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UWORLD
Overall review 5
| Question | Answer |
|---|---|
| What is the most common type of Renal Cell carcinoma (RCC)? | Clear cell carcinoma |
| What cells give rise to clear cell carcinoma? | Epithelial cell of the PCT |
| What common renal cell carcinoma is originated form the Epithelial cells of the proximal tubule? | Clear cell carcinoma |
| What are the gross pathologic findings of Clear Cell carcinoma? | Sphere-like mass composed of golden-yellow cells with areas of necrosis and hemorrhage |
| What gives the clear cell carcinoma is golden-yellow color? | High lipid content |
| What is Epidermolysis bullosa? | Group of inherited disorders characterized by epitelial fragility triggered by minor trauma |
| What are some examples of epithelial fragility of epidermolysis bullosa disorders? | Bullae, erosions, and ulcers |
| What are the most commonly related mutations in Epidermolysis bullosa? | Keratin mutation |
| What mutation gives rise to Epidermolysis bullosa? | Keratin mutation |
| What is Meconium ileus? | Distal small bowel obstruction due to abnormally dehydrated meconium |
| To what autosomal recessive condition is Meconium ileus strongly associated? | Cystic fibrosis |
| What is a common small bowel condition or complication seen in Cystic Fibrosis? | Meconium ileus |
| Which part, distal or proximal, of the small bowel is most affected by Meconium ileus? | Distal small bowel |
| What is the most common cause of death in patients with Cystic Fibrosis? | Persistent, treatment-resistant infections causing pneumonia, bronchiectasis, and cor pulmonae |
| Which bowel complication is associated with Down syndrome? | Hirschsprung disease |
| Which bowel complication is associated with Cystic fibrosis? | Meconium ileus |
| What medication or drug type is given to hospitalized patients to reduce risk of thromboembolisms? | Low molecular-weight heparins (LMWH) |
| What is a common prophylactic anticoagulation medication? | Low molecular-weight heparins (LMWH) |
| What are common features accompanying head and neck squamous cell cancers? | Cervical adenopathy and base tongue mass |
| What is a strong risk factor and/or environmental exposure, giving increase development of base tongue mass and cervical adenopathy, in a HNSCC patient? | Hx of tobacco smoking |
| What are the classic histologic findings of Head and Neck Squamous cell cancers? | 1. Intercellular bridges and, 2. Keratin pearls |
| What causes Hidradenitis suppurativa? | Occlusion of folliculopilosebaceous units |
| What is the most common cause of Long QT syndrome? | Mutations to K+ channel protein, that contributes to the outward-rectifying K+- current |
| What is the result of a decrease in K+ outward current? | Prolongation of the action potential duration and QT interval |
| What is the most serious arrhythmia in severe cases of Long QT syndrome? | Torsade de Pointes |
| What are complication or clinical features of Torsades? | Palpitations, syncope, seizures, or sudden cardiac arrest |
| Urinary complication of BPH | Progressive bladder outlet obstruction |
| What are chronic complication of BPH causing bladder outlet obstruction? | Hydronephrosis and renal parenchymal atrophy with scarring |
| What is the pathogenesis of Constrictive pericarditis? | Normal pericardium, is replaced with a dense, rigid pericardial tissue that restricts ventricular filling, leading to a low cardiac output (CO) adn progressive right-sided heart failure |
| What sided heart failure is expected in Constrictive pericarditis? | Right-sided heart failure |
| What are the symptoms and signs of Constrictive pericarditis with heart failure? | Elevated JVP, pericardial knock, pulsus paradoxus, and a paradoxical rise in JVP with inspiration (Kussmaul's sign) |
| What is the most likely pathogenesis of Alcoholic Liver disease? | Decrease in fatty acid oxidation secondary to excess NADH production by 2 major alcohol metabolism enzymes, alcohol dehydrogenase and aldehyde dehydrogenase |
| Which metabolic coenzyme is essential in the pathogenesis of Alcoholic liver disease? | NADH |
| Which are the two most significant enzymes involved in hepatic alcohol metabolism? | Alcohol dehydrogenase and aldehyde dehydrogenase |
