A previously healthy 10-year-old boy is brought to the emergency department for the evaluation of one episode of vomiting and severe headache since this morning. His mother says he also had difficulty getting dressed on his own. He has not had any trauma. The patient appears nervous. His temperature is 37°C (98.6°F), pulse is 100/min, and blood pressure is 185/125 mm Hg. He is confused and oriented only to person. Ophthalmic examination shows bilateral optic disc swelling. There is an abdominal bruit that is best heard at the right costovertebral angle. A complete blood count is within normal limits. Which of the following is most likely to confirm the diagnosis?
Q852
A 26-year-old male professional soccer player is brought to the emergency department due to an episode of syncope during a game. He has felt increasing shortness of breath during the past 3 months. During the past week, he has been feeling chest pain upon exertion. He also tells the doctor that his brother had a sudden death a couple of years ago. His heart rate is 98/min, respiratory rate is 18/min, temperature is 36.5°C (97.7°F), and blood pressure is 110/72 mm Hg. On physical examination, there is a harsh crescendo-decrescendo systolic murmur immediately after S1; it is best heard at the left lower sternal border. There is also a holosystolic murmur at the apex that radiates to the axilla. When the Valsalva maneuver is performed, the murmur becomes louder. An ECG and an echocardiogram are performed, which confirm the diagnosis. What is the most likely cause of this patient's condition?
Q853
A 39-year-old woman comes to the physician because of progressive pain and swelling of her wrists and hands for the past 2 months. Her hands are stiff in the morning; the stiffness decreases as she starts her chores. She also reports early-morning neck pain at rest for the past 3 weeks. She has no history of serious illness and takes no medications. Her sister has systemic lupus erythematosus. Vital signs are within normal limits. Examination shows bilateral swelling and tenderness of the wrists, second, third, and fourth metacarpophalangeal joints; range of motion is limited by pain. There is no vertebral tenderness. Cardiopulmonary examination shows no abnormalities. Neurologic examination shows no focal findings. Laboratory studies show:
Hemoglobin 12.8 g/dL
Leukocyte count 9,800/mm3
Erythrocyte sedimentation rate 44 mm/h
Serum
Glucose 77 mg/dL
Creatinine 1.1 mg/dL
Total bilirubin 0.7 mg/dL
Alkaline phosphatase 33 U/L
AST 14 U/L
ALT 13 U/L
Rheumatoid factor positive
Which of the following is the most appropriate next step in management?
Q854
A 62-year-old man returns to his physician for a follow-up examination. During his last visit 1 month ago splenomegaly was detected. He has had night sweats for the past several months and has lost 5 kg (11 lb) unintentionally during this period. He has no history of a serious illness and takes no medications. The vital signs are within normal limits. The physical examination shows no abnormalities other than splenomegaly. The laboratory studies show the following:
Hemoglobin 9 g/dL
Mean corpuscular volume 95 μm3
Leukocyte count 12,000/mm3
Platelet count 260,000/mm3
Ultrasound shows a spleen size of 15 cm (5.9 in) and mild hepatomegaly. The peripheral blood smear shows teardrop-shaped and nucleated red blood cells (RBCs) and immature myeloid cells. The marrow is very difficult to aspirate but reveals hyperplasia of all 3 lineages. The tartrate-resistant acid phosphatase (TRAP) test is negative. Clonal marrow plasma cells are not seen. JAK-2 is positive. The cytogenetic analysis is negative for translocation between chromosomes 9 and 22. Which of the following is the most appropriate curative management in this patient?
Q855
A 24-year-old previously healthy man comes to his physician because of dyspnea and hemoptysis for the past week. Examination shows inspiratory crackles at both lung bases. The remainder of the examination shows no abnormalities. His hemoglobin concentration is 14.2 g/dL, leukocyte count is 10,300/mm3, and platelet count is 205,000/mm3. Urine dipstick shows 2+ proteins. Urinalysis shows 80 RBC/hpf and 1–2 WBC/hpf. An x-ray of the chest shows pulmonary infiltrates. Further evaluation is most likely to show increased serum titers of which of the following?
Q856
A 42-year-old man presents to the clinic for a second evaluation of worsening blackened ulcers on the tips of his toes. His past medical history includes diabetes mellitus for which he takes metformin and his most recent HbA1c was 6.0, done 3 months ago. He also has hypertension for which he's prescribed amlodipine and chronic obstructive pulmonary disease (COPD) for which he uses an albuterol-ipratropium combination inhaler. He is also a chronic tobacco user with a 27-pack-year smoking history. He first noticed symptoms of a deep aching pain in his toes. Several months ago, he occasionally felt pain in his fingertips both at rest and with activity. Now he reports blackened skin at the tips of his fingers and toes. Evaluation shows: pulse of 82/min, blood pressure of 138/85 mm Hg, oral temperature 37.0°C (98.6°F). He is thin. Physical examination of his feet demonstrates the presence of 3, 0.5–0.8 cm, eschars over the tips of his bilateral second toes and right third toe. There is no surrounding erythema or exudate. Proprioception, vibratory sense, and monofilament examination are normal on both ventral aspects of his feet, but he lacks sensation over the eschars. Dorsal pedal pulses are diminished in both feet; the skin is shiny and hairless. Initial lab results include a C-reactive protein (CRP) level of 3.5 mg/dL, leukocytes of 6,000/mm3, erythrocyte sedimentation rate (ESR) of 34 mm/hr, and negative antinuclear antibodies. Which part of the patient's history is most directly associated with his current problem?
