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Designing USMLE-style scenarios and questions Eric Niederhoffer SIU-SOM Outline • USMLE style • Scenario template • Resources • Clinical pearls • Example designs USMLE Style • • • • • • • • Focus on important concept, not trivial facts Assess application of knowledge, not isolated recall Avoid giving facts that may be used elsewhere on exam Avoid defining terms or drugs that should be in knowledge base Pose a clear question with one best answer Provide homogeneous distractors (incorrect options) Avoid technical flaws or clueing Five answer choices (or balanced) Scenario Template • A n-year-old (Age) boy/girl/man/woman (Gender) comes/is brought to the physician/emergency department (Site of Care) for evaluation of/with a problem (Presenting Complaint including Duration and Patient/Family History). • Physical examination shows some combination of pertinent findings (Height/Weight/BMI/Percentile, Temperature/Heart Rate/Breathing Rate/Blood Pressure). • Laboratory studies show some combination of test results (Reference Ranges provided for non-adult age groups/special tests). • Which drug, toxic exposure, diet; predict physical findings, lab findings, sequelae; identify underlying cause/mechanism/diagnosis, cause of drug response, drug to administer (Questions) Resources • Textbooks with clinical correlations • Internet sites for selected diseases emedicine.com Harrison’s eSupplement NCBI endocrinology Online pathology cases • Primary literature • Reference ranges for ages/gender (pediatric) Clinical Pearls “Pink Puffer” A person where emphysema is the primary underlying pathology. There has been gradual destruction of the pulmonary capillary bed and decreased ability to oxygenate the blood. Less surface area for gas exchange, less vascular bed for gas exchange--but less ventilation-perfusion mismatch than blue bloaters. The person compensates by hyperventilation (the "puffer" part). Arterial blood gases are relatively normal. Because of the low cardiac output, patients develop muscle wasting and weight loss. Less hypoxemia (compared to blue bloaters) and appear to have a "pink" complexion. No right sided heart failure signs. pH increased PCO2 decreased-normal mm Hg PO2 >60 (70) mm Hg HCO3decreased mEq/L DLCO decreased Clinical Pearls “Blue Bloater” A person where the primary underlying lung pathology is chronic bronchitis. Caused by excessive mucus production with airway obstruction resulting from hyperplasia of mucus-producing glands, goblet cell metaplasia, and chronic inflammation around bronchi. Pulmonary capillary bed is undamaged. Body responds by decreasing ventilation and increasing cardiac output. Poor ventilationto-perfusion mismatch leading to hypoxemia and polycythemia. There is increased carbon dioxide retention (hypercapnia). Because of increasing obstruction, residual lung volume gradually increases (the "bloating" part). Hypoxemic/cyanotic manifests as bluish lips and faces--the "blue" part. Right-sided heart failure, peripheral edema, systemic venous congestion, elevated hematocrit and respiratory acidosis are characteristic. Sputum, coughing and infections are general features. pH decreased PCO2 >45 (increased) mm Hg PO2 <60 (decreased) mm Hg HCO3increased mEq/L Disease or Condition Approach (Piriformis Syndrome) • Find additional information about PS from emedicine • Search (piriformis syndrome “clinical case”) for an appropriate clinical case (review) to abstract • Consider what relevant concept or content to assess • Design a specific question (use diagrams) • Provide balanced homogeneous distractors Piriformis Syndrome A 36-year-old woman comes to the physician with a 1-week history of pain in the right buttocks that radiates to the right lower leg. She has tried aspirin, but the pain persists. Physical examination shows pain present in the vicinity of the greater sciatic notch during extension of the knee with the hip flexed to 90º, tenderness to palpation of the greater sciatic notch, and a sausage-shaped mass over the piriformis muscle. The woman is given an injection of bupivacaine