"High Yield or U-World Only" Step 1 Cards Flashcards
IV Benzodiazepines
- Main one
- Indication
- MOA
- Lorazepam
- Intitial drug of choice for status epilepticus
- They work by enhancing the effect of GABA at GABA -A receptor, leading to increased chloride influx and suppression of AP firing
What is Vernet syndrome?
It is when you have lesions of the jugular foramen, thus leading to CN Ix, X, and XI dysfunction
What are lamellar bodies?
They are organelles which contain parallel stacks of membrane lamellae and are a component of type II pneumocytes. They function to store and release pulmonary surfactant.
Normal Vitamin E function
Protection of fatty acids against oxidation
What is responsible for swelling in myocardial cells during ischemia?
Ion pump failure due to ATP loss, leading to increased intracellular Na+ and Ca2+
Between (Na+, K+, Cl-, and Ca2+):
Which are mostly located intracellularly? Extracellularly?
Intracellular: K+
Extracellular: Na+, Cl-, Ca2+
What path does fetal blood take from the placenta to the heart?
Umbilical vein - liver - ductus venosus - IVC - heart
What type of Bacteria are capable of surviving boiling?
Spore forming
What are Hamartomas? How do they present? What are they composed of?
The most common benign lung tumor
Present as asymptomatic peripherally located “coin lesion” in patients 50-60 y/o
Composed of disorganized cartilage, fibrous and adipose tissue
What is responsible for the clinical variability of mitochondrial diseases?
HETEROPLASMY
The mixture of (2) types of genetic material
Lidocaine
- What class drug?
- MOA?
- Effect?
- IB antiarrhythmic
- Binds (mostly) to inactivated sodium channels and rapidly dissociates
- Effective in suppressing v.tach induced by rapidly depolarizing and ischemic myocardium.
Primase
How is it related to uracil showing up in partially replicated DNA strands?
A DNA-dependent RNA polymerase that incorporates short RNA primers into replicating DNA
Because it is composed of RNA, this primer could have uracil in it
Lung mass + hyponatremia, is suggestive of what?
SIADH (secondary to Small cell lung carcinoma)
Pertussis (Whooping cough)
- Who gets it?
- Describe the (3) phases
- What causes it?
- Was mostly in kids prior to vaccinations. Now usually adolescents/adults w/o a booster
- (phases)
- Catarrhal stage- similar to many routine URI’s
- Paroxysmal stage- severe coughing spells w/ classic whoop (post-tussive emesis)
- Convalescent stage- during which cough improves
- Caused by gram-negative Bordetella pertussis.
Thiazide diuretics
- Indication
- MOA
- Effect on serum Ca2+
- Primary HTN
- Inhibit the Na+/Cl- cotransporter in the distal tubule, leading to inc. excretion of Na+ and H2O (as well as K+ and H+ ions)
- Increases distal tubular Ca2+ reabsorption, causing both hypercalcemia and hypocalciruria
What effect does left ventricular HF have on lung compliance?
Leads to fluid accumulation in the lung interstium resulting in decreased lung compliance.
What artery is responsible for supplying the occipital lobe?
Posterior Cerebral Artery
Linkage disequilibrium
When a pair of alleles are inherited together in the same gamete (haplotype) more or less often than would be expected given random chance
Which components of the skin drain to the superficial inguinal lymph nodes?
All skin from the umbilicus down, including the anus (below the pectinate line), excluding the testes, glans penis, and posterior calf
Name the muscles/areas that the musculocutaneous nerve innervates/provides sensory innervation to (3)
- Movement to Major forearm flexors
- Movement Corcobrachialis
- Sensory to lateral forearm
Theophylline
- Indication
- MOA
- Metabolization
- AE
- Used as an alternate therapy for asthma and COPD
- Adenosine receptor antagonist and phosphodiesterase inhibitor that causes bronchodilation by increasing cAMP levels and has mild anti-inflammatory effects
- Metabolized predominantly by hepatic cytochrome oxidase
- AE: Theophylline toxicity
After invading the nasopharnyx, how does N. meningitidis gain access to the brain?
Bloodstream followed by choroid plexus
Between positive/ negative predicative values, and sensitivity/ specificty, which are dependent on disease prevalence in the tested population?
Positive/ negative predictive values
Name the Glycogen Storage Diseases
Very Poor Carbohydrate Metabolism
- Von Gierke Disease
- Pompe Disease
- Cori Disease
- McArdle Disease
Describe the Chvostek sign and the Trousseasu sign associated with DiGeorge Syndrome
The hypocalcemia assc. with DGS leads to increased neuromuscular excitability.
Chvostek sign- tapping on the facial nerve elicits twitching of the nose and lips
Trousseau sign- inflation of the BP cuff leads to carpal spasm
Beta Blocker MOA
They dec. AV nodal conduction, leading to an increased AV nodal refractory period
What is the common clinical presentation for a pneumothorax?
What is often the cause of spontaneous pneumothorax?
- Sudden unilateral chest pain
- Hyperresonance
- Absent breath sounds
- Often seen in tall, thin males around age 20
Can be the spontaneous result of the rupture of an apical subpleural bleb
Describe the specific effect of the superantigens assc. with TSS
THey interact with MHC II on APCs (macrophages) and w/ T-cell receptor to widely activate T cells.
(this leads to IL2 release from T cells, and TNF / IL1 release from macrophages, ultimately causing shock symptoms)
Effect of ACE-I on GFR
Decreases GFR (because AT-II can no longer constrict the efferent arteriole)
Following focal ischemia in an MI, how long until there is a loss of cardiomyocyte contractility?
Loss of focal contractility occurs within 60secs!
Polyarteritis nodosa
- Pathology
- What organ is normally spared?
- Presentation
- Potential consequences
- Segmental, transmural, necrotizing nflammation of medium to small sized arteries in any organ
- Lungs are spared
- Can be cutaneous manifestations, including livedo reticularis and palpable purpura
- Inflammation can result in ischemia, infarcation, or hemorrhage and bead-like aneurysm formation
Presentation/ potential complications of femoral hernias
They can present with groin discomfort or manifest with a bulge on the upper thigh.
Incarceration and strangulation are common complications of femoral hernias.
Describe the essential differences in excitation-contraction coupling in cardiac vs skeletal muscle
Cardiac Muscle: surface membrane depolarization → opening of voltage-sensing DHPR channel → Ca2+ dependent activatin of RyR2 channel (calcium induced calcium release)
Skeletal Muscle: surface membrane depolarization → conformational change in voltage-sensing DHPR channel → allosteric activaton of RyR channel (mechanical coupling)
What is Trousseau sign? What does it indicate?
It is when superficial venous thromboses may appear in one site, resolve, and occur in another site. This often indicates visceral cancer.
The proximal ureter receives its blood supply from what artery? The distal ureter?
Proximal: renal artery
Distal: superior vesical artery
Consequences of chronic gout
Tophi (uric acid crystals in the soft tissues/joints), or renal failure due to uric acid deposition in tubules
Role of muscarinic agonists, in glaucoma tx
Increase trabecular outflow of aqueous humor
What drugs are/can be given with L-DOPA to reduce their peripheral metabolism?
Carbidopa- a DOPA decarboxylase inhibitor
Entacapone- a COMT inhibitor
These both increase levadopa bioavailability to the brain
What are the cutaneous neurofibromas assc. with NF1, derived from? What is its embryological derivation?
They are comprised mostly of Schwann cells, which are embryologically derived from the neural crest
Describe the main roles for IL-1 through IL-6 when secreted by macrophages
Hot T-Bone stEAK
IL-1: fever (hot)
IL-2: stimulates T cells
IL-3: stimulates bone marrow
IL-4: stimulates IgE production
IL-5: stimulates IgA production
IL-6: stimulates aKute-phase protein production
Fibrate medications
- Function
- MOA
- AE
- Dec. LDL and TG; Inc. HDL
- MOA: Upregulate LPL, resulting in inc. oxidation of fatty acids; also, inhibit cholesterol 7a-hydroxylase, which catalyzes rate-limiting step of bile acid synthesis
- Reduced bile acid production results in dec. cholesterol solubility, favoring cholesterol stone formation
Order the class one subcategories based on sodium-channel-binding strength (as measured by use dependence)
1C > 1A > 1B (least use dependent)
What receptors on the juxtaglomerular cells are responsible for stimulating renin release? What drugs take advantage of this fact, to decrease BP?
B-1 receptors (which is why B-blockers can help dec. blood pressure)
Nitroprusside
- MOA
- Effect
- Indication
- Short-acting balanced venous and arterial vasodilator
- Decreases both preload and afterload, thus maintaining stroke volume
- Indicated for hypertensive HF
How can severe chronic rheumatoid arthritis lead to spinal cord injury?
It can cause joint destruction with verterbal malalignment (subluxation), particularly at C1. Extension of neck (particularly during ET placement), can worsen the subluxation, leading to acute compression of the spinal cord and/or vertebral arteries
Osgood-Schlatter Dz
- Presentation
- Etiology
- Focal pain + swelling at the tibial tuberosity
- Repetitive quadriceps contractions in adolescent (quads are attached to the tibial tuberositt, via the patella)
Describe the Sx of Digoxin Toxicity
(what ion is often elevated?)
- Typically presents w/ cardiac arrhythmias and nonspecific GI, neuro and visual (color change) sx
- Elevated K+ is another sign of digoxin toxicity (due to inhibition of Na-K-ATPase)
- What is the cofactor of phenylalanine hydroxylase?
- What would be the presentation for a deficiency in either of these enzymes?
- Tetrahydrobiopterin
- Accumulation of phenylalanine in body fluids and the CNS. Homozygous infants are normal at birth but gradually develop severe intellectual disabilities and seizures. May also have hypopigmentation of skin, hair, eyes, and catecholaminergic brain nuclei
Maturity-onset Diabetes of the Young
- Presentation
- What is the main pathogenesis? Describe in detail
- Potential Consequences
- MIld, nonprogressive hyperglycemia that often worsens with pregnancy-induced insulin resistance
- Often a result of mutation of the glucokinase gene. Glucokinase = glucose sensor. Mutation leads to dec. beta cell metabolism of glucose, less ATP formation, and dec. insulin secretion
- Could lead to fetal growth retardation and sever hyperglycemia at birth
What are the (3) tx for C.diff and in what situation would you use each?
- Metronidazole (Initial mild/moderate C. Diff tx)
- Vancomycin (Severe or recurrent C. Diff)
- Fidaxomicin (recurrent C. Diff and inc. risk of recurrence)
Intraventricular Hemorrhage
- Most frequent occurance is in what population?
- Pathology?
- Presentation?
- Usually in infants born before 32 weeks gestation or with a low birth weight
- IVH in premies usually originates in germinal matrix, where neurons and glial cells migrate out during brain development
- Clinically silent or present with altered level of conciousness, hypotonia, and decreased movement. Symptoms of catstrophic bleeding are the same as those of increased ICP.
In Kallmann syndrome, there is an absence of GnRH secretory neurons in the _____________
hypothalamus
What is the affect of occluson of the anterior cerebral artery?
Disruption of sensory and motor function of the contralateral leg and foot, while sparing the contralateral arm/face
Presentation of Meckel diverticulum
Most often presents with painless melena (gastric tissue can cause ulceration leading to lower GI bleed). The diverticulum may also become inflamed and simulate presentation of acute appendicitis.
Treacher-Collins syndrome
- Path?
- What is the effect?
- Genetic disorder resulting in abnormal development of the first and second pharyngeal arches
- Result: (1) Craniofacial abnormalities which often compromise airway and feeding; (2) absent or abnormal ossicles leading to profound conductive hearing loss
What is the role of transketolase and transaldolase? What cells can use them and for what purpose?
They carry out the nonoxidative reactions of the HMP shunt (Pentose Phosphate Pathway)
All cells can synthesize ribose from fructose-6-phosphate using the nonoxidative rxns.
Path of mitochondrial myopathy
What does muscle biopsy show?
Myopathy + Lactic Acidosis due to failure of oxidative phosphorylation. Muscle biopsy often show “ragged red fibers”
What is the composition of fetal hemoglobin?
a2y2 (alpha 2, gamma 2)
Chloramphenicol AE
This antibiotic can lead to both dose-dependent (reversible) cytopenias and dose-independent (irreversible) aplastic anemia.
Th1 vs Th2
What do they secrete? What do they recruit/activate? How are they activated? How are they inhibited?
Both are CD4+ Helper T-cells
Th1
- Secretes IFN-y
- Activates macrophages and cytotoxic T cells
- Activated by INF-y and IL-12
- Inhibited by IL-4 and IL-10 (from Th2)
Th2
- Secretes IL-4, 5, 10, and 13
- Recruits eosinophils for parasite defense and promotes IgE production by B cells
- Activated by IL-4
- Inhibited by IFN-y (from Th1 cells)
What percentage of vessel must be blocked to qualify as stable angina?
> or equal to 75%
What tumor type(s) is the following tumor marker assc. with?:
HCG
- Choriocarcinoma
- Germ cell tumors
How can malignant, nonseminomatous germ cell testicular tumors cause hyperthyroidism?
They secrete very high levels of hCG, which can bind the TSH receptor (leading to paraneoplastic hyperthyroidism)
Ataxia-telangiectasia
- Inheritance
- Path
- Sx/Presentation (4)
- Autosomal-recessive
- Result of a defect in DNA-repair genes, making the genes hypersensitive to ionizing radiation
- Presents as child w/ (1) cerebellar ataxia, (2) oculocutanoeous telangiectasias, (3) repeated sinopulmonary infxn, (4) inc. incidence of malignancy
What does the candidal antigen test assess?
The activity of T cell-mediated immunity via recruitment of macrophages, and CD4+ and CD8+ T lymphocytes
What is the best non-surgical tx for cholesterol gallstones?
Hydrophilic bile acids (eg ursodeoxycholic acid). These decrease biliary cholesterol secretion and increase biliary bile acid concentration, improving cholesterol solubulity.
What enzyme is responsible for the conversion of NE to Epi? What upregulates this enzyme?
Phenylethanolamine-N-methyltransferase (PNMT)
It is upregulated by cortisol
What tumor type(s) is the following tumor marker assc. with?:
CA 19-9
Pancreatic
Diabetic Mononeuropathy
- Usually involves which CN?
- Pathology
- Sx (3)
- CN III is most commonly involved
- Caused predominantly by central ischemia
- Sx: ptosis, “down and out” gaze, normal light/accomodation reflexes
Buproprion
- Indication?
- What key side-effect does it NOT have?
- Contraindications/ Adverse Effects?
- First-line tx for major deppression disorder
- Does not cause sexual dysfunction (unlike the SSRIs)
- Can lead to seizures so contraindicated in any patient with seizure disorders or prior dx of bulimia/anorexia
Describe the Chloride Shift
- Carbonic Anhydrase activity w/in erythrocytes forms bicarb from CO2/water
- Many of the bicarb ions diffuse from the RBC to the plasma
- To maintain electroneutrality, chloride ions diffuse into the RBC
Which cells found in the lungs, contain elastase? How do they keep it controlled?
Macrophages (control w/ Tissue Inhibitos of Metalloproteinases- TIMPs)
and
Neutrophils (control w/ serum a1 antitrypsin)
Bisphonates have a chemical structure similar to _____________
Pyrophosphate
Paroxysmal Supraventricular Tachycardia
- Presentation
- Tx
- Potential SEs of tx (4)
- Sudden onset palpitations
- Tx w/ adenosine
- SE: (1) Flushing, (2) chest burning (from bronchospasm), (3) hypotension, (4) AV block
What is the indication for drugs which act on H1 receptors vs H2 receptors
H1 = allergies
H2 = acid (GERD, ZE, etc.)
Both are histamine receptors
Tx of Giant cell (temporal) arteritis
Tx with high dose corticosteroids (to prevent blindness), and then get a biopsy
What are the (4) stages associated with Lobar Pneumonia? Describe the exudate.
- Congestion (first 24 hours): Vascular dilation; exudate contains bacteria
- Red hepatization (days 2-3): erythrocytes, PMNs and fibrin in exudate
- Gray hepatization (days 4-6): RBCs disintegrate. Exudate contains PMNs and fibrin
- Resolution: Enzymatic digestion of exudate
What is the presentation for Hemolytic Uremic Syndrome? (3) What is the etiology?
- Microangiopathic hemolytic anemia
- Thrombocytopenia
- Renal insufficiency
Result of 0157:H7 E.coli, usually in undercooked ground beef
Describe the pathogenesis of Polymyositus
Over-expression of MHC-I on the sarcolemma leads CD8+ infiltration, and subsequent myocyte damage
What molecule does insulin promote activation of, in order to increase glycogen synthesis?
Protein phosphatase, via the PI3K pathway
The KRAS gene is a member of what gene subfamily?
Ras
- Tetrahydrobiopterin (BH4), serves as cofactor for which enzymes?
- What enzyme is responsible for forming BH4?
- What effect would a deficiency in BH4 have?
- Important cofactor for the Phenylalanine hydroxylase and Tyrosine hydroxylase
- Dihydropteridine reductase is responsible for converting BH2 to BH4
- Deficiency would lead to inc. phenylalanine (can be corrected by diet) and def in tyrosine conversion to its downstream products such as dopamine, NE, Epi, and serotonin (leading to neuro deterioration)
Describe Pompe Disease
- Deficient enzyme?
- Pathology
- Presentation
- Acid a-glucosidase
- Acid a-glucosidase is responsible for breakdown of glycogen w/in lysosomes. Its absence leads to glycogen accumulation w/in liver and muscle lysosomes
- Presentation: Pompe trashes the Pump (heart, liver, and muscle) – cardiomegally, hypertrophic cardiomyopathy, excercise intolerance, hypotonia, etc.
What (2) drugs are selective vasodilators of coronary vessels? How can they affect the heart negatively during ischemic events?
Adenosine and dipyridamole
They can lead to coronary steal because they vasodilate everything in the heart. The vessels going to ischemic areas are already maximumly dilated so they only lose out on blood.
Describe the hemodynamic profile of aortic regurg (3 key differences from normal)
- Higher pressure peaks (more blood in LV)
- Loss of dichrotic notch
- Steeper fall in aortic pressure
What are the only cells within atherosclerotic plaque which are capable of synthesizing collagen isoforms and ECM?
Vascular Smooth Muscle Cells (VSMCs)
The valgus stress test indicates injury where?
Injury with the MCL (test ability to passively abduct knee)
What is the most common location for acoustic schwannomas?
Cerebellopontine angle
Transudate vs exudate
Transudate- plasma only. Due to hemodynamic changes
Exudate- plasma + ions. Due to structural damage
Permissiveness
When one hormone allows another to exert its maximal effect (ex. cortisol allows NE to work even better although cortisol has no direct vascular effect itself)
What is the clearence mechanism for inhaled particles which are…
- 10-15 um?
- 2.5-10 um?
- less than 2um?
- 10-15 um: Trapped in the upper respiratory tract
- 2.5 - 10um: mucociliary transport
- <2.5um: phagocytized
Nocardiosis (Nocarda asteroides infection)
- Key micro characteristics
- Epidemiology (where is it, how does it get to people and who does it affect?)
- Clinical features (3)
- Tx
- Gram positive rod (beading/branching); acid-fast; Aerobic
- Endemic in soil; get disease from spore inhalation/ trauma inoculation; immunocomprimised/elderly
- Pneumonia (similar to TB); CNS (abscess); Cutaneous involvement
- Tx: Bactrim and surgical drainage of abscess
What is an appendage, in the context of the heart?
It is a small saclike structure that is particularly susceptible to thrombus formation
Presentation of Left-sided Colon CA
- Constipation
- Sx of Intestinal Obstruction
(Left sided colon CA tends to infiltrate the intestinal wall and encircle the lumen)
- Most common cause of homocystinuria
- What is the result of this defect?
- How might this present clinically?
- Homocystinuria is most commonly caused by a defect in cystathionine sythase
- The result is an inability to form cysteine from homocysteine. Cysteine then becomes essential and buildup of homocysteine leads to elevated methionine
- May present as premature thromboembolic events (atherosclerosis, acute coronary syndrome, etc.), because homocysteine is prothrombotic
Sensitivity vs Specificity
Sensitivity = true-positive rate
(the probability that a test detects disease when a disease is present)
Specificity= true-negative rate
(Probability that a test indicates no disease when disease is absent)
Role of prostaglandin agonists in glaucoma
Increase uveoscleral outflow of aqueous humor
PREFERED TX FOR OPEN ANGLE GLAUCOMA
Name the microscopic and macroscopic changes associated with Ischemic Brain Injury, during the following times after injury:
- 12-24 hours
- 24-72 hours
- 3-7 days
- 1-2 weeks
- >2 weeks
Clopidogrel
- MOA
- Indication
MOA: Irreversibly blocks the P2Y component of ADP receptors on the platelet surface and prevents platelet aggregation
Indication: Just as effective as aspirin for prevention of CV events and should be used if patient has aspirin allergy.
Formula for half life
Vd x .7/CL
Diazepam
- Indications (4)
- SEs (1 most common)
- Contraindications
- Indications: (1) anxiolytic, (2) sedative-hypnotic, (3) anticonvulsant, (4) muscle relaxant
- SE: Sedation
- Contraindications: Don’t give to patients on other CNS depressants (ex. chlorpheniramine)
Phenoxybenzamine
- MOA
- Indication
- Effect
- Irreversible a1 and a2 antagonist that effectively reduces the arterial vasoconstriction induced by NE.
- Pheochromocytoma
- Because it is irreversible, even very high concentrations of NE (like those seen in pheochromocytoma) cannot overcome its effects
Polymyositus
Presentation
Symmetrical proximal muscle weakness
What is the relationship between CO2 and cerebral perfusion?
CO2 is a potent cerebral vasodilator and therefore a drop in CO2 (due to hyperventilation and subsequent hypocapnia for example), causes a linear decrease in cerebral perfusion.
Niacin (nicotinic acid)
- Indication
- Adverse Effects
- What is the mechanism for the adverse effects and how can they be prevented?
- Used in the tx of hyperlipidemia. Effective in raising HDL cholesterol levels, and lowering LDLs and TGs
- SEs include cutaneous flushing, warmth and itching
- SEs are mediated by release of prostaglandins and can therefore be prevented by aspirin.
Kawasaki Disease
- What type of disease is this?
- Presentation
- Potential consequence
- Tx
- Medium-vessel vasculitis
-
CRASH and burn
- Conjuctival injection, Rash, Adenopathy, Strawberry tongue, Hand/foot changes (edema and erythema) and fever
- Risk of coronary artery aneurysm
- Tx w/ IV and aspirin
Primary Myelofibrosis
- Presentation
- Mutation
- Bone marrow fibrosis, severe fatigue, splenomegaly (often causing early satiety/abdominal discomfort), hepatomegaly, and anemia
- Mutation: JAK2 (of the JAK-STAT signaling pathway– tyrosine kinase)
Paradoxical Embolism
- Pathology
- Dx
- When a thrombus from the venous system crosses into arterial circulation (as oppose to the pulmonary) via an abnormal connection between the right and left cardiac chambers
- May see fixed splitting of S2 if a shunt is present
Inheritance of CF
Autosomal recessive
What is the negative predictive value and how do we calculate it?
NPV represents the probability of not having a disease given a negative test result. NPV = true negatives/total negative tests
Following cleavage of pro-insulin, what is the fate of insulin and C-peptide?
They are both stored in islet cell secretory granules unti they are secreted in equimolar amounts
ASD
- What is the usual pathology?
- What other cardiac condition is often also present?
- Dx
- Potential consequence
- What genetic disease is also associated?
- Failure of the endocardial cushions of the atrioventricular canal to fuse completely during embryonic development can lead to a lower interatrial septum defect
- Malformation of mitral valve leading to mitral regurg is often present
- Wide, fixed splitting of the second heart sound (S2)
- Pulmonary HTN
- Down syndrome
What is the role of NAD+ in glycolysis? How is NAD+ regenerated after reduction to NADH?
NAD+ is needed to convert G3P to 1,3-BPG
NADH then transfers electrons to pyruvate to form lactate and regenerate NAD+
Flattening of deltoid muscle + acromial prominence, suggests what type of injury?
Anterior humerous dislocation (most common type of shoulder dislocation)
What are the (3) options for Pyruvate metabolism and how do they occur?
- Lactic acid (via lactate dehydrogenase in anaerobic conditions)
- Acetyl-CoA (via pyruvate dehydrogenase in aerobic conditions)
- Oxaloacetate (via pyruvate carboxylase)
Mycobacterium Avium Complex
- What is it?
- Risk factors?
- Normal presentation?
- How do you differentiate MAC from disseminated TB?
- An infection caused by nontuberculous mycobacteria M avium and M intracellulare.
- Risk factor: CD4 count
- Presents w/ nonspecific symptoms- fever, weight loss, and diarrhea in HIV patient
- Differs from TB due to hepatosplenomegally, anemia, and elevated alk phosp/ LDH
How does hypothermia lead to a left shift of the O2-dissociation curve?
Decreased temps help stabilize the bonds between O2 and hemoglobin
Formula for Loading dose
Vd x Cpss (steady state plasma concentration)/ [bioavailability fraction]
What is the most common CFTR mutation associated w/ CF?
F508 mutation. This causes impaired postranslational processing (improper folding and glycosylation) of the CFTR. As a result, the abnomormal protein is targeted for degradation.
What key interleukin/cytokines are responsible for converting a helper T-cell into a Th1 cell? Th2 cell? Th17 cell?
Helper T to…
Th1 (via IL-12)
Th2 (via IL-4)
Th17 (via TGF-B and IL-6)
Embryologically, the dorsal pancreatic bud forms what?
The ventral pancreatic bud?
- Dorsal: majority of the pancreatic tissue (body, tail, and most of the head)
- Ventral: precursor of the uncinate process, inferior/posterior portion of the head, and the major pancreatic duct
What effect do Ach and adenosine have on cardiac pacemaker cells?
They reduce the rate of spontaneous depolarization of cardiac pacemaker cells by prolonging phase 4
Chronic lung transplant rejection
- Pathology/ histopathology
- Presentation
- Dx
- Lymphocytix inflammation + epithelial destruction in small airways. Exudate and granulation tissue later found ultimately leading to fibrosis. Leads to obstructive lung disease bronchiolitis obliterans.
- Dyspnea + dry cough
- Dx: Spirometry shows airflow limitation w/ a drop in FEV1 and FEV1/FVC
Cutaneous, strawberry-type capillary hemangiomas
- Pathology
- Prognosis
- Benign congenital tumor of unencapsulated aggregates of closely packed, thin-walled capillaries
- Initial growth followed by regression (excellent prognosis)
Enteropeptidase
- What secretes it?
- What does it do?
- What does its deficiency lead to?
- Comes from the jejunal brush border
- Activates trypsin from trypsinogen (needed for peptide breakdown and activation of other pancreatic enzymes)
- Deficiency impairs protein and fat absorption
What are the main causes of pulsus paradoxus. Name one option for tx
Pericardial dz, asthma and COPD
Beta-adrenergic agonisists are useful for the asthma/COPD etiologies
In an MI, when does loss of cardiomyocyte contractility occur?
Within the first 60 secs (1 minute) after the onset of total ischemia.
Tx of acute gout vs chronic gout
acute: NSAIDs (1st line), glucocorticoids, or colchicine (GI issues due to inhibition of microtubule formation)
chronic: Xanthine oxidase inhibitors
Clostridium tetani blocks the release of what?
The inhibitors Glycine and GABA
Prevention of Tumor Lysis Syndrome
Prevent w/ hydration + use of hypouricemic agents such as allopurinol or rasburicase
Tension Pneumothorax
- Pathology?
- Clinical presentation?
- Dx?
- Tx?
- Pathology: As an increasing volume of air accumulates w/in the pleural space, the lungs and mediastinum shift to the opposite side and this pressure drops systemic venous return to the heart, leading to decreased cardiac output
- Presentation: Tachycardia, hypotension, tachypnea, hypoxemia
- Dx: Hx, mediastinal shift on CXR, absent breath sounds and hyperresonance to percussion on affected side
- Tx: Emergency needle thoracostomy or chest tube
Describe Cori Dz
- Deficient enzyme
- Which other GSD is it similar to?
- Debranching enzyme
- It is a milder version of Von Gierke (type I)
Neurofibromatosis Type 1 (NF-1)
- Sx (4)
- Inheritance
- What type of cells are the main compent of cutaneous neurofibromas?
- (1) Cafe-au-lait spots, (2) Neurofibromas (short, sessile or pedunculated lesions), (3) Lisch nodules (pigmentated hamartomas of the iris), (4) pseudoarthritis
- Autosomal-dominate inheritance
- Schwann cells are the main component
How is carotid sinus massage useful for termination of paroxysmal SVT?
It leads to increased parasympathetic tone causing temporary inhibition of the SA node and the prolongation of AV node refractory period
What specifc joints are most commonly fused in Ankylosing Spondylitis?
What are potential complications of AS? (3 systems)
Fusion of the sacroiliac and apophyseal joints of the spine are most commonly affected
Complications:
- Respiratory: limited chest wall expansion leading to hypoventilation
- CV: ***most common!!!– aortitis, leading to dilation of the aortic ring and aortic insufficiency
- Uveitis (blurred vision, photophobia, conjunctival erythema, etc.)
What type of RNA can be infective, what type cannot, and why?
- Purified single-stranded positive-sense RNA can be infectious
- Single-stranded negative sense or double stranded RNA cannot be infectious
For a purified RNA molecule to induce viral protein synthesis in a host cell, it must be able to act DIRECTLY AS mRNA USING HOST INTRACELLULAR MACHINERY for translation.
How does the effect of Rheumatic Heart Disease on the mitral valve vary as the patient’s age changes?
