Step 1 Qank Flashcards
Which chemotherapuetic drugs act on the M-phase of the cell cycle?
Vinca alkaloids (vincristine, vinblastine) Paclitaxel
MOA of vinca alkaloids (vincristine, vinblastine)?
Inhibits microtubule function and spindle formation
MOA of paclitaxel
Reversibly binds to tubulin and hyperstabilizes microtubules that have already polymerized, preventing spindle breakdown
Which drugs are associated with prolongation of QT interval?
Class 1a and III antiarrhythmics
Fibrinous pericarditis is a complication that usually occurs _______ days post-MI.
1-3 days
Presentation of fibrinous pericarditis
Chest pain that is relieved when the patient leans forward; often a pericardial friction rub can be auscultated on cardiac examination
Free wall rupture is a complication that occurs ____ days after MI and can result in ______.
3-14; cardiac tamponade
Presentation of cardiac tamponade
Hypotension, distant heart sounds and elevated jugular venous pressure
B-blockers ________ end diastolic volume by decreasing ____ and _____, permitting greater filling time
increase; HR and contractility
Fibrates increase _______ by increasing PPAR-a activity
HDL levels
Effect of NE on B1 receptor: Positive dromotropic effect, which is an increase in conduction velocity through the AV node, is caused by increase in ___________
inward Ca2+ current
Effect of NE on B1 receptor: Positive inotropic effect, which is an increase in contractility in cardiac myocytes, is caused by an increase in ______
inward Ca2+ current
Effect of NE on B1 receptor: Positive chronotropic effect, which is an increase in HR via SA node, is caused by increase in _________
inward Ca2+ and Na+ current
Aortic stenosis requires increased pressure to open the aortic valve, prolonging the _______ ________ phase
isovolumetric contraction
A potent vasodilator that is first line tx for HTN in pregnancy
Hydralazine
What should hydralazine be taken with to prevent serious side effects?
Taken with B-blocker to prevent reflex tachycardia
Polymyalgia rheumatica is associated with what vascular disease?
Temporal arteritis
Polyarteritis nodosa is associated with what infection?
Hepatitis B infection
What disease is a necrotizing vasculitis which affects small vessels, all of which are typically at the same stage of inflammation, and is associated with MPO-ANCA (p-ANCA)? It commonly affects the lung and kidney and granulomas are absent
Microscopic polyangiitis
Which drug is given to treat Wegener’s granulomatosis and polyarteritis nodosa?
Cyclophosphamide
Diuresis with ____________ is effective in mitigating acute exacerbation of congestive heart failure marked by SOB secondary to pulmonary edema
loop diuretics such as furosemide
What type of virus causes mumps?
Single stranded, negative sense, enveloped RNA virus of the Paramyxoviridae family
Presentation of mumps
Inflammation/swelling of one or both parotid glands and also the testes (orchitis)
Lhermitte phenomenon
A transient shock-like sensation most often triggered by flexion of the neck, seen in MS
Tremor at rest is a symptom of what disease?
parkinsons disease
Intention tremor is a symptom of what disease?
MS
Treatment of CMV
Ganciclovir
Adverse effects of ganciclovir
Leukopenia, neutropenia, thrombocytopenia, renal toxicity
MOA of ganciclovir
Activated to its monophosphate form by viral thymidine kinase and then converted to the triphosphate form.
Ganciclovir triphosphate is used as a substrate for viral DNA polymerase, leading to formation of “faulty” DNA
Side effects of metoprolol
Mild sedation, depression, impotence and exercise intolerance
Treatment of absence seizures
Ethosuximide (first line), or valproic acid
MOA of ethosuximide
Blocks T-type Ca channels
MOA of valproate
Increase Na channel inactivation, increase GABA concentration by inhibiting GABA transaminase
Gabapentin, an analogue of _______, has been approved for the treatment of all seizure types except ________. Gabapentin functions to inhibit high voltage Ca channels
g-aminobutyric acid; absence seizures
Cholinesterase inhibitor toxicity is treated with what?