| What is primary myelofibrosis? | Myeloproliferative disorder associated with bone marrow fibrosis |
| What is a consequence of Extramedullary Hematopoiesis? | Marked expansion of the Splenic Red pulp with hematopoietic progenitor cells, resulting in massive splenomegaly |
| What is the result of the expansion of the splenic red pulp? | Massive splenomegaly |
| What are some common histologic findings in PBS of primary myelofibrosis? | Dacrocytes, nucleated red cells, and immature granulocytes |
| What gives rise to Pulmonary Arterial hypertension? | Endothelial dysfunction that leads to an increase in vasoconstriction, pro-proliferative mediators (endothelin, TXA2) and decrease in vasodilative mediators (Nitric oxide, Prostacyclin) |
| What are the effects on pulmonary vasculature of Endothelin and TXA2? | Vasoconstriction and increase cell proliferation |
| What are the effects on pulmonary vasculature of Nitric oxide and Prostacyclin? | Vasodilation and decreased cell proliferation |
| Which two vascular mediators are involved in vasodilation of pulmonary vasculature? | Nitric oxide and Prostacyclin |
| Which vascular mediators are known to cause pulmonary vasoconstriction? | Endothelin and Thromboxane (TXA2) |
| What leukocyte is involved in ARDS? | Neutrophilic lung infiltration with widespread alveolar damage |
| What causes the alveolar damage in ARDS? | Proteases and reactive oxygen species (ROS), leading to failure of the vascular barrier and exudative pulmonary edema |
| What is the primary mediator of Anemia of Chronic disease? | Hepcidin |
| What is Hepcidin? | Peptide released from the liver that inhibits iron channels on enterocytes and reticuloendothelial macrophages |
| What is the overall result of increased levels of Hepcidin? | Reduced availability of iron for Erythropoiesis, which leads to normocytic anemia with poor reticulocyte response |
| What is a possible effect of long term use of supraphysiologic doses of exogenous glucocorticoids? | Cushing syndrome |
| What are the effects of Cushing syndrome in the Adrenal gland? | Bilateral adrenocortical atrophy involving the zona fasciculata and Zona reticularis |
| Which zones of the adrenal gland (cortex) are involved in Cushing syndrome? | Atrophy of the Z. Fasciculata and Z. Reticularis |
| What is a possible effect of sudden cessation of exogenous corticosteroids? | Precipitation of an Adrenal crisis |
| Which type of Pulmonary function test is seen with Cystic Fibrosis? | Obstructive |
| Obstructive or Restrictive lung profile. Cystic fibrosis? | Obstructive |
| Obstructive or Restrictive lung profile. Decreased FEV1/FVC ratio? | Obstructive |
| Which volumes are increased in an Obstructive lung profile? | TLC and RV (residual volume) |
| Is Residual volume increased or decreased in a patient with Cystic fibrosis? | Increased |
| What are some key clinical features of Subacute de Quervain's thyroiditis? | 1. Fever 2. Painful and tender goiter 3. Elevated ESR |
| What is characteristic of the way Subacute (de Quervain's) thyroiditis presents? | Initially develop transient Hyperthyroidism due to destruction of thyroid follicles, and released of preformed thyroid hormone. Chronically it develops into hypothyroidism |
| How is the Radioactive iodine uptake in de Quervain's thyroiditis? | Low radioactive iodine uptake |
| Which two main fat soluble vitamins are insufficient in breast milk, as soon as 1 month after birth? | Vitamin D and Vitamin K |
| Which is a key supplement that must be given to a baby that is breast feeding after 1 month since birth? | Vitamin D |
| What is the physiology of an AV fistula? | Allows blood for the arterial circulation to venous circulation, bypassing the high resistance of systemic arterioles |
| What is the effect on SVT , afterload, and SV in an AV fistula? | Reduced SVR, increased venous return, and increased stroke volume |
| What is a common cause of an aplastic crisis in a patient with Sickle cell disease? | Parvo B19 infection |
| What are the classic signs of symptomatic anemia? | Exertional apnea, fatigue, and low hematocrit |