hydrochloride (amide anesthetic) intramuscularly and is treated with naproxen. She is provided instructions for rest along with gentle muscle stretching. Where do we go from here? – – Ask mechanism of action for medications. Ask which receptors are involved. Piriformis Syndrome #1 A 36-year-old woman comes to the physician with a 1-week history of pain in the right buttocks that radiates to the right lower leg. She has tried aspirin, but the pain persists. Physical examination shows pain present in the vicinity of the greater sciatic notch during extension of the knee with the hip flexed to 90º, tenderness to palpation of the greater sciatic notch, and a sausage-shaped mass over the piriformis muscle. The woman is given an injection of bupivacaine hydrochloride (amide anesthetic) intramuscularly and is treated with naproxen. She is provided instructions for rest along with gentle muscle stretching. The mechanism of action for the medication that the patient initially tried most likely increases the concentration of which of the following labeled molecules? A. B. C. D. E. A B C D E Piriformis Syndrome #2 A 36-year-old woman comes to the physician with a 1-week history of pain in the right buttocks that radiates to the right lower leg. She has tried aspirin, but the pain persists. Physical examination shows pain present in the vicinity of the greater sciatic notch during extension of the knee with the hip flexed to 90º, tenderness to palpation of the greater sciatic notch, and a sausage-shaped mass over the piriformis muscle. The woman is given an injection of bupivacaine hydrochloride (amide anesthetic) intramuscularly and is treated with naproxen. She is provided instructions for rest along with gentle muscle stretching. The mechanism of action for one of the medications used to treat the patient inhibits an enzyme that most likely synthesizes which of the following labeled molecules? A. B. C. D. E. F. G. A B C D E F G Piriformis Syndrome #3 A 36-year-old woman comes to the physician with a 1-week history of pain in the right buttocks that radiates to the right lower leg. She has tried aspirin, but the pain persists. Physical examination shows pain present in the vicinity of the greater sciatic notch during extension of the knee with the hip flexed to 90º, tenderness to palpation of the greater sciatic notch, and a sausage-shaped mass over the piriformis muscle. The woman is given an injection of bupivacaine hydrochloride (amide anesthetic) intramuscularly and is treated with naproxen. She is provided instructions for rest along with gentle muscle stretching. The mechanism of action for the medication that the patient initially tried increases the concentration of a molecule that most likely binds to which of the following labeled receptors? A. B. C. D. E. F. G. H. A B C D E F G H Relevant Concept Approach (Glycoprotein Structure/Function) • Find information about abnormal/defective glycoproteins from reference sources • Search emedicine for appropriate diseases to consider • Construct a brief scenario representative of common presentation • Design a specific question • Provide balanced homogeneous distractors Glycoproteins Glycoprotein Disease/Condition Antibodies immunoglobulin A deficiency, autosomal dominant hyperimmunoglobulin E (Job) syndrome Blood group antigens transfusion Collagen Alport syndrome, vitamin C deficiency (scurvy) Fibrillin Marfan syndrome, isolated ectopia lentis Glycoprotein IIb/IIIa immune thrombocytopenic purpura, Glanzmann thrombasthenia Mucins scleredema, asthma Thyroid stimulating hormone goiter, thyroxine-binding globulin deficiency Concept Approach #1 Goiter A 37-year-old woman comes to the physician with a 6-month history of neck discomfort. A photo of the patient is shown. Laboratory serum studies show: TSH 24 µU/mL (N=0.5-5.0) Thyroxine (T4) 22 µg/dL (N=5-12) Analysis of the protein hormone will most likely show which of the following co- and post-translational modifications? A. B. C. D. E. F. G. H. Acylation Alkylation Biotinylation 𝛾-Carboxylation Glycosylation Hydroxylation Isoprenylation S-Nitrosylation Concept