hllj/quesmed huggingface dataset:
A 33-year-old attends her GP after she noticed a white ring on the outer edge of her cornea. When questioned, she tells the GP her father died aged 42 from a "heart problem". The GP suspects familial hypercholesterolaemia and refers her to the lipid clinic. The GP starts the patient on some medication while waiting for the appointment.

What is the mechanism of action of the first-line cholesterol-lowering medication?

Options:
explanation: This describes the mechanism of action of ezetimibe, another cholesterol-lowering drug. Ezetimibe works by blocking the NPC1L1 protein, stopping the absorption of cholesterol across the gut wall. The first line cholesterol-lowering drug is a statin. This drug class works by inhibiting the HMG-CoA reductase enzyme. This decreases the synthesis of cholesterol causing an upregulation in LDL receptor expression which moves more LDL into cells and thus lowers the level of circulating LDL.
name: Inhibits absorption of cholesterol across the gut wall
label: b
answer: no
explanation: This describes the mechanism of action of fibrates, another group of cholesterol-lowering drugs. Activation of the PPARα transcription factor by fibrates increases the transcription of genes for lipoprotein lipase, apo-A1 and apo-A5. The first line cholesterol-lowering drug is a statin. This drug class works by inhibiting the HMG-CoA reductase enzyme. This decreases the synthesis of cholesterol causing an upregulation in LDL receptor expression which moves more LDL into cells and thus lowers the level of circulating LDL.
name: Activates nuclear transcription factor PPARα
label: c
answer: no
explanation: This describes the mechanism of action of PCSK-9 inhibitors, such as alirocumab. These are monoclonal antibodies against the PCSK-9 protein on the LDL receptor. Normally PCSK-9 breaks down the LDL receptor; inhibiting PCSK-9 allows LDL receptors to be reused by the cell, thus increasing the uptake of LDL from the blood. The first line cholesterol-lowering drug is a statin. This drug class works by inhibiting the HMG-CoA reductase enzyme. This decreases the synthesis of cholesterol causing an upregulation in LDL receptor expression which moves more LDL into cells and thus lowers the level of circulating LDL.
name: Inhibits PCSK-9 protein
label: d
answer: no
explanation: The first-line cholesterol-lowering drug is a statin. This drug class works by inhibiting the HMG-CoA reductase enzyme. This decreases the synthesis of cholesterol causing an upregulation in LDL receptor expression which moves more LDL into cells and thus lowers the level of circulating LDL.
name: Inhibits HMG-CoA reductase
label: a
answer: yes
explanation: This describes the mechanism of action of bempedoic acid, another cholesterol-lowering medication. The first line cholesterol-lowering drug is a statin. This drug class works by inhibiting the HMG-CoA reductase enzyme. This decreases the synthesis of cholesterol causing an upregulation in LDL receptor expression which moves more LDL into cells and thus lowers the level of circulating LDL.
name: Inhibits cholesterol synthesis in the liver
label: e
answer: no

Correct answer: a
A 34-year-old female attends her GP complaining of being tired all the time. On further questioning, she also admits to experiencing light-headedness and exertional dyspnoea. She also states she has been having heavy periods for the last year.

The GP orders further tests, including a blood film.

What is the blood film most likely to show?

Options:
explanation: The patient has iron-deficiency anaemia due to chronic blood loss from her heavy periods. This causes a microcytic hypochromic picture on the blood film. This means the red blood cells are smaller and paler than normal due to lower levels of haemoglobin.
name: Microcytic hyperchromic anaemia
label: e
answer: no
explanation: Macrocytic hyperchromic anaemia is seen in vitamin B12 and folate deficiency. This patient has iron-deficiency anaemia due to chronic blood loss from her heavy periods. This causes a microcytic hypochromic picture on the blood film. This means the red blood cells are smaller and paler than normal due to lower levels of haemoglobin.
name: Macrocytic hyperchromic anaemia
label: b
answer: no
explanation: This patient has iron-deficiency anaemia due to chronic blood loss from her heavy periods. This causes a microcytic hypochromic picture on the blood film. This means the red blood cells are smaller and paler than normal due to lower levels of haemoglobin.
name: Microcytic hypochromic anaemia
label: a
answer: yes
explanation: Normocytic normochromic anaemia is seen in haemorrhage or acutely in haemolytic anaemias. This patient has iron-deficiency anaemia due to chronic blood loss from her heavy periods. This causes a microcytic hypochromic picture on the blood film. This means the red blood cells are smaller and paler than normal due to lower levels of haemoglobin.
name: Normocytic normochromic anaemia
label: c
answer: no
explanation: Macrocytic normochromic blood films are seen in conditions such as aplastic anaemia. This patient has iron-deficiency anaemia due to chronic blood loss from her heavy periods. This causes a microcytic hypochromic picture on the blood film. This means the red blood cells are smaller and paler than normal due to lower levels of haemoglobin.
name: Macrocytic normochromic anaemia
label: d
answer: no