| What are the findings of bone marrow examination of a aplastic anemia sample? | Giant pronormoblasts with glassy, and intranuclear viral inclusions |
| What is Primary Biliary Cholangitis? | Autoimmune disorder that most commonly affects middle-age women and is characterized by fatigue, pruritis, jaundice, cholestasis, and anti-mitochondrial antibodies |
| WHat are the classic lab findings of Cholestasis? | Increased ALP, increased GGT, and direct hyperbilirubinemia |
| Histologic finding of Primary Biliary Cholangitis? | Dense lymphocytic portal tract inflammation with granulomatosis destruction of interlobular bile ducts |
| Is PBC is seen with lymphocytic or neutrophil infiltration? | Lymphocytic portal tract inflammation |
| Which neurodegenerative illness associated with Nigrostriatal degeneration? | Parkinson disease |
| What is the result of Nigrostriatal degeneration in Parkinson disease? | Reduces activity of the Thalamus and its projections to the cortex, resulting in bradykinesia and rigidity |
| At what level of the brain, deep brain stimulation is beneficial in Parkinson disease? | Globus Pallidus Internus or Subthalamic nucleus |
| What is promoted by deep brain stimulation of the Globus pallidus internus or subthalamic nucleus? | Promotes Thalamo-cortical disinhibition with improved mobility |
| What are the four phases of wound healing (in order)? | 1. Hemostasis (fibrin clot formation) 2. Inflammation (cellular infiltration) 3. Proliferation (re-epithelization, fibroplasia, and angiogenesis) 4. Maturation (collagen remodeling) |
| Fibrin clot formation is the _______________ step of wound healing. | First |
| What are symptomatic characteristics of ARDS? | Hypoxia and bilateral pulmonary infiltrates and is associated with pneumonia, sepsis, trauma, and pancreatitis |
| What is the effect on PCWP in ARDS patients? | Not affected and remain within normal range of 6-12 mmHg |
| CKD is associated with Hyper- or Hypophosphatemia? | Hyperphosphatemia |
| CKD causes: | - Hyperphosphatemia as it binds to serum Calcium, and - Low 1, 25 - (OH) vitamin D as ti ecrescred intestinal calcium absoption and calcium release from bone |
| CKD is seen with what type of PTH abnormality? | The hypocalcemia in CKD stimulates release of PTH causing secondary hyperparathyroidism |
| What nephrogenic condition is seen with Secondary Hyperparathyroidism? | CKD |
| Hypocalcemia + Hyperphosphatemia + Low vitamin D + secondary Hyper-PTH. Dx? | CKD |
| What is Synaptophysin? | Protein found in the presynaptic vesicles of neurons, neuroendocrine, and neuroectodermal cells |
| What is a common positive marker is some neuronal origin brain tumors? | Synaptophysin |
| What are brain neoplasms are + for GLIAL origin? | Astrocytomas, Ependymomas, and Oligodendrogliomas |
| Which brain tumors stain (+) GFAP? | Astrocytomas, Ependymomas, and Oligodendrogliomas |
| How is Dermatomyositis defined? | Proximal muscle weakness and dermal manifestations |
| What are the dermal manifestations associated with Dermatomyositis? | Heliotrope rash and Gottron papules |
| What is the most common muscle enzyme elevated in many muscle weakening illnesses? | Creatine kinase (CK) |
| - Elevated CK - (+) antinuclear antibody - (+) anti-Jo-1 antibody | Polymyositis and Dermatomyositis |
| What is the inheritance pattern of Huntington's disease? | Autosomal dominant |
| What is Huntington disease? | AD neurodegenerative disease that lead to the accumulation of abnormal toxic huntingtin protein in neural cells |
| Which neurons are most affected in Huntington's disease ? | Inhibitory GABAergic neurons in the Caudate nuclei |
| What are the main manifestations of Huntington's disease? | Chorea and behavioral abnormalities |
| What is a Melanoma? | Highly aggressive malignancy that often metastasizes early in the disease course |
| What is the histology of Melanoma? | Cellular atypia with cells containing brown pigment (melanin granules); and immunostaining for melanocyte markers (S-100, HMB- 45) |
| What are the most common melanocyte markers in Melanoma? | S-100 and HMB-45 |
| What is the most effective way to reduce Hepatocellular cancer in developing countries? | Universal vaccination of children against HBV |