Q857
A 17-year-old girl is brought to the physician for a physical examination prior to participating in sports. She has no history of serious illness. She is on the school's cheerleading team and is preparing for an upcoming competition. Menarche was at 13 years of age, and her last menstrual period was 4 months ago. She is 167 cm (5 ft 6 in) tall and weighs 45 kg (99 lb); BMI is 16.1 kg/m2. Examination shows pale skin with thin, soft body hair. The patient is at increased risk for which of the following complications?
Q858
A 59-year-old man is brought to the emergency department by his wife for a 1-hour history of sudden behavior changes. They were having lunch together when, at 1:07 PM, he suddenly dropped his sandwich on the floor. Since then, he has been unable to use his right arm. She also reports that he is slurring his speech and dragging his right foot when he walks. Nothing like this has ever happened before. The vital signs include: pulse 95/min, blood pressure 160/90 mm Hg, and respiratory rate 14/min. The physical exam is notable for an irregularly irregular rhythm on cardiac auscultation. On neurological exam, he has a facial droop on the right half of his face but is able to elevate his eyebrows symmetrically. He has 0/5 strength in his right arm, 2/5 strength in his right leg, and reports numbness throughout the right side of his body. Angiography of the brain will most likely show a lesion in which of the following vessels?
Q859
A 58-year-old woman presents to the clinic with an abnormal sensation on the left side of her body that has been present for the past several months. At first, the area seemed numb and she recalls touching a hot stove and accidentally burning herself but not feeling the heat. Now she is suffering from a constant, uncomfortable burning pain on her left side for the past week. The pain gets worse when someone even lightly touches that side. She has recently immigrated and her past medical records are unavailable. Last month she had a stroke but she cannot recall any details from the event. She confirms a history of hypertension, type II diabetes mellitus, and bilateral knee pain. She also had cardiac surgery 20 years ago. She denies fever, mood changes, weight changes, and trauma to the head, neck, or limbs. Her blood pressure is 162/90 mm Hg, the heart rate is 82/min, and the respiratory rate is 15/min. Multiple old burn marks are visible on the left hand and forearm. Muscle strength is mildly reduced in the left upper and lower limbs. Hyperesthesia is noted in the left upper and lower limbs. Laboratory results are significant for:
Hemoglobin 13.9 g/dL
MCV 92 fL
White blood cells 7,500/mm3
Platelets 278,000/mm3
Creatinine 1.3 U/L
BUN 38 mg/dL
TSH 2.5 uU/L
Hemoglobin A1c 7.9%
Vitamin B12 526 ng/L
What is the most likely diagnosis?
Q860
A 40-year-old man comes to the physician because of fatigue, increased sweating, and itching in his legs for the past 2 years. He has chronic bronchitis. He has smoked two packs of cigarettes daily for 24 years and drinks one to two beers every night. His only medication is a tiotropium bromide inhaler. His vital signs are within normal limits. He is 175 cm (5 ft 9 in) tall and weighs 116 kg (256 lb); BMI is 38 kg/m2. Physical examination shows facial flushing and bluish discoloration of the lips. Scattered expiratory wheezing and rhonchi are heard throughout both lung fields. Abdominal examination shows no abnormalities. Laboratory studies show:
Erythrocyte count 6.9 million/mm3
Hemoglobin 20 g/dL
Mean corpuscular volume 91 μm3
Leukocyte count 13,000/mm3
Platelet count 540,000/mm3
Serum
Ferritin 8 ng/mL
Iron 48 μg/dL
Iron binding capacity 402 μg/dL (N: 251 - 406 μg/dL)
Which of the following is the most appropriate next step in treatment?
Cardiology US Medical PG Practice Questions and MCQs
Question 851: A previously healthy 10-year-old boy is brought to the emergency department for the evaluation of one episode of vomiting and severe headache since this morning. His mother says he also had difficulty getting dressed on his own. He has not had any trauma. The patient appears nervous. His temperature is 37°C (98.6°F), pulse is 100/min, and blood pressure is 185/125 mm Hg. He is confused and oriented only to person. Ophthalmic examination shows bilateral optic disc swelling. There is an abdominal bruit that is best heard at the right costovertebral angle. A complete blood count is within normal limits. Which of the following is most likely to confirm the diagnosis?
A. Serum IGF-I level
B. Oral sodium loading test
C. Echocardiography
D. CT angiography (Correct Answer)
E. High-dose dexamethasone suppression test
Explanation: ***CT angiography***
- The patient presents with **malignant hypertension** (BP 185/125 mmHg, confusion, optic disc swelling) and an **abdominal bruit** especially at the **right costovertebral angle**, pointing strongly towards **renovascular hypertension** due to **renal artery stenosis**.