Correct answer: a
A 6-year-old male presents to the paediatric haematology team with his mother.

On examination, the child has a yellow colouring to his skin. The mother states that this has happened before, and the child is becoming increasingly lethargic and irritable.

The haematologist orders further tests.

Which of the following is the most likely test result for this patient?

Options:
explanation: This history is suggestive of a haemolytic anaemia. we would expect to see corresponding blood film changes such as the presence of schistocytes (red blood cell fragments), spherocytes (in hereditary spherocytosis), bite cells and Heinz bodies (in G6PD deficiency).
name: Normal blood film
label: e
answer: no
explanation: This history is suggestive of a haemolytic anaemia. In haemolytic anaemias the haptoglobin is low. Haptoglobin is a plasma protein that binds to free haemoglobin and then is removed from the body. It is low in haemolytic anaemias as red blood cell breakdown results in the release of lots of free haemoglobin which haptoglobin can bind to.
name: Low haptoglobin
label: a
answer: yes
explanation: This history is suggestive of a haemolytic anaemia. Reticulocytes are immature red blood cells and would be increased in haemolytic anaemias as a compensatory mechanism for the increased red blood cell breakdown.
name: Reduced reticulocytes
label: d
answer: no
explanation: This history is suggestive of a haemolytic anaemia. Bilirubin is a breakdown product of haem and would be raised in a haemolytic anaemia, not lowered. It is the increased level of bilirubin that is responsible for the yellow skin pigmentation in this patient (jaundice).
name: Low bilirubin
label: c
answer: no
explanation: This history is suggestive of a haemolytic anaemia. Lactate dehydrogenase (LDH) would be expected to be raised in a haemolytic anaemia. LDH is present in red cells and haemolysis causes its release into the plasma.
name: Low LDH
label: b
answer: no

Correct answer: a
A 32-year-old man receives a blood transfusion after experiencing bleeding following a renal biopsy. 4 hours later he develops rapid onset dyspnoea and tachypnoea. His blood pressure is 95/65 and his temperature is 37.8°C.

An ABG is carried out and shows a normal LDH.

Which of the following is the most likely cause of his symptoms?

Options:
explanation: Acute haemolytic reactions occur within 24 hours of transfusion due to ABO incompatibility and normally present with a triad of symptoms: fever, flank pain, and red or brown urine. Further investigations would show a raised bilirubin and LDH, and an acute fall in haemoglobin.
name: Acute haemolytic reaction
label: e
answer: no
explanation: Whilst renal biopsies can be carried out to investigate the cause of nephrotic syndrome, the patient's pattern of symptoms is not in keeping with nephrotic syndrome. Nephrotic syndrome is associated with hypoalbuminaemia (and fluid overload) and massive proteinuria.
name: Nephrotic syndrome
label: c
answer: no
explanation: This pattern of symptoms following blood transfusion are more in keeping with a diagnosis of transfusion-associated acute lung injury.
name: Heart failure
label: b
answer: no
explanation: TRALI causes acute pulmonary oedema within 6 hours of transfusion. It is caused by antibodies in the donor blood reacting with the patient’s white blood cells (neutrophils and monocytes) or pulmonary endothelium, causing inflammatory cells to be sequestered in the lungs. This results in the leakage of plasma into the alveolar spaces and acute respiratory distress.
name: Transfusion-associated acute lung injury (TRALI)
label: a
answer: yes
explanation: TACO can also present within 6 hours of transfusion, but it is associated with hypertension, not hypotension, and is generally in older patients. It also does not present with a high temperature.
name: Transfusion-associated circulatory overload (TACO)
label: d
answer: no

Correct answer: a
A 27-year-old female is brought to the Emergency Department after being involved in a road traffic collision. On arrival, she is noted to be hypotensive and has lost a lot of blood. The major haemorrhage protocol is activated. No blood has been crossmatched.