| What does "TAVI" stand for? | Transcatherer Aortic Valve Implantation |
| What is a common complication of TAVI? | Paravalvular Aortic Regurgitation |
| What is a procedure to treat severe Aortic stenosis if the patient is unable to sustain surgery? | Transcatherer Aortic Valve Implantation |
| What is the pathogenesis of Paravalvular Aortic regurgitation? | Complications TAVI, resulting from improper sealing of the prosthetic valve to the native aortic valve annulus |
| Improper sealing of the prosthetic valve to the native aortic valve annulus | Paravalvular Aortic Regurgitation |
| What is the common triad of Paroxysmal Nocturnal Hemoglobinuria (PNH)? | 1. Hemolytic anemia 2. Hypercoagulability 3. Pancytopenia |
| What gene is mutated in Paroxysmal Nocturnal hemoglobinuria? | PIGA gene |
| What cause the mutation of PIGA gene by PNH? | Absence of the glycosylphosphatidylinositol anchor and deficiency of CD55 and CD59 complement inhibitory proteins |
| Which CD complement proteins are deficient in PNH? | CD55 and CD59 complement inhibitor proteins |
| CD55 and CD59 complement inhibitor proteins deficiency. Dx? | Paroxysmal Nocturnal Hemoglobinuria |
| What is Postpartum thyroiditis? | Occurs within 12 months of pregnancy and is characterized by autoimmune destruction of thyroid follicles |
| What is the pattern of Postpartum thyroiditis? | Initially hyperthyroid profile due to release of preformed TH, followed by a hypothyroid phase due to depletion of TH, and eventually returning to a Euthyroid state |
| What is the common histological finding of Postpartum thyroiditis? | Lymphocytic infiltration with the formation of germinal centers, which is overall similar to chronic Hashimoto |
| What is the MCC of Primary Congenital Hypothyroidism? | Thyroid dysgenesis |
| What is the TSH and T4 levels in Primary congenital hypoTH? | TSH is elevated and Thyroxine (T4) is low |
| What are the main clinical (recognizable) features of Pulmonary Arterial Hypertension? | Loud pulmonic component of S2 and an accentuated palpable impulse of the Left-sternal border |
| What condition is associated with a Left Parasternal lift due to right ventricular heave? | Pulmonary Arterial Hypertension |
| What is Dactylitis? | Painful swelling of the hands and feet |
| What is a common and recognizable feature of Sickle cell disease in young children? | Dactylitis |
| To which hematologic condition is Dactylitis associated with? | Sickle cell disease |
| The "sickling" episodes in SCD result in what lab findings? | 1. Increased INDIRECT bilirubin 2. Lactate dehydrogenase 3. Decreased levels of haptoglobin |
| What are some hemodynamic and cardiologic structural changes caused by VSD? | Increased RV pressure and large Left sided volume overload with increased LV and LA pressures |
| What causes changes in membrane potential? | Changes or alterations in neuronal membrane permeability to various ions |
| What ions provide negativity to the membrane resting potential? | K+ and Cl- |
| Which ions provide positivity tohe membrane resting potential? | Na+ and Ca2+ |
| Which ions involved in membrane potential are "Ligand"-gated? | Na+ and Ca2+ |
| What nerve innervates the diaphragm? | Phrenic nerve |
| What are the origins (roots) or the Phrenic nerve? | C3-C5 nerve roots |
| Which muscles aid in active expiration? | Internal intercostals and abdominal mucles |
| What innervates the Internal intercostals? | Thoracic nerve rootlets |
| What nerve innervates the abdominal muscles that help in active expiration? | Thoracic and lumbar nerve roots |
| Damage to the Thoracic nerve roots may represent what respiratory deficient? | Decrease involvement of the internal intercostals and abdominal muscles to aid in active expiration |
| What autoimmune disorder is triggered by inability to metabolize gluten? | Celiac disease |
| What is Celiac disease? | Autoimmune disorder triggered by gluten, causing small intestinal intraepithelial lymphocytosis, villous atrophy, and crypt hyperplasia |
| What is rare but severe complication of Celiac disease? | Monoclonal T cell expansion in the small intestine mucosa, lead into the enteropathy-associated T-cell lymphoma |