Atropine, a muscarinic antagonist
_________ is a chelator used to treat lead, arsenic gold, or acute mercury poisoning
Dimercaprol
________ is a direct cholinergic agonist used to treat ileus and urinary retention because it stimulated GI motility and bladder contraction
bethanechol
_________ is a granulocyte colony-stimulating factor [G-CSF] that is used to augment the recovery of bone marrow, preventing infections and decreasing the duration of neutropenia associated with receiving myelosuppresive cancer chemo
Filgastrim
_______ are characteristic of LMN lesions. They are thought to be caused by release of ____________ from degenerating nerve terminal at the neuromuscular junction
Fasciculations; acetylcholinesterase
__________ is a progressive, stepwise cognitive decline. History of HTN, cerebrovascular accident, and neurologic symptoms makes this diagnosis more like likely
Multi-infarct vascular dementia
What is a watershed zone?
The zone supplied by the most distal sections of 2 different arteries
The right internal carotid artery supplies blood to both the right ___________ and _____________.
anterior cerebral artery and middle cerebral artery
In a sarcomere, the _________, which is the length of myosin, does not change upon contraction
A-band
Acoustic neuromas are thought to arise from loss of a tumor-suppressor gene on chromosome ___ and may be a manifestation of ___
Chromosome 22, NF2
___________ are eosinophillic inclusions bodies seen in the cytoplasm of neurons from patients infected with rabies.
Negri bodies
IFN-gamma activates macrophages and TH1 cells. A failure to produce this leads to ___________ (________) characterized by coarse facies, staphyloccal skin/respiratory infections and bone abnormalities
Hyper-IgE syndrome (Job syndrome)
Erbs palsy presents with “_________” arm position and is due to damage of the ___ and ____ nerve roots of brachial plexus
Waiters tip; C5 and C6
____________ is characterized by contralateral muscle weakness, ipsilateral tongue deviation, and contralateral loss of sensation from the body, sparing the face
Medial medullary syndrome
Medial medullary syndrome is caused by infarct of the ______________
Anterior spinal artery
What is the most common ligament affected in ankle sprains?
Anterior talofibular ligament
This disease is characterized by extensive remodeling of bone that results in drastically increased bone mass; often seen in older patients; this heavy bone is more prone to fracture
Pagets Disease
Describe the pertinent lab values seen in patients with Paget’s Disease
Normal calcium (sometimes elevated), normal phosphate, and elevated alkaline phosphatase
__________ is marked by proliferation of fibrous tissue, with occasional small foci of calcification, that causes a loss of elasticity of pericardial tissues; mimics sings if right heart failure
constrictive pericarditis
What are common causes of constrictive pericariditis?
Infections such as SLE, rheumatic fever; post-radiation changes, and viral and bacterial infections
____ is a key cytokine produced by TH1 cells and is a potent stimulator of macrophages. It also has antitumor and antiviral activity.
IFN-g
What interleukin does macrophages secrete?
TNF-a
_______ is diagnosed when an individual presents with several physical complaints in multiple organ systems. Such patients have often undergone several tests, none of which indicate an organic cause. The symptoms must have begun before age 30 and cannot be intentionally produced.
Somatic symptom disorder
Patients with ____________ often engage in self destructive behaviors such as cutting or burning themselves with cigarettes. They have extremely labile moods (mood swings), impulsivity, and unstable relationships. Often exhibit splitting
Borderline personality disorder
Hepatocyte congestion suggest ___________ and _______
right sided heart failure and nutmeg liver
_________ is classically associated with fatalities and fulmitant hepatitis in pregnant women, commonly seen in sub-saharan Africa, spread by fecal-oral route
Hepatitis E infection
Histologic finding of hepatitis infections?
Patchy necrosis of liver cells from focal hepatocyte damage
________ is associated with chronic liver disease and is a result of disorders such as Alcoholism, Wilson Disease, and hemochromomatosis
Micronodular cirrhosis
What disease is characterized by a tyrosine kinase signaling defect that arrests B lymphocytes at the pre-B-lymphocyte stage so they cannot yet produce immunoglobulins. Characterized by low serum IgG, IgM, and IgA levels after the first few months of life
Bruton x-linked agammaglobinemia
Classic physical exam finding of patients with Bruton x-linked agammaglobinemia?