First few decades of life: MR
Middle-aged: MS (most common cause of MS)
Elders: Mixed mitral disease (S and R)
(Leukocyte Accumulation:) What receptors are involved in:
- Rolling?
- Tight Adhesion/Crawling?
- Transmigration?
- Rolling: Selectin
- Adhesion/crawling: ICAM-1 and integrins
- Transmigration: PECAM-1 and integrins
When given with a Statin, what drug is most likely to lead to myopathy or even rhabdomyolosis? Why?
Fibrates (like Gemfibrozil). They impair the hepatic clearance of Statins leading to excessive blood levels.
X-linked agammaglobulinemia
Pathogenesis?
Diagnostics?
Presentation?
- A mutation in Bruton tyrosine kinase gene causes failure of BM pre-B cells to mature.
- These patients have:
- low B-cells in peripheral blood (CD19+, CD20+, CD21+)
- pan-hypogammaglobulinemia (low Ig’s)
- Increased risk of infection w/ encapsulated bugs and certain viruses and parasites
Allergic bronchopulmonary aspergillosis (ABPA)
- History commonly associated (2)?
- Chest imaging?
- Dx?
- Asthma and CF
- Recurrent infiltrates and Bronchiectasis
- Eosinophilia; Positive skin test and Ig for Aspergillus; Elevated IgE
Key presentation for cyanide poisoning
- Presentation: Reddish skin discoloration, tachypnea, HA. Lab studies indicate severe lactic acidosis and dec. venous-arterial PO2 gradient
What are the key associations with Multiple Myeloma? (7)
CRAB (hyperCalcemia, Renal involvement, Anemia, Bone lytic lesions/Back Pain)
+
Primary amyloidosis, Rouleaux formation, and numerous plasma cells w/in bone marrow sample
Familial Hypocalciuric Hypercalcemia (FHH)
What is it?
Describe the associated receptor?
A benign autosomal dominant disorder casued by a defective calcium-sensing receptor (G-protein coupled), which is suppose to regulate PTH secretion. Defect raises the Ca2+ threshold
Most common site of injury during a traumatic aortic rupture
Aortic isthmus
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Describe the pathogenesis of Sarcoidosis
Granuloma formation as a manifestation of cell-mediated immunity driven by products of Th1 type CD4 helper T-cells, particularly IL-2 and IFN-y, which stimulate Th1 type cell proliferation and macrophage activation, respectively.
Name the (4) nerves/ vessels which enter the orbit via the superior orbital fissure
OSATO
- Oculomotor nerve
- Superior Ophthalamic vein
- Abducens nerve
- Trochlear nerve
- Opthalmic nerve (CN V1) branches
Right Ventricular MI
- Presentation
- Pathology
- What does the hemodynamic assessment reveal? (CVP? Wedge pressure? CO?)
- Presents with hypotension, elevated JV pressure, clear lungs
- Most often occurs in the setting of acute inferior wall MI
- CVP= inc.; Wedge pressure = dec.; CO = dec.
What is the most common CV manifestation associated with SLE?
Pericarditis
Hypoglycemia with an elevated insulin and low C-peptide level, suggests what?
High C-peptide level?
Low: exogenous insulin injection
High: insulin secretagogue or insulin-secreting tumor
When amniocentesis is performed to check phospholipids, what are we really checking for?
Fetal lung maturity
Phospholipids (such as lecithin aka phosphatidylcholine) are a majory component of pulmonary surfactant
Structural cardiac changes due to aging are generally not prominent before the age of…
65y/o
Describe the relationship between Bile acid-binding resins and Statins
BAB resins (ex. cholestyramine) Inc. stimulation of HMg-CoA Reductase, which will need to be compensated by giving a Statin. Synergistic effects with the statin further reduce plasma LDL level.
What is the effect of G6PD deficiency?
What other enzyme deficiency might paint a similar picture?
G6PD deficiency is a defect in the HMP shunt (aka the Pentose Phosphate Pathway), which leads to impairment of glutathione reduction due to failure to produce NADPH
Glutathione reductase deficiency causes a similar clinical picture
What are the structural differences between MHC Class I and Class II?
MHC Class I: Heavy chain and B2-microglobulin
MHC Class II: Alpha and beta polypeptide chains
What is the most common congenital heart lesion?
- Dx/ Presentation?
- Effect on blood oxygenation
- Prognosis?
Ventricular Septal Defects
- Small VSDs have a loud, “blowing”, holosystolic murmur at the mid/lower left sternal border (louder with handgrip). Murmur is usually inaudible until 4-10 days when pulmonary vascular resistance declines enabling left-to-right shunt.
- Right ventriclular blood has increased O2 content
- Most are clinically insignficant and close spontaneously
New-onset odynophagia in the setting of chronic GERD usually indicates what?
Dx?
Erosive esophagitis and the formation of an ulcer
Dx via upper endoscopy
Jervell and Lange-Nielsen Syndrome
- Pathology
- Clinical presentation
- Mode of inheritance
- What other disease is this similar to?
- Congenital long-QT syndrome; thought to result from mutations in a K+ channel protein
- Syncopal episodes; sudden cardiac death (torsades de pointes);
- Autosomal recessive condition
- Similar to Romano-Ward syndrome
Dihydropyridine CCBs (Anti-hypertensives)
- Main examples
- Indications
- SEs
- Amlodipine and Nifedipine
- Effective for monotherapy or in combination with other agents for Tx of HTN
- Peripheral Edema and Dizziness/ Lightheadedness
Trousseau syndrome
Migratory superficial thrombophlebitis assc. with visceral CA
What is pancreas divisum?
Failure of the dorsal and ventral pancreatic buds to fuse
Interstitial lung disease has what effect on lung volumes? Lung elastic recoil?
How does this affect expiratory flow rates?
Decreased lung volumes
Increased lung elastic recoil
The increased recoil leads to radial traction (outward pulling) of airways, leading to increased corrected expiratory flow rates.
What is the role of Small nuclear RNA (snRNA)?
What is the name of autoantibodies which work against the function of snRNA and what dz are they associated with?
snRNA is synthesized by RNA pol II and complexes w/ specific proteins to form small nuclear ribonucleoproteins (snRNPs). These snRNPs are essential components fo the spliceosome and remove introns from to form mature mRNA.
Autoantibodies directed against snRNPs are called anti-Smith antibodies, and are assc. with SLE.
Type I Hypersensitivity
Explain the mechanism. What mediates these reactions?
Mediated by the interaction of allergen w/ preexisting IgE bound to basophils and mast cells. Cross-linking occurs allowing for degranulation and immediate allergy signs.
Which ligament is most commonly involved in a lateral ankle sprain?
Anterior talofibular ligament
Main potential AE of neonatal oxygen therapy?
Retinal damage
Name the potential sequelae of Obstructive Sleep Apnea (3)
Systemic and Pulmonary HTN, and right heart failure
What type of gating does the CFTR channel have?
ATP-gated
Cauda Equina Syndrome
Presentation?
Specific areas damaged?
Saddle anesthesia and loss of the anocutaneous reflex, assc. with damage to the S2 through S4 nerve roots
Cystic Medial Degeneration
- Characteristics
- What does it predispose you for?
- Who is it common in?
- Myxomatous (weakening of connective tissue) changes with pooling of proteoglycans in the media layer of large arteries
- Aortic dissections/ Aneurysms
- Younger patients with Marfan Syndrome
Walking pneumonia
- What bug causes it?
- Dx?
- What type of medium is required for this bug to grow?
- Sx?
- Mycoplasma pneumoniae
- CXR looks much worse than the clinical appearance of the patient indicates
- Cholesterol medium
- Nagging unproductive cough, low-grade fever, and malaise
Risks of secondhand smoke exposure (7)
- SIDS
- Low birth weight
- Dental caries
- Middle ear disease
- Asthma
- LRTI
- Decreased GFR
Ankylosing Spondylitis
- What is it?
- Presentation
- Chronic inflammatory condition assc. wtih HLA-B27 serotype
- Low back pain + stiffness in a young man. Fusion of axial joints (hence bamboo spine)
Adenocarcinoma In-situ (of the lung)
- Pathology
- Microscopy
- Presentation
- Imaging
- Arises from alveolar epithelium at the periphery of lung
- Microscopy shows well-differentiated, dysplastic columnar cells w/ or w/o mucin
- Similar to other lung cancers (SOB, cough, hemoptysis)
- Discrete mass or pneumonia-like consolidation on imaging
What is the most common condition predisposing a patient to infective endocarditis in…
- Adults in wealthier nations?
- Poorer nations/ children?
- Mitral valve prolapse/ mechanical valves
- Rheumatic fever
Ramelteon
- MOA
- Indications
- SEs
- MOA: melatonin agonist
- Initial insomnia treatment that demonstrates high safety and efficacy in older adults
- Very few SEs and no dosage adjustment needed
In what conditions do we see Cheyne-Stokes breathing? (2)
- CHF
- Neurologic disease
How do OC treat hirsutism?
They suppress pituitary LH secretion and subsequently decrease ovarian androgen production
Neural Tube Defects
- Pathophysiology
- Presentation
- Dx
- NTDs occur due to failure of fusion of the neural tube during the 4th week of development. This leaves an opening between the neurotube and the amniotic cavity.
- Can present with encephalocele (herniation), anencephaly (no brain), or the spina bifadas
- Leakage of alpha-fetpoprotein and AchE occurs, allowing for prenatal diagnosis
Idiopathic Pulmonary Artery Hypertension
Pathogenesis/pathophys?
Presentation?
Treatment?
Pathogenesis: If familial form, result of inactivation of the pro-apoptotic BMPR2 gene. Results in increased endothelial and smooth muscle cell proliferation and vascular remodeling
Presentation: Dyspnea, excercise intolerance in women 20-40
Treatment: Lung transplant and bosentan (endothelin-receptor antagonist) in the meantime
Formula for Maintenance dose
Cpss (steady-state plasma concentration) x CL/[bioavailability fraction]
What is the main difference in effect between unfractioned heparin and LMWH?
Both of them can bid to AT3 to increase its activity against factor Xa.
Only unfractionated heparin is able to bind to both AT3 and thrombin, thereby allowing antithrombin to inactivate thrombin.
PPO vs HMO vs POS (Point of Service)
- Compare the monthly premiums
- Compare copayments and deductibles
- Is a PCP referral required for specialist visits?
- Size of network
- May go outside network?
Statins
- Indication
- MOA
- Side effects
- What sort of things can help lead to adverse effects from Statins
- Indications: Tx of hypercholesterolemia
- Inhibit HMG-CoA reductase, thus blocking hepatic cholesterol synthesis. This forces the liver to increase surface expression of LDL receptors, thus pulling LDL from circulation
- SE: Myopathy, rhabdomyolysis, and hepatoxicity
- Drugs that interfere w/ statin metabolization, particuarly via cytochrome p450 enzymes (ex. fibrates)
Abetalipoproteinemia
- What is it?
- Consequence?
- Describe the histo
- An inherited inability to synthesize apoB, which is critical for chylomicrons and VLDL
- As a result, lipids absorbed by the small intestine cannot be transported into the blood and accumulate in the intestinal epithelium.
- Enterocytes w/ clear/ foamy cytoplasm
What is the effect of excercise on LV EDP?
On systemic vascular resistance?
LV EDP is increased due to the increased return
Systemic vascular resistance decreases due to large levels of vasodilation in muscles
Isoproterenol
- MOA
- Effect
- Non-selective Beta-_agonist_
- Increases myocardial contractility and decreases systemic vascular resistance
Isoproterenol
(MOA)
Nonselective B-adrenergicc agonist (leads to inc. vasodilation, inc. cardiac rate, and inc. contractility)
What are the (3) main dopaminergic systems? What are their functions? What diseases are they associated with?
Presentation of Right-Sided Colon CA
- Occult bleeding
- Sx of iron deficient anemia
Myasthenia Gravis
- Sx
- Disease associated with
- Pathogenesis
- Tensilin test result
- Nerve stimulation studies
- Sx: Weakenss that is worse at the end of the day/ with exertion; extraocular muscles are affected first (ptosis/diplopia)
- Assc. with risk of thymoma
- Path: antibodies against Ach receptors
- Improvement/ resolution w/ tensilon test
- Decremental response to nerve stim. test
Length constant
A measure of how far along an axon an electrical impulse can propagate
This is increased by myelination
Name the (2) classes of dopamine agonists (and examples).
What dz are they most often used for?
- Ergot compounds- Bromocriptine
- Nonergot compounds- Pramipexole, ropinirole
Histoplasma Capsulatum
- What is it/where is it found?
- How is it transmitted?
- Pathology?
- Presentation?
- Dx?
- A mold found in soils of Mississippi and the Ohio River Valley
- It is transmitted by the respiratory route when bird/rat droppings containing fungal spores are inhaled
- Pathology: In the lungs, the fungus is ingested by macrophages and a granuloma forms, similar to TB
- Presentation: Asymptomatic if immunocompetent. May develop acute pulmonary disease, or develop chronic pulmonary histoplasmosis (looks like TB). May see lymphadenopathy and hepatosplenomegally
-
Dx.: small oval bodies w/in macrophages
6.
What type of drug is ipratropium and what type of bronchoconstriction is affected by it?
It is an antimuscarinic agent
Only reverses vagally-mediated bronchoconstriction
Varicocele
- Pathophysiology?
- Presentation?
- Increased pressure in the left gonadal vein resulting in valve leaflet failure and varices of the testicular pampiniform plexus
- Presents with flank/abdominal pain and gross or microscopic hematuria
Bile acid-binding resins
- Give one major example
- MOA
- Cholestyramine
- Binds bile acids, forcing the liver to inc. uptake of LDL in order to create more bile.
At the FRC, the airway pressure is? alveolar pressure is? Intrapleural pressure is?
Airway pressure = 0
Alveolar pressure = 0
Intrapleural pressure = Negative, with a value of -5cm H2O (prevents pneumothorax)
- A subset of patients stricken by influenze got on to develop secondary…
- What types of patients are these?
- What are the most common causes for this secondary disease state?
- …Bacterial pneumonia
- Elderly
- S. Pneumo, S. Aureus, H. Flu
Maple Syrup Urine Disease (MSUD)
- Pathology
- Presentation
- Treatment
4.
- Path: defect in a-keto dehydrogenase, leading to inability to degrade branched chain amino acids (leucine, isoleucine, valine) beyond deaminated a-keto acid state
- Sx: Dystonia; poor feeding; maple syrup scent urine in first few days of life
- Tx: Dietary restriction of branched-chain AA’s
Hartnup Dz
- Path
- Sx
- Possible Consequences
- Tx
- Defective intestinal and renal tubular absorption of dietary tryptophan. This can result in Niacin deficiency, as Niacin is synthesized from tryptophan
- Sx: Often asymptomatic but may result in photosensitvity or pellegra-like skin rashes
- Tx w/ nicotinic acid/ nicotinamide and a high-protein diet
Tricuspid Valve Endocarditis
- Most common bug associated?
- Usual patient population?
- Potential complications?
- Staph aureus is #1. Pseudomonas is #2
- IV drug users
- These patients can develop multiple septic emboli in lungs. Resulting pulmonary infarcts will be hemorrhagic due to dual blood supply.
What are the key effects of uncontrolled gestational diabetes on the fetus?
- Beta cell hyperplasia due to increased trans-placental glucose delivery to the fetus (leads to TRANSIENT hypoglycemia at birth)
- Fetal macrosomia (large size at birth!)
When is anovulation most common?
In the first several years after menarche (immature H-P-Ovarian axis), and in menopause.
Blastomyces dermatitidis
- Endemic to where?
- Characterisitics?
- Presentation?
- Endemic to southeastern US (east of mississippi river)
- Characteristics: Dimorphic fungus which is a large yeast (in the human body) with a single, broad-based bud.
- Infxn follows inhalation and may present as a lung infection (with GRANULOMA) or cause flu-like illness/pneumonia in the immunocompetent. Can present as systemic disease in immunocomprimised patients.
What is the main side effect which limits the long-term efficacy of arteriolar vasodilators. Give (2) main examples of these drugs
Ex: Hydralazine and minoxidil
There can be reflex sympathetic stimulation (leading to inc. HR, contractility, and CO) and also stimulation of the RAA system leading to sodium and fluid retention/edema
What are the afferent and efferent limbs leading to/away the carotid bodies?
Afferent: Glossopharyngeal nerve
Efferent: Vagus nerve
Tetralogy of Fallot Pathophysiology and clinical presentation
VOIR:
- VSD
- Overiding aorta
- Infundibular pulmonary stenosis
- RVH
Presentation: Cyanosis; Improvement w/ squatting (increases SVR)
What types of procedures are associated with enterococcus endocarditis?
Genitourinary instrumentation or catheterization
Injury to the lower trunk of the brachial plexus
- What mechanism of injury which causes this?
- Consequence of this injury?
- Result of sudden upward jerking of the arm at the shoulder
- May causes paralysis of all intrinsic hand muscles (Klumpke’s palsy) w/ sparing to the extensors, leading to total claw hand deformity. Can also see sensory loss and weakness.
Blood flow is directly proportional to the vessel radius raised to what power?
The 4th power
Pulmonary Embolism
- Presentation
- Lab data (blood gases?acidosis or alkalosis?
- Other diagnostic tests
- Acute-onset dyspnea, calf-swelling (indicative of DVT), obesity, Hx of prolonged immobility
- Hypoxemia and respiratory alkalosis
- CT pulm angiography is image testing of choice
Name the (5) regions of the secondary structure of tRNA
- Acceptor stem (mediates corrct tRNA recognition)
- 3’ CCA tail (used as recognition sequence by proteins)
- D loop (facilitates correct tRNA recognition)
- Anticodon loop (used by the ribosome complex to select the right tRNA)
- T loop (facilitates binding of tRNA to ribosomes)
Describe the functions of each of the (3) domains of G protein-coupled receptors.
Describe composition of the transmembrane domain in detail.
- Extracellular domain- responsible for ligand binding
- Intracellular domain- coupled with heterotrimeric G proteins
- Transmembrane domain- composed of nonpolar, hydrophobic amino acids (alanine, valine, leucine, etc.), arranged in an alpha-helical fashion. Serve to anchor the proteinto the phospholipid bilayer. May also play role in cellular signaling and transport.
What dz is associated with incorrect assembly of snRNPs? What is the specific mutation associated?
Spinal Muscular Atrophy (delayed motor development + flaccid paralysis)
SMN1 gene
Thin (actin) filaments of the I band are bound to structural proteins, where? Thick (myosin) filaments?
Thin: Z-line
Thick: M-line
What is the most superficial layer in which you can appreciate an absence of ganglion cells in Hirschsprung dz?
The submucosa of the narrowed area
The anterior cerebral arteries supply which portions of the brain? (2)
What is the affect that occlusion can have?
- They supply the medial portions of the 2 hemispheres (frontal and parietal)
- Occlusion can cause contralateral motor and sensory deficits of the lower extremities, behavorial changes, and urinary incontinence
Name the a-ketoglutarate dehydrogenase co-enzymes which may be defective in Maple Syrup Urine Dz (MSUD)
Tender Loving Care For Nancy
Thiamine pyrophosphate
Lipoate
Coenzyme A
FAD
NAD
Why is raloxifene usually a better choice than tamoxifen for tx of osteoporosis in women?
Unlike tamoxifen, raloxifene has does not have agonist activity in the uterus (which inc. risk of endometrial hyperplasia/CA)
What is the most common location on the aorta for a traumatic aortic rupture (blunt aortic injury)?
The aortic isthmus (located just past the aortic arch)
Niemann-Pick Dz
- What type of dz is this?
- Deficient enzyme
- Inheritance
- Presentation
- LSD
- Sphingomyelinase
- AR (common in Ashkenazi Jew population)
- Sphingomyelin accumulation, leading to hepatosplenomegally , “Cherry-red” spot on macula, and progressive neurodegeneration
Hormone Sensitive Lipase (HSL)
- Where is it found?
- What is its function?
- Found in adipose tissue
- Functions to drive the breakdown of stored triglycerides into free fatty acids and glycerol. During times of starvation, this enzyme provides substrates for hepatic gluconeogenesis and ketone body formation
McArdle dz
- Deficient enzyme
- Path
- Presentation
- Skeletal muscle glycogen phosphorylase
- Muscles can’t breakdown glycogen in muscle (McArdle = Muscle)
- Present with painful muscle cramps, myoglobinuria w/ strenuous excercise, and arhytmia from electrolye abnormalities.
Kartagener Syndrome
- Pathology
- Presentation (3)
- Form of primary ciliary dyskinesia, that is autosomal recessive in nature
-
Presentation
- Recurrent respiratory infections (e.g., sinusitis, bronchiectasis)
- Situs inversus (reverse organ positioning)
- Infertility
Describe the work that activated Ras does.
It begins a phosphorylation cascare that results in the activation of mitogen-activated protein kinase, which enters the nucleus to influence gene transcription.
SE profile for ACE-Inhibitors (2)
- Bradykinin induced cough and angioedema if there is accumulation
- First dose hypotension due to volume depletion
Legionella pneumophila
- Description of bug
- Symptoms
- Dx info
- Pathogenesis
- Tx
- Facultative intracellular gram-negative bacillus
- Symptoms- high fever, elevated transaminases, cough, confusion, and diarrhea,
- Dx- Hyponatremia and sputum gram stain showing many neutrophils but few to no organisms
- Passed from contaminated water
- Respiratory fluroquinolones or newer macrolides
Length constant
(How is it affected by myelination?)
A measure of how far along an axon an electrical impulse can propagate. Myelination increases this constant.
Name the genes involved in each of the (3) steps of the “adenoma-to-carcinoma sequence”
- Progression from normal mucosa to a small polyp: mutation of the APC tumor suppressor gene
- Inc. in the size of the polyps: mutation of the K-ras protoncogene
- Malignant transformation: requires mutation of both p53 and DCC (Deleted in Colorectal Carcinoma)
What is rT3 and what is it created from?
An inactive form of T3, that is generated almost entirely from peripheral conversion of T4
Pure Red Cell Aplasia
- What is it?
- What dz’s is it associated with?
- Rare form of marrow failure that is characterized by severe hypoplasia of marrow erythroid elements in the setting of normal granulopoiesis and thrombopoiesis
- Assc. with Thymoma, lymphocytic leukemias and Parvovirus B19
Vitamin E deficiency
- Who does it occur in?
- What is defiency in this vitamin associated with?
- Clinical manifestations
- Can occur in individuals with fat malabsorption
- Assc. w/ inc. susceptibility of the neuronal and erythrocyte membranes to oxidative stress
- Clinical manifestations: ataxia, impaired proprioception and vibratory sensation, and hemolytic anemia
Why might patients with signficant renal dysfunction demonstrate prolonged bleeding time?
Accumulation of uremic toxins impair platelet aggregation and adhesion (no effect on platelet count, PT or aPTT)
Other than the small joints of the hand, what other joints/bones are often affected by RA?
The cervical spine
Function of the Posterior Cruciate Ligament
How is its integrity tested?
Prevents posterior displacement of the tibia relative to the femur. Tested via the posterior draw test.
Pulmonary Fibrosis
- Microscopic view
- Clinical presentation
- Dx
- What disease can help cause it?
- Microscopic: Progresive fibrosis an lead to cystically-dilated bronchioles that later coalesce to from “honeycomb” appearance
- Presents with gradual-onset dyspnea, first w/ exertion and then eventually even at rest
- Physical exam can show end-inspiratory crackles and PFTs show restriction (decreased FEV1 and FVC w/ normal ratio)
Why do patients with Antiphospholipid antibody syndrome often get false positive syphillus exams?
Presence of anticardiolipin antibody (which is tested for in Treponema pallidum, but is also present in this dz)
Almost 100% of cases of Hypertrophic Cardiomyopathy result from mutations in genes encoding what?
Beta-myosin heavy chain
(A cardiac sarcomere protein)
Why do you normally do cardiac catherization via the femoral artery, below the inguinal ligament and not above?
There is signficant risk for a retroperitoneal hemorrhage if you do it above the inguinal
Acute bacterial parotitis
- Occurs most commonly in who?
- Bacteria most commonly involved?
- Dx
- Common in elderly, postoperative patients who are intubated or dehydrated
- S. Aureus
- Elevated serum amylase (in the presence of normal serum lipase/ no evidence of pancreatitis)
Elastase from what cells, are inhibited by alpha-1 antitripsin?
Neutrophils
Which cell types, when metabolizing a single glucose molecule, will always ueild pyruvate but sometimes generate NO net ATP?
(Explain this mechanism)
Erythrocytes
2, 3- BPG dec. hemoglobin affinity for O2. When 2,3-BPG needs to be synthesized, the RBC bypasses the step which converts 1,3 BPG to 3-phosphoglycerate, instead forming 2,3 BPG in a process which does not create ATP.
Sx of trapezius weakness vs deltoid weakness
Trapezius- drooping of shoulder, impaired abduction above horizontal, winging of the scapula
Deltoid- impaired abduction at angles below the horizontal plane
Finasteride MOA and indication
MOA: 5-alpha-reductase inhibitor that suppresses conversion of testosterone to DHT
Ind: BPH, Androgenetic alopecia
When is an aortic stenosis murmur at its loudest?
The intensity is proportional to the magnitude of the LV to aorta pressure gradient during systole, (i.e. whenever aortic pressure is at its strongest)
- Propionyl CoA is derived from what?
- What enzyme is responsible for its conversion to methylmalonyl CoA?
- What would deficiency of this enzyme lead to?
- It is derived from amino acids (Val, Ile, Met, and Thr), as well as odd-numbered fatty acids and cholesterol side chains
- Propionyl CoA carboxylase is the enzyme responsible
- Deficient enzyme leads to development of proprionic acidemia
What are the (2) key functions of type II pneumocytes?
- Regeneration of the alveolar lining
- Surfactant production
Tabes Dorsalis
- Epidemiology?
- Pathogenesis?
- Clinical findings?
- Epidemiology: Increased incidence of syphilis in MSM/ HIV-infected patients
- Pathology: Treponema pallidum spirochetes directly damage the dorsal sensory roots
- Clinical findings: sensory ataxia, lancinating pains, neurogenic urinary incontinence, Argyll Robertson pupils
Which ventricle forms the apex of the heart?
The left ventricle
(All other chambers lie medial to the midclavicular line)
Pancoast tumors and Horner’s syndrome
Among other symptoms, Pancoast tumors can lead to Horner’s syndrome due to involvement of the cervical sympathetic ganglia.
How do PPIs affect osteoporosis?
Longterm PPI use may be associated with increased osteoporosis risk, most likely do to a decreased absorption of Ca2+ (acidic environment is needed)
T-test versus analysis of variance (ANOVA)
A t-test is used to compare the difference between the means of (2) groups. ANOVA compares bbetween the means of 2 or more groups.
What rxns does Thyroid peroxidase catalyze? (3)
- Oxidation of iodide
- Iodination of thyroglobuin
- Coupling rxn between 2 iodized-tyrosine residues
Which blood vessel carries blood with the lowest content of oxygen in the body and why?
Coronary sinus
Myocardial oxygen extraction is very high and has a high demand
What are the effects of occlusion of the MCA?
- Contralateral hemiplegia of the face/ upper limb
- Relative preservation of the lower limb
- Aphasia (if occlusion is in dominant hemisphere – usually left)
What is the cause of Myotonic Dystrophy?
An autosomal dominant disorder, leading to increased number of trinucleotide repeats on the myotonia-protein kinase gene
Why does listening to heart sounds at end expiration make them more audible?
Decreased lung volume, bringing the heart closer to the chest wall.
Fenoldopam
- MOA
- Indication
- Effects (3)
- MOA: selective peripheral D1 receptor agonist
- Indication: given IV ti lower BP in HTN crisis, especially in patients with renal insufficiency
- Effects: (1)Arteriolar dilation, (2) increased renal perfusion, and (3) promotion of diuresis/ natriuresis
Superior Vena Cava Syndrome
- Pathogenesis?
- Presentation?
- Path: Compression of SVC (often by an intrathoracic carcinoma– eg. mediastinal mass) leading to impaired venous return
- Presentation: Dyspnea, facial swelling, and dilated collateral veins in upper trunk
Inapparopriate activation of what enzyme, leads to pancreatitis?
Trypsinogen (to trypsin)
17a-hydroxylase def.
Presentation?
Boy appear phenotypically femaile at birth, but girls develop normally. (Result of dec. androgen, estrogen, and cortisol synthesis)
Patients present with hypogonadism, HTN, and hypokalemia
Why is it that calcium channel blockers affect smooth and cardiac muscle, but not skeletal?
Cardiac and smooth muscle cells depend on extracellular calcium influx into cells via L-type calcium channels. Skeletal muscle cells do not, because they have a direct mechanical coupling of the L-type channel and the RyR.
Lesions on the medial foot cause, what type of lymphadenopathy?
On the lateral foot?
Medial: Inguinal lympadenopathy
Lateral: popliteal and inguinal lymphadenopathy
Causes of oral thrush (3 main)
- Candida albicans
- Albuterol inhaler
- HIV
Coronary Sinus
- What does it serve as?
- Under what condition might it be dilated?
- Serves as the endpoint of venous drainage from the coronary blood supply, draining directly into the right atrium
- Will be dilated by any factor that dilates the right atrium (most commonly pulmonary HTN), because it freely communicates with the RA.
What is usually the most key component of the pathogenesis of AAAs?
Transmural aortic wall inflammation
What are the effects of nitrate? (3 general categories of effects)
Side Effects?
- Primarily venodilators (but also vaso) that increase peripheralvenous capacitance
- Reduce cardiac preload and afterload and LVEDP and volume, reducing work of heart
- Modest effect on arteriolar dilation
Side Effects: HA, cutaneous flushing, hypotension
Aspergillus fumigatus
- Describe its structure
- How do people get it?