Which of the following groups of blood is safe to give to this patient?

Options:
explanation: As the patient is a female of childbearing age, she should receive O- blood to ensure she will not form anti-D antibodies against Rhesus factor which could cause problems in future pregnancies.
name: A-
label: e
answer: no
explanation: As the patient is a female of childbearing age, she should receive O- blood to ensure she will not form anti-D antibodies against Rhesus factor which could cause problems in future pregnancies.
name: AB-
label: c
answer: no
explanation: As the patient is a female of childbearing age, she should receive O- blood to ensure she will not form anti-D antibodies against Rhesus factor which could cause problems in future pregnancies.
name: O+
label: d
answer: no
explanation: The patient's blood group is unknown and blood is urgently required so O- blood should be given. As the patient is a female of childbearing age, she shouldn't be given O+ blood as she will form anti-D antibodies against Rhesus factor and this has the potential to cause problems in future pregnancies. AB+ is the blood group that is a universal recipient.
name: AB+
label: b
answer: no
explanation: O- blood has no ABO and RhD antigens on the surface of the red blood cells, so is safe for all patients to receive.
name: O-
label: a
answer: yes

Correct answer: a
A 72-year-old man is on a surgical ward recovering from a Hartmann's procedure 5 days ago. His pain post-op has been poorly controlled. He now complains of a swollen and tender left leg. On examination, it is erythematous and a 5cm difference in circumference is noted from the right leg.

He receives an ultrasound scan which confirms the diagnosis of a deep vein thrombosis (DVT).

Which of the following factors is most likely to have contributed to the development of this condition?

Options:
explanation: Enoxaparin is an anticoagulant and is given to patients to reduce the risk of them developing DVTs in hospital. This means enoxaparin treatment cannot be a contributing factor to this patient developing a DVT. Considering the history given, immobility following surgery is the most likely contributing factor to his DVT.
name: Enoxaparin treatment
label: c
answer: no
explanation: Whilst smoking can contribute to endothelial damage - a part of Virchow's triad for thrombus formation - the history given in this situation makes immobility following surgery the most likely contributing factor to him developing a DVT.
name: Smoking
label: e
answer: no
explanation: Whilst long-haul flights can result in the development of DVTs due to stasis of blood flow, there is nothing in this patient's history that would suggest he has been on any recent long-haul flights. Considering the history given, immobility following surgery is the most likely contributing factor to his DVT.
name: Recent long-haul flight
label: b
answer: no
explanation: Whilst atherosclerosis can contribute to endothelial damage - a part of Virchow's triad for thrombus formation - the history given in this situation makes immobility following surgery the most likely contributing factor to him developing a DVT.
name: Atherosclerosis
label: d
answer: no
explanation: This patient has presented with a DVT 5 days post-operation and is likely to have been moving less due to his poorly controlled pain, making immobility the most likely contributing factor to his DVT. Stasis of blood is part of Virchow's triad for thrombus formation.
name: Immobility
label: a
answer: yes

Correct answer: a
A 72-year-old man attends his GP for an annual review. He has a past medical history of severe COPD, diagnosed 25 years ago. The GP orders routine blood tests and they come back showing an erythrocytosis. The GP suspects this has occurred secondary to chronic hypoxia from his COPD.

Hypoxia stimulates erythropoietin production, which in turn stimulates the red bone marrow to increase erythropoiesis.

Where is erythropoietin produced?