- **CT angiography** is the most appropriate imaging modality to confirm **renal artery stenosis** by visualizing the renal arteries and identifying any narrowing.
*Serum IGF-I level*
- This test is used to screen for **growth hormone disorders** like **acromegaly** or **gigantism**, which are not indicated by the patient's symptoms.
- The patient's presentation is focused on acute severe hypertension and neurological changes, rather than chronic growth disturbances.
*Oral sodium loading test*
- This test is used to confirm the diagnosis of **primary aldosteronism**, where **aldosterone levels** fail to suppress after a sodium load.
- While primary aldosteronism can cause hypertension, it typically doesn't present with an **abdominal bruit** or the acute, severe neurological symptoms seen here.
*Echocardiography*
- **Echocardiography** assesses the heart's structure and function, which could show signs of **hypertensive heart disease** (e.g., left ventricular hypertrophy) due to long-standing uncontrolled hypertension.
- However, it does not identify the underlying cause of the hypertension in this acute setting, especially when an **abdominal bruit** suggests a vascular origin.
*High-dose dexamethasone suppression test*
- This test is used to differentiate between **Cushing's disease** (pituitary ACTH-dependent) and other causes of **Cushing's syndrome** (e.g., ectopic ACTH production, adrenal tumor) due to excess cortisol.
- The patient's symptoms are inconsistent with Cushing's syndrome, and the **abdominal bruit** points away from this diagnosis.
Question 852: A 26-year-old male professional soccer player is brought to the emergency department due to an episode of syncope during a game. He has felt increasing shortness of breath during the past 3 months. During the past week, he has been feeling chest pain upon exertion. He also tells the doctor that his brother had a sudden death a couple of years ago. His heart rate is 98/min, respiratory rate is 18/min, temperature is 36.5°C (97.7°F), and blood pressure is 110/72 mm Hg. On physical examination, there is a harsh crescendo-decrescendo systolic murmur immediately after S1; it is best heard at the left lower sternal border. There is also a holosystolic murmur at the apex that radiates to the axilla. When the Valsalva maneuver is performed, the murmur becomes louder. An ECG and an echocardiogram are performed, which confirm the diagnosis. What is the most likely cause of this patient's condition?
A. First-degree heart block
B. Aortic stenosis
C. Hypertrophic cardiomyopathy (Correct Answer)
D. Third-degree heart block
E. Cardiac tamponade
Explanation: ***Hypertrophic cardiomyopathy***
- The patient's history of **syncope**, exertional **chest pain**, and **shortness of breath** in a young athlete, combined with a family history of sudden death, are classic signs of hypertrophic cardiomyopathy (HCM).
- The physical exam findings of a **harsh crescendo-decrescendo systolic murmur at the left lower sternal border** represent left ventricular outflow tract (LVOT) obstruction, the hallmark of obstructive HCM.
- The **holosystolic murmur at the apex radiating to the axilla** represents mitral regurgitation due to systolic anterior motion (SAM) of the mitral valve, a common finding in HCM.
- Both murmurs **increase with Valsalva maneuver** (due to decreased preload, exacerbating LVOT obstruction and worsening MR), which is highly characteristic of HCM.
*First-degree heart block*
- **First-degree heart block** is characterized by a prolonged PR interval on ECG and typically does not cause syncope, chest pain, or a murmur that changes with Valsalva.
- It is generally asymptomatic and not associated with sudden cardiac death in the same manner as HCM.
*Aortic stenosis*
- While **aortic stenosis** also presents with a harsh systolic murmur and can cause syncope, it typically **decreases with Valsalva maneuver** (decreased preload reduces stroke volume and murmur intensity).
- The murmur of aortic stenosis is typically heard best at the **right upper sternal border** and radiates to the carotid arteries, not the left lower sternal border.
- Aortic stenosis would not cause the additional holosystolic murmur of mitral regurgitation seen in this patient.
*Third-degree heart block*
- **Third-degree heart block** (complete heart block) involves a complete dissociation between atrial and ventricular activity, leading to severe bradycardia and symptoms like syncope.
- However, it does not typically produce the specific type of systolic murmurs described, nor does it fit the familial sudden death profile in the context of a young athlete's exertional symptoms.
*Cardiac tamponade*
- **Cardiac tamponade** is a medical emergency caused by fluid accumulation around the heart, leading to impaired ventricular filling.
- Its classical signs include **hypotension, jugular venous distension, and muffled heart sounds (Beck's triad)**, none of which are described in this patient.