- Describe associated disease processes
- Mold that froms septate hyphae that branch at 45-degree angles
- Spores are inhaled and particularly impact those in immunocompromised states
- Wide spectrum of diseases:
- Invasive pulmonary aspergillosis (for the immunosuppressed/ neutropenic)
- Aspergillus colonization in preexisting lung cavities
- Allergic bronchopulmonary aspergillosis (lung hypersensitivity reaction) for patients w/ asthma
“Red ragged” muscle fibers on Gomori trichome are seen in what kinds of disease…?
Mitochondrial diseases
The mitochondria accumulate under sarcolemma
Of all major vascular beds, which (2) are most susceptible to athersclerosis?
- Lower abdominal aorta
- Coronary arteries
Aortic Regurgitation
- Presentation
- Describe how the pulses may feel
- Describe the murmur and the best way to hear it
- What is the state of the pulse pressure?
- Presentation: progressive fatigue + dypsnea
- Pulses: “water-hammer” pulses (bounding femoral and carotids) and head-bobbing with each heartbeat (de Musset sign)
- Murmur:
- Decrescendo murmur after A2
- High-pitched, blowing quality
- Best heard at left sternal border, at 3rd/4th intercostal space, with the patient sitting up and leaning forward
- The pulse pressure is widened
How does sepsis lead to ARDS? Describe step by step.
During sepsis, cytokines circulate in response to infection, activate pulmonary epithelium and provoke an inflammatory response mediated by neutrophils. This leads to capillary damage and the leakage of protein/fluid into the alveolar space.
What is the marker for hematopoietic stem cells?
CD34+
Function of the suprachiasmatic nucleus (of the hypothalamus)
Circadian rhythm regulation and pineal gland function
Blockage of what vein causes symptoms similar to those in SVC syndrome, except only on one side of the body? What are those symptoms?
Blocking of the Brachiocephalic vein
Shows one sided face-swelling, arm swelling and engorgement of subcutaneous veins .
What types of tissues are comonly found in a Meckel diverticulum? Which is most common?
The presence of these tissues is an example of what?
Gastric (most common), pancreatic, colonic, endometrial, small bowell, etc.
This is an example of ectopy
MOA of class 3 antiarrhytmic agents
They block K+ efflux from cardiac myocytes and prolong phase 3 of the myocyte AP
Describe Von Gierke Dz
- Deficient enzyme
- Presentation
- Tx
Type I Glycogen Storage Dz
- Glucose 6- phosphatase
- Severe fasting hypoglycemia (can’t convert G6P to glucose); hepatomegaly
- Tx: frequent oral glcose/cornstarch; avoidance of fructose and galactose
What is NE extravasation and what is the treatment?
Blanching of vein into which NE is being infused due to NE leakage causing major a1 receptor activation and subsequent constriction.
Use phentolamine (alpha-receptor blocker)
Major SEs of Corticosteroids
- Immunosuppression (except with neutrophilia!!)
- Corticosteroid-induced psychosis
Fibrates
- MOA
- Effect
- Compare this to fish oil
- Activates peroxisome proliferator-activated receptor alpha (PPAR-a).
- This leads to decreased hepatic VLDL production and increased Lipoprotein lipase activity, thus lowering triglyceride levels
- Fish oil supplements w/ O3FA dec. VLDL and ApoB production
If a patient with HIV has rin-enhancing lesions, and treated for toxoplasmosis is negative, what dz should you suspect?
What cell typically compose this dz?
Suspect Primary Central Nervous System Lymphom__a
It is typically composed of B-lymphocytes, and is associated with EBV.
G protein-couple receptors
- What do they bind?
- What are the domains which make them up? (3)
- Bind glycoprotein hormones (eg TSH, LH, FSH)
- 3 major domains: Extacellular domain, transmembrane domain, intracellular domain
- Name the anthracycline chemotherapeutic agents.
- What is their most severe side effect
- How does it present?
- How is this SE prevented?
- Doxorubicin, daunorubicin, epirubicin and idarubicin
- Their most severe side effect is a cumalitive dose-related dilated cardiomyopathy due to free-radical formation
- Presents w/ right and left ventricular CHF
- Prevented via dexrazoxane- iron-chelating drug which reduces free radical formation
X-linked (Bruton) agammaglobulinemia
- Caused by what?
- Effect on germinal center formation
- Presentation?
- Caused by a defect in B cell maturation, resulting in the absence of mature B cells with severe defiency of all Ig types. T cells remain fxnal and intact!
- No B cells = no germinal center formation
- Recurrent infxn in setting of intact T Lymphocyte function
Narcolepsy
- Pathology
- Presentation
- Tx
- Pathology: Due to low levels o stimulatory neurotransmitter orexin (hypocretin) which is involved in maintaining wakefulness and suppressing REM sleep
- Episodes of refreshing sleep during the daytime + at least one REM sleep-related phenomena (cataplexy, hypogogic/hyponopompic halluciantions, and sleep paralysis)
- Psychostimulants: Modafinil
Retinal Artery Occlusion
Presentation?
Pathogenesis?
What is the path most likely taken to occlude the artery?
Presentation: Acute, painless, monocular vision loss
Pathogenesis: Thromboembolic complications of athersclerosis in the internal carotid.
Path: Internal carotid –> Ophthalmic artery –> retinal artery
Other than trauma, what is one major cause for cardiac tamponade?
Viral pericarditis w/ signficant pericardial fluid accumulation
(following a respiratory infection)
Sarcoidosis
Pathophys?
Presentation?
Dx?
Treatment?
- Path:Immune mediated, widespread noncaseating granulomas
- Pres: Black women w/ possible erythema nodusum, arthralgia, elevated ACE levels, etc.
- Dx: Bilateral hilar adenopathy on CXR
- Tx: Steroids
Myocardial hibernation
A state of chronic myocardial ischemia in which myocardial metabolism and function is reversibly reduced in order to match a reduction in coronary blood flow, thus preventing myocardial necrosis
What does Prussian blue stain detect?
Intracellular iron
Antiarrhythmic drugs
Drugs + Predominant actions
Class IA, IB, IC
- IA: Dispyramide, Procainamide, Quinidine- slows AP conduction velocity; prolongs APD
- 1B: Lidocaine, Mexiletine, Phenytoin- Shortens APD (no effect on AP conduction velocity)
- 1C: Flecainide, Propafenone- Slows AP conduction velocity; minimal effect on APD
“Double Quarter Pounder; Light Mayo and Pickles; Fries Please”
What illnesses can result in cold agglutinins (3)
- Mycoplasma pneumoniae
- EBV infection
- Hematologic malignancy
What role does NFK-B play in osteoclast differentiation?
RANK-L is the receptor for activated NFK-B
What is the most effective preventative intervention in almost all patients?
Smoking cessation
Describe the structure of the mealtime insulin analogs
AA substitution at the C terminal end of the B chain
Name the A’s of small cell (oat cell) carcinoma
- May produce ACTH
- SIADH
- Antibodies against presynaptic Ca2+ channels (Lambert-Eaton)
- Amplification of myc oncogenes
What receptors is epinephrine capable of affecting?
a1, B1, and B2
Where does the gastroduodenal artery sit? How can it be affected by ulceration?
It lies along the posterior wall of the duodenal bulb. It is likely to be eroded by posterior duodenal ulcers, leading to potential life-threatening hemorrhage.
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Xanthomas are found where? What are they associated with?
These yellowish macules/papules are found on the medial eyelids. They are accumulations of macrophages + cholesterol/ TG and are assc. with hyperlipidemia/ dyslipidemia
Hairy Cell Leukemia
- What type of neoplasm?
- Presentation?
- Indolent B-cell neoplasm
- MIddle-aged men w/ bone marrow /fibrosis (leading to dry tap) + massive splenomegaly (due to red pulp infilitration) + cytoplasmic projections on lymphocytes
Diphteria
- Epidemiology
- Micro/pathology
- Clinical Sx (4)
- Complications (3)
- Endemic in developing countries
- Corynebacterium diphtheriae colonize repiratory tract and secrete diphtheria toxin (inhibits protein synthesis via ribosylation of EF-2)
- (1) Pseudomembrane; (2) cervical adenopathy (3)sore throat (4) fever
- (1) Suffocation due to edema/pseudomembrane aspiration; (2) Heart failure/ (3) neuro toxicity from the toxin
Paroxysmal nocurnal hemoglobinuria is result of an acquired mutation in what gene? What inhibitor proteins is absent and/or deficient as a result?
Mutation in the PIGA gene. This leads to absence of the GPI anchor that normally protects the RBC from complement, and associated deficiency of CD55/CD59 complement inhibitor proteins.
What is the difference in the response generated by inactivated vs live-attenuated viral vaccines?
Inactivated: Generate a humoral response, inducing neutralizing antibodies which keep virus from entering the cell membrane
Live-attenuated: Generate a strong cell-mediated response, in addition to a humoral response
What is the MOA for ANP and BNP? How is this similar to sildenafil?
ANP and BNP are similar to NO. They activate guanyl cyclase which increases the levels of cGMP. cGMP leads to relaxation of vascular smooth muscle and vasodilation, via myosin light-chain dephosphorylation
Sildenafil is a phosphodiesterase inhibitor and therefore decreases degradation of cGMP, ultimately causing the same result.
Role of the musculocutaneous nerve (2)
- Innervates the major forearm flexors and the coracobrachialis
- Provides sensory innervation to the lateral forearm
Complications of Paget dz (2)
- High Output cardiac failure
- Osteosarcoma
Thiazide diuretics
- MOA
- Effects
- AEs
- Inhibit Na+/Cl- co-transporter in the DCT, thereby decreasing reabsorption of NaCl
- Lower BP by decreasing intravascular volume, reduce CO, and lower systemic vascular resistance
- Dec. insulin secretion and glucose uptake, and increase LDL cholesterol and TG levels
What is the (4) step route that N. meningitidis uses to cause meningitis?
Pharynx –> blood –> choroid plexus –> meninges
Lesch-Nyhan Syndrome
- Inheritance
- Specific defect
- What are the results of this defect?
- X-linked recessive
- Defect in hypoxanthine-guanine phosphoribosyltransferase (HGPRT)
- Results in failure of the purine salvage pathway leading to inc. degradation of hypoxanthine and guanine to uric acid. De novo purine synthesis must inc. to compensate via inc. PRPP amidotransferase
Tay-Sachs Dz
- Inheritance
- Enzyme that is deficient
- What accumulates?
- Presentation
- Autosomal recessive disorder
- B-hexosaminidase A deficiency
- GM2 ganglioside accumulation
- Presentation: progressive neurodegeneration and cherry-red macular spot, NO HEPATOSPLENOMEGALY!!! (unlike Niemann-Pick dz)
How does eithanol block gluconeogenesis?
Ethanol metabolism reduces NAD+ to NADH and increases the NADH/NAD+ ratio.
This inhibits all other pathways requiring NAD+, including reactions required for gluconeogenesis.
What bones meet at the pterion? What vessel lies underneath it?
Frontal, parietal, temporal, and sphenoid bones
Middle meningeal artery (branch of the maxillary artery)
Most severe AE of the anthracyclines (rubicins)
Cumulative dose-related dilated cardiomyopathy, due to formation of free radicals in the myocardium
Subthalamic nucleus
- What pathology can cause damage to this structure?
- What is the consequence of this damage?
- Lacunar stroke can cause damage
- Damage may result in contralateral hemiballism, characterized by wild, involuntary, large-amplitude, flinging movements of proximal limbs on one side of the body
What is ARDS?
- Pathology?
- Presentation?
- Histo?
- What main diseases/issues can lead to ARDS?
Acute Respiratory Distress Syndrome
- Characterized by diffuse injury to the pulmonary microvascular endothelium and alveolar epithelium, resulting in increased pulmonary capillary permability and a leaky alveolocapillary membrane
- Presents as progressive hypoxemia refractory to O2 therapy and diffuse interstitial edema w/o cardiogenic cause.
- Hyaline membranes on histo
- Pancreatitis
What (3) types of drugs disrupt the peptidoglycan cell wall of gram-positive/ gram-negative organisms?
- Penicillans
- Cephalosporins
- Vancomycin
For neonates infected with Hep B, what is their…
- level of HBV replication?
- level of liver enzymes?
- risk of chronic infection?
- High HBV replication
- mildly elevated liver enzymes
- very high (90%) risk of chronic infection
Metabolism of 1g of protein or carbohydrate, produces ______ Calories of energy.
Metabolism of 1g of fat produces _____ Calories of energy.
P/C: 4 C
F: 9 C
Naltrexone
- MOA
- Indication
- Mu-opiod receptor
- First line tx for moderate to severe alcohol use disorder (blocks the rewarding and reinforcing effects)
Main Side Effect difference between ACE-I’s and ARBs
ACE-I’s raise the level of Bradykinin causing non-productive cough.
ARBs do not.
The two drugs are very similar otherwise
Myotonic dystrophy presentation (5)
Sustained muscle contraction (can’t release doorknob) + weakness/atrophy
(often also see cataracts, frontal balding and gonadal atrophy)
What is moa of mineralocorticoid receptor antagonists? What diseases are they good for and who should they not be used for?
Give (2) examples
(Spironolactone and Eplerenone)
They prevent aldosterone from binding to its receptor in the distal renal tubules, leading to increased sodium excretion.
Indications: CHF and reduced left ventricular EF
Contraindications: Hyperkalemia and renal failure
Kussmaul sign
Paradoxical rise in JVP during inspiration, because volume-restricted right ventricle is unable to accomadte the inspiratory increase in venous return.
Associated with constrictive pericarditis
Name the key characteristics of which distinguish Slicosis from other forms of pneumoconiosis (2)
How can silicosis impair immune function?
- Eggshell calcification at the hilar nodes
- Birefringent silica particles surround by fibrous tissue on histology
Silicosis impairs the macrophages, by disrupting them with internalized silica particles
Where are dietary lipids digested? Where are they absorbed?
- Digestion: in the duodenum via pancreatic enzymes
- Absorbed: in the jejunum in the form of water-soluble micelles
What are the (2) main effects of nitrates?
- Venodilation (as well as general vasodilation)
- Decreased preload (i.e. decreased LV EDV and EDP)
All told, this results in decreased left ventricular systolic wall stress and myocardial oxygen demand
What is the neurological effect of vitamin B12 deficiency?
Subacute combined degeneration!!!
demyelination of dorsal columns, lateral CST, and spinocerebellar tracts, leading to: ataxic gait, paresthesia, and impaired position/vibration sense
Why is N-myc capable of being detected by DNA probes?
It is a transcription factor and thus is capable of binding DNA.
Describe Type IV Hypersensitivity
How are antibodies involved?
What are the (4) T’s of this hypersensitivity?
Aka delayed (T-cell mediated) type
Sensitized T cells encounter antigen and then release cytokines (leading to macrophage activation)
No antibodies involved
4 T’s:
T cells
Transplant rejections
TB skin tests
Touching (contact dermatitis)
What is the relationship between Cystic Fibrosis, ADEK, and squamous metaplasia?
- CF can lead to pancreatic insufficency, fat malabsorption, and an ADEK deficiency.
- Vitamin A maintains orderly differentiation of specialized epithelia.
- Avitaminosis A can cause squamous metaplasia of such epithelia to keratinizing epithelium
What tumor type(s) is the following tumor marker assc. with?:
Alpha fetoprotein
- Hepatocellular carcinoma
- Germ cell tumors
Conversion disorder
Neurological symptoms that are incompatible with a neurological disease. Often stressed associated.
At what point in the respiratory cycle is the total pulmonary vascular resistance at its lowest?
The functional residual capacity (the trough of the tidal volume)
Frontal Lobe Syndromes
- Cause
- Clinical manifestation
- Left sided vs Right sided
- Dx
- Cause: Damage to frontal lobe
- Presentation: Issues with executive fxn (complex tasks, motivation, organization, etc.) and personality
- Left sided: apathy/depression | Right sided: disinhibtion
- Dx: info from fam/friends, neuropsych testing and structural brain imaging
What effect might fibrinolytics have on heart rhythm?
They may cause a repurfusion arrhythmia on arterial reopening. These arrhytmias are usually benign.
AV Shunts
- What affect do they have on preload/afterload?
- What might the physical exam reveal?
- AV shunt increases the preload and decreases the afterload by routing blood directly from the arterial system to the venous system
- Physical exam may reveal pulsatile mass w/ thrill on palpation. Ausculation reveals a constant bruit over the site.
Stimulation of the vagus nerve would have what effect on the lungs?
It would cause bronchoconstriction and increased bronchial mucus secretion via Ach binding to M3 receptors. This ultimately increases the work of breathing.
Ulcers in which of these locations are NOT associated with an increased risk of carcinoma in that location?
- Duodenum
- Esophagus
- Stomach
- Colon/Rectum
Of these, only Duodenal ulcers are not associated with an inc. risk of CA
What organelle serves as the primary site of ribosome synthesis and assembl?
The nucleolus (All ribosomal RNA except 5S RNA is transcribed here)
CREST Syndrome
- What is this disease a subset of?
- Pathology?
- What are the sx?
- Limited scleroderma
- Increased proliferation and accumulation of monoclonal T-cells in affected tissues. Leads to increased TGF-B release and thus increased production of collagen and ECM
- Calcinosis, Raynaud’s, Esophageal dysmotility, Sclerodactyly, Telangiectasia
Ortner syndrome
When mitral stenosis causes left atrial dilatation sufficient to impinge on the left laryngeal nerve
Wilson Dz
- Sx (3)
- Dx
- Sx: Liver injury (sometimes asymptomatic), psych sx’s (impulsive and moody), Basal ganglia injury (leading to parkinsonism)
- Dx: Kayser-Fleischer rings; labs show increased transaminases (liver injury) and low serum ceruloplasmin
- Acid-fast stans ID organisms that have ______ ______ (2) in their cell walls.
- How do acid fast stains specifically work?
- Mycolic Acid (like mycobacterium and some Nocardia species)
- Aniline dye is applied to a smear and then is decolorized w/ acid alcohol
What are the two ways in which Beta-blokers ameliorate the sx of thyrotoxicosis?
- Decreasing the effect of adrenergic impulses on target organs
- Decreasing the rate of peripheral T4 to T3 conversion
What type of injury does Carbon tetrachloride (CCL4) cause, and how?
It causes free radical injury, after being oxidized by the P450 system. The result is CCl3 which causes lipid degradation and formation of H2O2
PaO2
A measurement of the partial pressure of oxygen dissolved in the plasma only! (not in the RBCs)
Lesch-Nyhan Syndrome
- Inheritance?
- Presentation?
- Pathology?
- The [] of what enzyme increases as a result?
- X-linked recessive
- Presentation: self-mutilation, hyperuricemia, dystonia, and choreoathetosis in the first few years of life
- Pathology: HGPRT deficiency (used in purine salvage pathway). Leads to degradation of hypoxanthine bases into uric acid.
- Increased demand for de novo purine synthesis leads to increased PRPP activity
Hepcidin
- What is it/ what does it do?
- When is it released?
- Hepcidin is an acute phase reactant synthesized by the liver that acts as the central regulator of iron homeostasis. It prevents release of iron bound by ferritin
- High iron levels/ inflammatory conditions increase hepcidin synthesis. Hypoxia and increased EPO lower hepcidin levels
Subarachnoid Hemorrhage (SAH)
- Presentation
- Pathology and associated diseases (3)
- Dx
- Sx: “Worst headache of my life” + fever/nuchal rigidity
- Path: Saccular (berry) aneurysms are most common cause. Associated with Ehlers-Danlos syndrome, ADPKD, and AV malformations
- Dx: Non-contrast CT showing hyperdensity in cisterns/sulci; LP showing gross blood or xanthochromia (yellow discoloration of the CSF)
What is the main dz Primary Biliary Cirrhosis (PBC) is associated with?
Sjrogren’s syndrome (and other autoimmune disorders)
What are the normal adult pressures in the cardiac chambers, the pulmonary artery and the aorta (minimum and maximum)
RA: 0 and 8 mmHg
RV: 4 and 25 mmHg
PA: 9 and 25 mmHg
LA: 2 and 12 mmHg
LV: 9 and 130 mmHg
Aorta: systolic BP
What is responsible for the green discolortion of pus or sputum during bacterial infections?
Myeloperoxidase (MPO) from neutrophil azurophilic granules. MPO is a heme-containing pigmented molecule.
What type of polyps are most likely to undergo malignant transformation?
Villous adenomas
What type of epithelium makes up the true vocal cords?
Stratified squamous
Prostacyclin vs Thromboxane A2
Compare their effects
Prostacyclin: vasodilates, inhibiting platelet aggregation and increasing vascular permeability
Thromboxane A2: prostaglandin which enhances platelet aggregation and causes vasoconstriction
McCune-Albright Syndrome
Name the triad of clinical features
- Fibrous dysplasia (multiple osteolytic-appearing lesions of the hip/ pelvis)
- Cafe-au-lait spots
- Endocrine abnormalities (precocious puberty, hyperthyroidism, etc.)
What is responsible for the clinical variability of mitochondrial dz?
The mixture of two types of genetic material, known as heteroplasmy. In other words, during mitosis there is a random distribution of mitochondria, some containing lots of damaged mitochondria, others containing very few.
What key structures form from the third pharyngeal pouch?
Thymus and inferior parathyroid glands
The venous component of internal hemorrhoids drain where?
External hemorrhoids?
Internal: drain into the middle and superior rectal veins, which communicate with the internal iliac and inferior mesenteric veins
External: drain via the inferior rectal vein into the internal pudendal vein, which communicates with the internal iliac veins
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Describe the echocardiogram assc. with Transposition of the Great Arteries (TGA)
Aorta lying anterior to the pulmonary artery
Where is ADH synthesized? Is this the same location, or a different location from where it is stored?
In the hypothalamus. It is then transported, to the posterior pituitary for storage and later release into the circulation.
Only hypothalamic damage will be enough to cause central Diabetes insipidus
- Pernicious anemia (Vit B12 def.) is caused by autoimmune destruction of what cells?
- What do these cells secrete and where are they located?
- Destruction of parietal cells (chronic atrophic gastritis).
- They secrete HCL and IF, and are found primarily in the superficial region of the gastric glands
Deletions or additions of a number of base pairs which are not a multiple of three, indicate that a ___________ mutation has occured.
Frameshift
What prompt eosinophils to release their cytoplasmic granules to kill a parasite?
When parasites enter the body they are coated by IgG and IgE which bind to the Fc receptor on the eosinophil, prompting it to degranulate.
(Antibody-dependent cell-mediated cytotoxicity)
Eos also contribute to late stage hypersensitivity
If a pneumonia patient has a CD4+ count greater than 400cells/uL, what bug do you expect to be the cause?
>400?: strep pneumo
pneumo jiroveci (PCP)
What effect does expgenous steroid abuse have on hematocrit?
Increases hematocrit
Meckel diverticulum
- What is it/ How common is it?
- What causes it?
- The most common congenital anomaly of the small intestine
- It forms due to incomplete obliteration of the omphalomesenteric duct that connects the midgut lumen and yolk sac cavity
For an otherwise health child, what is the most common cause of osteomyelitis?
Staph aureus
How is the pathogenesis of Non-bacterial thrombotic endocarditis (NBTE) related to that of Trousseau’s?
They can both be induced by disseminated cancers, usually due to some hypercoagulable state.
Location of femoral hernias
Inferior to the inguinal ligament, lateral to the pubic tubercle, and medial to the femoral vein
Coordinate each artery with the leads associated with them
- LAD
- LCX
- RCA
- Anterior and Septal
- Left lateral
- Inferior and Right
Aschoff bodies
What are they and with what dz process are they found?
What is another name for the cellular component?
They are interstitial myocardial granulomas, found in myocarditis due to acute rheumatic fever
The macrophages are aka as caterpillar cells
Phosphorylation of what residues (and via what?), leads to insulin resistance?
Phosph. of serine and threonine residues of insulin receptor and insulin receptor substrate by serine kinase, leads to insulin resistance
Prinzmetal’s (variant) Angina
- Pathology?
- When does it occur?
- Dx?
- Tx?
- What drug can make it worse?
- Episodic, transient attacks of coronary vasospasm
- Usually occurs at rest and during late night/early morning
- Temporary transumral MI w/ ST elevation on EKG
- Tx with vasodilators and CCBs
- Ergonovine can provoke vasospasm and can aid in diagnosis
Aortic stenosis
- Common murmur
- Sx
- Main causes
- Crescendo-decrescendo murmur right sternal border
- Typically asymptomatic. If advanced can present w/ exertion and inlcude syncope, dizzyness, angina or even HF
- Main causes: abnormal valve w/ calcification (e.g. bicuspid aortic); calcified normal valve; or rhemuatic heart disease
Adult type Aortic Coarctation
- Key triad
- View on imaging
- (1) upper body hypertension; (2) diminished lower extremity pulses; (3) enlarged intercostal artery collaterals
- Notching of ribs as a result of the enlarged, tortuous intercostal arteries
What drug group is responsible for most of the overdoses in the country?
Opioids
Chronic Granulomatous Disease
- Pathogenesis?
- Presentation?
- Pathogenesis: A genetic defect in the NADPH oxidase complex decreases the formation of reactive oxygen species which have a direct microbicidal activity and also activate granule proteases.
- Presentation: Child with recurrent bacterial and fungal infections
Coccidioides immitis
- Describe the (2) forms
- Where is it endemic to?
- How is it transmitted?
- How does it present (5)
- Mold in the cold, endospore in the heat
- Southwest US, northern Mexico, regions of Central and South America
- Transmitted via spore inhalation which form endospores that rupture and disseminate
-
Presentation:
- acute pneumonia (most common)
- chronic pneumonia
- pulmonary nodules and cavities
- extrapulmonary nonmeningeal disease
- meningitis
Colonic Diverticula
- Involve what part of the colon?
- What process leads to their development?
- Usually involve the sigmoid colon
- The result of increased intraluminal pressure (pulsion) created during strained bowel movements
What is the MOA and Indication for both Cromolyn and Nedocromil? How effective are they compared to drugs with a similar indication?
They are mast cell stabilizing agents which inhibit mast cell degranulation independent of stimuli.
They are a second-line treatment for allergic rhinitis and bronchial asthma. Glucocorticoids are the first line prophylactic and are a superior drug.
Primary Carnitine Deficiency
- What is the effect at the level of the cell?
- Presentation
- Deficiency in carnitine impairs fatty acid transport from the cytoplasm into mitochondria, preventing B-oxidation of fatty acids into acetyl CoA
- Sx: Cardiac and skeletal myocyte injury (lack of ATP from Citric acid cycle) and impaired ketone body production by the liver during fasting periods
Describe the process of Infective Endocarditis (4)
- Disruption of normal endocardial surface (usually in areas of maximum turbulence)
- Focal adherence of fibrin and platelets, forming sterile fibrin-platelet nidus
- Colonization by microorganisms (strep to damaged areas; staph to damaged or normal areas)
- Formation of macroscopic vegetations made of debris
Aortic Dissection
- Most important risk factor
- What specific layer tears?
- What genetic issue predisposes for aortic dissection?
- HTN is most important risk factor
- Tunica intima is the layer which tears away
- Marfan’s which leads to cystic medial degeneration
What is the most abundant AA in collagen?
Glycine (occupies every thrid AA position)
Osler-Weber-Rendu Syndrome
- What is the other name?
- Genetics
- Clinical Presentation
- Possible consequences
- Hereditary hemorrhagic telangiectasia
- Autosomal dominat
- Presence of telangiectasias in the skin and mucous membraes of the lips, oropharynx, respiratory tract, GI tract and urinary tract
- Rupture of these vessels may cause epistaxis (nosebleeds), GI bleed, or hematuria
What is the effect of low hepcidin levels?
Low hepcidin levels increase intestinal iron absorptin and stimulate iron release by macrophages
Lambort-Eaton syndrome
- Sx
- Disease associated with
- Pathogenesis
- Tensilin test result
- Nerve stimulation studies
- Sx: weakness improves during the day and with excercise. Weakness of proximal muscles
- Assc. with pre-existing malignancy
- Path: antibodies against pre-synaptic calcium channels
- No improvement from tensilon test
- Incremental response from nerve stim. test
How is Campylobacter infection spread?
Via domestic animals or from contaminated food
Tumor lysis syndrome
- When can it develop?
- What characterizes it?
- Develops during chemo for CAs with rapid cell turnover, high tumor burden, or high chemo sensitivity
- Characterized by a leak of intracellular ions (such as hyperphosphatemia, hypocalcemia, hyperkalemia, and hyperuricemia)
What is the most common cause of bacterial meningitis in adults of all ages?
Strep Pneumo
Serotonin syndrome
- How do we get this syndrome?
- Sx (3)
- What is the antidote?
- What is the precursor to serotonin?
- The result of either serotonin overdose or co-prescription with an MAOI or triptan
- (1) neuromuscular excitation, (2) autonomic stimulation, (3) altered mental status
- Antidote- Cyproheptadine ( a first generation histamine antagonist)
- Tryptophan is the precursor to serotonin
Dopamine: What is the effect of…
- Low doses?
- Medium doses?
- Very high doses?
- Low dose: stimulate D1 receptors in the renal and mesenteric vasculature
- Medium dose: stimulate B-1 receptors, increasing cardiac contractility
- High dose: stimulate a-1 receptors, producing generalized vasoconstriction
First generation anti-histamines
- Name them
- In addition to blocking histamine receptors, what other affects?
- How can the eye be affected?
- Ex. Chlorpheniramine, diphenhydramine
- Also have antimuscarinic, anti-alpha adrenergic, and anti-serotonergic properties
- Anticholinergic effects on occular ciliary muscles impair accomdation and cause blurring of vision for close obects
Vitamin A toxicity
- Acute affects (3)
- Chronic affects (5)
- Teratogenic affects (3)
- Acute: N/V, vertigo, blurred vision
- Chronic: alopecia, dry skin, hyperlipidemia, hepatotoxicity/ hepatosplenomegaly, and visual difficulties
- Teratogenic: Microcephaly, cardiac anomalies, fetal death
MOA of B-Blockers
B-Blockers decrease AV nodal conduction, leading to an increased AV nodal refractory period
What are the (5) general dz types associated with clubbing?
