol use
post-op ileus, neurogenic ileus, urinary retention
carbachol use
constricts pupil, relieves intraocular pressure in glaucomoa
methacholine use
challenge test for asthma dx
pilocarpine use
open angle and closed angle glaucoma
donepezil use
alzheimer disease
galantamine use
alzheimer disease
rivastigmine use
alzheimer disease
edrophonium use
historically used to diagnose myasthenia gravis
neostigmine use
post-op ileus, neurogenic ileus, urinary retention, myasthenia gravis, reversal of neuromuscular junction blockade post-op
physostigmine use
used to treat anticholinergic toxicity (ie atropine tox, TCA od); is able to cross BBB
pyridostigmine use
myasthenia gravis
atropine use
eye: mydriasis (pupillary dilation), hard to seeairway: decreased secretionsstomach: decreased acid secretiongut: decreased motilitybladder: decreased urgency
homatropine use
eye: mydriasis (pupillary dilation), hard to see
tropicamide use
eye: mydriasis (pupillary dilation), hard to see
benztropine use
Parkinson disease; acute dystonia
glycopyrrolate use
GI (oral administration): drooling, peptic ulcerrespiratory (parenteral administration): pre-op to reduce secretions
hyoscyamine use
GI: antispasmotic for IBS
dicyclomine use
GI: antispasmotic for IBS
ipatropium use
respiratory: COPD, asthma
tiopropium use
respiratory: COPD, asthma
oxybutynin use
GU: reduce bladder spasm and urge incontinence (overactive bladder)
solifenacin use
GU: reduce bladder spasm and urge incontinence (overactive bladder)
tolterodine use
GU: reduce bladder spasm and urge incontinence (overactive bladder)
scopalamine use
CNS: motion sickness
tetrodotoxin symptoms
nausea, vomiting, paresthesias, weakness, dizziness, loss of reflexes after eating poorly prepared pufferfish
ciguatoxin symptoms
temperature related dysesthesia (Cold feels hot)= specific finding; otherwise looks like cholinergic poisoning (diarrhea, urination, miosis, bradycardia, lacrimation, sweating salivation) after eating reef fish
scombroid poisoning
burning sensation in mouth, flushing of face, erythema, urticaria, pruritus, headache; anaphylaxis-like presentation
albuterol use
acute asthma
salmeterol use
long-term asthma, COPD control
dobutamine use
heart failure (inotropic> chronotropic), cardiac stress testing
dopamine use
unstable bradycardia, HF, shock; inotropic/chronotropic effects predominate at high dosese
epinephrine use
anaphylaxis, asthma, open-angle glaucoma (alpha effects predominate at high doses)
isoproterenol use
electrophysiologic evaluation of tachyarrhythmias
norepinephrine use
hypotension
phenylephrine use
hypotension (vasoconstrictor), dilates pupils, decongestant
amphetamine use
narcolepsy, obesity, ADHD
cocaine use
vasoconstriction, local anesthesia
ephedrine use
nasal decongestant, urinary incontinence, hypotension
clonidine use
hypertensive urgency, ADHD, Tourettes
_-methyldopa use
hypertension in pregnancy
phenoxybenzamine use
used pre-op before pheochromocytoma surgery to prevent catecholamine/hypertensive crisis
phentolamine use
for patients on MAO inhibitors who eat tyramine-containing foods
prazosin use
BPH urinary symptoms; PTSD; hypertension
terazosin use
BPH urinary symptoms; hypertension
doxazosin use
BPH urinary symptoms; hypertension
tamsulosin use
BPH urinary symptoms
mirtazapine use
depresson, increases appetite
metoprolol use
angina pectoris, MI (dec mortaility), SVT, hypertension, HF
esmolol use
angina pectoris, SVT, hypertension, HF
carvedilol use
angina pectoris, MI (dec mortaility), hypertension, HF
timolol use
glaucoma
toxin use
antidote/treatment
acetaminophen antidote
N-acetylcysteine
AchE inhibitors antidote
atropine, pralidoxime
organophosphates antidote
atropine, pralidoxime
amphetamines antidote
NH4Cl
antimuscarinic agents antidote
physostigmine salicylate
anticholinergic agents antidote
physostigmine salicylate
benzodiazepines antidote
flumazenil
beta blockers antidote
glucagon
carbon monoxide antidote
100% O2, hyperbaric O2
copper, arsenic, gold antidote
penicillamine
cyanide antidote
nitrite+ thiosulfate, hydroxocobalamin
digitalis (Digoxin) antidote
anti-dig Fab fragments
heparin antidote
protamine sulfate
iron antidote
deferoxamine, deferasirox
lead antidote
EDTA, dimercaprol, succimer, pencillamine
mercury, arsenic, gold antidote
dimercaprol (BAL), succimer
methanol antidote
fomepizole, ethanol
ethylene glycol antidote
fomepizole, ethanol
methemoglobin antidote
methylene blue, vitamin c
opioids antidote
naloxone, naltrexone
salicylates antidote
sodium bicarb
TCAs antidote
sodium bicarb
tPA, streptokinase, urokinase antidote
aminocaproic acid
warfarin antidote
FFP, vit K
Inspiration does what to flow to the heart
increased venous return to RA
Hand Grip does what to flow to the heart
increases afterload
valsalva (phase II)does what to flow to the heart
decreased preload
standing up does what to flow to the heart
decreased preload
rapid squatting does what to flow to the heart
increased venous return, increased preload
Inspiration changes heart sounds how?
increased intensity of right heart sounds
Hand Grip changes heart sounds how?
increased intensity of MR, AR, VSD murmursdecreased intensity of hypertrophic cardiomyopathy murmur MVP: later onset of click/murmur
valsalva (phase II) changes heart sounds how?
decreased intensity of most murmurs (inc. AS)increased intensity of hypertrophic cardiomyopathy murmurMVP: earlier onset of click/murmur
standing up changes heart sounds how?
decreased intensity of most murmurs (inc. AS)increased intensity of hypertrophic cardiomyopathy murmurMVP: earlier onset of click/murmur
rapid squatting changes heart sounds how?
decreased intensity of hypertrophic cardiomyopathy murmur increased intensity of AS murmurMVP: later onset of click/murmur
Alcohol exposure in utero
VSD, PDA, ASD, TOF
congenital rubella
septal defects, PDA, pulmonary artery stenosis
Down syndrome
AV septal defect (endocardial cushion defect), ASD, VSD
Infant of Diabetic Mother
Transposition of the great vessels
Marfan
MVP, thoracic aortic aneurysm and dissection, aortic regurg
prenatal lithium exposure
Ebstein anomaly (tricuspid valve anomaly)
Turner syndrome
coarctation of the aorta, bicuspid aortic valve
Williams Syndrome
supravalvular aortic stenosis
22q11 (Di George)
truncus arteriosus, TOF
VSD is associated with
alcohol exposure, Down syndrome
PDA is associated with
alcohol exposure, Down syndrome, congenital rubella
TGA is associated with
infants of diabetic mother
truncus arteriosus is associated with
digeorge
coarctation of the aorta is associated with
Turner syndrome