Approach #2 Goiter A 37-year-old woman comes to the physician with a 6-month history of neck discomfort. A photo of the patient is shown. Laboratory serum studies show: TSH 24 µU/mL (N=0.5-5.0) Thyroxine (T4) 22 µg/dL (N=5-12) Analysis of the protein hormone will most likely show co- and post-translational modifications of which of the following amino acids? A. Asparagine B. Glycine C. Histidine D. Lysine E. Serine F. Tyrosine Concept Approach #3 Goiter A 37-year-old woman comes to the physician with a 6-month history of neck discomfort. A photo of the patient is shown. Laboratory serum studies show: TSH 24 µU/mL (N=0.5-5.0) Thyroxine (T4) 22 µg/dL (N=5-12) Post-translational modifications of the protein hormone will most likely occur in which of the following cellular locations? A. Endoplasmic reticulum A. Golgi complex B. Lysosome B. Lysosome C. Mitochondrion C. Mitochondrion D. Nuclear membrane D. Nuclear membrane E. Peroxisome E. Peroxisome F. Plasma membrane F. Plasma membrane Respiratory Acidosis #1 A 64-year-old man is brought to the emergency department complaining of a persistent headache, feeling weak and drowsy, and having a chronic productive cough. He has been smoking 2 packs of cigarettes a day for over 40 years. A photograph of the patient is shown. He is 170 cm (5 ft 7 in) tall and weighs 50 kg (110 lb); BMI is 18 kg/m2. His temperature is 38.3°C (101°F), pulse is 105/min, respirations are 22/min, and blood pressure is 130/90 mm Hg. Physical examination shows cyanosis, an A-P to lateral ratio of 1:1, use of accessory muscles to inspire, and use of abdominal muscles to expire. Upon ascultation, scattered rhonchi and wheezes are heard bilaterally. A chest x-ray is shown. Laboratory studies (room air) show: pH 7.32 (N=7.35-7.45) PCO2 56 mm Hg (N=33-44) PO2 35 mm Hg (N=75-105) HCO3 28 mEq/L (N=22-28) O2 saturation 60% (N=96-100) α1-antitrypsin 96 mg/dL (N=110-200) Based upon the findings, which of the following is the most likely description of the patient’s acid base status? A. Acute respiratory acidosis B. Acute respiratory alkalosis C. Metabolic acidosis with appropriate compensation D. Metabolic alkalosis with appropriate compensation E. Respiratory acidosis with appropriate compensation F. Respiratory alkalosis with appropriate compensation Respiratory Acidosis #2 A 64-year-old man is brought to the emergency department complaining of a persistent headache, feeling weak and drowsy, and having a chronic productive cough. He has been smoking 2 packs of cigarettes a day for over 40 years. A photograph of the patient is shown. He is 170 cm (5 ft 7 in) tall and weighs 50 kg (110 lb); BMI is 18 kg/m2. His temperature is 38.3°C (101°F), pulse is 105/min, respirations are 22/min, and blood pressure is 130/90 mm Hg. Physical examination shows cyanosis, an A-P to lateral ratio of 1:1, use of accessory muscles to inspire, and use of abdominal muscles to expire. Upon ascultation, scattered rhonchi and wheezes are heard bilaterally. A chest x-ray is shown. Laboratory studies (room air) show: pH 7.32 (N=7.35-7.45) PCO2 56 mm Hg (N=33-44) PO2 35 mm Hg (N=75-105) HCO3 28 mEq/L (N=22-28) O2 saturation 60% (N=96-100) α1-antitrypsin 96 mg/dL (N=110-200) Compensation for the acid base condition in this patient will most likely increase which of the following? A. α1-Antitrypsin concentration B. Carbonic anhydrase concentration C. H+ concentration D. HCO3- concentration E. PCO2 F. PO2 Ketogenesis An investigator is studying the effect of nutrition on pediatric epilepsy. A matched set of children with a history of epilepsy are provided either a diet containing 60% medium-chain triglyceride oil, 20% protein, 10% carbohydrate and 10% other dietary fats or an unrestricted diet. Over the course of the 12-month study, there is a 20% decrease in the frequency of seizures in the experimental group of children. An increased serum concentration of which of the following is most likely in the experimental group of children? A. Glucose B. Insulin C. Insulin-like growth factor 1 D. Ketone bodies E. Sorbitol F. Triglycerides Iodine Deficiency An investigator is studying the