- Lung dzs (oft associated with hypoxia)
- Heart dzs (especially cynotic congenitals dzs and bacterial endocarditis)
- IBD
- Hyperthyroidism
- Malabsorption
Main phase “0” difference between regular cardiac cell and a pacemaker cell
Regular cardiac cell: 0 = Na+ rush
Pacemaker: 0 = Ca2+ rush
Vascular and Immunologic Manifestations of Infective Endocarditis
(3) Vascular
(2) Immunological
Vascular:
- Systemic emboli
- Mycotic aneurysm
- Janewy lesions (nontender- palms/soles)
Immunologic:
- Osler nodes (Painful- toes/fingertips)
- Roth spots
Viral Laryngotracheitis
- Also known as?
- Presentation?
- Most common virus responsible?
- Croup
- Brassy, barking cough; Dyspnea; Recent history of upper respiratory infection (URI)
- Parainfluenza virus is the most common cause
Elevated prolactin levels (from a prolactinoma), directly suppress what?
GnRH
Capsaicin causes depletion of what neurotransmitter? What does this lead to?
Substance P
An intitial burning/stinging sensation, but chronic exposure leads to reduced pain transmission
Omalizumab MOA/ indication
Anti-IgE antibody
Add-on therapy for patients with severe allergic asthma
What are the (2) major effects of Clostridium perfingens?
- Late-onset food poisoning (consisting of transient watery diarrhea)
- Clostridial myonecrosis (gas gangrene)
Which blood vessels does the third part of the duodenum interact with?
It crosses horizontally acros the abdominal aorta and the IVC at the level of L3.
The Superior mesenteric vessels lie anterior to the duodenum at this location.
What are the (4) major causes of hypoxemia? Which has a normal A-a gradient?
- Alveolar hypotension (normal A-a gradient)
- V/Q mismatch
- Diffusion impairment
- Right-to-left shunting
What kind of drug are theophylline and aminophylline? What is their MOA?
They are Methylxanthines
They cause bronchial dilatation by decreasing phosphodiesterase enzyme activity, thereby increasing intracellular camp.
Compare cardiac tissue conduction velocity between the:
AV node, purkinje system, ventricular muscle, and atrial system
_P_ark _At_ _Vent_ure _Av_enue
Fastest- Purkinje system, Atrial system, Ventricular system, AV node- slowest
Wilson Disease
- Inheritance
- Path
- Autosomal recessive mutation of the ATP7B gene
- Path: Mutation leads to a decrease in copper incorporation into ceruloplasmin and reduced biliary copper excretion leading to copper accumulation in liver, brain, and eye
Hyperacute rejection vs acute rejection vs chronic rejection
(particularly in the lung)
Time-frame? What part of the lung is affected?
Hyperacute: Occurs in minutess. Preformed antibodies lead to graft blood vessel spasm and diffuse intravascular coagulation (white graft rejection)
Acute: 1-2 weeks. Due to recipients rection to the HLA of the graft. Causes vascular damage and bronchial infiltration. Perihilar and lower lobeopacities on CXR.
Chronic: months to years after transplant. Inflmmation of the small bronchioles (lungs) or vessels (kidneys) leading to narrowing and obstruction
Which cell types have B1 receptors?
Cardiac tissue and renal juxtaglomerular cells
Name the main organisms that can cause diarrhea with only a small inoculum (4)
- Shigella
- Entamoeba histolytica
- Giardia lamblia
- Campylobacter jejuni
Gallstone ileus
- What is it?
- Presentation
- Dx
- Obstruction in the ileum due to passage of a large gallstone through a cholecystenteric fistula in the small bowel
- SBO
- Dx: Ab XR may reveal gas within the gallbladder and biliary tree
Mechanisms of bacterial transfer: Transformation vs Conjugation vs Transduction
Transformation: Direct uptake of naked DNA form the environment
Conjugation: One way transfer of chromosomal or plasmid DNA between bacteria via direct physical contact (one of them must have F factor which codes for a sex pilus).
Transduction: Transfer via bacteriophage
Name the pentad for Thrombotic thrombocytopenic purpura
- Neurologic symptoms
- Renal failure
- Fever
- Thrombocytopenia
- Microangiopathic hemolytic anemia
What is the most common genetic cause for:
Hypertrophic cardiomyopathy?
Dilated cardiomyopathy?
- Hypertrophic: Autosomal dominant mutations in cardiac sarcomere proteins (usually beta-myosin heavy chain)
- Dilated: Autosomal dominant mutations of myocyte cytoskeleton (dystrophin) or mitochondrial enzymes
Migraine
- Sx
- Abortive therapy (1)
- Prophylactic therapy (3)
- Unilateral HAs, with pulsating/throbbing quality, associated with photophobia, phonophobia, and nausea
- Abortive: Triptans (serotonin agonists)
- Prophylactic: Beta-blockers, antidepressantts, and anti-convulsants
How is tolerance to nitrates avoided?
You must provide a nitrate-free interval every day in patients w/ long acting nitrates.
Fat Embolism
- Presentation?
- Staining?
- Severe resp. distress + diffuse neurological impairment + upperbody petechial rash, following long bone injury
- Fat emboli turns block from stain w/ osmium tetroxide
The 22q11.2 microdeletion associated with DiGeorge, causes what embryological component(s) not to develop?
3rd and 4th branchial pouches
What cranial nerves and what aortic arch derivatives are associated with each pharyngeal/aortic arch (1-6)
- CN V; Maxillary artery
- CN VII; Stapedial artery (regresses)
- CN IX; Common carotid and prox. internal carotid
- CN X (superior laryngeal); True aortic arch and Subcalvian arteries
- Obliterated
- CN X (recurrent branch); Pulmonary arteries and Ductus arteriosus
In patients with mitral regurg, what is the most reliable ausculatory finding to assess severity
The presence of a left-sided S3 gallop. This indicates high regurgitant volume and left ventricular volume overload
Isotretinoin
Indication and Contraindication
Indication: Used to tx severe acne w/ significant scarring
Contraindication: Pregnancy, due to high risk of teratogenicity
Describe the biopsy specimen associated giant cell arteritis. What other dz is this identical to?
Scattered, focal granulomatous inflammation centered on the media with :
- intimal thickening
- elastic lamina fragmentation
- giant cell formation (not granulomatous)
Same histo as Takayasu arteritis
Describe the process of staphylococcal food poisoning
It often occurs after a food handler inoculates food (usually a mayonnaise containing product), w/ S.aureus that is allowed to incubate at room temp, producing heat-stable exotoxin that causes rapid-onset N/V and abdominal cramping
Describe the mechanism with which Sarcoidosis can lead to hypercalcemia
1-a hydroxylase expression in activated macrophages, leads to PTH independent Vit D activation and subsequent inc. intestinal absorption of Ca2+
- Who should be vaccinated against Strep Pneumo?
- What are the (2) main vaccines?
- Elderly/ young children are most at risk
- Both affect the outer polysaccharide covering:
- Pneumococcal polysacharide vaccine
- Pneumococcal conjugate vaccine
What type of drug is useful for nausea assc. with GI insults?
For nausea assc. with migraine?
GI: 5-HT3 receptor antagonists
Migraine: Dopamine antagonists
What disease are caused by “exotoxin” release by S. aureus?
- Toxic shock syndrome
- Staphylococcal scalded syndrome
- Gastroenteritis
What is the role of protein A in staph aureus?
Protein A is a virulence factor found in the peptidoglycan wall of Staph Aureus that binds the Fc portion of IgG, leading to impaired complement activation, opsonization, and phagocytosis.
Ehlers-Danlos syndrome
- What is it?
- Usual cause?
- Presentation
- Group of rare hereditary disorders charcterized by defective collagen synthesis
- The result of procollagen peptidase deficiency, which results in impaired cleavage of terminal propeptides in the extracellular space
- Patients present with joint laxity, hyperextensible skin, and tissue fragility, due to collagen which does not crosslink
What is the indication for an ace-inhibitor? For a thiazide?
ACE-inhibitors: inhibit chronic angiotensin II-mediated remodeling that occurs in association w/ MI and CHF
Thiazides: useful as an initial treatment for essential HTN w/o CHF or diabetes
What cell type composes the wall of the cyst in the years after a stroke?
Astrocytic processes (glial scar)
Selective IgA Deficiency
- Pathology
- Presentation
- What are these patients at risk for?
- Pathology: Failure of B-cells to switch from IgM to IgA production. Very common
- Presentation: Usually asymptomatic but can have recurrent sinopulmonary and GI tract infections
- Risk: Many of these patients form IgG antibodies directed against IgA so risk anaphylaxis during transfusion
What does the smooth ER contain? What type of cells contain lots of smooth ER?
(In contrast to the RER,) the smooth ER contains enzymes for steroid and phospholipid biosynthesis. ALL steroid-producing cells (adrenals, gonads, liver, etc.) contain a well-developed smooth ER.
Mesothelioma
What is it?
Risk factor(s)?
Presentation?
Dx factors? (3)
- Rare malignant neoplasm of mesothelial cells.
- Primary risk factor is consistent Asbestos exposure
- Symptoms include: dyspnea and chest pain
- Dx tests will show:
- Hemorrhagic pleural effusion
- Pleural thickening on radiographic study
- Long slender microvilli and abundunt tonofilaments on histo.
Dystrophic vs Metastatic calcification
What conditions do they each occur in?
Dystrophic: Occurs normally, w/ age, in damaged or necrotic tissues in the setting of normal calcium levels.
Metastatic: Occurs in normal tissue in the setting of hypercalcemia
Klebsiella
Encapsulated or not?
Lactose-fermenting or not?
Usual presentation/ patients affected/characteristics?
- Encapsulated
- Lactose fermenting (grows pink on MacConkey agar)
- It causes pneumonia in subjects with impaired host defenses, especially alcoholics
- Characterized by tissue necrosis, early abscess formation and currant jelly sputum
How can Isoniazid lead to sideroblastic anemia? Describe the process in detail (3 main steps)
- Isoniazid (TB drug) inhibits pyridoxine phosphokinase, leading to a vitamin B6 deficiency (pyridoxine).
- The active form of pyridoxine is a cofactor for delta-aminolevulinate synthase, an enzyme which catalyzes the rate-limiting step of heme synthesis.
- Inhibition leads to sideroblastic anemia.
What drugs do you give to reverse heparin effects? Warfarin effects?
Heparin: protamine sulfate
Warfarin: Vitamin K and Fresh Frozen Plasma (FFP)
Describe the pathogenesis of centriacinar emphysema
- Oxidative injury to the respiratory bronchioles by smoking
- Macrophage activation
- Inflammatory recruitment of neutrophils
- Release of proteases by neutrophils and macrophages
What sort of procedure is associated with enterococcal endocarditis
Genitourinary instrumentation or catherization (enterococcus is a component of normal colonic and GU flora)
Antiarrhythmic drugs
- Drugs + Predominant actions
- Potential SEs
Class IV
Verapamil; Dilitiazem
- Slows sinus node discharge rate; slows AV nodal conduction and prolongs refractoriness
- Can lead to severe bradycardia and hypotension (especially in combo with a beta-blocker)
Hexokinase vs Glucokinase
- Location
- Affinity vs Capacity?
- Induced by insulin?
- Mutation assc. with Maturity-onset diabetes of the young?
- H: Everywhere but liver and pancreatic B cells; G: Liver and pancreatic B cells
- H: High affinity, low capicity; G: low affinity, high capacity
- H: Not induced by insulin; G: induced by insulin
- H: Not assc. with MODY; G: Assc. w/ MODY
What components are needed to do PCR? (4)
- Primers that are complementary to the regions of DNA flanking the segment of interest
- Thermostable DNA polymerase
- Deoxynucleotide triphosphates
- A source DNA template strand
Ghon Complex and TB reactivation (who is it in and what characterizes it?)
This complex characterizes the initial stages of M. tuberculosis infection. It consists of a lower lobe lung lesion (Ghon focus) and ipsilateral hilar adenopathy.
Reactivation of TB occurs most often in immunosuppressed patients and is characterized by apical cavitary lesions and hemoptysis.
What sometimes occurs between days 2-4 following a transmural MI? Why?
Early-onset pericarditis develops in 10-20% of patients. It represents an inflammatory reaction to adjacent cardiac muscle necrosis.
Define Preventable Adverse Effect
An injury to a patient due to failure to follow evidence-based best practice guidlines.
(Not to be confused with malpractice, which is a legal determination)
What is the usual clinical presentation for Hyper-IgM syndrome? (2)
- Lymphoid hyperplasia
- Recurrent sinopulmonary infections
Patient presents due to lightheadedness/ passing out while buttoning a tight shirt collar.
- Most likely dz process
- What nerve is most assc. with this dz process?
- Carotid sinus hypersensitivity (triggered by pressure from the shirt)
- The afferent limb of the carotid sinus reflex is a branch of the glossopharyngeal nerve
McCune-Albright Syndrome
Pathogenesis
Mutation in the GNAS gene which leads to constitutive activation of the G protein/cAMP/ adenylate cyclase signaling cascade
Digoxin MOA
Digoxin directly blocks the Na/K+ pump in myocardial cells, leading to increased intracellular Na. This slows functioning of Na+/Ca+ exchanger, thereby keeping Ca+ trapped in the the myocardial cell as well, increasing contractility.
Huntington disease
- Describe the genetic/cellular level cause of this disease.
- How are histones involved?
- Increased CAG trinucleotide repeats in the gene that codes for the huntingtin protein, leading to gain-of-fxn mutation.
- Transcriptinal repression (silencing), via histone deacetylation, is thought to be one of the main mechanisms in which this mutation causes disease
What is the most common viral cause for aplastic crisis for sickle cell patients?
Parvovirus B19
Tetrodotoxin
- Where does it come from?
- What effect does it have?
- Comes from pufferfish
- Binds to voltage-gated sodium channels in nerve and cardiac tissue, preventing sodium influx and depolarization – leads to paralysis
Pulsus Paradoxus
- What is it?
- How is it detected?
- Why does it happen?
- What disease processes is it associated with? (4)
- An exaggerated drop (>10mmHg) in systolic BP during inspiration
- Detected when taking the BP, by listening to the difference between when korotkoff sounds are first heard during expiration and the pressure at which they are heard throughout all phases of respiration
- Inspiration inc. venous return and normally this expands RV into pericardium but if this expansion can’t happen, the interventricular septum pushes into the LV, dropping LV EDV and subsquently the stroke volume
- Pericardial disease, acute cardiac tamponade, asthma, COPD
What tumor type(s) is the following tumor marker assc. with?:
Carcinoembryonic antigen
GI (colorectal)
Achalasia
- Cause
- What is seen on esophageal manometry?
- Caused by reduced numbers of inhibitory ganglion cells in the esophageal wall
- Manometry shows dec. amplitude of peristalsis in the mid esophagus, w/ increased tone and incomplete relaxation at the LES
What pathology does this histological image indicate?
Coccidioides immitis
List the (6) steps for Ggucose-induced insulin release from beta cells
- Glucose enters the beta cell through GLUT-2
- Glucose is metabolized by glucokinase to glucose-6-phosphate
- Glucose 6-phosphate is further metabolized by glycolysis and the Krebs cycle to produce ATP
- High ATP to ADP ratio causes closure of ATP-sensitive K+ channels
- Subsequently, depolarization of beta cells results in opening of voltage-dependent Ca2+ channels
- High intracellular Ca2+ leads to insulin release
Patients with Down Syndrome are at an increased risk for what CA types?
ALL and AML
Torsades de Pointes
- Pathology
- What are the most common precipitants? (3 categories + examples)
- Polymorphic v.tach that occurs in the setting of a congenital or acquired prolonged QT interval
- Medications such as certain (1) antiarrhythmics (sotalol, quinidine); (2) antipsychotics (haloperidol); (3) antibiotics (macrolides, fluoroquinolones)
When providing a nerve block of the brachial plexus between scalene muscles, what must you be weary of?
Transient ipsilateral diaphragmatic paralysis due to involvement of the phrenic nerve roots as they pass through the interscalene sheath.
What is the site of lowest osmolarity in the nephron?
DCT
Tx of Acute Adrenal insufficiency
Hydrocortisone or dexamethasone
What tumor type(s) is the following tumor marker assc. with?:
PSA
Prostate
Describe the peripheral blood smear associated with lead poisoning
Coarse erythrocyte basophilic stippling and microcytic hypochromic anemia
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Attributable Risk Percent (definition + calculation)
The excess risk in the exposed population that can be attributed to the risk factor.
ARPexposed = 100 x [(RR-1)/RR], where RR = relative risk
MOA of SSRIs
Blockade of the serotonin transporter
Patent Ductus Arteriosus (PDA)
- Pathology
- Why is it patent
- Clinical manifestations
- Typical age at presentation
- Tx.
- Vascular connection between the main pulmonary artery and the aorta still remains after birth
- Patent due to prostaglandin E2 production by the placenta
- Clinical features vary by size: Small: continuous machine-like murmur w/ no other symptoms. Large: progressive pulmonary HTN, reversal of shunt (now right-to-left), ultimately leading to HF and cyanosis (Eisenmenger syndrome), particularly in lower extemities.
- Childhood
- Tx: Indomethacin (prostaglandin E@ inhibitors)
Name the mealtime insulin analogs
Describe their onset/ offset
Glulisine, Aspart, Lispro
They have rapid onset and offset of action, mimicking physiologic postprandial insulin secretion
Maple Syrup Urine Disease
- Pathogenesis
- Sx
- Dx
- Tx
5.
- Blocked degradation of branched amino acids– Isoleucine, Leucine, and Valine (I Love Vermont) due to decreased a-ketoacid dehydrogenase
- Sx: severe CNS defect, intellectual disabiity, and death
- Urine smells like maple syrup/ burnt sugar
- Tx: restriction from branched amino acids in diet, and thiamine supplementation
Describe the key presentations associated with thiamine deficiency?
Beriberi and Werknicke-Korsakoff syndrome
- Wernicke-Korsakoff- Confusion + occulomotor abnormalities + memory deficita + ataxia (COMA)
- Dry beriberi- characterized by symmetrical peripheral neuropathy
- Wet beriberi- includes the addition of high-output CHF
Tissue damage and resultant abscess formation is primarily caused by…
Lyosomal enzyme release from neutrophils
Where do the ureters sit in respect to the common, external, and internal iliac?
Anterior (right on top of them)
Describe Aspergillus fumigatus colonization
- What is it?
- How does it look on imaging?
- Sx
- Aspergillus develops in old lung cavities (from TB, emphysema, sarcoidosis, etc) and it forms a fungus ball w/o tissue invasion
- Appears on CXR as radioopaque structure which shifts when Pt changes postion
- Can cause hemoptysis (otherwise asymptomatic)
POMC, a polypeptide precursor, goes through enzymatic cleavage and modification to produce what (3) proteins?
- Beta-endorphins (endogenous opioid peptide)
- ACTH
- MSH
Ethosuximide MOA
Blocks T-type Ca2+ channels in thalamic neurons
(used to treat absence seizures)
What is Kinesin and what does it do?
A microtubule-associated, ATP-powered motor protein that facilitates the anterograde transport of neurotranmsitter-containing secretory vesicles down axons to synaptic terminals.
Describe the activation of Ras proteins (2 steps)
- Growth factor ligand binds to a receptor tyrosine kinase on the cell membrane, causing autophosphorylation
- Proteins interact with Ras, promoting GDP removal and GTP binding!
What are the (2) mechanisms by which beta-blockers lower blood pressure?
- Reducing myocardial contractility and HR
- Decreasing renin release by the kidney
Antiarrhythmic drugs
Drugs + Predominant actions
Class III
Amiodarone, Dronedarone, Dofetilide, Sotalol (also class II)
Prolongs APD by blocking K+ channels (no effect on AP conduction velocity)
What effect does sickling (in SCD) have on labs?
Leads to
- Increased indirect bilirubin
- Increased lactate dehydrogenase
- Decreased haptoglobin (binds free hemoglobin and is then removed by the spleen)
Buerger’s disease (thromboangiitis oblierans)
- Main population demographics
- Pathophysiology
- Presentation
- Tx
- Heavy smokers, males
- Path- Segmental thromboding vasculitis
- Presentation- Intermittent claudication which can lead to gangrene, autoamputation, and superficial nodular phlebitis. Also associated w/ Raynauds
- Tx: smoking cessation
How is nitric oxide synthesized?
It is synthesized from arginine by NO synthase
Ariginase
- Fxn
- Sx of Arginase deficiency
- Tx of Arginase deficiency
- Fxn: urea cycle enzyme that produces urea and ornithine from arginine
- Sx: (1) Progressive spastic diplegia, (2) growth delay, (3) abnormal movements
- Tx: Arginine-free, low protein diet
Propionyl CoA is derived from which amino acids? (4)
Val, Ile, Met, and Thr
What types of drugs are not effective against organisms of the mycoplasma species? What type are? Why?
All of these organisms lack peptidoglycan cell walls and are therefore resistant to agents that attack this wall, uch as penicllins, cephalosporins, carbapenems, and vanco.
Must treat with anti-ribosomal agents (tetracycline and macrolides)
Describe themost important biochemical abnormality in Alzheimer dz
Where is this decline most notable?
A dec. in Ach level due to the deficiency of choline acetyltransferase, needed to synthesize Ach.
Most notable in the basal nucleus of Meynert or the hippocampus
Why doesn’t Wallerian degeneration occur in the CNS?
Due to the persistence of myelin debris, secretion of neuronal inhibitory factors, and development of dense glial scarring.
What is the best determinate of oral bioavailability of a drug?
AUC (Oral)/ AUC (IV)
This assumes they’ve been administered at equal doses
Lipofuscin
Yellow-brown, granular product of lipid peroxidation and considered to be a sign of “wear and tear”/aging.
Key AE associated with lamotrigine
SJS (30% of body surface)
Relationship between blood flow and radius
Blood flow is directly proportional to vessel radius raised to the fourth power.
What virus is the pictured biopsy associated with?
What is the normal type of patient who has this virus?
Describe the virus?
This biopsy is associated with cytomegalovirus (CMV).
This is particuarly common among patients who have recently had lung transplants or are generally immunocompromised.
It is an enveloped double-stranded DNA virus
Familial chylomicronemia syndrome
- Which protein is defected? (2)
- Which lipoprotein is elevated?
- Major manifestations?
- Defected: Lipoprotein lipase; ApoC-II
- Elevated: Chylomicrons
- Manifestations: Acute pancreatitis; lipemia retinalis (milky lipids in retinal vasculature); eruptive skin xanthomas; hepatosplenomegaly
a-1 blockers are useful for the treatment of … (2 conditions)
What natural secretion has these same effects?
HTN and benign prostatic hyperplasia (relaxes the bladder)
ANP/BNP has the same effects
What drug is used as abortive therapy during an acute migraine?
What is its MOA?
Triptans
They are serotonin 5-HT1B/5-HT1D agonists, and thus inhibit the release of vasoactive peptides, promoting vasoconstriction, and blocking pain pathways in the brainstem.
Fick Principle
An alternative means for calculating cardiac output.
CO = O2 consumption/ AV O2 difference
What is the role of atypical T-cells in assc. with EBV?
They function to destroy virally-infected B-lymphocytes
What is the foundation of management for RA? Why are anti-inflammatory drugs often used?
DMARDs (Dz-modifying antirheumatic drugs) such as methotrexate and sulfasalazine are the most key tx.
DMARDs take weeks to kick in so short-term tx with anti-inflammatory tx is often used in the meantime.
MOA of Sotalol
It has both beta-adrenergic blocking properties and class 3 antiarrhtmic (K+ channel blocking) properties. It prolongs the PR interval and the QT interval.
- What do Central Chemoreceptors sense?
- What do Peripheral Chemoreceptors sense?
- What do Pulmonary Stretch Receptors sense?
- Central: Increased PaCO2
- Peripheral (carotid and aortic bodies): PaO2 – stimulated by hypoxemia
- Pulm Stretch: Regulates duration of inspiration based on degree of lung distension
You see a large cell with multiple nuclei in the shape of a horshoe. What is it called and what disease process is it associated with?
Langhans Giant Cells. Formed from macrophages which are activated in response to pulmonary TB.
Drug-induced Lupus Erythematosus
- Presentation
- Dx
- What is almost never seen?
- Drugs normally implicated
- New onset of lupus symptoms
- Anti-nuclear antibodies (ANA) and anti-histone antibodies
- Anti-dsDNA ALMOST NEVER SEEN
- HIP: Hydralazine, Isoniazid, Procainamide
Amatoxins
- Where are they found?
- What is there MOA?
- Found ina variety of posionous mushrooms
- Potent inhibitors of RNA pol, thus halting mRNA synthesis
What (3) parts of the body does the iliohypogastric nerve innervate?
Sensation to the suprapubic and gluteal regions
Motor function to the anterolateral abdominal wall muscles
Potential complications from hereditary spherocytosis (2)
- Pigmented gallstones
- Aplastic crises from parvovirus B19 infection
What type of cells are seen in the pulmonary vasculature in cases of an Amniotic fluid embolism?
Fetal squamous cells
Path and Presentation of patients with 21-hydroxylase deficiency
Deficient cortisol and aldosterone synthesis + adrenal androgen overproduction
Males: normal genitalia + vomiting, hypoTN, hyponatremia, hyperkalemia
Females: ambiguous genitalia (+ sx above)
Meningiomas
- Pathophysiology?
- Who is usually affected?
- Where do these tumors occur?
- Presentation?
- Common, slow-growing (benign), intracranial tumors
- Typically adults
- Arise in regions of dural reflection (falx cerenri, tentorium cerebelli, etc.)
- Patients are often asymptomatic but may present w/ HA, seizure or focal neurologic deficits depending on size/ location of tumor
What molecules take on a trple helical conformation?
Collagen, due to repetitive AA sequences within each alpha chain
- Name the non-selective beta-blockers
- Name the selective beta-blockers
- Why would you choose selective over non-selective?
- Non-Selective- Propanolol, timolol, and nadolol
- Selective- metoprolol, atenolol, acebutolol and esmolol
- Selective only target B1, so if you have patients w/ COPD/asthma, you want to only use these.
What are the type II Anti-arrhythmics?
Beta-blockers
The (2) most signficant risk factors for development of esophageal SCC in the US
Smoking tobacco and drinking alcohol
What is Annular Pancreas? What might it cause?
The ventral pancreatic bud abnormally encircles the 2nd part of the duodenum, forming ring of pancreatic tissue. May cause duodenal narrowing.
Amyotrophic Lateral Sclerosis
- Sx
- Gross anatomy
- Histo/ microscopic
- Genetics
- Tx
- Sx: LMN- muscle weakness, atrophy, fasiculations; UMN- spasticity, hypereflexia, pathologic reflexes
- Gross: (1)Thin anterior roots; (2) mild atrophy of precentral gyrus
- Histo/micro: loss of anterior horn neurons, lateral CSTs, motor nuclei neurons, and denervation atrophy of muscles
- Genetics: mutation of gene for superoxide dismutatse (SOD1) may be implicated
- Tx: Riluzole (decreases glutamate release)
- Peroxisomes are responsible for metabolizing what?
- What does Peroxisomal disease commonly lead to?
- Metabolizes very long chain fatty acids or fatty acids with branch points at odd-numbered carbons since they cannot undergo mitochondrial beta-oxidation
- Can lead to neurologic defects from improper CNS myelination
Name the muscles of the rotator cuff and what each one does
SITS
Supraspinatus- abducts arm initially (before deltoid takes over)
Infraspinatus (laterally rotates arm)
Teres minor (adducts and laterally rotates arm)
Subscapularis (medially rotates and adducts arm)
Ataxia Telangiectasia
- Classic triad
- Specific defect
- Cerebellar ataxia + telangiectasias + inc. risk of sinopulmonary infections
- Defect in the gene that encodes for the ATM gene, which plays a role in DNA break repair
Cryptococcus neoformans
- How do they look?
- Who do they affect/ how is it passed?
- Most common disease processes caused
- Dx
- Budding yeasts w/ thick capsules
- Passed via pigeons/soil and affect only immunocompromised
- Meningoencephalitis is the most common disesase caused
- India Ink/ Mucarmine
Hypertrophic Cardiomyopathy
- Key potential clinical consequences (2)?
- Histo
- Pathology
- Dx of this consequence?
- What actions make those sx better or worse?
- Left ventricular outflow obstruction; sudden death in stressful situation
- Extreme myofiber disarray w/ interstitial fibrosis
- Mutations in genes encoding cardiac sarcomere proteins
- Harsh crescendo-decrescendo systolic ejection-type murmur best heard along left sternal border and apex
- Mechanisms dec. preload or afterload increase obstruction – such as sudden standing or nitro.
- Mechanisms inc. preload or afterload decrease obstruction – such as squatting, sustained hand grip, or passive leg raise
Describe the pathology of Good Pasture Syndrome
It is a Type II hypersensitivity, in which there are antibodies to the glomerulus basement membrane and alveolar basement membrae
Asbestos exposure
- Potential clinical manifestations? (3)
- What two cancer types can Asbestos lead to? Which is most common?
- (1) Pleural thickening + (2) calcified lesins of the posterolateral midlung zones/diaphragm + (3) occasional pleural effusions
- Bronchogenic carcinoma (most common and can occur w/o asbestos) and malignant mesothelioma (rare but more specific to heavy asbestos exposure)
What antibodies have a high specificity for Rhematoid arthritis?
Antibodies to citrullinated peptides/ proteins (anti-CCP)
- Inhibition of uterine contractons is a result of stimulation of which adrenergic receptor?