Absence or near absence of tonsils and adenoids, which are B-lymphocyte rich tissues
MOA of clozapine
Blockade of serotonin and dopamine receptors
MOA of chloramphenicol
Inhibits protein synthesis by binding to and inhibiting the 50S ribosomal subunit
MOA of sulfonamide
Inhibits dihyropteroate synthetase, an enzyme involved in folate synthesis
___________ competes with K at the Na/K ATP pump. Thus in hypokalemia, this drug binds more readily to its target, leading to possible toxicity
Digoxin
Adverse effects of chloramphenicol
Aplastic anemia: suppression of all blood lines: erythroid, myeloid and thrombocytes
Results in anemia, neutropenia and thrombocytopenia which present as fatigue, predisposition to infection and excessive bleeding
Hypotonia
also known as floppy baby syndrome, a state of low muscle tone
Which drug do you use for diuresis in patients that are allergic to sulfa drugs
Loop diuretic ethacrynic acid
Angiosarcomas are composed of malignant cells forming vascular channels and are predominantly found in the ___________
right atrium
T or F: Procarbazine is an antineoplastic agent that is also a MAOI
True
The muscles of facial expression are derived from what?
Second branchial arch
The muscles of mastication are derived from what?
First branchial arch
Duchennes muscular dystrophy, which produces weakness starting before age 5, is usually due to what mutation
Frame shift mutation
Beckers muscular dystrophy, which is a less severe form of DMD, results from what mutation
Point mutation of dystrophin gene
Congenital CMV manifests as what? What would you see in CT of the head?
petechial rash, hepatosplenomegaly and microcephaly;
Intracranial calcification
Patients with autism spectrum disorder have an association with which conditions?
Tuberous sclerosis and seizures
MOA of aminoglycosides (gentamicin, neomycin, amikacin, tobramycin, streptomycin)
Bactericidal; irreversible inhibition of initiation complex through binding of the 30S subunit; causes misreading of mRNA
Clinical use of aminoglycosides
Severe gram negative rod infections, synergistic with B-lactam antibiotics
Adverse effects of aminoglycosides
nephrotoxicity, neuromuscular blockade, ototoxicity, teratogen
_________, produced by platelets, stimulates platelet aggregation and vasoconstriction, and will be elevated at the site of a clot.
Thromboxane A2
__________ is synthesized by vascular endothelium and smooth muscle. It decreases platelet aggregation and causes vasodilation
Prostaglandin I2
What are some anticholinergic adverse effects
urinary retention, dry mouth, tachycardia, constipation, and blurry vision
what are cowdry a inclusion bodies?
Intranuclear eosinophilic inclusions surrounded by a clear halo, seen in cells infected with VZV, CMV, HSV
What is locked in syndrome?
Locked in syndrome consists of almost complete motor paralysis (sparing vertical eye movements) with total preservation of sensation, consciousness and reason.
How do patients get locked in syndrome?
It results from occlusion of the basilar artery, disrupting blood supply to the ventral pons
What type of drug is busulfan?
DNA alkylation agent; chemotherapeutic
What type of drug is cisplatin?
Platinum based chemotherapeutic drug that is a DNA crosslinking agent
MOA of doxorubicin
Doxorubicin generates free radicals and results in DNA double stranded breaks
MOA of chemotherapeutic drug etoposide
Inhibits type 2 topoisomerase
Treatment of extrapyrimidal side effects of atypical antipsychotics
Anticholinergic agents like benztropine
_______, which acts by inhibiting mycolic acid synthesis, is the treatment of choice for TB prophylaxis
Isonaizid
____________ is used to help break up mucus plugs in patients with cystic fibrosis
N-acetylcysteine
Patients with CF are at risk for developing ____-______ vitamin deficiencies
Fat soluble vitamin deficiencies (vitamins A, D, E, K)
MOA of carbamazepine
Antiepileptic drug that inactivates Na channels and inhibits N-methyl-D-aspartate receptors
Adverse effects of antiepileptic carbamazepine
Blood dyscrasias (agranulocytosis or aplastic anemia), liver toxicity, diplopia, ataxia and teratogenesis
As the prevalence of disease increases, the PPV ________ and the NPV ________
increases, decreases
Guillan-Barre syndrome is an autoimmune disease characterized by an ascending paralysis caused by ________ and _______ of the PNS
inflammation and demyelination
Intracytoplasmic eosinophilic inclusions, also known as _____________, are seen in ________________
Mallory bodies; alcohol-induced hepatitis
Patients with sickle cell anemia are at risk for osteomyelitis due to ________ or ________
salmonella or staphyloccus
__________ is a major risk factor for osteomyelitis caused by Pseudomonas
IV drug abuse
In multiple myeloma, a common finding seen on peripheral blood smear is a ____________________
Rouleaux formation - single file stacking of RBCs
_________ causes pneumonia in older patients with a history of alcoholism, tobacco use, diabetes, chronic illness or immunosuppression and can be cultured on buffered charcoal yeast extract containing cysteine and iron
Legionella pneumophilla
Mycoplasma pneumonia requires media containing ________
sterols
What lysosomal storage disorder is characterized by a defect in N-acetylglucosaminyl-1-phosphotransferase?