| What is the first step in pathogenesis of Appendicitis? | Obstruction of the lumen of the appendix |
| What are some common causes of appendix lumen obstruction? | Fecaliths, hyperplastic lymphoid follicles, foreign bodies and tumors |
| What are the acute clinical signs of Appendicitis? | RLQ pain, nausea, vomiting, diarrhea, and fever |
| What is Seborrheic keratosis? | Pigmented macules or plaques with greasy surface and well-demarcated borders |
| What is the Leser-Trelat sign? | Rapid onset of numerous lesions is an indication of internal malignancy |
| Which is an important malignancy associated with Leser-Trelat sign? | Gastric adenocarcinoma |
| In heart failure, what causes the pulmonary edema? | Increased pulmonary venous pressure |
| What is the result in HF patients of elevated pulmonary venous pressure? | Pulmonary edema |
| How does the pulmonary edema appear within the alveoli in HF patients? | Pink, acellular material within the alveoli |
| What process or actions lead to pulmonary edema? | The elevated pulmonary venous pressure causes alveolar capillaries to be engorged with blood, which transudates fluid plasma across the alveolar-capillary membrane, leading to the pink, acellular material seen inside the alveloi |
| What does presence of erythroid precursors in the liver and spleen, are indicative of? | Extramedullary hematopoiesis |
| What is Extramedullary hematopoiesis? | Condition characterized by EPO-stimulating , hyperplastic marrow cell invasion of extramedullary organs |
| Which condition is often seen to develop extramedullary hematopoiesis? | Beta Thalassemia |
| What is the gross and microscopic description of sample of acute pancreatitis? | Grossly edematous, which areas of fat necrosis, calcium deposition, and interstitial edema |
| Which condition is associated with "chalky-white areas" of fat nerosi? | Necrotizing (hemorrhagic) pancreatitis |
| Chalky-white areas in pancreas. Dx? | Necrotizing (hemorrhagic) pancreatitis |
| What are varicose veins? | DIlated, tortuous veins most commonly found in superficial veins of the leg |
| What causes development of varicose veins? | Increased intraluminal pressure and/or loss of tensile strength i the vessel wall, leading to incompetence of the venous valves |
| Are the venous or arterial vessels involved in varicose vein development? | Veins |
| What are common complication of Varicose veins? | Edema, stasis dermatitis, skin ulcerations, poor wound healing and infections |
| What is the most common consequence of an MI affecting the left ventricle? | Pulmonary edema |
| What does the presence of Hemosiderin-laden macrophages indicate? | Chronic lung congestion due to heart failure |
| Are Hemosiderin-laden macrophages, an indication of chronic or acute lung congestion in heart failure? | Chronic lung congestion |
| What actions and/or functions are carried by the frontal lobes? | Personality, language, motor functions, and executive functions |
| Abstraction is an example of an _________________ function, carried by the frontal lobe. | Executive |
| How is frontal lobe function often tested? | Test abstraction, by asking the patient to indicate similarities in two related objects |
| If a physician asks the patient to indicate relations between two related objects, it probably to diagnose what type of lobe dementia? | Frontotemporal lobe dementia |
| What is the pathophysiology of NRDS? | Inadequate surfactant production, resulting in increased alveolar surface tension and decreased alveolar compliance |
| What is Atelectasis? | Diffuse alveolar collapse |
| What is the CXR findings of Atelectasis? | Ground-glass opacities with air bronchograms |
| What are ganglion cysts? | Benign, fluid-filled masses that occur due to mucoid degeneration of Periarticular tissue |
| How are ganglion (dermal) cysts clinically presented? | Painless, smooth, rubbery, round structures that overlie joints (wrist) or tendons and transilluminate |
| What is the main clinical triad of Ataxia Telangiectasia? | 1. Cerebellar ataxia, 2. Telangiectasia, and, 3. Sinopulmonary infections |
| What is the mode of inheritance of Ataxia Telangiectasia? | Autosomal recessive |
| What gene is mutated or defective Ataxia Telangiectasia? | ATM gene |