- Pupillary dilation is the result of stimulation of which adrenergic receptor?
- B2 (inhibits uterus)
- a2 (dilates pupils)
Dactylitis
(What is it and what is it a common presentation of?)
Painful swelling of the hands and feet
It is a common presentation of sickle cell in young children.
What does the radial nerve do? Damage to it may result in what?
- Innervation to the skin of the posterior arm, forearm, and dorsal lateral hand
- Motor innervation to all of the extensors of the upper limb (below the shoulder)
Damage leads to wrist drop
What phase of the AP corresponds with the QRS of the EKG?
QT?
QRS = Phase 0
QT = Phase 3
Inherited defects involving what signaling pathway, result in disseminated mycobacterial dz in infancy/ early childhood?
Interferon-gamma
(needed to activate macrophages)
What hormone is often increased in liver cirrhosis? What can this lead to?
Estrogen (leading to gynecomastia + spider angiomas + hair loss + testiclar atrophy)
Which neural structure is most affected by the thiamine deficiency associated with Wernicke encephalopathy?
Mamillary bodies
What specific component of the H. Flu capsule is the vaccine set against?
Polyribosyl-ribitol-phosphate (PRP)
What is the “Number Needed to Treat”, and how is it calculated?
It’s the number of patients that need to be treated with a medicatin to avoid a negative outcome. It is 1 divided by the percent difference in outcome between the control and the experiment
What are the main effects of a glossopharyngeal nerve lesion?
- Loss of gag reflex
- Loss of general sensation of the upper pharynx, posterior tongue, tonsils and middle ear cavity
- Loss of taste on posterior third of tongue
Diffuse muscle pain + fatigue +neuropsych disturbances, w/ negative labs = what dz?
Fibromyalgia
What is a pancoast tumor? Where is the most likely location for a pancoast tumor to occur? What is the most common presentation?
Carcinoma in the apex of the lung. These usually arise in the superior sulcus (groove fromed by the subcalvian vessels)
Common presentation:
- Pt. w/ extensive smoking history
- Shoulder pain- most common symptom
- Horner syndrome
- Pain in distribution of C8-T2 roots (spinal cord compression)
What is the stabilizing force for the secondary structure of proteins?
Hydrogen bonds
Lesch-Nyhan syndrome
Key presentation components (2)
- Self-mutilation
- Hyperuricemia
What are the classic sputum findings for Extrinsic Allergic Asthma
Eosinophils and Charcot-Leyden crystals (crystalloid bodies containing eosinophil membrane proteins).
H. flu
- What does it require in order to grow?
- What does its pathogenicity depend on?
- Which strain is most invasive?
- How does it present in unvaccinated individuals?
- Requires both X factor (hematin) and V factor (NAD+) to grow
- Pathogenicity depends on the presence of a capsule
- Type B strain = most invasive and virulent
- Type B causes- severe epiglottitis, meningitis, and bacteremia; Non-type B causes noninvasive sinusitis, bronchitis, otitis media and conjunctivitis
Streptococcus gallolyticus (formely S. Bovis) can cause endocarditis and bactermia. When this bug is cultured in the blood, workup for what, is absolutely essential?
Colonic malignancy with colonoscopy
Explain the rapid onset and short duration of action for drugs similar to propofol
They are lipophilic and thus readily diffuse across membranes.
First accumulate in tissues with high blood flow (like brain, hence rapid onset), then dubsequently redistributed to organs receiving less blood flow, like fat or muscles (hence short duration)
What substances are capable of inactivating enveloped viruses?
Organic solvents (ex. Ether)
[Non-enveloped viruses are generally resistant to the action of ether]
Aspirin
MOA
Aspirin impairs prostaglandin synthesis by irreversibly inhibiting COX. Inhibition of COX-1 in platelets prevents synthesis of thromboxane A2, a potent stimulator of platelet aggregation and vasoconstriction.
Under normal cricumstances, how does Ras protein become inactivated?
It has intrinsic GTPase activity , that allows it to hydrolyze the attached GTP
Verrucous endocarditis
- Who does it occur in?
- What is it?
- Potential consequence?
- Occurs in up to 25% of patients with SLE
- Can cause small cardiac valvular vegetations on either side of a valve, resulting in fibrotic valve thickening and deformity
- May cause acute coronary syndrom in young patients with normal coronary arteries
Abciximab MOA
It blocks the GP IIb/IIIa receptor, which normally promots platelet binding to fibrinogen
Antiphospholipid Antibody Syndrome
- Type/ cause of antibodies
- Sx (2)
- Antiphospholipid antibodies (either primary or due to SLE)
- Venous or arterial thromboembolism (in the presence of paradoxical inc. PTT) + recurrent pregnancy loss
Carbamazepine
- Indication
- MOA
- Adverse Effects
- Seizure tx
- Blockage of voltage-gated sodium channels in neuronal membranes
- Can cause bone marrow suppression
What is the pathognomnic presentation for a large patent ductus arteriosus complicated by Eisenmenger syndrome?
Differential clubbing and cyanosis w/o blood pressure or pulse discrepancy
Giant Cell Arteritis
- Also known as?
- Main Sxs (4)
- What does the artery look like on biopsy?
- aka Temporal arteritis
- Jaw Claudication, HA, facial pain, and vision loss
- Temporal artery biopsy demonstrates granulomatous inflammation of the media
Leuprolide MOA
Continuous GnRH agonist
What is the specific deficiency in patients with Glanzmann thrombasthenia?
(GP) IIb/IIIa, needed for platelet aggregation
Name the (4) key neutrophil chemotactic agents during inflammation
- Leukotriene B4
- 5-HETE (leukotriene precursor)
- C5a (complement component)
- IL-8
Which bug is associated with right-sided endocarditis in IV drug users?
Staph aureus
What is the best ausculatory indicator of the severity of a patient’s mitral regurgitation?
Presence of an audible S3 (indicating a large volume of regurgitant flow re-entering the ventricle during mid-diastole)
Describe the histo of myxomas
Scattered cells within a mucopolysaccharide stroma, with abnormal blood vessels and hemorrhaging
Name the precursor protein/peptide responsible for localized amyloidosis for each of the following organs:
- Cardiac atria
- Thyroid gland
- Pancreatic islets
- Cerebrum/cerebral blood vessels
- Pituitary gland
- Multi-organ amyloid deposition?
- Cardiac atria: atrial natriuretic peptide
- Thyroid gland: calcitonin
- Pancreatic islets: islet amyloid protein
- Cerebrum/cerebral blood vessels: B-amyloid protein
- Pituitary gland: prolactin
- Multi-organ amyloid deposition? Immune globulin light chains
What do RAS genes code for?
A family of small G-proteins involved in signal transduction in the Ras-MAPK pathway
Distinguish Precursor B-ALL from precursor T-ALL
This can only be done via immunophenotyping
B-ALL: TdT+, CD10+ and CD19+
T-ALL: TdT+, CD1a+, and + for T-cell markers (CD2 , 3, 4, 5, 7, and 8)
Chronic Granulomatous Disease
- Pathogenesis?
- Clinical Manifestations?
- Pathogenesis: Inactivating mutation affecting NADPH oxidase. Impaired respiratory burst inhibits phagocytic intracellular killing
- Clinical Manifestation: Recurrent infections in child w/ catalase (+) bacteria/fungi (commonly in lungs, skin, LNs and liver). Diffuse granuloma formation
Acute Arsenic Posioning
- Presentation
- Source
- Antidote
- Garlic odor breath + diarrhea + N/V
- Insecticides
- Cure = Dimercaprol
What is the most common cause of death in patients who have been struck by lightning?
Cardiac arrhythmias/ arrest
Oligodendrocytes Vs Schwann Cells
Oligodendrocytes- CNS myelin (oligarchy means central power)
Schwann- Peripheral Myelin
Pigment gallstones
- Composition
- Appearence
- Cause (including key enzyme)
- What organism is often associated
- Composed of Ca2+ salts of unconjugated bilirubin
- Soft, dark brown or black
- Typically arise secondary to bacterial or helminthic infection of the biliary tract. Beta-glucuronidase, released by injured hepatocytes and bacteria, hydrolyzes bilirubin glucorinides to unconjugated bilirubin
- The liver fluke Clonorchis sinensis (high prevalence in East Asian countries), is a common cause
How do chronically elevated FFA levels contribute to insulin resistance?
By impairing insulin-dependent glucose uptake and increasing hepatic gluconeogenesis
What is the most frequent cause of lung abscess and what type of conditions increase their risk.
Name (1) key example of this cause.
Tx (2)?
Anaerobic bacteria normally found in the oral cavity are the most common cause. Risk factors include anything that increases aspiration risk (seizure, alcholism, drug abuse, stroke, dementia, etc.)
Ex: Actinomycosis
Tx: Penicillin or Clindamycin (if hypersensitive to Penicillin)
What is the primary site for complement production?
The liver
Kehr sign
Referred shoulder pain due to irritation of the phrenic nerve sensory fibers around the diaphragm as the result of some abdominal process (ruptured spleen, peritonitis, hemoperitoneum)
Mitral Stenosis
- What is the best/most reliable asuculatory indicator of the severity?
- The interval between A2 and the opening snap (OS). Shorter interval = more severe stenosis
Germinoma
- What type of mass is it?
- Classical manifestations (2) and the Sx associated with each (3)
- Dx
- Pineal gland mass
- (1) Obstructive hydrocephalus due to aqueductal stenosis - Sx: papilledema, HA, vomiting (2) Dorsal midbrain (Parinaud) syndrome due to direct compression of the pretectal region of the midbrain- Sx: paralysis of upward gaze, ptosis, pupillary abnormalities
- Dx via MRI
Define accuracy vs reliability
Accuracy: the degree to which the aerage measurment value matches that of the gold standard
Reliability: reproducibility of a result
What is hypocapnia and what does it imply for a patient?
A state of reduced CO2 in the blood. Always implies alveolar hyperventilation.
Meconium Ileus
What is it and what disease is it associated w/?
Small bowel obstruction due to abnormally dehydrated meconium in a patient w/ cystic fibrosis
What are “Lichtenberg figures” and what are they associated with?
Erythematous cutaneous marks in a fern-leaf pattern.
They are pathognomonic of lightning strikes
Primary Biliary Cirrhosis/ Cholangitis
- What characterizes this dz?
- Who is it common in ?
- Presentation?
- Chronic liver dz characterized by autoimmune destruction of the intrahepatic bile ducts and cholestasis (elevated alk phos)
- Most common in middle aged women
- Sx: Severe pruritis (especially at night)
How does A-fib look on an EKG? In this condition, what ultimately regulates the number of atrial pulses which reach the ventricle?
- On EKG, it is characterized by: absent P waves, irregularly irregular R-R intervals, and narrow QRS
- Ventricular response is based on transmission of the abnormal impulses through the AV node. The AV node refractory period regulates the number of impulses.
Naltrexone
- Indication
- MOA
- Effect
- 1st line treatment for moderate to severe alcohol use disorder
- Blocks the mu-opiod receptor
- Effect: blocks the “rewarding and reinforcing” effects of alcohol, thus reducing cravings
What type of MHC do APCs express?
MHC Class II so that they can present antigen to CD4 cells
Zolpidem
- Indication
- SImilar to what drug? How does it differ?
- Short-acting hypnotic used for short-term insomnia
- Same MOA as benzos but a much lower risk of tolerance and dependence
Alkaptonuria
- Inheritance
- Enzyme that is deficient
- Presentation (3)
- Autosomal-recessive disorder
- Deficiency of homogentisic acid dioxygenase (an enzyme involved in tyrosine metabolism)
- Presentation: (1) diffuse blue-black deposits in connective tissues, (2) adults have slerae and ear cartilage hyperpigmentation, (3) osteoarthropathy of spine and large joints
- What is the cause of umbilical hernias?
- What conditions are they associated with?
- Incomplete closure of the umbilical ring
- Dat Big Hernia –> assc. w/ Down Syndrome, Beckwith-Wiedmann syndrome, Hypothyroidism
What tumor type(s) is the following tumor marker assc. with?:
CA 125
Ovarian
Candida Albicans
- What does it give rise to when heated?
- Where is it found normally in humans?
- Gives rise to true hyphae when incubated at 37 degrees C
- Normally found in the GI tract (including the oral cavity) and thus is a common contaminant of sputum cultures
Positive Predictive Value
The proportion of indivudals with positive test results who actually have the dz
PPV = TP/ (TP + FP)
Dobutamine
- MOA
- Indication
- Effect (3)
- AE
- B-agonist (predominant activity on B1 receptors)
- Management of refractory HF w/ severe LV systolic dysfunction/ cardiogenic shock
- (1) Positive inotropic effect (2) weakly positive chronotropic effect (3) mild vasodilation
- Some increased O2 consumption due to increased chronotropy
Role of B-blockers and a2 agonists in tx of glaucoma
Decrease secretion of aqueous humor via the ciliary epithelium
In the pathogenesis of atherotic plaques, What is responsible for promoting migration of smooth muscle cells from the media into the intima, and subsequent proliferation?
The release of Platelet-derived growth factor (PDGF) by locally adherrent platelets, endothelial cells, and macrophages.
Describe the common presentation for sarcoidosis
- Hilar adenopathy
- Pulmonary infiltrates
- Non-caseating granuloma
- AA Woman
What are the (3) main sx of vitamin A overuse?
- Intracranial HTN
- skin changes
- Hepatosplenomegaly
Describe the process of granuloma formation for TB
- Macrophages eat m. tuberculosis
- Macrophages present to T cells and also secrete IL-12
- T-cells differentiate to Th1
- Th1 produces IFN-y which activates macrophages
- Activated macrophages produce TNF-a, increasing recruitment, allowing for walling off of infection
Resistance in the airway is maximal between which bronchial generations?
Generations 2-5 (then drops off due to high cross-sectional area)
Main difference between MDD and Persistent depressive disorder (dysthymia)
Persistent DD is > 2y, while MDD is greater than 2 weeks (usually 6-12mo)
Also Pers. DD tends to be milder
After cutting through fascia, what else is cut during a cricothyrotomy?
The cricothyroid membrane
Achlorydia
Lack of HCl in gastric secretions
_______________ is almost always needed in order to develop acute pyelonephritis (as opposed to regular UTI)
Vesicoureteral reflux
What key structure is assc. with ovarian torsion (other than the ovary)?
The infundibulopelvic ligament (IP).
It suspends the ovaries and has blood vessels/nerves for the ovary running through it.
How can untreated hydrocephalus in an infant lead to UMN issues?
Stretching of the periventricular pyramidal tracts
Acute acalculous cholecystitis
- What is it?
- Who does it occur in?
- Acute inflammation of GB in absence of gallstones
- Occurs in critically ill patients (and thus has very poor prognosis)
Why is Piperacillin-tazobactam such an effective tx for Bacteroides?
Bacteroides (gram-negative, anaerobic rods) produce B-lactamase, an enzyme that breaks down penicillins.
Tazo inhibits the B-lactamase, so that piperacillin can do its job.
What type of brain damage leads to decerebrate (extensor) posturing?
Damage at or below the midbrain tegmentum, pons (below the red nucleus)
What type of brain damage leads to decorticate (flexor) posturing?
Damage w/in the cerebral hemispheres (above the red nucleus)
What hormone is ultimately responsible for causing gestational diabetes?
Human placental lactogen (hPL)
It decreases maternal fatty acid stores and increases maternal serum glucose, but also increases insulin resistance
Describe the path the median nerve takes prior to the carpal tunnel
- Courses between the humeral and ulnar heads of the pronator teres muscle
- Runs between the flexor digitorum superficialis and the flexor digitorum profundus muscles (like meat in a sandwich)
- Crosses wrist w/n carpal tunnel
What is the most sensitive screening test for dx od primary hypothyroidism?
Serum TSH
(even more sensitive than T3/T4)
The production of what cytokine is a very important driver of Giant Cell Arteritis?
IL-6 (cell mediated immunity is the primary mechanism underlying this dz)
Why should protein be restricted in an ornithine transport deficiency?
Ornithine is a crucial part of the urea cycle, needed to breakdown protein. Defects can lead to neurological damage, due to ammonia accumulation.
Which antibiotic groups disrupt the peptidoglycan cell wall of bacteria? (3)
- Penicillins
- Cephalosporins
- Vancomycin
What compensatory pathway can help convert fructose to a useable form, in cases of essential fructosuria?
The hexokinase pathway can be used, if the fructokinase pathway is broken (as it is in the case of essential frucosuria)
First-order kinetics vs zero-order kinetics
First: a constant proportion of the drug is metabolized per unit time (based on serum levels)
Zero: a constant amount of drug is metabolized per unit time (independent of serum levels)
(2) key histo findings in Glioblastoma Multiforme
- Pseudopalisading
- Central areas of necrosis + hemorrhage (hence, multiforme)
Cholesteatoma
- What is it?
- Etiology
- Presentation
- Collection of squamous cell debris that forms a pearly mass behind the tympanic membrane
- Congenital or acquired following infxn, trauma or middle ear surgery
- Can cause hearing loss
What are the (2) normal arrest points for oocytes?
- Prophase of meiosis I (prior to ovulation)
- Metaphase of meiosis II (prior to fertilization)
What tissue types have B1 receptors?
Cardiac and Renal JGA cells
What is the major immune mechanism against Giardia?
secretory IgA production
Viral bronchiolitis
- Cause
- Presentation (4)
- Dx
- RSV
- Low-grade fever, cough, tachypnea, inc. work of breathing
- Wheezing + crackles
What type of bacteria is Gardenella vaginalis?
An anaerobic gram-variable rod
Describe the morphology of cryptococcus neoformans
Budding yeast
What nerve innervates the cricothyroid muscle?
The external branch of the superior laryngal nerve
What is the optimal site for a femoral nerve block and what becomes anesthetized?
- Inguinal crease = optimal site
- Skin and muscles of the anterior thigh (e.g. quads), femur, and knee
Why can PAH be used to estimate Effective Renal Plasma Flow (eRPF)?
Because it is both filtered and secreted in the proximal collecting tube (PCT), resulting in nearly 100% excretion of PAH by the kidneys.
Progressive onset of heart failure in the setting of recent viral infection should raise suspicion for what?
Dilated cardiomyopathy caused by viral myocarditis
What are the values for serum Ca2+, phosphorous, and PTH in primary osteoporosis? (low, normal, high– for each)
All normal
What is the most efficient way to decrease cerebral blood flow/ ICP in a patient?
Hyperventilate. This will blow off CO2, leading to a vasoconstriction.
Presentation of Porphyria cutanea tarda
Blistering cutaneous photosensitivity
Cardiac defect assc. with Turner’s syndrome
Bicuspid aortic valve + coarctation of the aorta
What is the triad of sx assc. with Serotonin Syndrome?
- Autonomic instability (hyperthermia, HTN, tachycardia)
- AMS
- Neuromuscular hyperactivity
How is Linezolid able to cause Serotonin syndrome?
It has MAOI activity, so combined with an SSRI, it can precipitate serotonin syndrome
Why are thiazides effective for some patients with nephrolithiasis?
They increase Ca2+ reabsorption from the nephron, so are effective for calcium based nephroliths
What does arteriovenous concentration gradient tell us about the tissue solubility and onset of action of an anesthetic?
Anesthetics with high tissue solubility are characterized by large AV concentration gradients and slower onsets of action.
Ultimately, what are vegetations assc. with infective endocarditis, composed of?
(1) sterile fibrin-platelet nidus + (2) bacterial colonization
Craniopharyngiomas commonly show what?
Calcification
CN III courses between which 2 vessels?
SCA and PCA
Put Alcohol w/drawal symptoms in order (4)
Mild w/drawal (tremors, diaphoresis, etc.) –> seizures –> alcoholic hallucinosis –> delirium tremens
Describe the breakdown of heme to unconjugated bilirubin
Heme –> Biliverdin (via heme oxygenase)
Biliverdin –> unconjugated bilirubin (via biliverdin reductase)
A patient has hypoxemia, in the setting of a normal A-a gradient. What (2) differentials should you consider?
- Alveolar hypoventilation
- Inspiration of air at high altitude
Which vessels are most likely to be penetrated by gastric ulcers? Why?
The left and right gastric arteries.
They run along the lesser curvature of the stomach, where the great majority of ulcers occur.
Why do individuals who’ve experienced excessive weight loss or who excercise strenously, often miss menstruation?
Decreased leptin, leads to decreased GnRH secretion via the hypothalamus
What is most responsible for the increase in nosocomial bloodstream infections?
Intravascular devices (indwelling catheters)
Where does fluid accumulate in cases of communicating hydrocele?
W/in the tunica vaginalis
Is S pyogenes PYR-positive or PYR-negative?
(PYR)-positive
In a pregnant woman, which drugs are the best tx choice for for DVTs?
Low Molecular Weight Heparins
What substances allow for a prenatal dx of NTDs?
The presence of alpha fetoprotein and AchE in the amniotic fluid (or even serum for AFP)
For what reason does Tamoxifen rely on CYP2D?
Tamoxifen is a prodrug and relies on CYP2d to become activated, as endoxifen
What region of the LN is populated primarily by T lymphocytes (as well as dendritic cells)?
The paracortex region
The transverse portion of the duodenum (the bottom part of the C), runs between which (2) vessels?
Aorta and SMA
Why is their a major increase in the density of connexins in the uterus just before delivery?
Connexins assemble into gap junctions which are needed to facilitate communication and coordination between cells preparing for labor contractions.
They increase in response to rising estrogen levels.
What component of the tRNA serves as the AA binding site?
3’CCA tail (loaded by aminoacyl tRNA synthetase)
Where does lymph from the testes drain?
The scrotum?
Testes: para-aortic LNs
Scrotum: superficial inguinal LNs
Dissection of what nerve leads to “winged scapula”, and why?
The long thoracic nerve. The results in weakness of the serratus anterior muscle.
(leading to winged scapula and impaired abduction of the shoulder beyond the horizonal)
What types of renal injury can be caused by NSAIDs? (2)
Chronic interstitial nephritis + papillary necrosis
What role do neurophysins play for oxytocin and vasopressin?
They are involved in posttranslational processing and stabilization w/in neurosecretory vesicles during transport to the posterior pituitary.
What are the (2) main roles that NADPH plays?
- Reduction of glutathione (i.e. prevention of oxidative damage)
- Biosynthesis of cholesterol, fatty acids, and steroids
Why are patients who’ve experienced organophosphate poisoning still at risk for muscle paralysis, even after being given atropine?
Atropine only reverses muscarinic sx of the poisoning. It has no effect on the nicotinic receptors, which cause the muscle paralysis (need pralidoxime→PAM)
Why must you be careful not to overuse a-adrenergic agonists as decongestants?
Overuse causes negative feedback, resulting in dec. NE synthesis and release from nerve endings, diminishing their effect
Candida albicans is a normal inhabitant of what?
The GI tract (including the oral cavity)
Morphine MOA
Binds to mu receptors, resulting in G-protein activation of potassium conductance.
This hyperpolarizes the postsynaptic neurons, blocking pain transmission
Most common cause of galactosemia
Deficiency in galactose-1-phosphate uridyl transferase (GALT)
Describe the characteristic histopathological findings assc. with HIV-associated dementia
Microglial nodules (activated macrophages/giant cells around center of necrosis)
Thoracic outlet syndrome
- Pathlogy
- Presentation
- What is it associated with?
- Compression of lower trunk of brachial plexus in the scalene triangle (bordered by anterior/ medial scalene and first rib)
- Sx: uper extremity numbness, tingling, and weakness
- Having an extra rib
Gout or pseudogout: associated with myeloproliferative disorders
Gout
Tx of Wilson Dz
D-penicillamine (copper chelating agent)
In females, the paramesonephric ducts fuse to form, what? (4 things)
- Fallopian tubes
- Uterus
- Cervix
- Upper vagina
What does the liver do in attempts to compensate for protein loss in nephrotic syndromes?
Increase its synthesis of a bunch of other proteins (including VLDL, LDL, and HDL). This often leads to lipiduria.
P bodies play an important role in protein synthesis. Where do they operate/ what is their general role?
They work in the cytoplasm. Play an important role in mRNA translation regulation and mRNA degradation
If a patient presents with mono, but has a negative agglutination to horse erythrocytes, what is the likely dx?
CMV (agglutination to horse erythrocytes = monospot test)
Tx for Chlamydia trachomatis
Doxycycline or azithromycin
IV tx to lower BP in hypertensive emergency.
What is its effect (3)
Fenoldopam
(dopamine-1 receptor agonist)
- Arteriolar dilation
- Inc. renal perfusion
- Promotes diuresis and natriuresis
Neuroleptic Malignant Syndrome
- Etiology
- Sx
- Tx
- Rxn to antipsychotic drugs
FEVER (Fever, Encephalopathy, Vitals unstable, Enzyme inc., Rigidity of muscles)
Tx w/ Dantrolene
Rapid correction of chronic hyponatremia, may lead to what?
Central pontine myelinolysis (osmotic demyelination of the axons in the central pons). Can cause quadriplegia.
How do you reduce the risk of “wrong-site surgery”?
Dual identifiers (usually a nurse and physician)
What is the appropriate attire for C-dif?
Nonsterile gloves + gown
Describe the morphology of acute transplant rejection
It can have a humoral and cellular component
- Humoral- necrotizing vasculitis with a neutrophilic infiltrate
- Cellular- lymphocytic interstitial infiltrate and endotheliitis
What does it mean when we say that the lac operon is polycistronic?
It means that the one mRNA codes for several proteins
What is the specific defect associated with Fragile X syndrome?
An X-linked trinucleotide repeat disorder (CGG)n which affects methylation and expression of the FMR1 gene
Explain how A fib, with subsequent abdominal pain, can lead to acidic urine.
- A fib can lead to acute ischemic colitis
- Ischemia forces bowel to use anaerobic respiration
- Anion gap acidosis occurs
- Kidneys generate ammonia and bicarb (via conversion of glutamide to glutamine), so bicarb can be used to buffer blood
- Ammonia is urinated out (hence acidic urine!!!)
What is the difference in fxn between free and attached ribosomes?
Free = produce proteins for the cytosol
Attached = produce proteins for cell membrane/ secretion
Which diuretics are best for improving survival in patients with CHF and decreased EF?
Mineralocorticoids (eg, spironolactone, eplerenone, etc.)
If the rectus abdominis muscle is incised laterally, what vessel is at greatest risk of injury?
The inferior epigastric artery
How can left-sided heart dz, lead to pulmonary vasoconstriction?
The backflow of blood causes damage to the endothelium, which causes impaired NO function and endothelin release.
Sx associated with hep C
Hep C is typically asymptomatic! There may be skin manifestations in chronic Hep C.
What determines which side of the coronary circulation is dominant?
Attachment to the Posterior descending/interventricular artery (PDA)
The PDA (posterior descending/interventricular artery) supplies blood to what key structure?
The AV node (via the AV nodal artery)
Osteocytes send signals and nutrients/waste to one another via what structure?
Gap junctions
What is the major cause of morbidity and mortality in patients recovering from SAH? How do you prevent this?
Severe vasospasm
Tx w/ nimodipine to prevent
Biliary Atresia
- Presentation
- Dx
- What is seen on Biopsy?
- dark urine + jaundice + clay colored stools in first months of life
- Dx: conjugated hyperbilirubinemia and take biopsy
- Biopsy reveals duct prolifeation, portal tract edema, and fibrosis
Name the only medication which selectively decreases HR but has no effect on myocardial contractility or relaxation.
What is its MOA?
Ivabradine
Selective inhibition of funny sodium channels (If)
Name the toxicities associated with aminoglycosides (2)
- nephrotoxicity
- ototoxicity
a2 receptor effect on insulin secretion?
B2 receptor effect?
Which predominates
a2: inhibits insulin secretion
B2: promotes insulin secretion
a2 predominates when both are activated
Tx of Restless Leg Syndrome
Dopamine agonists
Cytokeratin is a commonly-used immunohistochemical marker for what?
Epithelial-derived tissues
What symptoms characterize Atypical Depression?
Tx?
- Mood reactivity (ex. feeling better n response to positive events)
- Leaden paralysis (heavy arms/legs)
- Rejection sensitivity
- Increased sleep/appetite
Tx: MAOIs
What is one of the rare tissues to have an anabolic increase in protein synthesis in response to longterm glucocorticoid tx?
The liver, due to increased hepatic gluconeogenesis and glycogenesis (contributes to the hyperglycemia seen).
What is the main MOA of all hormone containing Oral Contraceptives?
Inhibition of ovulation by dec. FSH and LH synthesis
Ristocetin aggregation test is used to test for what?
von Willebrand dz
(should show decreased aggregation of platelets)
A rxn to Poison Ivy, is a form of what?
Allergic contact dermatitis (type IV hypersensitivity)
A patient has weakness during finger and thumb extension (w/o wrist drop or sensory deficits).
Most likely site of injury?
Radial nerve during its passage through the supinator canal
Most important tx for Diptheria?
Diphtheria antitoxin (passive immunization)
Nitroglycerin acts primarily on which vessels?
LARGE VEINS!!!
(It’s a venodilator, which allows it to decrease preload)
Best mechanism for Dx of vertebral osteomyelitis
MRI of the spine
What type of drugs are preferentially metabolized and eliminated by the liver?
Drugs w/ high lipophilicity
What connects the 3rd ventricle to the 4th ventricle?
Cerebral aqueduct
What drains the 4th ventricle?
Lateral foramina of Luschka and Median foramen of Magendie
DRESS Syndrome
- What does it stand for?
- When does it occur?
- Drug rxn with eosinophilia and systemic symptoms
- 2-8 weeks after exposure to high risk drugs (ex. anticonvulsants, allopurinol, sulfonamides, and antibiotics)
DRESS Syndrome presentation
- Fever
- Eosinophilia
- Diffuse rash
- Internal organ Dysfunction
- Facial edema
You will be FED IF you get DRESSed
Increased intake of what substances, helps to prevent renal calculi formation?