I-cell disease
What happens in I-cell disease
Failure of golgi apparatus to phosphorylate mannose residues on glycoproteins -> proteins are secreted extracellularly rather than delivered to lysosomes
Presentation of I-cell disease
Coarse facial features, clouded corneas, restricted joint movement, and high plasma lysosomal enzymes; often fatal in childhood
__________ is a genetic disease in which fatty substances (sphingolipids) accumulate in cells and certain organs; caused by hereditary deficiency of enzyme glucocerebrosidase (aka glucosylceramidase)
Gaucher Disease
C. diptheriae produces diphtheria toxin. Diphtheria toxin inhibits ______, inhibiting ______ and protein synthesis
EF-2a; translocation
Intracytoplasmic eosinophilic inclusion bodies describe what?
Lewy bodies
Patients with ________ personality disorder are submissive and clinging; they have low self-esteem and have an excessive need to be taken care of
dependent
Patients with ________ personality disorder display excessive emotionality, somatisization, attention-seeking and sexually provacative behavior
Histrionic
Patients with _______ personality disorder are sensitive to rejection and are socially inhibited and timid.
avoidant
Within the carotid sheath, the common carotid artery is _______, the internal jugular vein is _________ and the vagus nerve lies ________
medial, lateral, posterior
_____________ is rapidly fatal and is characterized by hemorrhagic mediastinitis, mediastial widening and bloody pleural effusions
inhalational anthrax; B. anthracis, a gram positive rod in chains with protein capsule
What neurological deficits are seen in syringomyelia (an enlargement of the central canal of the spinal cord, commonly occurring at C8-T1
The crossing fibers of the spinothalamic tract are damaged (bilateral loss of pain and temperature sensation), but dorsal column function remains intact (position and vibration sense)
What is the most common cause of pyridoxine deficiency (Vitamin B6)?
Isoniazid
MEN-1 (Wermer syndrome) is inherited as an autosomal dominant mutation of chromosome 11, as causes a tumor in the “3Ps”: ____________ __________ __________
Pituitary, Parathyroid and pancreas
Arsenic exerts its toxic effects via inhibition of _________, a required cofactor for the pyruvate dehydrogenase complex
lipoic acid
How do you differentiate between child with osteogenesis imperfecta (defect in type 1 collagen synthesis) and victim of abuse?
Look for blue sclera or miscolored and damaged teeth (both seen in osteogenesis imperfecta)
_________ is a gram negative rod that is usually associated with cat scratches. Chronic lymphadenitis would most commonly be seen in young children
Bartonella henselae
_________ is a gram negative coccoid rod that is normally found in dogs, can cause fever, malaise and HSM in humans. Veterinarians are at increased risk
Brucella canis
A-amanitin, toxin found in Amanita phalloides (death cap mushrooms) inhibits _________________, which is responsible for the production of mRNA.
RNA polymersase II
RNA polymerase I is responsible for what?
synthesis of rRNA
RNA polymerase III is responsible for what?
synthesis of tRNA
Acyclovir is an acyclic guanosine derivative that inhibits DNA synthesis in viruses after activation by viral ____________
thymidine kinase
The subscapularis acts to ____________________ the arm
medially rotate and adduct
In addition to causing glossitis, macrocytic megaloblastic anemia, and dementia, vitamin B12 deficiency causes neurological symptoms due to degeneration of what?
Degeneration of dorsal columns, lateral corticospinal tracts, and spinocerebellar tracts
Patients who develop polio experience injury to the nerve roots that supply the ___________
superior gluteal nerve
Areteriovascular malformation and HTN are associated more with which type of brain injury?
Intraparenchymal hemorrhage, more so than subarachnoid hemorrhage which is often due to rupture of aneurysm
What does Km (michealis menton constant) reflect?
the Km inversely reflects the affinity of the enzyme for its substrate
- the higher the Km, the lower the affinity and vice versa
Competitive inhibitors have what effect on Km? How can competitive inhibition be overcome?