Options:
explanation: Erythropoietin (EPO) is a hormone produced by the kidney in response to hypoxia. It stimulates the red bone marrow to increase erythropoiesis, increasing red blood cell production. The spleen functions to sequester and destroy worn-out red blood cells. The spleen also has an important role in immune function by detecting and responding to blood-borne antigens.
name: Spleen
label: c
answer: no
explanation: This is the correct answer. Erythropoietin (EPO) is a hormone produced by the kidney in response to hypoxia. It stimulates the red bone marrow to increase erythropoiesis, increasing red blood cell production.
name: Kidney
label: a
answer: yes
explanation: Erythropoietin (EPO) is a hormone produced by the kidney in response to hypoxia. It stimulates the red bone marrow to increase erythropoiesis, increasing red blood cell production. The red bone marrow also contains stem cells that can differentiate to form white blood cells and platelets.
name: Red bone marrow
label: b
answer: no
explanation: Erythropoietin (EPO) is a hormone produced by the kidney in response to hypoxia. It stimulates the red bone marrow to increase erythropoiesis, increasing red blood cell production. The liver has many functions including being a glucose and fat buffer, as well as protein and drug metabolism.
name: Liver
label: d
answer: no
explanation: Erythropoietin (EPO) is a hormone produced by the kidney in response to hypoxia. It stimulates the red bone marrow to increase erythropoiesis, increasing red blood cell production. The thyroid's primary function is to produce thyroid hormone in the form of T4 (mainly) and T3.
name: Thyroid
label: e
answer: no

Correct answer: a
A 32-year-old pregnant female attends the Emergency Department with shortness of breath, pleuritic chest pain and haemoptysis. She is also complaining of a swollen left leg that is warm to touch. Pitting oedema is present. A chest x-ray and ECG have been performed.

Considering the most likely diagnosis, what is the next best investigation to carry out?

Options:
explanation: D-dimer is a highly sensitive but non-specific marker of clotting. This test is useful for ruling out DVTs and PEs, but not confirming the diagnosis. According to the Wells' Score, this patient has a high risk of having a pulmonary embolism, and as such a d-dimer test is not recommended. Furthermore, d-dimer is elevated in pregnancy, and would not provide a meaningful result in this patient.
name: D-Dimer
label: b
answer: no
explanation: As this patient has signs and symptoms of a DVT as well as a PE, compression duplex ultrasound is the next best investigation. If the compression duplex ultrasound was negative or the woman did not have features of DVT, then a CT pulmonary angiogram or V/Q scan could be considered. A V/Q scan is a ventilation-perfusion scan, and it looks at the blood and air supply to the lungs. In pregnant women, V/Q scanning is associated with a mildly increased risk of childhood cancer, and a lower risk of maternal breast cancer compared to CT pulmonary angiogram. This is why it is not used first line for diagnosis of PE in pregnant women who also show features of DVT. However, the absolute risk for both these scans is very low.
name: V/Q scan
label: c
answer: no
explanation: As this patient has signs and symptoms of a DVT as well as a PE, compression duplex ultrasound is the next best investigation. If the compression duplex ultrasound was negative, then a CT pulmonary angiogram or VQ scan could be considered. A CT pulmonary angiogram looks at the blood vessels from the heart to the lungs and can identify any blockages. As CT scans use radiation, this poses risks to the foetus, however, the absolute risks are very small.
name: CT pulmonary angiogram
label: d
answer: no
explanation: As this patient has signs and symptoms of a DVT as well as a PE, compression duplex ultrasound is the next best investigation. Fibrinogen is a protein that is essential for clot formation. It is most often requested after a patient has prolonged or unexplained bleeding, not clots. However, it can also be used to help diagnose disseminated intravascular coagulation (DIC).
name: Fibrinogen levels
label: e
answer: no
explanation: In pregnant women with suspected pulmonary embolism (PE) who also have symptoms and signs of deep vein thrombosis (DVT), compression duplex ultrasound should be performed. This is due to the risks of radiation exposure to the foetus in CTPA and VQ scans. The management would also be the same for both DVT and PE.
name: Compression duplex ultrasound
label: a
answer: yes

Correct answer: a
A 63-year-old male presents to the Emergency Department with acute onset severe chest pain that radiates to the back. He describes it as a "tearing" pain. His blood pressure is 96/62 and his heart rate is 109. The chest x-ray shows a widened mediastinum. An urgent CT aortogram is ordered.