Question 853: A 39-year-old woman comes to the physician because of progressive pain and swelling of her wrists and hands for the past 2 months. Her hands are stiff in the morning; the stiffness decreases as she starts her chores. She also reports early-morning neck pain at rest for the past 3 weeks. She has no history of serious illness and takes no medications. Her sister has systemic lupus erythematosus. Vital signs are within normal limits. Examination shows bilateral swelling and tenderness of the wrists, second, third, and fourth metacarpophalangeal joints; range of motion is limited by pain. There is no vertebral tenderness. Cardiopulmonary examination shows no abnormalities. Neurologic examination shows no focal findings. Laboratory studies show:
Hemoglobin 12.8 g/dL
Leukocyte count 9,800/mm3
Erythrocyte sedimentation rate 44 mm/h
Serum
Glucose 77 mg/dL
Creatinine 1.1 mg/dL
Total bilirubin 0.7 mg/dL
Alkaline phosphatase 33 U/L
AST 14 U/L
ALT 13 U/L
Rheumatoid factor positive
Which of the following is the most appropriate next step in management?
A. Adalimumab
B. CT scan of the chest
C. Measurement of anti-CCP antibodies (Correct Answer)
D. X-ray of the cervical spine
E. Measurement of anti-Smith antibodies
Explanation: ***Measurement of anti-CCP antibodies***
- This patient presents with **classic rheumatoid arthritis (RA)**: symmetric polyarthritis affecting wrists and MCP joints, morning stiffness improving with activity, elevated ESR, and positive rheumatoid factor.
- **Anti-CCP (anti-cyclic citrullinated peptide) antibodies** are highly specific for RA (~95% specificity) and should be measured to **confirm the diagnosis**.
- Anti-CCP positivity helps with **prognostication** (associated with more aggressive disease and erosive changes) and guides early initiation of disease-modifying antirheumatic drugs (DMARDs) like methotrexate.
- Early diagnosis and treatment of RA is crucial to prevent irreversible joint damage and disability.
*Measurement of anti-Smith antibodies*
- **Anti-Smith antibodies** are highly specific for **systemic lupus erythematosus (SLE)**, not RA.
- This patient has **no clinical features of SLE** (no malar rash, photosensitivity, oral ulcers, serositis, renal involvement, or cytopenias).
- Family history of SLE alone does not warrant testing when the clinical presentation clearly points to RA.
- The neck pain is consistent with early RA involvement of cervical spine, not SLE.
*Adalimumab*
- **Adalimumab** is a TNF-alpha inhibitor used for RA, but it is **not first-line therapy**.
- The diagnosis must first be confirmed, and conventional DMARDs (especially **methotrexate**) should be tried first.
- Biologics like adalimumab are reserved for patients who fail conventional DMARDs or have severe disease at presentation.
*CT scan of the chest*
- There are **no respiratory symptoms** or abnormal cardiopulmonary findings on examination.
- While RA can cause interstitial lung disease or pleural effusions, screening imaging is not indicated without clinical signs or symptoms.
- The priority is confirming the diagnosis of RA.
*X-ray of the cervical spine*
- While the patient has neck pain, the **neurological examination is normal** and there is no vertebral tenderness.
- Cervical spine involvement in RA (atlantoaxial subluxation) typically occurs in longstanding disease, not at presentation.
- Imaging would be indicated if there were neurological deficits, severe pain, or mechanical symptoms suggesting instability.
Question 854: A 62-year-old man returns to his physician for a follow-up examination. During his last visit 1 month ago splenomegaly was detected. He has had night sweats for the past several months and has lost 5 kg (11 lb) unintentionally during this period. He has no history of a serious illness and takes no medications. The vital signs are within normal limits. The physical examination shows no abnormalities other than splenomegaly. The laboratory studies show the following:
Hemoglobin 9 g/dL
Mean corpuscular volume 95 μm3
Leukocyte count 12,000/mm3
Platelet count 260,000/mm3
Ultrasound shows a spleen size of 15 cm (5.9 in) and mild hepatomegaly. The peripheral blood smear shows teardrop-shaped and nucleated red blood cells (RBCs) and immature myeloid cells. The marrow is very difficult to aspirate but reveals hyperplasia of all 3 lineages. The tartrate-resistant acid phosphatase (TRAP) test is negative. Clonal marrow plasma cells are not seen. JAK-2 is positive. The cytogenetic analysis is negative for translocation between chromosomes 9 and 22. Which of the following is the most appropriate curative management in this patient?
A. Adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD)
B. Allogeneic bone marrow transplantation (Correct Answer)
C. Splenectomy
D. Splenic irradiation
E. Imatinib mesylate
Explanation: ***Allogeneic bone marrow transplantation***
- The patient's presentation with **splenomegaly**, night sweats, weight loss, anemia, **teardrop cells**, **nucleated RBCs**, **immature myeloid cells** in peripheral blood, **dry tap** on marrow aspiration, and **JAK2 positivity** is highly suggestive of **primary myelofibrosis**.
- For younger patients with intermediate to high-risk primary myelofibrosis, allogeneic stem cell transplantation is the only potentially curative treatment.
*Adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD)*
- This is a chemotherapy regimen primarily used for the treatment of **Hodgkin lymphoma**.
- It is not indicated for the management of primary myelofibrosis.