Increase Km = decrease the affinity
Competitive inhibition can be overcome by adding more substrate
MTX is administered with what to adverse effects?
Leucovorin (folinic acid)
What enzyme is needed to convert testosterone to DHT
5a-reductase
What enzyme is needed to convert testosterone to estrogen
Aromatase
Where does the lymphatic drainage of the testes go?
para-aortic lymph nodes
What drugs are first line therapy for severe hypertriglyceridemia?
Fibrate and niacin
MOA of fibrates
Increase LPL activity -> increase TG clearance; activates PPARa to induce HDL synthesis
MOA of niacin
Inhibits lipolysis (hormone sensitive lipase) in adipose tissue; reduces hepatic VLDL synthesis
_____________ (degeneration of the dorsal columns of the spinal cord and the dorsal roots) is on the possible manifestations of tertiary syphilis. Symptoms include impaired proprioception and ataxia
Tabes dorsalis
HLA B27 predisposes individuals to what conditions?
Psoriasis, ankylosing spondylitis, inflammatory bowel disease and Reiters syndrome (reactive arthritis)
____________ is an amide anesthetic capable of causing cardiac arrythmias and hypotension
Bupivacaine
________ are macrophages of the central nervous system that, like other tissue macrophages, arise from monocytes in the bone marrow
microglia
HIV encephalitis is caused by infection of the __________ and ________ in the brain
macrophages and microglia
_______________ can be identified on a bone marrow smear by looking for macrophages engulfing RBCs
Hemophagocytic lymphohistiocytosis
Achilles tendon reflex tests which nerve roots?
S1-2 (S1,2 buckle my shoe)
________ reduces alveolar surface tension by disrupting the hydrogen bonds between molecules of water
surfactant
___________ are intermediate cells that immediately precede the terminally differentiated neutrophil and are present during a systemic inflammatory response
band cells
Adverse effects of lithium (which is used to treat bipolar disorder)
Tremor, hypothyroidism, birth defects, polyuria and nephrogenic diabetes insipidus due to ADH antagonism
Amphotericin B toxicity include?
Fever and chills, hypotension, nephrotoxicity, and arrhytmias
SCC is associated with excessive _______, leading to hypercalcemia, and is suggested by a large hilar mass with cavitation in a smoker
PTHrP (parathyroid hormone related peptide)
Polyarteritis nodosa is a systemic necrotizing vasculitis of small or medium sized arteries characterized histologically by _____________. PAN, which is generally antineutrophil cytoplasmic antibody (ANCA) negative, has a strong association with HBV
fibrinoid necrosis
A-hemolytic form what color colonies on blood agar?
green colonies
What is the cofactor required in the hydroxylation of proline and lysine residues in collagen synthesis
Vitamin C, deficiency leads to scurvy
Scarlet fever is caused by?
Strep pyogenes (GAS)
Scalded skin syndrome is caused by?
Staph aureus
Diagnosis of Bipolar II requires?
History of BOTH a major depressive disorder and a hypomanic episode
Diagnosis of Bipolar I requires?
Presence of an elevated, expansive or irritable mood for more than 1 week that causes marked impairment in functioning socially at work, or at home
Vascular type of Ehlers Danlos syndrome is caused by deficiency of what?
Type 3 collagen
What are the trinucleotide repeat diseases?
Huntingtons Disease, Myotonic dystrophy, Friedrich ataxia, Fragile X syndrome
SLE is associated with what renal disease?
Diffuse Proliferative Glomerulonephritis
Which IL is responsible for fever?
IL-1, IL-6, TNFa
IL-3 function
Responsible for stimulating the growth and differentiation of bone marrow.
IL-4 function
Responsible for growth of B cell and enhancing class switching
IL-5 function
Promotes the differentiation of B cells and enhancing class switching to IgA. Also produced and activation of eosinophils, which play a role in defending in parasitic infections
The deltaF508 mutation in cystic fibrosis results in what defect?
Defective processing from the ER to the golgi apparatus
E. coli ferments what?
Lactose
What is first line treatment for pneumococcus infection?
Penicillin or amoxicillin
The reduced GFR in chronic renal disease leads to accumulation of phosphate which does what?