Citrate and fluids
Effect of DM on urinary continence
Can cause overflow incontinence due to inability to sense a full bladder and due to incomplete bladder emptying.
SE: Hyponatremia, Hypercalcemia, Hypocalcemia
Thiazides are more likely to cause which of these?
Loop Diuretics are more likely to cause which of these?
Thiazides: Hyponatremia and hypercalcemia
Loop diuretics: Hypocalcemia
Presentation of Intestinal Malrotation
- Intestinal obstruction (due to compression by the adhesive bands)
- Midgut volvulus (intestinal ischemia due to twisiting around the blood vessels)
What aspect of oral administration of a drug is responsible for its decreased bioavailability?
First-pass metabolism by the liver
Ezetimibe action
Reduces intestinal absorption of cholesterol at the brush border (this leads to inc. LDL expression pulling more cholesterol out of the circulation)
Name the organisms most often involved in Chronic Granulomatous Dz (5)
Catalase-postive bugs which destroy their own hydrogen peroxide
Specifically:
- S. aureus
- Burkholderia cepacia
- Serratia marcescens
- Nocardia
- Aspergillus
Which checkpoint does Rb regulate?
G1 → S
What causes Humoral Hypercalcemia of Malignancy?
Secretion of Parathyroid hormone-related protein (PTHrP).
It closely resembles parathyroid hormone and causes increased bone resorption and decreased renal excretion of calcium.
Lynch Syndrome (HNPCC)
- What does it lead to?
- What is responsible?
- What is the key gene affliated?
- Leads to occurence of colonic adenocarcinomas at a young age (
- Mutations in DNA mismatch repair is responsible
- MSH = key gene
What is responsible for the outflow block associated with hypertrophic cardiomyopathy?
The septum touches the mitral valve leaflets, and blocks the aortic valve
Other than iodide, what other ions are absorbed into the thyroid gland via the sodium-iodide symporter?
Perchlorate and pertechnetate ions
Injury of what nerve, leads to foot drop and weak eversion/ toe extension?
Common peroneal nerve
In what part of the cell does Vitamin C function?
The RER, where it is a cofactor for hydroxylation of proline and lysine.
What is the most common COD in patients with TCA overdose, and why?
Cardiac arrhythmias and refractory hypotension. Due to Inhibition of fast sodium channels in cardiac myocytes.
KRAS
Proto- oncogene or tumor suppressor?
Proto-oncogene
What is used for prevention of hemorrhagic cystitis, during tx with systemic chemo (w/ cyclophosphamide or ifosfamide)?
Mesna
In addition to looking completely different from their tissue of origin, what other characteristics might anaplastic cells possess?
- abnormal mitoses
- giant, multinucleated tumor cells
In addition to trisomy 21, what else can lead to Down syndrome?
Unbalanced Robertsonian Translocations [46, XX, t(14;21)]
or
Mosaicism (some cells have extra copy of chromosome 21)
What is the specific mechanism implicated in the pathophysiology of cataracts and peripheral neuropathy in DM?
During hyperglycemia, excess plasma glucose is converted to sorbitol by aldose reductase.
Sorbitol accumulates w/in some cells and attracts water, leading to osmotic cellular injury.
Dx of Chronic Granulomatous Dz
Diagnosis: DHR flow cytometry (prefered); Nitroblue tetrazolium (NBT) testing
What is the role of short non-coding RNA sequences (microRNA and small interfering RNA)?
Induce posttranscriptional gene silencing by base-pairing w/ complementary sequences w/in target mRNA molecules
What causes appendicitis pain to eventually localize?
Irritation of the parietal peritoneum
What is the inheritance for classical galactosemia?
Autosomal recessive
What bug/virus causes encephalitis that can lead to hemorrhagic necrosis of the inferior and medial temporal lobes?
Herpes simplex virus
What happens if obliteration of the omphalomesenteric (vitelline) duct fails?
- Persistent vitelline duct
- Meckel diverticulum
- Vitelline sinus
- Vitelline duct cyst
Classic triad of sx for RCC?
- Hematuria
- Flank pain
- Palpable mass
Sx of angioedema (and what drug can cause it?)
- Swelling of tongue, lips, eyelids, or sometimes larynx
- Caused by bradykinin accumulation from ACE-I’s
Equation for absolute risk reduction
ARR= (event rate in the control group) - (event rate in the tx group)
What structure is cut in tx of carpal tunnel syndrome?
Transverse carpal ligament
Aliskiren MOA
Direct renin inhibitor
(Blocks conversion of angiotensinogen to angiotensin I)
What can creatinine be used to measure?
Approximate measure of GFR (secreted 10%)
What can inulin be used to measure?
GFR (freely filtered and neither reabsorbed or secreted)
(can also estimate extracellular volume)
What would be the result of, loss of CYP21A2 (21-hydroxylase) on:
Cortisol
Aldosterone
Androgens
Loss of cortisol and aldosterone, increased androgens (most common casued of CAH)
What would be the result of, loss of CYP11B1 or B2 (11-hydroxylase) on:
Cortisol
Aldosterone
Androgens
Loss of cortisol and aldosterone, increased androgen and increased aldosterone precursor, 11DOC (binds strongly to mineralocorticoid receptor, simulating increased aldosterone)
What would be the result of, loss of CYP17 (17-hydroxylase) on:
Cortisol
Aldosterone
Androgens
Loss of Cortisol and androgens. Increased aldosterone.
Non-rhytmic conjugate eye movements + myoclonus (opsoclonus-myoclonus syndrome) is assc. with what dx?
Neuroblastoma
Orotic aciduria
- What is it?
- What causes it?
- Rare autosomal recessive disorder of de novo pyrimidine synthesis
- Defect in uridine 5’-monophosphate (UMP) synthase
Presentation of individuals with Orotic aciduria
Tx?
Children with
- Physical/mental retardation
- Megaloblastic anemia
- Large amounts of urinary orotic acid
Tx w/ uridine
What are the dendritic cells of the skin called? Describe them? (cell line? structure?)
Langerhans cells
(derived from myeloid cell line and possess racquet-shaped intracytoplasmic granules called Birbeck granules)
Which Carcinoid tumor types are capable of causing carcinoid syndrome?
Metastatic
(The cytokines of local carcinoid tumors confined to the intestine, are metabolized by the liver before reaching systemic circulation)
How do cancers of the pelvis spread to the lumboscral spine?
Via the vertebral venous plexus
Attachement point for greater and lesser omentum
Greater = Greater curvature of stomach
Lesser = lesser curvature of stomach (to liver/duodenum)
In cases of Small Intestinal Bacterial Overgrowth (SIBO), what vitamins/minerals…
… become deficient? (5)
…increase in production? (2)
Deficient: vitamins B12, A, D, E, and iron
Increased: folic acid and vitamin K
What is the most important virulence factor for E. Coli?
Fimbrae (allows it to stick to uroepithelial cells)
Prophalictic procedures for Group B Strep (S. agalactia)
- Prenatal screening (35-37 weeks)
- INTRAPARTUM antibiotics
Colchicine
- Indication
- MOA
- SE
- Tx of acute gouty arthritis in patients who can’t take NSAIDs
- Blocks tubulin polymerization, thus inhibiting leukocyte migration/phagocytosis
- N/Dia
Main AE of Phenytoin (3)
- Ataxia
- Nystagmus
- Gingival hyperplasia
Hematogenous osteomyelitis
- Most common in who?
- What part of the bone is affected?
- Most common in children
- The metaphysis of long bones (slower blood flow)
Drug of choice for B-blocker overdose
Glucagon (causes inc. of intracellular cAMP)
What is carboxyhemoglobin?
Carbon monoxide (CO) -bound hemoglobin
What’s the worst long-term prognostic factor for PSGN?
Adult onset
What hormone stimulates release of Bi-carb rich secretions from the exocrine pancreas?
Secretin, produced in the S cells of the duodenum
What happens to the ratio of Lecithin to sphingomyelin during the third trimester?
Increases
Lecithin is a component of surfactant (greatly inc. during third trimester), while sphingomyelin is a general amniotic fluid component which should remain constant.
What is the cause of Acute hemolytic transfusion Rxn?
Type II HSN rxn, w/ subsequent complement activation
What differentiates eccrine and adrenal medulla neural input, from the rest of the sympathetic responding glands/organs?
No adrenergic postganglionic neurons
If bone conduction is greater than air conduction, what type of hearing loss is it?
Conduction hearing loss
Innervation of external hemorrhoids
Inferior rectal nerve (branch of the pudendal)
What volume counts as low Vd?
3-5L
What protein is most involved in the transport process, leading to HSV-1 recurrence?
Kinesin
What is the Sudan III stain, and what is it the best test for?
It is a stain for fat and it is the most sensitive strategy for malabsorptive disorders
What is a homeobox and what do they code for?
It is an 180 nucleotide, highly conserved gene, which codes for transcription factors
What does acid-fast stain specifically identify?
Organisms with mycolic acid present in their cell walls
What (4) CAs are associated with dermatomyositis?
- Ovarian
- Lung
- Colorectal
- non-Hodgkin
Piriformis Syndrome
Sciatica-like sx as a result of compression of the sciatic nerve, due to muscle injury or hypertrophy of the piriformis muscle
What type of gland is assc. with inflammatory acne?
Sebaceous (a holocrine gland)
Most frequent mechanism of sudden death in the first 48h after acute MI?
V. Fib (due to electrical instability in the ischemic myocardium)
What is given to women at risk of premature delivery, to prevent NRDS?
Steroids (ex. dexamethasone)
What occurs in a crossover study?
Subjects are randomly allocated to a sequence of 2 or more tx given consecutively
What do sertoli cells produce and what does it do?
Inhibin B, which feedsback to inhibit FSH secretion
What causes Familial dysbetalipoproteinemia? What does it lead to?
Defects in ApoE2 and ApoE4. Leads to dec. clearance of chylomicrons and VLDL remnants
The enzymes responsible for glycolysis, fatty acid synthesis, and the PPP, all reside where?
The cytosol
How far down into the airway do cilia persist?
Through the length of the respiratory bronchioles
Pleiotropy definition
Instances where multiple phenotypic manifestations result from a single genetic mutation
Which histone type is outside of the histone core?
H1
What can injury of the prostatic plexus (often during a prostatectomy) lead to?
Erectile dysfunction (it innervates the corpus cavernosa of the penis)
What cells do melanocytes derive from?
Neural crest cells
What is the “Thayer-Martin” selective medium, and what is it used for?
aka VPN (vanco, polymyxin, and nystatin + trimethoprim)
Kills everything except Neisseria
Most common cause of aseptic meningitis
Enteroviruses (polio/ coxsackie)
What are the (3) main chemotactic agents for neutrophils?
- Leukotriene (and its precursor)
- C5a
- IL-8
Most common cause of cystathionine synthase deficiency? How do you tx this issue?
Homocystinuria (responds dramatically to vit B6– pyridoxine)
HBsAg and HBeAg
Which is the marker for high infectivity?
HBeAg
Drug of Choice for Tx of resistant schizophrenia?
Clozapine
RF for C__andidemia
Presence of a central vascular catheter + receipt of parenteral nutrition
Lymphatic drainage of the rectum proximal to the anal dentate line? Distal?
Proximal: inferior mesenteric and internal iliac LNs
Distal: inguinal nodes
Describe the anemia assc. with Mycoplasma pneumoniae
Autoimmune hemolytic anemia (it looks like human erythrocytes)
What is the mechanism of liver injury for Hep B Pts?
The presence of viral HBsAg and HBcAg on the cell surface stimulates the host’s cytotoxic CD8+ cells to destroy infected hepatocytes.
When is glucose excreted?
When it gets beyond the “Transport Maximum of Glucose”, which is around 200 mg/dL.
Prior to this value, it is completely reabsorbed.
What complicates the long term tx medicating of Parkinson’s patients?
Unpredictable fluctuations in motor function
How do neutrophils often look in Leukemoid rxn?
Granulated (Dohle bodies)
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Conn’s Syndrome?
Tx?
Primary Hyperaldosteronism
(tx w/ eplerenone or spironolactone)
What (3) drugs work via inhibition of dihydrofolate?
- Trimethoprim
- Methotrexate
- Pyrimethamine
What makes up the superficial ring?
Openings in the external abdominal oblique
What makes up the deep inguinal ring?
Physiologic openings in the transversalis fascia
Rituximab MOA
Monoclonal antibody directed against the CD20 antigen
Valproate indication
Bipolar maintenance drug
What causes malignant hyperthermia? Tx?
Caused by admin of inhalation anesthetics/ succinylcholine to genetically susceptible individuals.
Tx w/ Dantrolene (Ryr receptor blocker)
What effect can HPV have on newborns?
Respiratory papillomatosis, w/ infection of larynx (true vocal cords)
Patients w/ familial retinoblastoma have increased risk for what type of tumor?
Secondary tumors, especially osteosarcomas
What is phencyclidine?
PCP (angeldust)
How does schizophrenia differ from schizoaffective disorder?
In schizoaffective disorder, there are signficant mood symptoms (ie mania or depression) in conjunction with the hallucinations.
Equation for true positives
(Sensitivity) * (number of patients actually with the dz)
Rifampin MOA
Blocks the action of bacterial DNA-dependent RNA polymerase
False negatives
(1-sensitivity) * number of patients actually with the dz
Most common cause of bloody nipple discharge?
Intraductal papilloma (typically presents w/o breast mass or skin changes)
What is responsible for the phosphorylation of Rb?
CDK4, after exposure to proliferation signals
Riboflavin is a precursor of what coenzymes? What are they used for?
FMN and FAD
(electron acceptor for succinate dehydrogenase)
What effect does sarcoidosis have on calcium levels? How?
Hypercalcemia
Activated macrophages produce excess 1,25 dihydroxyvitamin D
Name the gene mutations assc. w/ early onset Alzheimer
- APP (chromosome 21)
- Presenilin 1
- Presenilin 2
Name the gene mutations assc. with late-onset Alzheimer dz
Apoliporotein E4
Best drugs for alcholol w/drawal
Benzos
What forms the inferior surface of the heart? What vessel supplies it?
The inferior wall of the left ventricle. Fed by the PDA, which usually derives from the right heart
Primary cause of morbidity in acute rheumatic fever?
Severe pancarditis
(inflammation of the entire heart)
How can total parenteral nutrtion lead to gallstones?
Because there is no normal enteral stimulation, there is dec. CCK release and subsequent billiary stasis.
Presentation of primary HSV-1 infection in children
Gingivostomatitis
Systematic desensitization is an example of what type of therapy?
Behavioral therapy
Listeriosis Sx (3)
- Gastroenteritis
- Septicemia
- Meningoencephalitis
Key AE of Niacin
It can decrease renal excretion of uric acid, precipitating acute gouty arthritis
What are the (2) most important buffers in settings of diabetic ketoacidosis?
NH3 and HPO42-
(are urinated out as NH4 and H2PO4)
Penicillin is structurally similar to what part of the pepidoglycan?
D-alanine-D-alanine
(penicillin looks like it, vanco directly binds to it)
Effects of a Pudendal Nerve Injury
When does it usually occur?
- Fecal/urinary incontinence
- Perineal pain
- Sexual Dysfunction
Often occurs as a stretch injury during labor
Name the common complications of Psoriasis (3)
- Psoriatic arthritis
- Nail pitting
- Uveitis
What does Hep B look like on liver Biopsy?
Large hepatocytes folled with finely granular, homogenous, pale pink cytoplasm (eosinophilic inclusions), often described as ground glass.
This is from the accumulation of Hep B surface antigen w/in affected hepatocytes.
Pathogenesis of Hirschsprung dz
What organ is always involved?
The result of abnormal migration of neural crest cells during embryogenesis.
These cells are ganglion precursors, and always involve the rectum because they migrate caudally
Folate deficiency can lead to erythroid precursor cell apoptosis. What can be given to help allieviate this?
Thymidine supplementation
Who should you avoid giving first gen. antihistamines to, and why?
Older patients, with cognitive or fxnal impairments
Other than histamine, what is the best marker for mast cell activation?
Tryptase
(it is a preformed inflammatory mediator released by the mast cell)
On what part of the collecting duct, does ADH work?
The medullary segment
Differentiate between the following:
- Western Blot
- Northern Blot
- Southern Blot
- Western: id’s proteins
- Northern: id’s RNA
- Southern: id’s DNA
Cocaine MOA
Inhibits synaptic reuptake of NE, DA, and Ser
What is the prefered longterm tx for panic disorder?
SSRIs
Describe the hormone status of patients w/ Klinefelter
Sperm count?
Elevated FSH and LH (no feedback), and decreased testosterone due to Leydig damage
Azoospermia (no sperm)
What parameter correlates with the potency of an inhaled anesthetic?
The minimal alveolar concentration.
This is the ED50 and is inversly proportional to the MAC (the lower the MAC, the higher the potency)
Where are the juxtaglomerular cells located? What is the effect of chronic renal hypoperfusion on them?
They are modifed SM cells of the afferent glomerular arteries. They secrete renin in response to the Macular Densa.
Chronic hypoperfusion will cause hypertrophy and hyperplasia.
How does Hep B facilitate the infectivity of Hep D
The hep B surface antigen of the hep B virus must coat the hep D antigen of the hep D virus, before it can infect hepatocytes and multiply.
How does prolonged exposure to loud noises cause hearing loss?
Damage to the sterociliiated hair cells of the Organ of Corti
What are mutations in the KIT receptor tyrosine kinase, often assc. with?
Mast cell proliferation
Systemic mastocytosis
- Path
- Presentation
- Abnormal proliferation of mast cells and inc. histamine release
- Histamine causes hypersecretion of gastric acid by parietal cells of the stomach, as well as a variety of other symptoms (hypotension, flushing, pruritus, etc.)
Which (2) drugs have the highest risk of causing drug-induced lupus erythematosus (DILE)?
- Procainamide
- Hydralazine
Baclofen
- MOA
- Indication
- Agonist at the GABA-B receptor
- Effective monotherapy for spasticity secondary to both brain and spinal cord dz, including MS
What mutation is assc. with medullary thyroid CA?
Activating mutations of the RET proto-oncogene
Inactivating mutations of p53 are fairly common in what typeof thyroid CA?
Anaplastic thyroid CA
RAS mutations are common in what type of thyroid CA?
Follicular thyroid CA (and some follicular adenomas)
What is Congenital Pyloric Stenosis?
How does it present?
Smooth muscle hypertrohy of the pyloric muscularis mucosae
Presentation
- projectile nonbilious vomit
- olive-sized mass in distal stomach
Interaction with what drugs can lead to chronic lithium toxicity?
- Thiazides
- ACE inhibitors
- NSAIDs
Potential serious complication of antithyroid drugs
What is the presentation and how should you respond?
Agranulocytosis
Sudden onset of fever and sore throat
(discontinue drug and take white count)
Tx of CAH
Low doses of corticosteroids to suppress excessive ACTH secretion
What does hemoglobin electrophoresis show for B-Thalassemia minor?
Slightly dec. HbA with inc. HbA2 and HbF
Identify this microorganism
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N. gonorrhea
(gram-negative intracellular diplococcus)
What factors does H. flu require to grow, and what other microorganism can provide them?
- Factor X (hematin)
- Factor V (NAD+)
S. aureus
What organism?
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Histoplasma capsulatum (in the form of oval bodies w/in a macrophage)
Pt presents w/ foot drop + inability to evert
Name the damaged nerve
Common peroneal
COX-1 vs COX-2
COX-1: House keeping fxns such as platelet aggregation, gastric mucosal protection, etc. (consitutively expressed)
COX-2: Inducible enzyme only expressed by inflammatory tissues
Celecoxib MOA
Selective COX-2 inhibitor
What type of muscle fibers compose postural skeletal muscles? (Type I or Type II)
Type I (slow twitch, aerobic, lots of mitochondria)
[this differs from type II, fast twitch, which is anaerobic, and is for rapid forceful pulses of movement]
Describe the relationship between BMI and Leptin
Directly correlated
Leptin is produced by adipocytes in proportion to the quantity of fat stored. Increased leptin leads to decreased appetite and increased satiety
Tryptophan is a precursor for what chemical?
Serotonin
What is the antidote for serotonin syndrome?
Cyproheptadine (an antihistamine)
Compare the embryological derivations of the anterior and posterior pituitary
Anterior: Surface ectoderm
Posterior: Neural tube
(Both are derivations of the ectoderm)
Mesothelioma
- Main finding on radiographic studies
- Histopathology
- CT/MRI: pleural thickening
- Histo: long, slender microvilli and abundant tonofilaments
Kids with Xeroderma pigmentosum are very sensitive to, what?
UV-light (due to an endonuclease deficiency)
Why is dietary fructose rapidly metabolized?
It bypasses PFK-1, the major rate-limiting enzyme of glycolysis
Caudal regression syndrome
- What is it?
- What condition is it commonly associated with?
- Sacral agenesis causing lower extremity paralysis and urinary incontinence in a newborn
- Poorly controlled maternal diabetes
Pudenal nerve block
Blocks sensory to what area?
Motor to what area?
- Sensory: Perineum
- Motor: Urethral and anal sphincters
MOA of inactivated viral vaccines
MOA of live-attenuated viral vaccines
Inactivated: humoral response (antibodies against hemagglutin, thus preventing viral entry into the cell)
Live-attenuated: cell-mediated + humoral
What nerve runs along the orbital floor? What would be the result of damage to this nerve?
Infraorbital nerve
Damage can result in paresthesia of the upper cheek, upper lip, and uper gingiva.
What does the oxygen-dissociation curve of myoglobin look like?
Hyperbolic (not sigmoid)
The common cardinal veins of the developing embryo ultimately give rise to what?
The superior vena cava, and other constituents of the systemic venous circulation
What effect do almost all volatile (inhaled) anesthetics have on cerebral blood flow? What potential risk does this hold?
They increase cerebral blood flow, which can sometimes lead to increased ICP.
What is the classic triad assc. with Parocysmal nocturnal hemoglobinuria?
- Hemolytic anemia
- Pancytopenia
- Thrombosis at atypical sites
What is the role of beta-lactamase inhibitors? Give (3) examples.
Given with penicillin family antibiotics, so that they can work against beta-lactamase inhibitor secreting bacteria.
Ex: Clavulanic acid, sulbactam, and tazobactam
Bleeding after a tooth extraction is suggestive of what? What do you give to stop the acute bleeding?
Hemophilia (dec. factor 8 or 9)
Give thrombin
What accounts for the fistulas and strictures associated with Crohn’s Dz?
Transmural inflammation
Acute Dystonic Reaction
- What is it?
- What causes it?
- Sudden, involuntary contraction of a major muscle group
- Recent initiation of a first gen. antipsychotic medication, leading to D2 antagonism in he nigrostriatal pathway
What is Patent Foramen Ovale? What can it lead to?
Incomplete fusion of atrial septum primum and secundum
Can lead to paradoxical embolization of venous clots into the systemic arterial circulation
Scaphoid fractures most frequently result from what? What is the largest risk associated with them?
Fall onto an outstretched hand
Large risk of avascular necrosis due to their weak blood supply
How does chronic bronchitis appear on biopsy? (4)
- Thickened bronchial walls
- Leukocyte infilitrate
- Mucus gland enlargement
- Squamous metaplasia
What is the most common benign liver tumor? (include description)
Should you biopsy?
Cavernous hemangioma
Cavernous, blood-filled vascular spaces of variable size lined by single epithelial layer.
DON’T BIOPSY! (likely to bleed)
What type of residue is attached to lysosome-bound proteins?
Mannose-residue
What specific serotype is Ankylosing Spondylitis associated with?
HLA B27 (a specific human leulocyte antigen class II serotype)
Emphasis on class TWO!!
What other atopic dzs is eczema associated with? (2)
Allergic rhinitis and asthma
Ca-125 serves as a marker for what CA type(s)
Epithelial ovarian CA
Characteristic CXR for bronchial obstructive lesion
Unilateral pulmonary opacification and deviation of the mediastinum toward the opacificed lung
What vessel of the portal circulation is associated with esophgeal varices?
Left gastric vein
Give the layers of the stomach, in order.
Which layer does an erosion not go beyond?
- Mucosa
- Muscularis mucosa (erosion doesn’t go past this point)
- Submucosa
- Muscularis propria
- Serosa
Name the autoantibodies assc. w/ SLE
- Anti-smith (snRNPs)
- Anti-DS-DNA
- Anti-nuclear
How does Lead cause anemia?
Inhibition of the heme synthesis pathway, leading to a microcytic, hypochromic anemia
What is one of the main pathological causes for a uniformly enlarged uterus?
Presentation?
Adenomyosis
The presence of endometrial glandular tissue within the myometrium (presents w/ menorrhagia and dysmenorrhea)
What test is used to confirm dx of menopause?
Elevated serum FSH (due to loss of feedback)
How can opiods lead to increased pressures in the commmon bile duct/ gallbladder?
They can cause contraction of smooth muscle in the sphincter of Oddi
Where does iron absorption primarily occur?
In the duodenum and the proximal jejunum
Etiology for Mallory-Weiss Tears
Increased intraluminal gastric pressure due to retching/vomiting/other abdominal strain
Which ovarian tumor secrtes testosterone?
Sertoli-Leydig
Crigler-Najjar syndrome
- Inheritance
- Path
- Aut. recessive
- Genetic lack of UGT enzyme, leads to inability to conjugate bilirubin via glucuronidation (infants often die early)
t(15;17)
What CA?
APL (M3 AML variant)
Methylmalonic acidemia
- Etiology
- Presentation
- Deficiency of methylmalonyl-CoA mutase
- Sx: lethargy + vomiting + tachypnea
Methylnalonic Acidemia
- Labs
- Dx
- Labs: hyperammonemia, ketotic hypoglycemia, metabolic acidosis
- Dx: elevated urine methylmalonic acid and proprionic acid
Most common cause of unilateral fetal hydronephrosis
Inadequalte canalization of the Utereropelvic Jxn
What mediates the glomerulonephritis assc. with PSGN?
Type III HSNT (IC deposition)
In Cystinuria, there a defective transporter causes build up of what AAs in the intestinal lumen/kidneys?
COLA
Cysteine, Ornithine, Lysine, Arginine
(leads to nephrolithiasis)
What does the following suffix indicate about a medication?
- -cept
- -nib
- -cept: receptor molecule
- -nib: kinase inhibitor
Patients with hemochromatosis are at incrased risk for what?
Liver cirrhosis + hepatocellular carcinoma
What is the substitution associated with Sickle Cell?
Valine for glutamic acid
What is the triad associated with Wiskott-Aldrich syndrome?
- Eczema
- Thrombocytopenia
- B-cell/T-cell deficiency
C1 inhibitor deficiency (C1INH)
What happens?
- Inappropriate activation of the complement cascade (due to increased C2 and C4 cleavage)
- Also leads to inc. Bradykinin because C1INH blocks its formation
Somatostatin Function
Inhibits release of many other hormones/enzymes (Secretin, CCK, glucagon, insulin, and gastrin)
Phentolamine vs Phenoxybenzamine
Both are a-antagonists
Phentolamine: reversible, competitive (effects can be overcome by dose)
Phenoxybenzamine: irreversible (effects cannot be overcome by dose)
Pilocytic Astrocytoma
- View on biopsy
- View on gross
- Biopsy: rosenthal fibers (eosinophilic, corkscrew fibers)
- Gross: cystic + solid
CHILDREN ONLY!
Tx of Drug-induced Parkisonism
- Decrease/discontinue drug
- Give anticholinergic
3 tx for cyanide toxicity
- Direct binding of cyanide (via hydroxocobalamin)
- Use of detoxifying sulfur donors (via sodium thiosulfate)
- Induction of methemoglobinemia (via sodium nitrate)
DUI
Glomangioma
- Etiology
- Presentation
- Originates from modified smooth muscle cells that control thermoregulatory functions
- Very tender, small, red-blue lesion under the nail bed
A decrease in what component of collagen is responsible for wrinkling?
Collagen fibril production
Common tx for bradycardia?
Associated side effect?
Atropine
May increase introcular pressure, precipitating closed-angle glaucoma
Why must alcohol be avoided when on metronidazole?
Can cause disulfiram-like effects, such as abdominal cramps, nausea, HA due to acetaldehyde accumulation
Acetyl-CoA serves as an allosteric activator of what?
Gluconeogenesis (increases activity of pyruvate carboxylase)
Porcelain gallbladder is associated with an increased risk of what?
Adenocarcinoma of the gallbladder
What effect might an ASD have on pulmonary circulation?
Can lead to chronic pulmonary hypertension due to left-to-right intracardiac shunting
What is Niacin (vit B3) synthesized from? What other cofactors are needed?
Synthesized from Tryptophan
Requires vitamins B2 and B6 as cofactors (B6/B2 = B3)
High levels of what, serve to increase cholesterol solubility and decrease the risk of gallstones? (2)
- Bile salts
- Phosphatidylcholine
What does FSH stimulate in males?
What does LH stimulate in males?
FSH: Sertoli to release Inhibin (feedsback)
LH: Leydig to release Testosterone (feedsback)
Key differences between the pneumococcal conjugate and pneumococcal polysaccharide vaccines
Conjugate: Good for infants; more robust immune response via B and T cell activation
Polysaccaride: Good for adults; more serotypes covered (23 vs 13)
A child falls while holding someone’s hand. On presentation, refuses to move elbow.
What is the injury?
The annular ligament is torn and displaced
What arteries supply the head and neck of the femur?
The medial femoral circumflex and its branches
Describe the alteration in resting respiratory function in obesity
Extrinsic restrictive pulmonary function tests are seen
(everything decreases except RV which remains normal)
Less capacity + air gets out faster
What are cavernous hemangiomas (neuronal)?