*Splenectomy*
- Splenectomy can be considered for patients with **severe, symptomatic splenomegaly** or refractory anemia/thrombocytopenia in myelofibrosis.
- However, it is a **palliative measure** to alleviate symptoms, not a curative treatment for the underlying bone marrow disorder.
*Splenic irradiation*
- Splenic irradiation is used to reduce spleen size and relieve symptoms like pain or early satiety in patients with **myelofibrosis** who are not candidates for splenectomy.
- It provides **symptomatic relief** but does not alter the disease course or offer a cure.
*Imatinib mesylate*
- Imatinib is a **tyrosine kinase inhibitor** primarily used to treat **chronic myelogenous leukemia (CML)**, specifically targeting the **BCR-ABL1 fusion gene**.
- The patient's cytogenetic analysis was negative for translocation between chromosomes 9 and 22, ruling out CML, and Imatinib is not effective for JAK2-positive myelofibrosis.
Question 855: A 24-year-old previously healthy man comes to his physician because of dyspnea and hemoptysis for the past week. Examination shows inspiratory crackles at both lung bases. The remainder of the examination shows no abnormalities. His hemoglobin concentration is 14.2 g/dL, leukocyte count is 10,300/mm3, and platelet count is 205,000/mm3. Urine dipstick shows 2+ proteins. Urinalysis shows 80 RBC/hpf and 1–2 WBC/hpf. An x-ray of the chest shows pulmonary infiltrates. Further evaluation is most likely to show increased serum titers of which of the following?
A. Anti-PLA2R antibody
B. P-ANCA
C. Anti-GBM antibody (Correct Answer)
D. Immunoglobulin A
E. Anti-dsDNA antibody
Explanation: ***Anti-GBM antibody***
- The patient's presentation with **dyspnea**, **hemoptysis**, **pulmonary infiltrates**, **proteinuria**, and **hematuria** is highly suggestive of **Goodpasture syndrome**, a rapidly progressive glomerulonephritis with pulmonary hemorrhage.
- Goodpasture syndrome is characterized by the presence of autoantibodies directed against the **collagen type IV** of the glomerular and alveolar basement membranes, specifically the **α3 chain of type IV collagen**.
*Anti-PLA2R antibody*
- **Anti-PLA2R antibodies** are associated with **membranous nephropathy**, a cause of nephrotic syndrome, which is not indicated by the patient's primary symptoms of dyspnea and hemoptysis, nor by the urinalysis showing significant hematuria.
- While membranous nephropathy can cause proteinuria, it typically does not present with **pulmonary-renal syndrome** or significant hematuria (red blood cell casts are more indicative of glomerulonephritis).
*P-ANCA*
- **P-ANCA** (perinuclear anti-neutrophil cytoplasmic antibodies) are typically associated with **microscopic polyangiitis** or **eosinophilic granulomatosis with polyangiitis (Churg-Strauss)**, which can cause pulmonary-renal syndrome but usually present with systemic vasculitis symptoms like skin lesions or nerve involvement.
- While microscopic polyangiitis can cause pulmonary hemorrhage and glomerulonephritis, the classic presentation of Goodpasture syndrome with its specific autoantibody profile is a more direct fit given the acute and severe pulmonary and renal symptoms.
*Immunoglobulin A*
- Increased serum **IgA** levels are characteristic of **IgA nephropathy (Berger's disease)** or **Henoch-Schönlein purpura**, which primarily cause glomerulonephritis.
- These conditions do not typically cause severe **pulmonary hemorrhage** or the acute pulmonary-renal syndrome seen in this patient.
*Anti-dsDNA antibody*
- **Anti-dsDNA antibodies** are a hallmark of **systemic lupus erythematosus (SLE)**, which can affect multiple organ systems, including the kidneys (lupus nephritis) and lungs.
- However, the acute and severe **pulmonary-renal syndrome** with hemoptysis and pulmonary infiltrates, without other systemic lupus symptoms, makes Goodpasture syndrome a more specific diagnosis in this context.
Question 856: A 42-year-old man presents to the clinic for a second evaluation of worsening blackened ulcers on the tips of his toes. His past medical history includes diabetes mellitus for which he takes metformin and his most recent HbA1c was 6.0, done 3 months ago. He also has hypertension for which he's prescribed amlodipine and chronic obstructive pulmonary disease (COPD) for which he uses an albuterol-ipratropium combination inhaler. He is also a chronic tobacco user with a 27-pack-year smoking history. He first noticed symptoms of a deep aching pain in his toes. Several months ago, he occasionally felt pain in his fingertips both at rest and with activity. Now he reports blackened skin at the tips of his fingers and toes. Evaluation shows: pulse of 82/min, blood pressure of 138/85 mm Hg, oral temperature 37.0°C (98.6°F). He is thin. Physical examination of his feet demonstrates the presence of 3, 0.5–0.8 cm, eschars over the tips of his bilateral second toes and right third toe. There is no surrounding erythema or exudate. Proprioception, vibratory sense, and monofilament examination are normal on both ventral aspects of his feet, but he lacks sensation over the eschars. Dorsal pedal pulses are diminished in both feet; the skin is shiny and hairless. Initial lab results include a C-reactive protein (CRP) level of 3.5 mg/dL, leukocytes of 6,000/mm3, erythrocyte sedimentation rate (ESR) of 34 mm/hr, and negative antinuclear antibodies. Which part of the patient's history is most directly associated with his current problem?