Directly stimulated PTH, suppress renal production of vitamin D3
What genetic disorder is characterized by a constitutive activation of the epithelial sodium channel (ENaC) in the collecting tubules of the kidney. This disease leads to early HTN characterized by low levels of renin and angiotensin leading to hypokalemia and metabolic alkalosis
Liddle syndrome
Why is mannitol, an osmotic diuretic, contraindicated in patients with CHF?
It can cause pulmonary edema
Small cell carcinomas of the lung is associated with what paraneoplastic syndrome?
Cushing syndome via increased ACTH
Squamous cell carcinoma of the lung is associated with paraneoplastic syndrome?
Produces PTHrP -> HYPERCALCEMIA
A palpable thrill of the abdomen just to the left of the midline could indicate what?
Renal artery stenosis
MOA of cyclosporine
Binds to cyclophilin, thus inhibiting calcineurin, blocking production of IL-2 and blocking activation of T-lymphocytes
What immunosuppresant inhibits T-lymphocyte proliferation by inhibiting nucleotide production and DNA synthesis?
Azathioprine
What are some common causes of dilated cardiomyopathy?
Alcohol abuse, wet BeriBeri, Coxsackie B virus, cocaine, Chagas disease, Doxorubicin, hemochromatosis, sarcoidosis, peripartum cardiomyopathy
What is used to treat heparin OD?
Protamine
Pertussis toxin does what?
Inhibition of Gi
Lethal factor, which is released by Bacillus anthracis, is a zinc metalloprotrease that inactivated protein kinase and that stimulated macrophages to release _____
TNFa
A postive quellung test (positive reaction with capsular swelling) indicates what?
S. pneumo pneumonia
MOA of macrolides
Inhibit protein synthesis by blocking translocation of the 23S ribosomal RNA of the 50S ribosomal subunit
MOA of aminoglycosides
Inhibits formation of the initiation complex, causes misreading of mRNA (binds to 30S)
Which antiretroviral drug is associated with crystal induced nephropathy?
Indinavir - protease inhibitor
Obstructive lung diseases is characterized by decreased vital capacity and an increased _________, ________, _________
Increased residual volume, total lung capacity, and functional residual capacity
Restrictive lung disease is characterized by decreased vital capacity, _________, _________, _________
decreased residual volume, total lung capacity and functional residual capacity
Why do both obstructive and restrictive lung disease result in decrease vital capacity?
Because of reduced ability to expire air in obstructive lung disease and reduced ability to inspire in restrictive lung disease
SCID can result from what deficiency?
Adenosine deaminase deficiency
Osmotic demyelination, AKA central pontine myelinosis, can result from overaggressive treatment of ___________
hyponatremia
Unfractionated heparin tends to have dramatic affect on ________________
activated partial thromboplastin time (aPTT)
MOA of amantidine in treatment and prophylaxis of influenza?
Binds to M2 protein on virus to prevent viral uncoating
Aschoff bodies (granulomas with giant cells) are seen in what disease?
rheumatic fever
Negative reaction to the nitroblue tetrazolium test is diagnostic for what disease?
Chronic Granulomatous Disease
What is the substrate for the NADPH oxidase enzyme complex? Phagocytes use NADPH oxidase to convert this to reactive oxygen species.
O2
The fibers of the ___________ that cross at the anterior white commissure can be damaged by the enlargement of the central canal seen in syringomyelia.
Spinothalamic tract
______________ is clinically characterized by progressive bone marrow failure and multiple congenital abnormalities, including radial hypoplasia and thumb deformity
Fanconi anema
What is the dominant component of surfactant
phosphatidylcholine (lecitin)
All the sensory modalities of the face, except for olfactory sensation, pass through the _________________ of the thalamus on their way to the cortex
ventral posteromedial nucleus
__________ is a multisystem disorder caused by defect in DNA repair enzymes. It usually presents with cerebellar problems (ataxia), IgA deficiency and spider angiomas (telangiectasia) of the eye and skin
Ataxia-telangiectasia
____________ is a autosomal recessive disorder characterized by destruction of the anterior horn cells; it presents with diffuse muscle atrophy, hypotonia, fasciculations, and decreased DTR
Werdnig-Hoffman Disease
Imatinib inhibits BCR-ABL _____________ created by t(9:22), which is the defining cytogenic lesion in CML
tyrosine kinase
A noncompetitive inhibitor will have what affect on Vmax and Km?
Decrease Vmax, no chance in Km
Adverse effects of aspirin?