| What is the role of the ATM gene? | Plays role in DNA break repair |
| In Ataxia Telangiectasia, which is the main immunoglobulin deficiency presented? | IgA deficiency which predisposes to upper and lower respiratory infections |
| What is the result on the kidneys of chronic heart failure having a decreased cardiac output? | Decreased renal perfusion and thus consequence activation of RAAS in maladaptive effort to maintain effective blood volume |
| Where is AT II synthesized? | AT I is converted in the lungs to AT II by ACE |
| What are some normal changes in liver function with age? | Decreased liver mass and blood flow, decreased CYP450 expression, concentration, and reduced hepatic regeneration after injury |
| How are hepatic aminotransferase levels are changed in normal aging? | Unchanged |
| What type of cardiac hypertrophy is seen in normal aging? | Concentric LV hypertrophy |
| Why is Concentric LV hypertrophy seen in elderly? | Allow for maintenance of Left-Ventricular contractility and ejection fraction despite cardiomyocyte CO and increased LV afterload |
| What is secreted in response of hyperphosphatemia in CKD? | Fibroblast growth factor 23 (FGF23) |
| What is the function of FGF-23? | Secreted in response to hyperphosphatemia and lowers plasma phosphate by reducing intestinal absorption and renal reabsorption of phosphate |
| Is CKD seen with hyper or hypo-phosphatemia? | Hyperphosphatemia |
| Why is there hyperphosphatemia in CKD? | Develop due to decreased filtration of phosphate |
| What are the nerve roots of the Phrenic nerve? | C3 and C5 |
| Which nerve has roots in the C3 and C5 roots? | Phrenic nerve |
| What is innervated by the Phrenic nerve? | Ipsilateral hemidiaphragm |
| What are symptoms caused by lung cancer affecting the Phrenic nerve? | Hiccups and diaphragmatic paralysis with dyspnea |
| What nerve is often affected by metastatic lung cancer which produces hiccups and diaphragmatic paralysis with difficulty breathing? | Phrenic nerve |
| What is the most significant symptom of Recurrent Pharyngeal nerve involvement in any type of pathology? | Hoarseness of voice |
| Affection of which nerve is often manifested with hoarseness of voice? | Recurrent Laryngeal nerve |
| What is Vitiligo? | Condition characterized by the loss of Epidermal Melanocytes |
| What is the diagnosis of a person with loss of epidermal melanocytes? | Vitiligo |
| What conditions are strongly associated with Vitiligo? | Autoimmune disorders such as DM type 1 and autoimmune thyroiditis |
| How is G6PD deficiency clinically presented? | Episodes of hemolytic anemia following oxidative stress |
| What are the histological findings of G6PD deficiency? | Reticulocytosis, and evidence of Bite cells and Heinz bodies on PBS |
| What is the most common associated mutated in hereditary cancer? | BRCA1 and BRCA2 |
| What is the role and function of BRCA1/2? | DNA repair, and the mutation increases the chances of developing breast and ovarian cancer |
| 1 hit gain of function | Oncogenes |
| What are Lacunar infarcts? | Small, ischemic infarct (usually <15mm) involving the deep brain structures (basal ganglia, pons) and subcortical white matter (internal capsule, corona radiata). |
| Wht is the most common cause of Lacunar infarcts? | Hypertension |
| What is Peripartum cardiomyopathy? | Rare form of DCM that may be related to impaired funtion of angiogenic growth factors |
| What kind of hypertrophy is seen with dilated cardiomyopathy? | Eccentric hypertrophy |
| What occurs in chronic DCM which leads to overwhelming cardiac wall stress? | Left ventricular failure with reduced ejection fraction an symptomatic heart failure |
| What is Celiac disease? | Autoimmune disorder triggered by dietary gluten that develops almost exclusively in patients with HLA-DQ2 or -DQ8 sertotys |
| What are the HLA serotypes involved in Celiac disease? | HLA-DQ2 & HLA-DQ8 |
| What is the earliest histologic finding of Celiac disease? | Duodenal intraepithelial lymphocytosis |
| What are the late gross findings of Celiac disease? | Crypt hyperplasia and villous blunting of the small intestine |
Created by:
rakomi
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