A. Diabetes mellitus
B. Hypertension
C. Autoimmune disorder
D. Chronic obstructive pulmonary disease
E. Tobacco smoking (Correct Answer)
Explanation: ***Tobacco smoking***
- The patient exhibits symptoms consistent with **Buerger's disease (thromboangiitis obliterans)**, which is strongly associated with **heavy tobacco use**. The blackened ulcers on the fingertips and toes ("blackened skin" and "eschars") are indicative of **ischemia** due to **vasculitis** of small and medium-sized arteries.
- The "deep aching pain" in his toes and fingertips preceding the ulcers, along with diminished peripheral pulses and shiny, hairless skin, further supports a diagnosis of severe peripheral vascular disease primarily driven by smoking.
*Diabetes mellitus*
- While diabetes can cause **peripheral neuropathy** and **vascular disease**, this patient's **HbA1c of 6.0** indicates good glycemic control, making it less likely to be the primary cause of his extensive, severe ischemic ulcers.
- The normal proprioception, vibratory sense, and monofilament examination (except over the eschars) suggest that **diabetic neuropathy** is not the direct cause of the current ischemic problem.
*Hypertension*
- Hypertension is a risk factor for **atherosclerosis** and cardiovascular disease, but it typically affects larger arteries and does not directly explain the specific pattern of **vasculitis** and **digital ischemia** seen in this patient (blackened fingertips and toes).
- The patient's blood pressure is controlled with medication, and while it contributes to overall vascular risk, it's not as directly associated with the presented condition as tobacco use is.
*Autoimmune disorder*
- The negative antinuclear antibodies (ANA), near-normal ESR (34 mm/hr) and CRP (3.5 mg/dL) make a systemic **autoimmune disorder**, such as systemic lupus erythematosus or scleroderma, less likely to be the primary cause of his ulcers.
- While some autoimmune conditions can cause vasculitis, the clinical picture, particularly the strong association with tobacco use and the distribution of lesions, points away from a primary autoimmune etiology.
*Chronic obstructive pulmonary disease*
- COPD is a **pulmonary condition** primarily affecting the lungs and is strongly associated with smoking, but it does not directly cause **peripheral ischemic ulcers** on the fingers and toes.
- While COPD indicates the patient's long-standing smoking habit, it is the smoking itself, not the COPD directly, that causes the vascular pathology leading to the blackened ulcers.
Question 857: A 17-year-old girl is brought to the physician for a physical examination prior to participating in sports. She has no history of serious illness. She is on the school's cheerleading team and is preparing for an upcoming competition. Menarche was at 13 years of age, and her last menstrual period was 4 months ago. She is 167 cm (5 ft 6 in) tall and weighs 45 kg (99 lb); BMI is 16.1 kg/m2. Examination shows pale skin with thin, soft body hair. The patient is at increased risk for which of the following complications?
A. Shortened QT interval
B. Hyperkalemia
C. Fractures (Correct Answer)
D. Hyperthyroidism
E. Hyperphosphatemia
Explanation: ***Fractures***
- This patient presents with signs and symptoms highly suggestive of **anorexia nervosa**, including a **low BMI (16.1 kg/m2)**, **amenorrhea (last menstrual period 4 months ago)**, and physical findings like **pale skin** and **thin, soft body hair**.
- **Estrogen deficiency** due to amenorrhea in anorexia nervosa leads to accelerated **bone loss** and **osteoporosis**, significantly increasing the risk of **pathological fractures**.
*Shortened QT interval*
- Anorexia nervosa is typically associated with **electrolyte imbalances** like **hypokalemia**, **hypomagnesemia**, and **hypophosphatemia**, which can lead to a **prolonged QT interval**, not a shortened one.
- A shortened QT interval is rare and usually associated with **hypercalcemia** or genetic disorders.
*Hyperkalemia*
- Patients with anorexia nervosa often experience **hypokalemia** due to gastrointestinal losses (e.g., vomiting, laxative abuse) or diuretic use, which is commonly associated with eating disorders.
- **Hyperkalemia** is less common unless there is kidney dysfunction, refeeding syndrome with rapid fluid shifts, or certain medications.
*Hyperthyroidism*
- Anorexia nervosa is more often associated with **euthyroid sick syndrome** or **hypothyroidism**, characterized by **low T3 levels** and normal or slightly low T4 and TSH.
- **Hyperthyroidism** is an accelerated metabolic state, which is contrary to the reduced metabolic rate seen in severe malnutrition.
*Hyperphosphatemia*
- **Refeeding syndrome**, a potential complication of refeeding in severely malnourished patients like this one, is characterized by **hypophosphatemia** (not hyperphosphatemia), hypokalemia, and hypomagnesemia.