effect of dietary supplements on endocrine function. Three women from a remote location in Asia are enrolled as part of the study. A photo of the women is shown. Laboratory studies show: TSH increased The women are treated with an over-the-counter dietary supplement. During the next 12 months, serum TSH concentration decreases to the reference range. Which of the following supplements is most likely used to treat the patients? A. Calcium carbonate B. Copper histidine C. Ferrous gluconate D. Magnesium oxide E. Potassium iodide Transcription of Gluconeogenesis Genes An investigator is studying the effect of nutrition on serum glucose concentrations. After one week of decreased food intake, serum cortisol concentrations are increased in the experimental group. Transcription of which of the following hepatic genes shown in the diagram is most likely increased in the experimental group? A. B. C. D. E. F. A B C D E F Urea Cycle Defects An investigator is studying the effect of specific defects associated with metabolic pathways. After eating a protein containing diet, laboratory studies show elevated serum concentrations of arginosuccinate and arginine but no hyperammonemia in the experimental group. A diagram of the metabolic pathway is shown. This experimental group most likely has an enzyme deficiency in which of the following marked locations? A. A B. B C. C D. D E. E F. F Dehydrogenase Complex Subunits A five-month-old infant is brought to the physician with severe lethargy, poor feeding, and tachypnea. Physical examination shows the presence of mental, psychomotor, and growth delays. Laboratory studies show increased blood and cerebrospinal fluid lactate and pyruvate concentrations. Molecular genetic studies show a defect in the riboflavin-dependent E3 component of the pyruvate dehydrogenase complex. Which of the following additional dehydrogenase-catalyzed steps shown in the diagram is most likely defective? A. B. C. D. E. F. G. A B C D E F G Ischemic Forearm Test A 22-year-old man comes to the physician with muscle pain and fatigue. Laboratory studies show compensated hemolytic anemia with serum lactate and ammonia concentrations within the reference ranges. An ischemic forearm test shows decreased serum concentrations of lactate and increased serum concentrations of ammonia (N=increased lactate and ammonia). Which of the following enzyme-catalyzed reaction steps in the diagram shown is most likely deficient in this patient? A. B. C. D. E. F. A B C D E F Regulation of Glycolysis When a patient is resting and energy demands are low in skeletal muscles, ATP and citrate bind to and inhibit which of the following labeled glycolytic enzymes? A. B. C. D. E. A B C D E Phototransduction Amplification Increased enzymatic activity of which of the following labeled locations is most directly responsible for generating the increased signal amplification associated with phototransduction? A. B. C. D. E. A B C D E Reference Ranges K+ ClHCO3Ca2+ Urea nitrogen Glucose, fasting Creatinine Phosphorus Total CO2 Total protein Albumin Osmolality Uric acid Serum 135-147 mEq/L 3.5-5.0 mEq/L 95-105 mEq/L 22-28 mEq/L 8.4-10.2 mg/dL 7-18 mg/dL 70-110 mg/dL 0.6-1.2 mg/dL 3-4.5 mg/dL 24-30 mEq/L 6-7.8 g/dL 3.5-5.5 g/dL 275-295 mOsm/kg water 3-7 mg/dL Total cholesterol LDL HDL Triglycerides Lipids <200 mg/dL <130 mg/dL >40 mg/dL <160 mg/dL Na+ Arterial Blood Gases pH 7.35-7.45 Pa.CO2 (PCO2) 33-44 mm Hg Pa.O2 (PO2) 75-105 mm Hg HCO3 22-28 mEq/L O2 saturation 96-100% + Anion gap (no K ) 8-16 mEq/L + with K 12-20 mEq/L Bicarbonate gap -6-+6 mEq/L Blood Hemoglobin, male 13-16 g/dL female 12-15 g/dL Hematocrit, male 42-50% female 40-48% O2 binding capacity 1.34 mL O2/g Hb Creatinine clearance Osmolality Specific gravity Protein Urine 90-140 mL/min 50-1400 mOsm/kg water 1.003-1.030 <150 mg/24 h Questions • How should important content and concepts should be assessed? • What is the structure of a clinical or scientific scenario? • What are examples of effective stems and options/distractors? • When should images be used in place of descriptions? • How should laboratory studies be reported?