Gastic ulcers, tinitis, reyes syndrome in kids, acute renal failure
OD initially causes hyperventilation -> respiratory alkalosis and later transitions to mixed metabolic acidosis-respiratory alkalosis
MOA of sumatriptan
Serotonin receptor agonist
Ras proteins belong in which families of molecules?
GTP binding proteins
First line treatment for otitis externa is what?
Flouroquinolone
Glucose intolerace, coarsening of facial features, thickened fingers, fatigue and sweating are signs of excessive ____________
GH synthesis
__________ are the only output cells of the cerebellum, and they are all what type of neurons?
Purkinje cells; GABAergic
What is oligohyramnios?
Toot little amniotic fluid
Unlike folate deficiency, vitamin B12 deficiency causes ___________
Neurological deficits
The first branchial arch develops what?
The muscles of mastication
The first branchial cleft gives rise to what?
the external auditory meatus
The 2nd branchial arch develops what?
Muscles of facial expression
The permanent metanephric kidneys found in humans originate from what?
the ureteric bud
Paroxysmal nocturnal hemoglobinuria, caused by ____________, leads to intravascular hemolysis and hemosideren in urine
PIG-A GPI anchor mutation
PE of a patient results in almond breath odor, confusion and headache. What is the patient suffering from?
Cyanide poisoning
What is characterized by degeneration of the anterior horn cells, not tertiary syphillis?
Polio
What is characterized by degeneration of posterior column?
Tertiary syphilis (Tabes dorsalis)
Degeneration of the anterior white commissure results from what?
Syringomyelia
Deficiency of adenosine deaminase can cause what?
SCID
Mutation in ATM kinase, which in involved in dsDNA break repair leads to what?
Ataxia-telangiectasia
MOA of amphetamines?
Promotes extravesicular release of catecholamines into the synaptic cleft
Acute transplant rejection is mediated by what?
CD8 cytotoxic T lymphocytes
Massive blood transfusions carry the risk of _________ caused by the concomittant administration of a large dose of anticoagulant citrate with the blood
hypocalcemia
_________ is a condition that results from injury to the subthalamic nucleus, which is a basal ganglia nucleus
Hemiballismus
MEN 1 neoplasms
Parathyroid tumors, Pituitary tumors, Pancreatic endocrine tumors;
associated with mutation in MEN1 gene (menin, a tumor suppressor)
MEN 2a neoplasms
Parathyroid hyperplasia, Pheochromocytoma, Medullary thyroid carcinoma;
associated with mutation in RET gene
MEN 2b neoplasms
Pheochromocytoma, medullary thyroid carcinoma, mucosal neoplasms;
associated with mutation in RET gene
Injury of what nerve should be suspected in a patient with midshaft humerus break?
radial nerve
What artery runs along the radial groove of the humerus with the radial nerve?
Radial collateral artery
What common thyroid cancer has histo characteristics of “orphan annie eye nuclei” (empty looking ground glass nuclei) and psammoma bodies?
Papillary carcinoma
MOA of phenytoin
Increases Na channel activation, thereby decreasing the flux of Na ions across the neuronal membrane
Toxicity of phenytoin
Gingival hyperplasia, megaloblastic anemia, induction of cytochrome p-450 system
Treatment of choice for tonic clonic seizures?
phenytoin
___________ is characterized by ipsi loss motor function, vibration, and proprioception and the contralateral loss of pain and temp sensation
Brown-Sequard Syndrome, or hemisection of spinal cord
What is the rate limiting step in glycolysis?
Conversion of F-6-phosphate to F-1,6-bisphosphate by phosphofructokinase
(This step is inhibited by citrate and increased ATP)
___________________ promotes the proliferation, differentiation and recruitment of lymphoid cells. It is used to treat RCC and is thought to promote immune mediated antitumor effects
Recombinant IL-2 (aldesleukin)
Tay sachs disease is due to a deficiency of ________________, causing an accumulation of GM2 ganglioside
hexosaminidase A
Oocytes remain in ___________ from birth to ovulation
prophase 1
After ovulation, the oocyte progresses through meiosis 1 and is arrested in ___________ until after fertilization occurs
metaphase II
Which amino acid residue is needed to create thyroid hormone?
Tyrosine
Congenital diaphragmatic hernia is due to failure of what?