- **Hypophosphatemia** occurs as glucose refeeding stimulates insulin release, leading to increased cellular uptake of phosphate.
Question 858: A 59-year-old man is brought to the emergency department by his wife for a 1-hour history of sudden behavior changes. They were having lunch together when, at 1:07 PM, he suddenly dropped his sandwich on the floor. Since then, he has been unable to use his right arm. She also reports that he is slurring his speech and dragging his right foot when he walks. Nothing like this has ever happened before. The vital signs include: pulse 95/min, blood pressure 160/90 mm Hg, and respiratory rate 14/min. The physical exam is notable for an irregularly irregular rhythm on cardiac auscultation. On neurological exam, he has a facial droop on the right half of his face but is able to elevate his eyebrows symmetrically. He has 0/5 strength in his right arm, 2/5 strength in his right leg, and reports numbness throughout the right side of his body. Angiography of the brain will most likely show a lesion in which of the following vessels?
A. Posterior cerebral artery
B. Internal carotid artery
C. Middle cerebral artery (Correct Answer)
D. Basilar artery
E. Anterior cerebral artery
Explanation: ***Middle cerebral artery***
- The patient's symptoms, including **right-sided weakness with arm > leg involvement** (0/5 arm, 2/5 leg), **facial droop** (lower face sparing the forehead), and **slurred speech (dysarthria/aphasia)**, are classic signs of an **MCA stroke**.
- The **arm > leg pattern** is the key distinguishing feature of MCA territory infarction, as the MCA supplies the **lateral motor cortex** (which controls arm and face).
- The finding of an **irregularly irregular rhythm** suggests **atrial fibrillation**, a common cause of **embolic stroke** to the MCA.
*Posterior cerebral artery*
- PCA strokes primarily affect the **occipital lobe** and **medial temporal lobe**, leading to **visual field defects** (e.g., homonymous hemianopsia) or memory deficits.
- While it can cause sensory loss, it typically does not present with the **prominent motor deficits** and **facial droop** seen in this patient.
*Internal carotid artery*
- ICA occlusion can cause symptoms similar to MCA stroke, especially if the **MCA is a direct branch of the ICA**, or it can cause both MCA and ACA symptoms simultaneously.
- However, the specific constellation of symptoms described (predominant motor and sensory deficits, speech issues) points more directly to the **MCA territory downstream**.
*Basilar artery*
- Basilar artery strokes affect the **brainstem** and often present with a combination of **cranial nerve palsies**, **ataxia**, bilateral weakness, **vertigo**, and sometimes **"locked-in" syndrome**.
- The patient's symptoms are more consistent with a **hemispheric lesion**, not a brainstem lesion.
*Anterior cerebral artery*
- ACA strokes typically cause **contralateral leg > arm weakness** (opposite pattern from MCA), as the ACA supplies the **medial motor cortex**.
- ACA strokes may also present with **behavioral changes** (e.g., abulia, apathy) due to involvement of the frontal lobe.
- The patient's prominent **right arm weakness** and **facial droop** are not characteristic of an ACA stroke.
Question 859: A 58-year-old woman presents to the clinic with an abnormal sensation on the left side of her body that has been present for the past several months. At first, the area seemed numb and she recalls touching a hot stove and accidentally burning herself but not feeling the heat. Now she is suffering from a constant, uncomfortable burning pain on her left side for the past week. The pain gets worse when someone even lightly touches that side. She has recently immigrated and her past medical records are unavailable. Last month she had a stroke but she cannot recall any details from the event. She confirms a history of hypertension, type II diabetes mellitus, and bilateral knee pain. She also had cardiac surgery 20 years ago. She denies fever, mood changes, weight changes, and trauma to the head, neck, or limbs. Her blood pressure is 162/90 mm Hg, the heart rate is 82/min, and the respiratory rate is 15/min. Multiple old burn marks are visible on the left hand and forearm. Muscle strength is mildly reduced in the left upper and lower limbs. Hyperesthesia is noted in the left upper and lower limbs. Laboratory results are significant for:
Hemoglobin 13.9 g/dL
MCV 92 fL
White blood cells 7,500/mm3
Platelets 278,000/mm3
Creatinine 1.3 U/L
BUN 38 mg/dL
TSH 2.5 uU/L
Hemoglobin A1c 7.9%
Vitamin B12 526 ng/L
What is the most likely diagnosis?
A. Conversion disorder
B. Complex regional pain syndrome
C. Dejerine-Roussy syndrome (Correct Answer)
D. Medial medullary syndrome
E. Subacute combined degeneration of spinal cord
Explanation: ***Dejerine-Roussy syndrome***
- The patient's symptoms of **contralateral hemianesthesia**, followed by **dysesthesia**, **spontaneous burning pain**, and **allodynia/hyperalgesia**, developing after a stroke a month prior, are classic for **Dejerine-Roussy syndrome** (thalamic pain syndrome). The history of unnoticed burns and hyperesthesia supports this.