Considering the most likely diagnosis, which of the following is a risk factor for this condition?

Options:
explanation: Marfan's Syndrome is a connective tissue disorder that is associated with an increased risk of aortic dissections. It occurs as a result of a point mutation in the fibrillin gene, preventing the normal deposition of elastin in the extracellular matrix. Other significant risk factors for aortic dissection are hypertension and valvular heart disease.
name: Marfan's Syndrome
label: a
answer: yes
explanation: The most likely diagnosis in this patient is aortic dissection. The main risk factors for this condition are hypertension, connective tissue disease, e.g. Marfan's syndrome, and valvular heart disease. Some studies have suggested that diabetes is actually a protective factor against aortic dissection.
name: Diabetes
label: b
answer: no
explanation: The most likely diagnosis in this patient is aortic dissection. The main risk factors for this condition are hypertension (not hypotension), connective tissue disease, e.g. Marfan's syndrome, and valvular heart disease.
name: Hypotension
label: c
answer: no
explanation: Normally the aortic valve is tricuspid, with a bicuspid aortic valve being a congenital heart defect. The most likely diagnosis in this patient is aortic dissection. The main risk factors for this condition are hypertension (not hypotension), connective tissue disease, e.g. Marfan's syndrome, and valvular heart disease (such as a bicuspid aortic valve).
name: Tricuspid aortic valve
label: d
answer: no
explanation: The most likely diagnosis in this patient is aortic dissection. The main risk factors for this condition are hypertension (not hypotension), connective tissue disease, e.g. Marfan's syndrome, and valvular heart disease. Aortic dissections usually present in men over the age of 50.
name: Younger age
label: e
answer: no

Correct answer: a
A 19-year-old female is brought to the Emergency Department by ambulance after suffering a stab wound to the chest. She has lost a significant amount of blood and is tachycardic and dyspnoeic. The clinician treating her suspects cardiac tamponade and an echocardiogram confirms the diagnosis.

Which of the following signs is the clinician most likely to see in this patient?

Options:
explanation: Cardiac tamponade is associated with Beck's triad: hypotension, muffled heart sounds and a raised JVP. It occurs when there is an accumulation of fluid or blood in the pericardial cavity. This compresses the heart, preventing diastolic filling and thus reducing cardiac output. Causes of symmetrically reduced chest expansion include pulmonary fibrosis, whereas pneumothorax, pneumonia and pleural effusions all cause asymmetrical reduced chest expansion.
name: Reduced chest expansion
label: d
answer: no
explanation: Cardiac tamponade is associated with Beck's triad: hypotension, muffled heart sounds and a raised JVP. It occurs when there is an accumulation of fluid or blood in the pericardial cavity. This compresses the heart, preventing diastolic filling and thus reducing cardiac output.
name: Hypertension
label: b
answer: no
explanation: Cardiac tamponade is associated with Beck's triad: hypotension, muffled heart sounds and a raised JVP. It occurs when there is an accumulation of fluid or blood in the pericardial cavity. This compresses the heart, preventing diastolic filling and thus reducing cardiac output. No observable JVP is a normal examination finding.
name: No observable JVP
label: c
answer: no
explanation: This is the correct answer. Cardiac tamponade occurs when there is an accumulation of fluid or blood in the pericardial cavity. This compresses the heart, preventing diastolic filling and thus reducing cardiac output. Cardiac tamponade is associated with Beck's triad: hypotension, muffled heart sounds and a raised JVP.
name: Muffled heart sounds
label: a
answer: yes
explanation: Cardiac tamponade is associated with Beck's triad: hypotension, muffled heart sounds and a raised JVP. It occurs when there is an accumulation of fluid or blood in the pericardial cavity. This compresses the heart, preventing diastolic filling and thus reducing cardiac output. A deviated trachea can be seen in many conditions, most notably in tension pneumothoraces.
name: Deviated trachea
label: e
answer: no

Correct answer: a