Failure of the pleuroperitoneal canal to close completely; leads to protrusion of the abdominal viscera into the chest, usually the left side
What megaloblastic anemia results in BOTH increased MMA and homocysteine?
Vitamin B12 deficiency`
Leukocyte adhesion deficiency is caused by deficiency in what?
Deficiency of B2-integrin and subsequent defects in several proteins including LFA-1
MOA of tetracyclines
Binds at 30S subunite and prevents attachment of the aminoacyl-tRNA
What is Meigs syndrome?
Triad of benign ovarian tumor, ascites and hydrothorax (pleural effusion)
_________ is characterized by proximal muscle weakness and delayed muscle relaxation and may manifest in childhood or adulthood. Caused by nucleotide expansion of CCTG
Myotonic dystrophy
On peripheral blood smear, dacrocytes are a sign of what?
Extramedullary hematopoiesis
What type of pneumocyte proliferates during lung damage?
Type 2 pneumocytes
The presence of abnormal lymphocytes with cytoplasmic projections on peripheral blood smear in addition to signs and symptoms of fatigue, weight loss, splenomegaly and pancytopenia should raise suspicion of what?
Hairy Cell leukemia
Absence of a-ketodehydrogenase leads to what?
Maple syrup urine disease - inability to degrade branched amino acids
A deficiency of metionine synthase would lead to development of what?
Homocysinuria, a disorder characterized by mental retardation, osteoporosis, tall stature, kyphosis and lens subluxation
Function of LH?
Stimulate the production of androgens by theca cells
Familial adenomatous polyposis, which is characterized by rectal bleeding in a 20-40 year old with a family history of colon polyps and/or colorectal cancer, is caused by mutation in what gene?
Mutation on chromosome 5 of APC gene
MOA of bleomycin
Inhibits DNA synthesis by binding to DNA and causing single strand and double strand breaks
The cremaster muscle is formed from what abdominal muscle?
The internal oblique muscle
What is heteroplasmy?
The presence of both normal and mutated mitochondrial DNA. The condition results in variable expression of mitochondrial inherited diseases
What is Trousseau syndrome?
A unique migratory thrombophlebitis that occurs as a results of the liberation of clotting factors, such as tissue factor, by some pancreatic tumors
What antiviral is used to treat Hep C infection? This antiviral is associated with hemolytic anemia
Ribavirin
What is conversion disorder?
Loss of sensory or motor function, often following an acute stressor
Patient presents with painful vaginal bleeding in the third trimester. She smokes, has HTN and uses cocaine. What does she have?
Placental abruption (abruptio placentae)
The cardinal ligaments located at the base of the broad ligements of uterus contain what?
Uterine arteries and veins
How do you calculate attributable risk?
The risk difference: calculated by subtracting the incidence of disease in nonexposed from incidence of disease in exposed persons
___________________ is an autosomal dominant disorder that affects a number of systems secondary to accumulation of porphobilinogen and d-aminolevulinate?
Acute intermittent porphyria
What disease is associated with vertigo, sensorineural hearing loss and tinnitus?
Meniere Disease
B-hCG is structurely similar to what hormones?
FSH, LH, TSH
What autoimmune disease is associated with HLA-DR4?
Rheumatoid arthritis
Adverse effect of milk of magnesia?
Diarrhea
Triple therapy for H. pylori infection?
Clarithromycin, amoxicillin (metronidazole if pt has penicillin allergy), proton pump inhibitor
Where in the spleen are the T cells located?
The periarterial lymphoid sheath
What marker is elevated in hepatocellular carcinoma?
A-fetoprotein
In the vast majority of cases, Down syndrome is due to nondisjunction during what phase?
Anaphase of meiosis 1
What is treatment of E coli O15:H7?
Hydration and avoidance of antibiotics
Antidote for heparin OD?
Protamine sulfate (positively charges molecule that binds negatively charges heparin)
Diagnosis of cryptosporidium infection can be confirmed by what?
Oocytes on an acid fast stain of stool smear
____________ is characterized by the absence or a decreased amount of the normal vWF metalloprotease ADAMTS13
Thrombotic thrombocytopenic purpura
MS can be treated with what cytokine?
B-interferon
What is IL-11 (also known as oprelvekin) used for?
Stimulate megakaryocyte and platelet growth to treat thrombocytopenia
What are the neoplastic cells in multiple myeloma?
plasma cells