- This syndrome is caused by a lesion in the **thalamus**, typically due to a stroke, which disrupts sensory pathways and leads to an abnormal processing of sensory information.
*Conversion disorder*
- Conversion disorder involves neurological symptoms that are **incompatible with recognized neurological or medical conditions** and are often associated with psychological stress.
- The patient's symptoms are clearly attributable to a known neurological etiology (a recent stroke) and specific anatomical location (thalamus), ruling out conversion disorder.
*Complex regional pain syndrome*
- **Complex regional pain syndrome (CRPS)** is characterized by severe pain, swelling, and autonomic dysfunction, typically affecting a **single limb distal to an injury**, but not necessarily associated with a stroke to the brain.
- While the patient has burning pain and hyperesthesia, the **hemibody distribution** and clear link to a prior stroke make CRPS less likely than a central lesion.
*Medial medullary syndrome*
- **Medial medullary syndrome** results from damage to the medial medulla and typically presents with **ipsilateral tongue weakness**, **contralateral hemiparesis**, and **contralateral loss of vibratory and proprioceptive sensation**, but not the prominent burning pain and allodynia described.
- The patient's primary sensory complaints of burning pain and hyperesthesia across an entire hemibody are not characteristic of medial medullary syndrome.
*Subacute combined degeneration of spinal cord*
- **Subacute combined degeneration** is caused by **vitamin B12 deficiency** and affects the dorsal and lateral columns of the spinal cord, leading to **paresthesias**, weakness, gait ataxia, and impaired proprioception.
- The patient's **vitamin B12 level is normal**, and the symptoms are acute-onset and unilateral, clearly linked to a stroke, not a progressive, symmetrical myelopathy.
Question 860: A 40-year-old man comes to the physician because of fatigue, increased sweating, and itching in his legs for the past 2 years. He has chronic bronchitis. He has smoked two packs of cigarettes daily for 24 years and drinks one to two beers every night. His only medication is a tiotropium bromide inhaler. His vital signs are within normal limits. He is 175 cm (5 ft 9 in) tall and weighs 116 kg (256 lb); BMI is 38 kg/m2. Physical examination shows facial flushing and bluish discoloration of the lips. Scattered expiratory wheezing and rhonchi are heard throughout both lung fields. Abdominal examination shows no abnormalities. Laboratory studies show:
Erythrocyte count 6.9 million/mm3
Hemoglobin 20 g/dL
Mean corpuscular volume 91 μm3
Leukocyte count 13,000/mm3
Platelet count 540,000/mm3
Serum
Ferritin 8 ng/mL
Iron 48 μg/dL
Iron binding capacity 402 μg/dL (N: 251 - 406 μg/dL)
Which of the following is the most appropriate next step in treatment?
A. Weight loss
B. Inhaled budesonide
C. Hydroxyurea
D. Allogeneic stem cell transplantation
E. Phlebotomy (Correct Answer)
Explanation: ***Phlebotomy***
- This patient has **secondary erythrocytosis** due to **chronic hypoxemia** from COPD (chronic bronchitis, 48 pack-year smoking history, cyanosis, wheezing).
- Severe erythrocytosis (Hb 20 g/dL, Hct likely >60%) causes **hyperviscosity syndrome** manifesting as fatigue, headaches, and pruritus.
- **Phlebotomy** is indicated when Hct >55% in secondary polycythemia to reduce thrombotic risk and improve symptoms related to hyperviscosity.
- The goal is to reduce hematocrit to <55% while addressing the underlying hypoxemia with oxygen therapy and COPD optimization.
- Iron deficiency (ferritin 8 ng/mL) may be present from prior phlebotomy or dietary causes; iron should NOT be supplemented as it would worsen erythrocytosis.
*Weight loss*
- While obesity (BMI 38) contributes to hypoventilation and worsens hypoxemia, weight loss is a long-term intervention that does not address the **acute hyperviscosity** and thrombotic risk.
- Weight loss would be an appropriate adjunctive measure but not the most immediate next step.
*Inhaled budesonide*
- Inhaled corticosteroids are used for COPD patients with frequent exacerbations or asthma-COPD overlap.
- This patient is already on appropriate bronchodilator therapy (tiotropium); adding corticosteroids does not address the primary issue of **severe erythrocytosis and hyperviscosity**.
- Optimizing COPD therapy and oxygen supplementation would be more appropriate than adding inhaled steroids at this point.
*Hydroxyurea*
- Hydroxyurea is a cytoreductive agent used primarily in **polycythemia vera**, not secondary polycythemia.
- This patient's erythrocytosis is driven by **hypoxia** (physiologic EPO response), not autonomous JAK2-driven proliferation.
- Treating the underlying hypoxemia and using phlebotomy is more appropriate than cytoreductive therapy.
*Allogeneic stem cell transplantation*
- Stem cell transplantation is reserved for advanced myeloproliferative neoplasms or myelofibrosis with poor prognosis.
- This is **secondary erythrocytosis**, not a malignant hematologic disorder, and does not warrant such aggressive treatment.
- This option is completely inappropriate for this clinical scenario.