What increased risk is associated with them?
- Vascular malformations mostly in the brain parenchyma
- Inc. risk of intracerebral hemorrhage and seizure
Physostigmine
- MOA
- Indication
- Cholinesterase inhibitor
- Atropine poisoning (ex. via jimson weed)
Neurofibromatosis Type 1
- Inheritance
- Mutation
- Presentation (3)
- Autosomal-dominant
- Mutation to NF1 (Ch. 17)
- Cafe-au-lait spots + multiple neurofibromas + Lisch nodules
Where is the cortisol receptor located?
Within the cytoplasm
It then translocates to the nucleus after binding its substrate.
How does vWF lead to increased PTT and bleeding time?
vWF deficiency leads to impaired platelet function (prolonged bleeding time) and coagulation pathway abnormalities because it is a carrier for factor VIII (prolonged PTT)
Define Statistical Power
(1-Beta)
A study’s ability to detect a difference when one exists
What is the relationship between transpeptidases and peniclllins/cephalosporins
Transpeptidases are an example of a “penicillin-binding protein”, which penicillins/cephalosporins bind to
What artery/nerve run along the midshact of the humerus?
Radial nerve + deep brachial artery
Name the triad associated with Hemochromatosis
- Hyperpigmentation (bronze diabetes)
- DM
- Pigment cirrhosis leading to hepatomegally
Follicular Lymphoma
- Presentation
- Associated translocation
- Painless waxing and waning lymphadenopathy
- t(14;18) w/ bcl-2 overexpression
Efficacy
Max effect achievable
During what portion of the TCA is GTP synthesized?
It is synthesized by succinyl-CoA synthetase during conversion of succinyl-CoA → succinate
Key GI manifestation of lead poisoning
Constipation
What is the result of non-fusion of the urethral folds in a male?
Hypospadia
(opening along ventral shaft of penis)
Most common adverse effects of ADHD meds
Dec. appetite and insomnia
What enzyme is most commonly deficient in impaired B-oxidation of fatty acids?
Acyl-CoA dehydrogenase (catalyzes the first step)
Best tx for patients w/ PCOS who wish to become pregnant
Explain the MOA
Clomiphene
(estrogen receptor modulator which decreases negative feedback inhibition on the hypothalamus, thus inc. GnRH)
Patients w/ CD4 counts
Prophylactic azithromycin to prevent Mycobacterium Avium Complex (MAC)
What tx can be used to partially overcome food poisoning with C. botulinum?
Rapid stimulation
Individual presents with vertical and torsional diplopia and an eye that is deviated upward.
What muscle is the issue?
Superior oblique
The presence of which serologic marker is most likely to increase the risk of vertical Hep B transmission?
HBeAg (a marker of viral replication and increased infectivity)
What process is directly stimulated by the NF-kB pathway?
NF-kB is a proinflammatory transcription factor that increases cytokine production
What is the etiology for trendelenburg gait (hip drop)?
Injury to the contralateral superior gluteal nerve (superomedial quandrant)
Where does bicarb to neutralize gastric acid come from?
Bicarb comes from the submucosal glands of the duodenum (Brunner glands) and from the pancreatic duct secretions
What cell type composes the ovaries?
Simple cuboidal
What female repro organs have simple columnar epithelium?
- Fallopian
- Uterus
- Endocervix
What female repro components have stratified squamous non-keratinized epithelium?
- Ectocervix
- Vagina
What is the major virulence factor for S. pyogenes? What does it do?
Protein M
(Inhibits phagocytosis and complement activation)
Streptomycin MOA
Inhibits protein synthesis by inactivating the 30S (small) ribosomal subunit
Define spongiosus
An accumulation of edema fluid in the intercellular spaces of the epidermis
What (3) organs/systems does stimulation of B2 receptors, relax?
- Peripheral vasculature
- Bronchi
- Uterus
Common COD for patients w/ adult-type coarctation of the aorta (3)
- LV failure
- Ruptured aortic aneurysm
- intracranial hemorrhage
(HTN associated complications)
RNA produced by RNA Pol I
rRNA
(forms ribosome components)
RNA produced by RNA Pol II
mRNA, snRNA, microRNA (gene silencing)
RNA produced by RNA Pol III
- tRNA
- 5s rRNA
Result of early antibiotics on preventing PSGN
Ineffective
(though useful for prevention of Acute RF)
Inheritance of G6P dehydrogenase deficiency
X-linked recessive
Other than IL-1, and IL-6, what other cytokine is critical in inducing a systemic inflammatory response?
TNF-a
What is the effect of Phospholipase C activation?
Leads to activation of protein kinase C, via direct DAG activation or indirect IP3 induced, Ca2+
(Phospholipase C breaks PIP2 into DAG and IP3)
Why does silicosis lead to an increased risk of TB?
Silicosis impairs macrophage function (disrupted by internalized silica particles)
Name the key E.coli virulence factor assc. w/ neonatal menigitis
K1 capsular polysaccharide
Name the stop codons
UAA
UAG
UGA
In polymyositis, what autoantibodies are common? (2)
ANA
and Anti-Jo-1 (Histidyl-tRNA synthetase)
Name the only sx of grave’s dz which is not improved via B-Blockers
Exophtalmos
Equation for Odds Ratio
OR = ad/bc
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What is the first step of base excision repair?
Removal of the defective base via glycosylase
(the empty spot on the sugar backbone is then cleaved and removed by endonuclease + lyase)
Explain the Haldane Effect
In the lungs, binding of oxygen drives hemoglobin to release H+ (protons) and CO2
- Where in the lungs do supine patients tend to aspirate?
- Upright patients?
- Supine: posterior segment of upper lobes (and superior segment of lower lobes)
- Upright: Base
RIGHT SIDE FOR BOTH
What keeps a PDA open? What closes it?
Open: Prostaglandin E2 (E, kEEps it open)
Closes: Indomethacin
In cases of Echinococcus granulosus, what can be the result of spilling of cyst contents during surgery?
Anaphylactic shock
Li-Fraumeni Syndrome
- Inheritance
- Mutation
- Family Hx
- Autosomal dominant
- Mutated TP53
- Family Hx of many CA types (sarcoma, leukemia, breast, adrenal)
What phenomenon facilitates dischare of urine from the umbilicus?
Failure of the urachus (allantois remnant), to obliterate at birth
Methotrexate
- What is it?
- Indications
- A folic acid analog that competitively inhibits dihydrofolate reductase (normally converts folic acid to THF).
- Serves as a chemotherapeutic and anti-psoriasis agent
Role of metalloproteinases during wound healing
Contracture (along with myofibroblasts)
What is the most common cause of spontaneous lobar hemorrhage in the elderly?
What are the most common sites of this hemorrhage?
CEREBRAL AMYLOID ANGIOPATHY
(Commonly in the occipital and parietal lobes)
What dz’s are Lewy bodies assc. w/?
Parkinson dz and Lewy body dementia
What conditions lead to polyhydramnios (excessive amniotic fluid)?
- GI obstruction
- Anencephaly
What kind of receptors contain zinc-finger binding domains?
Intracellular (ex. steroids, thyroid hormone, fat-soluble)
What is the role of releasing factors?
Recognize stop codons and terminate protein synthesis
What nerve handles plantar foot sensory?
Tibial
Factitious Disorder
Intentional falsification or inducement of sx w/ goal to assume sick role
Etanercept
- What is it?
- Indication
- What must you do first?
- Decoy receptor for TNF-a
- Tx for extreme RA
First must eval for TB
Metyrapone MOA
Blocks 11-B-hydroxylase (steroid pathway)
How do most enveloped nucleocapsid viruses acquire their lipid bilayer envelope?
(exception?)
By budding through the plasma membrane of the host cell.
(except for herpesviruses)
What commonly occurs following an SAH? How can it be prevented?
Vasospasm (prevent with CCB)
Patients w/ Actinic keratoses are at inc. risk for what?
Invasive squamous cell carcinoma
Lab tests show no CD18 on leukocyte surfaces.
Dx?
Leukocyte Adhesion Deficiency
(They can’t cross into tissues)
Classic presentation for Phenylketonuria
- Seizures
- Light pigmentation
- Intellectual disability
- “Musty” odor
Phenylketonuria
Pathology
What becomes an essential AA as a result?
Results from the inability to convert phenylalanine to tyrosine (via the phenylalanine hydroxylase system).
Tyrosine becomes an essential AA
Glucagonoma
- Presentation
- Dx
- Sx: (1) Necrolytic migratory erythema - bronze-colored, central indurated area w/ peripheral blistering/scaling around face, perineum, and extremities (2) DM
- Dx: Elevated glucagon levels
TOC for OCD
SSRIs
Congenital torticollis
Congenital contraction of unilateral SCM, usually due to malposition in utero, or birth trauma.
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How does VZV look on light microscopy?
Intranuclear inclusions in keratinocytes + multinucleated giant cells
Tx of Graves’ ophthalmopathy
High-dose glucocorticoids (ex. prednisone)
What mold has spherules packed with endosphores?
Coccidioides immites
What viruses does the Aedes aegypti mosquito carry?
Dengue + Chikungunya
A mass in the parotid gland, risks compressing what nerve?
Facial
(leading to ipsillateral facial droop)
Transmission of congenital toxoplasmosis
Transplacental (in utero)
Kiesselbach Plexus
Point at the nasal septum where the anterior ethmoidal, sphenopalatine, and superior labbial arteries all meet.
(most common location for epistaxis)
What type of kidney stones are assc. w/ Crohn dz?
Oxalate
(bowel damage leads to dec. Ca2+ absorption, and thus inc. free Oxlatate excretion in the urine)
Key pathogenesis for Alcohol-induced Hepatic Steatosis
Dec. Free fatty acid oxidation secondary to excess NADH production by alcohol dehydrogenase and aldehyde dehydrogenase
What drug is useful for tx of both BPH and HTN?
a1-blockers
Where do the intercostal nerves/vessels lie?
Lower border of the ribs (subcostal)
What structural change indicates irreversible myocardial cell injury?
Mitochondrial vacuolization
(loss of ability to further generate ATP)
Reid index
Ratio of thickness of the mucous gland layer in the bronchial wall, compared to everything in the wall above the level of the cartilage
(measures bronchitis progression)
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Mechanism of resistance against aminoglycosides
Methylation of the aminoglycosidee-binding portion of the 30S ribosome
When does cleft lip occur?
When the maxillary prominence fails to fuse with the intermaxillary segment
When does cleft palate occur?
When the palatine shelves fail to fuse w/ one another or w/ the primary palate
Clinical manifestations of Babesiosis
Flulike symptoms
(And labs show anemia + thrombocytopenia)
Dx of Babesiosis
Blood smear shows Intraerythrocytic pleimorphic ring forms (maltese cross)
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Echinocandin MOA
Antifungal which inhibits synthesis of the plysaccharide glucan, an essential component of the fungal cell wall.
Schizophrenia
- What is sx are required for dx
- Duration?
- At least (2) of the following (5): delusions, hallucinations, disorganized speech, grossly disorganized behavior, and negative sx (at least one of the bolded must be present)
- >6 months w/ >1 month of active sx’s
What is the role of IgA on mucosal surfaces?
It binds/inhibits the action of pili as well as other cell surface antigens that normally mediate mucosal adherence and subsequent penetration by bacteria
What order of IV medications should be given to alcoholics/malnourished patients? Why?
IV thiamine supplementation prior to IV dextrose
(or else risk precipitating Wernicke encephalopathy)
Genetic Recombination vs Reassortment
Recombination: Crossover between 2 chromosomes from different viruses
Reassortment: Exchange of entire genome segments (segmented viruses only)
How is vision affected by age?
Loss of structural proteins in the lens leads to presbyopia (far-sightedness), but also tends to slightly correct pre-existing myopia
What are the (5) P’s of Acute Intermittent Porpyria?
- Painful abdomen
- Port wine-colored urine
- Polyneuropathy
- Psych disturbances
- Precipitated by drugs (CYP450), alcohol, starvation
Tx for Acute Intermittent Porphyria
Glucose + heme
(Inhibits ALA synthase, halting build-up of PBG and ALA)
Jujenual/Ileal Atresia
- Path
- Sx
- Gross
- Vascular occlusion in utero leads to ischemia of segments of bowel, and subsequent narrowing/obliteration
- Bilious emesis
- Absence of large portions of the gut, potential winding of gut remnants winding around vascular stalk
What does PAS staining, stain for?
Glycoprotein
What role does desmopressin play in clotting?
Increases circulating factor VIII and endothelial secretion of vWF
Arginase Deficiency
Presentation
- Spastic diplegia
- Growth delay
- Abnormal movements
Injury to what portion of the male genitalia is assc. with pelvic fractures?
Posterior urethra
Sirolimus MOA
Immunosuppressant
(binds to FKBP in cytoplasm, inhibiting mTOR thus blocking IL-2)
What is PPAR-gamma?
An intracellular nuclear receptor that acts as a transcriptiona regulator of many genes involved in glucose and lipid metabolism
Adhesion of cells to the ECM involves integrin-mediated binding to what?
- Fibronectin
- Collagen
- Laminin
Tissue damage and resultant abscess formation is primarily caused by…
Lysosomal enzyme release from neutrophils and macrophages
What tissue type is unable to use ketones and why?
Erythrocytes
(lack mitchondria)
Zenker (false) diverticulum
- How does it form?
- Presentation?
- Diminished relaxation of cricopharyngeal muscles during swallowing, leading to increased intraluminal pressure in the oropharynx (forming outpouching)
- Dysphagia + halitosis (food stuck) + regurg + recurrent aspiration
Why must you be cautious when using B-blockers for patients w/ DM?
It excarcebates and masks the NE/epi mediated sx of hypoglycemia
Budd-Chiari Syndrome
Path
Occlusion of hepatic vein
What happens if a tRNA is mischarged w/ an incorrect AA?
It is either corrected by the aminoacyl-tRNA synthetase proofreader, or that incorrect AA is incorporated into the growing chain.
First line tx for general anxiety disorder
SSRIs
What defines Major Depressive Disorder w/ Psych Features?
MDD psych sx which are only present during the episode of MDD
How does Trypanosoma cruzi cause its effects?
It can destroy the myenteric plexi in the esophagus, intestines, and ureters (leading to achalasia, megacolon, and megaureter)
What effect do muscarinic agents have on the detrusor muscle?
They constrict it!! This pushes urine out.
What effect does estrogen have on the H-P-Thyroid axis?
Estrogen inc. the level of thyroxine-binding globulin which leads to a corrective, transient increase in TSH and T4
What effect does prolonged ACTH stimulation have on the adrenal glands?
HYPERPLASIA of the Zona fasiculata and reticularis
Describe the teratogenicity assc. w/ valproate
Decreases levels of folic acid, thus increasing risk of NTDs
Describe the relationship between the solubility of gas, and the onset of action
The more soluble a gas, the slower its onset of action
(less soluble gases saturate the brain quicker)
Name the (2) watershed ares of the colon, which are most susceptible to ischemic damage in hypotensive states
- Splenic flexure
- Rectosigmoid colon
Stress-related Mucosal Dz
- What is it?
- Sx?
- Acute gastric mucosal defects that develop in response to severe physiological stress
- Sx: multiple, small gastric erosions/ulcers, which may perforate or bleed
Friedreich ataxia
- Sx
- Path
- Cerebellar ataxia + dorsal column issues + scoliosis/foot abnormalities + hypertrophic cardiomyopathy
- AR disorder caused by mutation of the FXN gene
What dz is described as spotty necrosis on histo? What else does the histo show?
Hep A
(also shows ballooning degeneration and mononuclear infiltrates)
What makes up the walls of Pancreatic Pseudocysts? How is this different from true cysts?
Formed from granulation tissue and fibrosis
(true cysts are lined by epithelium)
Long term effect of Vibrio cholerae on cells of the colon
None
(does not cause cell death at all)
Effect modification vs Confounding
Effect modification: When the effect of the main exposure on the outcome is modified by the presence of another variable
Confounding: It looks similar to effect modification but appropriate stratification reveals there is actually no true difference that exists
CD14 is the surface marker for what?
Monocyte-macrophage cell lineage
Where does the left ovarian vein drain?
The right ovarian vein?
Left: drains into the left renal vein
Right: drains directly into the IVC
Digoxin Clearence
Renal
Describe the “preparation” stage in the stages of change model
Patient is in the process of planning to make a change in the near future, and mapping out how to do that
Which Benzos are preferred for patients w/ impaired hepatic metabolism?
LOT
Lorazepam
Oxazepam
Temazepam
Relationship between Azathioprine and Xanthine oxidase
Xanthine oxidase catalyzes one of the main mechanisms for azathioprine metabolism
(Any drug blocking XO will thus inc. the level of Aza)
How do you dx acute cholecystitis?
Ultrasound or otherwise nuclear medicine hepatobiliary scanning
Identify #8
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Psoas muscle
Methotrexate AE
Bone marrow suppression + liver function abnormalities
What mediates paraneoplastic cachexia
TNF-a
Costosternal Syndrome (Costochondritis)
- What causes it?
- Sx
- Caused by repetitive activity, leading to inflammation of the upper costal cartilage at the costochondral or costosternal jxn
- Pain is reproducable w/ palpation and worsened w/ movement or changes in position
What group does “reactive arthritis” from chlamydia, belong to? What does this mean?
It belongs to the HLA-B27 assc., seronagtive spondyloarthropathies
(thus there is risk for sacroiliitis)
What organs synthesize heme?
Virtually every organ (just need mitochondria). But principally in the BM (erythrocyte precursor cells) and hepatocytes
What is responsible for the hypercoagulative state of nephrotic syndrome?
The loss of anticoagulant factors in the urine
(especially antithrombin III)
Thombotic Thrombocytopenic Purpura
- What is missing?
- What happens as a result?
- Loss of ADAMTS13 (aka vWF cleaving protease), which is normally responsible for breaking vWF into smaller multimers
- Inc. large vWF multimers leads to inc. platelet adhesion and subsequent aggregation/thrombosis
TTP
- Presentation
- Tx
- Hemolytic anemia (schistocytes, inc. LDH, dec. haptoglobin)
- Give plasmapherisis + steroids
Stimulation of what adrenergic receptor causes increased IP3 release?
a-1
Male born w/ feminized external genitalia (small phallus and hypospadia) that masculinizes at puberty.
Dx?
5a-reductase deficiency
(converts testosterone to dihydrotestosterone, which mediates development of the external genitalia in the male fetus)
What must we be careful of early in tx w/ an ACE-Inhibitor?
First dose hypotension
Presentation of patients w/ von Willebrand dz
Lifelong Hx of mucosal bleeding (gingival, epistaxis, and/or menorrhagia)
Platelet levels are normal but they don’t fxn well
Hydroxyurea
MOA + Indication
For sickle cell patients w/ frequent pain crises
Increases fetal hemoglobin (mechanism unknown)
Gardos channel blocker
- Indication
- MOA
- Sickle cell
- Hinder the efflux of potassium and water from the cell, thus preventing dehydration of erythrocytes and subsequent polymerization of Hb S
Which ribs overlie the spleen?
Ribs 9, 10, and 11
Which ribs overlie the left kidney? Right kidney?
Left: Ribs 11 and 12
Right: 12
Hemolytic uremic syndrome
Etiology/pathology
Shiga toxin (E. Coli O157:H7 or Shigella ) induces capillary endotheial damage, resulting in platelet activation and formation of microthrombi
Hemolytic Uremic Syndrome
Clinical features
Hemolytic anemia (w/ schistocytes)
Acute kidney injury
Antecedent diarrheal illness
Thrombocytopenia
(HUS- HAAT)
What gene is abnormal w/ inherited Pulmonary Arterial HTN?
BMPR2
What charaterizes Drug-induced lupus erythematosus?
Abrupt onset of lupus sx w/ positive anti-histone antibodies
What type of individuals are genetically predisposed for Drug-induced lupus srythematosus (DILE)?
Patients w/ expression of slow hepatic acetylators
In general, what is a Chiari malformation?
Congenital disorder that results from the underdevelopment of the posterior fossa. Causes parts of the cerebellum and medulla to herniate through the foramen magnum.
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Describe Chiari type I
(prognosis and sx)
Relatively benign and presents in adulthood w/ occipital HA and cerebellar dysfxn
(type of Chiari malformation)
Describe Chiari type II
(prognosis and sx)
Severe form. Affects neonates and often assc. w/ myelomeningocele and hydrocephalus
(Type of Chiari malformation)
Describe the onset of action and risk of dependence for Buspirone
Slow onset and no risk of dependence
Tx of somatic symptom disorder
Regularly schedule medical visits w/ focus on fxnal improvement. Avoid unecessary dx testing.
What is the dx?
Frontal bossing
Delayed fontanel closure
Femoral/tibial bowing
Rickets
What is required to activate 6-MP?
To deactivate it?
Activate: HGPRT
Deactivate Xanthine Oxidase
Describe the biopsy findings of Subacute granulomatous (de Quervain) thyroiditis
MIxed, cellular infilitration w/ occasional multinucleated giant cells
Describe the clinical features of Subacute granulomatous thyroiditis
Painful thyroid enlargement and transient hyperthyroid sx, following a viral illness
Typical genotype of a complete mole and why
46, XX
(haploid sperm enters empty egg and duplicates. At least one X required for survival– hence no 46, YY)
What is responsible for destruction of cells w/ decreased or absent MhC class I proteins on their surface?
NK cells
What is Delusional disorder?
How does this differ from paranoid personality disorder?
Delusional disorder: > or = 1 delusion in absence of other psychotic sx
Paranoid: general suspiciousness or distrust of everyone, w/o odd fixed belief
- Potential consequences of CREST syndrome
- Assc. antibodies
- Can have sclerosis of renal, pulm, CV, and GI systems (due to small vessel deposits)
- Antibodies: (anti-centromere)
Describe the pathogenesis of prion dz
Give the (2) major examples
Prion protein (PrP) is normally a-helical in neurons. If converted to B-pleated sheets, it becomes resistant to proteases and accumulates in the brain matter creating vacuoles (spongiform encephalopathy)
ex. Creutzfeldt-Jakob and Bovine Spongiform
How (time-frame) does pheochromocytoma present?
Sx are often episodic in nature, coming and going with fluctuations in catecholamine synthesis
Anal fissures
- What are they/ what causes them?
- Most common location (be specific)?
- Longitudinal tears in the mucosa, usually due to passage of hard stool in patients w/ chronic constipation
- Usually at the posterior midline (low blood flow)
Obturator nerve
- What path does it follow?
- How is it injured?
- How does injury present?
- Only major nerve that exits the pelvis via obturator foramen
- Injury via compression
- Sx: weakness in thigh adduction + sensory loss over distal medial thigh
Where do the arteries going to the testicles/ovaries originate from?
The gonadal arteries originate from the AA
Septic abortion
- Presentation
- Common pathogens assc.
- Fever, uterine tenderness, and foul-smelling discharge after a pregnancy
- S. aureus or E.coli
What is the targetoid skin disorder assc. w/ HSV?
Erythema multiforme
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Key AE of dihydropiridine CCBs (2)
Peripheral Edema + Dizziness/lightheadedness
Rabies
- Main source in the US
- Prevention
- Bats
- Killed vaccine
Rabies
Presentation
Agitation + spasms progressing to coma
What is the most characteritic biochemical change in Huntington dz?
Dec. GABA in the brain
- What role does TGF-B play in the healing process?
- What happens in the body if there is too much TGF-B
- It is critical for fibroblast migration, proliferation, and connective tissue synthesis
- Increase is responsible for hypertrophic/keloid scarring (as well as fibrosis of the lung, liver and kidney in chronic inflammation)
How do patients w/ familial chylomicroenmia syndrome frequently present?
In childhood w/ recurrent episodes of acute pancreatitis
High levels of dietary aflatoxin exposure (produced by aspergillus) leads to what? What does this increase risk for?
G:C → T:A transversion in codon 249 of the p53 gene
(Greatly increases risk for HEPATOCELLULAR CARCINOMA)
What is AA transamination and what serves as the cofactor for it?
An amino group from an AA, is transfered to an a-keto acid, which in turn becomes an AA
Catalyzed by vitamin B6 (pyridoxal phosphate)
What kind of diet must you place a patient on who has Pyruvate Dehydrogenase Comlex Deficiency and why?
A ketogenic diet (high fat, low carbs)
This near-absence of glucose in the diet decreases the amount of pyruvate enerated, thereby decreasing lactate levels
Name the (2) exclusively ketogenic AA’s (can’t be metabolized to pyruvate)
Lysine and Leucine
First line tx of ADHD
Stimulant medications! (inc. NE and Epi to prefrontal cortex)
Local cutaneous AE of chronic topical corticosteroid administration (2 major ones)
- Atrophy/ thinning of dermis
- Tightening of skin
When a nerve is severed, what happens to the segment of axon still connectd to the cell body?
Axonal reaction (sets the stage for regeneration)
- Swelling
- Nuclei displaced to periphery
- Nissl substance becomes fine, granular, and dispersed
Who do Ovarian teratomas most comonly occur in?
Females age 10-30
Describe the histology of Schwannomas (2 key components)
- Biphasic cellularity
- S-100 positivity (neural crest derived protein)
What runs through the hepatoduodenal ligament?
The portal triad
(hepatic artery, portal vein, common bile duct)
Where in the peritoneum does the bladder sit?
The bladder is extraperitoneal
Mucormycosis
- Presentation
- Dx
- Patient w/ diabetic ketoacidosis, presenting w/ facial pain, HA, and black necrotic eschar
- Biopsy needed for dx
Where do the aspiration contents come from that cause lung abscesses?
They are oropharyngeal contents (NOT STOMACH!)
Key translocation for Burkitt lymphoma
t(8;14)
(c-Myc to Ig heavy chain)
c-Myc fxn
Transcription activator
Tx for oral/esophageal candidiasis
Nystatin
How do RBCs look in G6PD deficiency?
Heinz bodies (Hb precipitation), which can be “bitten” from the cell forming bite cells
What role do metalloproteinases of inflammatory macrophages, play in plaque stability?
These zinc-containing enzymes (Metalloproteinases) degrade ECM, making plaques more unstable
Reaction Formation
Immature defense mechanism that involves replacing unacceptable feelings and impulses w/ their extreme opposite.
Renal Angiomyolipoma
- What is it composed of?
- What dz is it associated with?
- Benign tumor composed of blood vessels, SM, and fat
- Assc. w/ tuberous sclerosis
Tuberous sclerosis
Presentation
- Hamartomas in the brain
- Facial angiofibroma
- Leaf-shaped patches of skin w/o pigment
- Seizures/ Cognitive disability
How does excess ammonia disrupt excitatory neurotransmission in the brain?
It crosses the BBB and is taken up by astrocytes, inc. glutamine production. This ultimately leads to inc. intracellular osmolarity, swelling, and impaired glutamine release for conversion to glutamate
What drugs for herpes virus do not require viral phosphorylation?
Cidofavir and Foscarnet
What characterizes Borderline Personality Disorder?
- Peristent pattern of unstable relationships
- Mood lability (possible suicidal ideation)
- Impulsivity
Achondroplasia
Path (including key mutation)
Exaggerated inhibition of chondrocyte proliferation in the growth plates of long bones, due to FGFR3 mutation.
What side of the body does each cerebellar hemisphere coincide with?
Ipsilateral
Type I (alpha) error
Falsely concludes there is a stastical difference
Type II (Beta) error
Falsely concludes that there is NO statistical difference
Berkson’s bias
Selection bias that can be created by selecting hospitalized patients as the control group
A high mitotic index (Ki-67 fraction) approaching 100% is typical for what CA type?
Burkitt lymphoma
Where does Burkitt lymphoma happen in adults and what adults are most likely to have it?
Pelvis/ abdomen
(HIV+ or generally immunocomprimsed adults)
Most common cause of Hyper-IgM syndrome
Absence of the CD40 ligand, resulting in defective immunoglobulin class switching
Which vessels come together to form the IVC
The right and left common iliac veins
Foreign bodies lodged in the piriform recess, put what nerve/reflex at risk?
The internal laryngeal nerve, which mediates the afferent limb of the cough reflex
Dubin-Johnson Syndrome
Path
Defective hepatic excretion of bilirubin glucuronides across the canaicular membrane, resulting in direct hyperbilirubinemia and jaundice
Dubin-Johnson Syndrome
Gross and Histo
Gross: liver appears black due to impaired excretion of epinephrine metabolites
Histo: Epi metabolites apprear as dense pigments w/in lysosomes
What is the most specific test for S. pyogenes?
The pyrrolidonyl arylamidase test (PYR)
Hydralazine
- MOA
- Indication
- MOA: Increases cGMP, leading to vasodilation (A > V) and afterload reduction
- Indication: severe HTN
What is the most common congenital heart defect associated w/ Down Syndrome
A complete atrioventricular septal defect
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What messenger system does Glucgaon use to exert its effects?
Adenylate cyclase second messenger system (Gs, cAMP, PKA)
What hormone receptors use PKA?
TSH, glucagon, and PTH receptors
Uniparental disomy
- What is it?
- Give (2) examples
- When an individual inherits 2 coies of a chromosome from one parent and none from the other
- Ex. Prader-WIlli syndrome or Angelman syndrome
What is the most common cause of Turner syndrome?
Paternal meiotic nondisjuntion
What z-score represents 95% of the statistical distribution? 99%?
95%: z-score = 1.96
99%: z-score= 2.58
What does a liver biopsy w/ green-brown plugs and pigment accumulation, signify?
Cholestasis
First line tx for narcolepsy
Modafinil (or other psychostimulants)
Rett Syndrome
Presentation
- Loss of speech and motor skills
- deceleration of head growth
- stereotypic hand movements
(Usually in girls, following period of normal development)
Rett syndrome = REGRESS syndrome
Most common mutation assc. w/ Rett Syndrome
MECP2 gene
Describe the histo and immunohisto for Small Cell Lung Carcinoma
Histo: clusters of small, ovoid cells w/ scant cytoplasm and high mitotic count
Immunohisto: stains positive for neuroendocrine markers, especially chromagranin
Describe the murmur assc. w/ ASD
Fixed splitting of S2
(because right side of heart now ALWAYS gets more blood, not just during respiration)
Metabolic effect of vomiting
Metabolic acidosis
What is this dx?
Necrotizing vasculitis of upper/lower resp tract (causing ulcers, sinusitis, hemoptysis) + renal issues
Wegener’s
HSP
Path
It is an IgA-mediated type III HSN Rxn
(which is why it often follows an URT infxn)
What is the role of sunlight exposure in vitamin D activation?
It catalyzes conversion of 7-dehydrocholesterol to cholecalciferol (vitamin D3)
Benzos
MOA
Positive allosteric modulation of GABAA
Common eye-related injury w/ congenital CMV
Chorioretinitis
What effect does pyruvate kinase deficiency have on RBCs and the spleen?
Leads to a hemolytic anemia due to loss of ATP and subsequent loss of cation transport.
Spleen must dispose of these parts, leading to red pulp hyperplasia.
Oseltamivir
MOA
Neuraminidase inhibitor
What causes hypersensitivity to intradermally injected tobacco extract?
Being a heavy smoker
What type of vaculitis is Buerger dz?
Segmenting (and medium sized vessels)
Decribe the (2) morphological variants of gastric adenocarcinoma
- Intestinal- solid mass that projects into stomach (glandular cuboidal/columnar cells)
- Diffuse- linitis plastica. Infiltrates stomach wall + displays signet-ring cells
Most prominent organisms associated w/ intraabdominal infxns
Bacteroides fragilis and E. coli
Major SE of ganciclovir, especially in combination w/ bactrim or zidovudine
Bone marrow suppression
What sort of antibodies can be expected in Pauci-immune RPGN?
p-ANCA/c-ANCA
(will not show up on light micro or IF, unlike IC’s or antiglomerular BM antibodies)
Axillary Nerve
- How is it injured?
- How does injury present?
- Injured via shoulder trauma
- Presentation: sensory loss over lateral shoulder + deltoid weakness
How does synchronization of glycogen degradation w/ skeletal muscle contraction occur?
Release of sarcoplasmic calcium following neuromuscular stimulation, causes activation of phosphorylase kinase, stimulating glycogen phosphorylase to inc. glycogenolysis
Neprilysin inhibitors (MOA)
Prevent degradation of ANP
(Neprilysin normally breaks down natriuretic peptides)
In addition to the eosinophilia and respiratory issues, what is a major sx assc. w/ Churg-Strauss?
Peripheral neuropathy
Capitate
When a payor (often an employer) pays a fixed, predetermined fee (to an insurance company, or physician group), to provide all services required by a patient (often employees)
(payment structure of HMOs)
DNA gyrase is aka…?
Topoisomerase II
Fxn of DNA polymerase I
The removal of RNA primers and replacement w/ DNA
What is responsible for “dimpling” that can occur in breast CA?
Malignant infiltration of the suspensory ligaments, leading to fibrosis, shortening, and thus skin traction.
Affect of inhaled anesthetics on the liver? What lab values are affected?
Can cause highly lethal fulminant hepatitis. Elevated AST and PT
What predisposes individuals for angiosarcoma?
Axillary LN dissection which leads to chronic lymphedema.
(angiosarcoma is a misnomer, as it is technically a lymphangiosarcoma)
Best tx for severe hypoglycemia in a nonmedical setting
Intramuscular glucagon
(IV dextrose in-hospital)
Effect of gallbladder hypomotility
Bile concentration (since gallbladder absorbs water), leading to formation of BILE SLUDGE
What is the major determinant of cyanosis severity in tetralogy of fallot?
The degree of obstruction of the right ventricular outflow tract
Daptomycin MOA
Depolarizes the bacterial cell membrane
What is the most common site for acute compartment syndrome? What is housed in this site? (2)
The anterior comportment of the leg, which contains:
- Deep peroneal nerve
- Anterior tibial artery/veins
Idenitifying characteristics of a craniopharyngioma (4)
- Suprasellar (rathke’s pouch)
- Calcified Cysts
- Cholesterol rich fluid w/in cysts
- Children
During continous infusion, how long does it take to reach steady state?
4-5 half-lives
What is the translocation assc. w/ follicular lymphoma? What does it lead to?
t (14;18)
BCL-2
Most likely AE of SSRIs?
Sexual dysfunction
Other name for “mole”
Nevus
Osteoblastic lesions are also known as…?
Osteolytic?
Osteblastic: sclerotic
Osteolytic: lucent (assc. w/ osteoclasts)
Elastin
What gives it its rubber-like properties?
Extensive cross-linking between elastin monomers, which is facilitated by lysyl oxidase (related to lysine)
Tennis Elbow
- What is injured?
- What attaches to this injured point?
- It is also known as lateral epicondylitis (the lateral epicondyle is injured)
- Attach point for extensor carpi radialis brevis and extensor digitorum (both involved in wrist extension)
At what point does coagulative necrosis begin after an acute MI?
4 hours
Multiple Myeloma
Sx/ presentation
CRAB eating a Roll of M & M’s
hyperCalcemia
Renal involvement
Anemia
Bone lytic lesions
Rouleaux formation
Monoclonal
M spike
How does the glu → val change in HbS lead to sickling?
Glu has a negative charge and val is neutral. Changing out the charge for neutral leads to hydrophobic interactions between deoxygentated Hb, causing the sickling shape
(+) VDRL of the spinal fluid indicates, what?
neurosyphilis
Medullablastoma
- Location
- Cell types
- Who?
- Cerebellum
- Primitive, blue cells (homer-wright rosettes)
- Children
If uncorrected, what can vesicoureteral reflux lead to?
Loss of nephrons and secondary HTN (due to all the inflammation)
What does EBV bind on the B-cell to gain entry?
CD21
Effect of lactose intolerance on stool pH
Bacterial fermentation of lactose lowers stool pH
Serum sickness
- What is it?
- What causes it?
- Type III HSN rxn to non human proteins, resulting in IC deposition
- Fever, pruritis, skin rash, arthralgia, dec. complement levels
For patients w/ IBD, how are mutations leading to colorectal cancer different from the ones which lead to sporodic CRC?
IBC patients develo early p53 and late APC mutations
Characteristic histo assc. w/ Reye syndrome
Microvesicular steatosis of hepatocytes
(no inflammation or edema)
What dz is this associated with?
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Gaucher
(Most common LSD)
What portions of the nephron are most suceptible to ATN and why?
The straight proximal tubule and thick ascending Loop of Henle
(higher oxygen demands due to active transport of ions)
Where are indirect and direct hernias located (relative to the inferior epigastric)?
Indirect: lateral
Direct: medial
Prevention of neonatal tetanus
Maternal vaccine
(passive immunity via transplacental IgG)
Describe the ulcers assc. w/ chlamydia
small and shallow + large, painful LNs
Describe the ulcers assc. w/ syphillus
single, well-circumbscribed
What is this?
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Candida albicans
(germ tube formation at 37 degrees C)
Calcipotriene
MOA and indication
Psoriasis med that is a vitamin D analog, which binds to the vit D receptor and inhibits keratinocyte proliferation
What medications should be avoided for individuals with hypertrophic cardiomyopathy?
Any that decrease preload and/or systemic vascular resistance
(you want both to be relatively high)
Where does the majority of water reabsorption occur (regardless of hydration status)?
PCT
Differentiate pilocytic astrocytoma and medullablastoma
Both are in kids
- Pilo is more common and imaging shows both solid and cystic components
- Medullo is always solid
What is the job of fructose 2,6 bisphosphonate?
Helps control the balance between gluconeogenesis and glycolysis
Temporomandibular Disorder
- What is wrong?
- Presentation
- Damage/issue with the mandibular division of the trigeminal nerve
- Pain/sx within the middle ear issues and muscles of mastication
Why do patients w/ MS develop urinary incontinence?
Loss of inhibition of detrusor contraction in the bladder
What is the main way in which UV rays cause DNA damage?
(How is it corrected?)
Formation of abnormal pyrimidine-pyrimidine covalent bonds (pyrimidine dimers)
Corrected via nucleotide excision repair
Lynch Syndrome
- What is it?
- What genes are mutated?
- Autosomal dominant dz caused by nucleotide mismatch repair
- Mutation in MSH2 and MLH1
Calcineurin
Essential for what?
What (2) drugs block it?
Essential for IL-2 activation
Blocked by the immunosuppressants cyclosporine and tacrolimus
Effect of high altitude exposure on blood gases
Hypoxemia + respiratory alkalosis
What is HER2?
Tyrosine kinase receptor
Most important environmental risk factor for pancreatic CA
Smoking
Name this psych disorder:
Eccentric, odd beliefs/magical thinking, interpersonal awkwardness/anxiety (despite familiarity)
Schizotypal
How might alpha-1 antitrypsin deficiency appear on histo?
PAS (+) granules of unsecreted, polymerized AAT in the hepatocytes
What is responsible for local defense against Candida?
Systemic defense?
Local: T cells
Systemic: Neutrophils
What role can CYP450 play in carcinogenesis?
Many pro-carcinogens rely on CYP450 monoxygenase metabolization to become activated (and thus dangerous)
Rifaximin
- MOA
- Indication
- Antibiotic which alters GI flora to decrease intestinal production and absorption of ammonia
- Hepatic Encephalopathy (too much ammonia due to liver damage)
TOC for trigeminal neuralgia
Carbamazepine
What values characterize the dyslipidemia seen in insulin resistance?
High TGs and low HDL
(no LDL involvement)
What kind of receptor is JAK2?
Cytoplasmic tyrosine kinase receptor
Path of HbC dz
Missence mutation leading to glutamate → lysine, change
What nerve provides sensation to the external auditory canal?
Vagus branch (auricular branch)
Name the (3) muscles/ muscle groups involved in sitting up
- hip flexors (iliopsoas)
- external abdominal oblliques
- rectus abdominus
(I got up for HER)
Achondraplasia inheritance
Autosomal dominant
Where do most RCCs originate from?
The epithelial cells of the proximal renal tubules
Factor V Leiden
- What is it?
- Consequences?
- Inherited mutation causing factor Va resistance to inactivation by Protein C
- Thrombosis in young person (usually pulmonary)
Role of N. meningitidis pili (fimbriae)
Adherence to nasopharynx epithelium
What parts of the brain are most susceptible to “global ischemia”?
Pyramidal cells of the neocortex or Hippocampus (#1!!!) and Purkinje cells of the cerebellum
PKU inheritance
Autosomal recessive
What is responsible for the presentation assc. w/ Pemphigus Vulgaris?
IgG autoantibodies against desmosomal proteins (eg, desmoglein– connects epithelial cells to epithelial cells)
What is responsible for the presentation assc. w/ bullous pemphigoid?
IgG antibodies against hemidesmosomes (connect epithelium to BM)
Describe the potassium levels in patients w/ DKA
Most have normal/high serum levels of potassium, despite a total body (i.e. intracellular) deficit.
dKa = dec. K+
(you must replace potassium as you manage these patients)
What accounts for the bimodal distribution of isoniazid metabolism speed in the population?
The presence of genetically “fast” and “slow” acetylators, as INH is metabolized by acetylation.
(slow acetylators = inc. risk of AE)
What are metalloproteinases composed of?
Zinc
Which SITS muscle is most vulnerable in a rotator cuff injury and why?
Supraspinatus
(Due to impingement between the acromion and the head of the humerus)
BRAF V600E mutation is associated w/ dz?
Melanoma
Dengue Fever
Presentation
- Retro-orbital pain
- Break-bone fever (hemorrhagic)
- Rash
- Thrombocytopenia
Primary vs secondary infxn of Dengue
Primary: often asymptomatic
Secondary: often much worse (different serotype)
Shiga-like toxin MOA
Inactivates 60S ribosomal subunit
What is often seen in renal tubular lumen for patients with multiple myeloma?
Bence-Jones proteins (large eosinophilic casts in tubular lumen)
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Why does Hep C not integrate into the host genome?
It lacks reverse transcriptase
What are the (2) key growth factors that promote angiogenesis in neoplastic and granulation tissues?
- VEGF
- Fibroblast growth factor
Why is the H-flu vaccine conjugated to a tetanus toxoid or n. menigitidis membrane protein?
Needed to elicit Th2 response
In addition to its anti-seizure effects, what other use does valproate have?
It can tx manic episodes assc. w/ bi-polar dz
What is the common mechanism for the protective factors against ovarian CA?
Anything htat decreases the amount of repair at the ovarian surface which ust take place as a result of lifetime ovulatory frequency (ex. oral contraceptives, multiparity, breastfeeding)
How does the effect of exogenous secretin differ between normal gastric G cells and ZE gastrinomas?
Secretin (in addition to secreting biochem), normally serves to decrease gastrin secretion from G cells.
In ZE, it paradoxically causes an increase in gastrin
Interferons a and B
- What produces them?
- What is their role?
- Produced by most human cells in response to viral infections
- Halts viral protein synthesis and promotes apoptosis of infected cels
Splenic Vein Thrombosis
- How might it occur?
- How might it present in imaging?
- Chronic pancreatitis can push a clot into the splenic vein
- It would show varices belonging to only the veins of the gastric fundus
What do you give to treat TCA overdose?
Sodium bicarb
The toxin for traveler’s diarrhea is similar to what other toxin?
Cholera toxin
What enzymes require thiamine as a cofactor? (2)
Pyruvate dehydrogenase and a-ketoglutarate dehydrogenase
What route does shigella use for invasion?
Microfold (M) cells of the Peyer patches
Superoxide dismutase
Function
Neutralizes ROS, preventing cell injury
What is myoedema and what is it associated with?
Focal “mounding” of muscle (lump formation) following percussion.
Assc. w/ hypothyroid myopathy
How do you distinguish between tuberculoid leprosy and lepromatous leprosy?
Give the lepromin skin test (similar to a PPD)
(+) in patients with tuberculoid leprosy (strong Th1 cell-mediated response to mycobacterium leprae)
(-) in patients w/ lepromatous leprosy (they have a weak Th1 response and a strong Th2 response to m. leprae)
What compound tends to be low in patients w/ narcolepsy?
Orexin (hypocretin)
Ondansetron
MOA/indication
Inhibits serotonin receptors and is used to treat N/V following chemo
Subclavian Steal Syndrome
Severe stenosis of proximal subclavian artery, leading to reversal in blood flow from the contralateral vetebral artery to the ipsilateral vertebral artery
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Damage to what structure leads to a pure sensory stroke?
Thalamus
Main cause for Lacunar infarcts
Small vessel occlusion/lipohyalinosis in the deep brain vessels, usually due to DM or HTN
Which fungi?
Mississippi and Ohio River Basins
Histoplasma capsulatum
Common cause of acute bacterial arthritis in sexually active young adults.
N. gonorrhoeae
Auer rods are chrystalized, what?
PEROXIDASE!!! (MPO+)
How do the particles look in silicosis
Birefringent silica particles surrounded by fibrous tissue
Transitional Cell Carcinoma (of the bladder)
Presentation
Painless, gross hematuria in an older male
(Usually do to smoking or occupation)
Goodpasture syndrome
Pathology? Presentation?
Anti GBM antibodies and anti-alveolar antibodies
(Present w/ hemotysis and renal failure)
What does Aldolase B deficiency cause?
Hereditary fructose intolerance
Milirinone/inamrinone
- Drug type
- Drug effect
- Phosphodiesterase-3 inhibitor
- Increases cAMP concentration leading to inc. cardiac contractility and vasodilation
Why is Primaquine added to the tx regimen w/ Chloroquine?
Chloroquine only kills malaria in the blood stream, but has no effect on the latent hepatic infxns assc. w/ P vivax and P ovale. Primaquine can eradicate these hypnozoites, preventing relapse.
In sarcoidosis what enzyme is found to be increased in the serum?
ACE
Name the Retroperitoneal Abdominal Organs
SAD PUCKER
Suprarenal glands
Aorta/IVC
Duodenum (except 1st part)
Pancreas (head/body)
Ureters and bladder
Colon (ascending and descending)
Kidneys
Esophagus
Rectum
Damage to what side of the brain can lead to neglect?
The non-dominant side
What is the status of sodium in the blood for CF patients?
Hyponatremia (they lose sodium via sweat because it is normally reabsorbed with Cl-)
What’s the main difference in symptoms between low-potency and high-potency anti-psychotics?
Low: non-neurological (sedation, anticholinergic, orthostatic hypotension)
High: extrapyramidal
What component of n. meningitidis assist it in resisting phagocytosis? What part is responsible for its toxic effects?
anti-phagocytosis: polysacharide capsule
toxicity: outer membrane lipooligosaccharide (LOS), [analogous to LPS of enteric rods]
What parts of the spinal cord can be affected by syringomyelia?
Commonly begins w/ the STT, but later stages will also compromise the DCT and/or the CST
Serum antibodies to the phospholipase A2 receptor is indicative of what?
Membranous nephropathy
(PLA2R is found in the podocytes)
How can Chron’s lead to gallstones?
Bile acids are normalaly reabsorbed in the terminal ileum, which is often inflamed in Crohn dz. Dec. bile acids = inc. cholesterol precipitation and stone formation.
Why doesn’t axonal regeneration occur in the CNS?
Persistence of myelin debris, secretion of neuroinhibitory factors, and development of dense glial scars.
Psoas abscess
- Presentation
- Dx
- Fever, back pain, inguinal mass, difficulty walking
- (+) psoas sign– pain when hip is extended
(2) key vascular signs of abusive head trauma
- Subdural hemorrhage
- Retinal hemorrhage
What is the triad associated w/ Normal pressure hydrocephalus?
Wet, Wobbly, Wacky
(urinary incontinence, ataxia, cognitive dysfunction)
Synovial fluid showing leukocyte count > 100,000 and no crystals
Dx?
Septic arthritis
How can hypertriglyceridemia lead to acute pancreatitis?
If a patient has >1000 mg/dL of TGs, the [FFA] exceeds the binding capacity of albumin and leads to direct tissue injury
What (3) enzymes/rxns require Biotin (vit. B7) as a cofactor?
What can often block the fxning of biotin?
- Pyruvate carboxylase: pyruvate → oxaloacetate
- Acetyl-CoA carboxylase: acetyl-CoA → malonyl-CoA
- Propionyl-CoA carboxylase: propionyl-CoA → methylmalonyl-CoA
(Blocked by Avidin found in egg whites)
Pick’s Dz
- Gross presentation
- Microscopic presentation
- Pronounced atrophy of frontal and temporal lobes
- Pick bodies (cytoplasmic inclusions of microtubule-associated protein tau)– silver staining
Mutation of what gene leads to Bilateral acoustic Neuroma?
NF2
(NF2 = bilateral acoustic neuroma)
AE of Ethambutol
Optic neuropathy
Presentation assc. w/ weakness in the trapezius muscle
- Impaired abduction above the horizontal
- Shoulder drooping
Central Retinal Artery Occlusion
- Presentation
- Funduscopic exam
- Sudden, painless, monocular blindness
- “Cherry-red macula” (has its own blood supply) and pale retina
Why is hypernatremia not seen in patients w/ hyperaldosteronism?
Aldosterone escape:
Inc. sodium → inc. GFR → filtration → dec. sodium retention
(this prevents volume overload)
Loss of which neurons is characteristic of Huntington Dz?
Caudate/Putamen
(indirect path of the motor circuit)
Most common disorder of the urea cycle
Ornithine transcarbamylase deficiency
(hyperammonemia and elevated urinary orotic acid)
Where does the great saphenous vein originate? Where does it travel up?
Originates at the medial side of the foot, courses anterior to the medial malleolus and then travels up the medial leg/thigh
Hepatic Angiosarcoma
- Associated with what?
- What do the tumor cells express?
- Assc. w/ carcinogens such as arsenic, thorotrast, and polyvinyl chloride
- Express CD31 (PECAM)
What is nonbacterial thrombotic endocarditis most commonly associated with?
Advanced malignancy or inflammatory dz/sepsis
What effect might carcinoid syndrome have on the heart?
Plaque like deposits of fibrous tissue on the right heart endocardium, which may progress to pulmonic stenosis and/or restrictive cardiomyopathy
What is the job of arachnoid granulations?
To absorb CSF, to remove it from the subarachnoid space.
(A malfunction here could cause communicating [normal pressure] hydrocephalus)
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What predisposes patients to lymphangiosarcoma?
Persistent lymphedema (due to chronic dilatation of lymphatic channels)
What is an interscalene nerve block used for?
(what must you be careful of?)
To anesthetize the brachial plexus for shoulder/upper arm procedure.
(Must be careful because the phrenic is almost always affected)
Anti-Yo, Anti-P/Q, and anti-Hu are the most common antibodies associated with what dz?
Paraneoplastic Cerebellar Degeneration
(an autoimmune attack of the cerebellum for CA patients)
Bell’s palsy
Idiopathic paresis of the facial nerve
What is Hypoxic-ischemic Encephalopathy and what would it show on autopsy?
It is global cerebral ischemia, due to profound cerebral hypoperfusion.
(Shows triangular watershed infarcts at locations between the zones of perfusion of the major cerebral arteries)
First line tx of essential tremor
Propranolol
What cells stain (+) for synaptophysin?
Neurons
(and thus tumors of neuronal origin)
Is ulnar deviation wrist aBduction or wrist aDduction?
aDduction
Buprenorphine
MOA and potential effect
Partial opiod agonist w/ low activity but high affinity to mu-receptor– thus it can precipitate w/drawal
More than 1/2 of patients w/ Temporal Arteritis, have what sx associated?
Polymyalgia Rheumatica!!
Most common COD in Friedreich Ataxia
Hypertrophic cardiomyopathy
Phrenic nerve arises from which spinal cord segments?
C3-C5
Options to assist patients in quiting smoking
- Varenicline (partial agonist of nicotinic Ach receptors
- Bupropion
What characterizes Adenocarcinoma in situ of the lung?
This preinvasive lesion is characterized by growth along intact alveolar septa, w/o vascular or stromal invasion.
Can spread aerogenously and be invasive!
What do Granulosa Cell Tumors of the Ovary, secrete?
Estrogen
Cavernous Sinus Thrombosis
Presentation
Deficits in CN’s III, IV, V and VI, following an infection
Primary mechanism for excess copper removal in the healthy human body?
Hepatic excretion in the bile
Role of glycerol kinase
Breakdown glycerol (from TGs) to eventually create energy/glucose
Prostacyclin action
- Vasodilates
- Inhibits platelet aggreggation
- Increases vascular permeability
(All the opposite of thromboxane A2)
Diphenoxylate
MOA and indication
Anti-diarrheal
Binds mu-opiod receptor to slow motility of the gut
What role can proteosomes play in tx of multiple myeloma?
Plasma cells in MM are producing lots of protein so they are particlular susceptible to proteosomes. The result is apoptosis.
How does INH lead to peripheral neuropathy?
By decreasing vitamin B6 levels.
It is NOT DIRECTLY TOXIC ITSELF!
The PI3K/Akt/mTOR pathway, is an intracellular pathway for what?
- Anti-apoptosis
- Pro-cell proliferation
- Pro-angiogenesis
(All play a role in CA)
What is the best follow-up test for an elevated alk phosph of unknown etiology?
y-glutamyl transpeptidase
Anti-mitochondrial antibodies are typically found in what disease?
Primary biliary cirrhosis
What is the most common trigger for DIC in pregnancy?
The release of tissue factor (thromboplastin) from an injured placenta, into maternal circulation
Prolonged QT + Neurosensory deafness
What Dz?
Jervell and Lange-Nielsen Syndrome
Where is H. pyolori found at the greatest concentration?
The prepyloric area of the gastric antrum (just before the pyloric sphincter)
What are the (2) types of Chronic Gastritis?
Type 1: (fundus/body) Autoantibodies to parietal cells → pernicious anemia + achloridia
Type 2: (antrum) H. pylori Bacteria → inc. CA risk (MALT or adenocarcinoma)
“A” comes before “B”
Toxic megacolon is mostly associated with which IBD?
Sx + Dx?
Ulcerative colitis
Abdominal pain/distension, bloody diarrhea, signs of shock.
Abdominal XR (colonoscopy and barium contrast study are contraindicated due to perforation risk)
What are the (2) distinct regulatory mechanisms of the lac operon?
- Negative via binding of the repressor protein to the Operator locus
- Positively via binding by cAMP-CAP binding upstream from the promoter region
N-O = NO
positive = promoter
Chronic Mesenteric Ischemia
Atherosclerosis involving the mesenteric arteries, which leads to bowel hypoperfusion during meals and subsequent pain.
What causes the esophageal dysmotility associated w/ CREST syndrome?
Atrophy and fibrous replacement of the muscularis in the lower esophagus. Leads to the LES becoming atonic and dilated (can result in GERD)
CEA levels are useful for the monitoring of what dz?
Colon CA
Necrotizing enterocolitis
- Path
- Pt demographic
- Bacterial invasion + ischemic necrosis of the bowel wall
- Asc. w/ premature newborns and enteral feeding (tube to stomach)
Necrotizing enterocolitis
Dx
Abdominal XR showing air in the bowel wall
The presence of erythroid precursors in organs such as the liver and spleen is indicative of what?
Extramedullary hematopoiesis (oft assc. w/ severe chronic hemolytic anemia, such as B-thalassemia)
What compound is added to stored blood and what effect might it have on a patient?
Citrate
It chelates Ca2+ and Mg, and thus may reduce their plasma levels
Lipid A
The toxic component of LPS
(Causes macrophage activation leading to sx of septic shock)
Autism spectrum disorder is typically recognized by what age?
2y
What determines whether or not an enveloped virus can attach to a specific host cell?
Whether or not a viral envelope glycoprotein w/ a high binding affinity for a host cell surface glycoprotein is present
EBV commonly stimulates B cells to do what?
Proliferate continously
(ie immortalization)
Monospot test is aka what?
Heterophile Antibody Test
Pseudomonas
Motile or non-motile?
MOTILE!!
Key difference between B-ALL and T-ALL
T-ALL has thymic mass
What are the (2) major causes of concern w/ theophylline intoxication?
- Seizures
- Tachyarrhythmias
What are the (2) most important steps in workup of metabolic alkalosis?
Ascertaining the patient’s volume status and urine Cl-
Longterm risk w/ PCOS (and why?)
Endometrial hyperplasia/carcinoma
(anovulation leads to progesterone-estrogen misbalance)
Meglitinides MOA
Close the ATP-dependent K+ channel in the pancreatic beta cell membrane, inducing depolarization and thus stimulating insulin release
What drugs tx N/V from…
Chemo?
GI issues?
Chemo: dopamine receptor antagonists, serotonin antagonists, Neurokinin 1 (nK1) receptor antagonists
GI: antimuscarinics, antihistamines
Activating mutations of the KRAS gene lead to what?
Constitutive activation of the epidermal growth factor (EGFR) pathway, promoting increased cell proliferation and growth.
Drug-Induced Interstitial Nephritis
Presentation
Fever, rash, and acute renal failure following administration of certain drugs (ex. penicillin derivatives). Also see pus (often eosinophils) in the urine
Wet vs Dry Age-related Macular Degeneration
Dry: basic gradual vision loss in one or both eyes
Wet: Inc. VEGF, in response to Dry AMD
Dx of Wet Age-related Macular Degeneration
Fundoscopy showing grayish-green subretinal discoloration w/ adjacent fluid/hemorrhage
Key difference between dysplasia and carcinoma
Dysplasia is reversible
What does bone-specific alkaline phosphatase reflect?
Osteoblastic activity
What do TRAP, urinary hydroxyproline, andurinary deoxypyridinoline reflect?
Osteoclastic activity
(urinary hydroxyproline is the most reliable)
Phencyclidine MOA
PCP is an NMDA receptor antagonist
Why do reticulocytes stain blue w/ Wright-Giemsa stain?
Residual ribosomal RNA
How does PSGN look on light microscopy?
On IF?
On EM?
light: enlarged, hypercellular glomeruli
IF: “lumpy-bumpy” granular deposits of IgG and C3
EM: electron dense deposits on the epithelial side of the BM (camel hump)
What is the role of T-tubules?
They bring AP into the interior of the muscle fibers
Effect of severe htN in the retinal precapillary arterioles
Endothelial disruption, necrosis and subsequent hemorrhage
Main dose-limiting AE of vincristine?
Neurotoxicity
What happens to phosphate levels in kidney dz?
They increase, due to a decreased filtered load
What does a stool guiac test screen for?
Occult blood
Rat poison has the same toxicities as what drug? How do you tx?
WARFARIN (so tx w/ FFP)
Terbinafine MOA
Antifungal which inhibits squalene epoxidase
What can reverse the effects of methotrexate?
N-f-THF (folinic acid, leucovorin) supplementation
Essential fructosuria
- What is it?
Benign autosomal recessive disorder which causes some dietary fructose load to be secreted in the urine unchanged
(due to defective fructokinase metabolism)
Testing for fructose in the urine
(+) copper reduction test
(-) urine dipstick
What is one of the most serious complications of the recovery phase of ATN?
Hypokalemia (can cause arrhythmia)
What are the (2) most characteristic signs of dermatomyositus?
Gottron papules (pictured) and heliotrope rash (rash on eyelids)
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Presentation of IgA nephropathy (Berger dz)
Recurrent, self-limited, painless hematuria w/in 5 days after a URI
List the cephalosporin-resistant organisms
LAME
Listeria, MRSA, Enterococci, Atypicals
Placenta accreta
When the placenta adheres to the myometrium as opposed to the endometrium (can cause severe postpartum hemmorhage)