hllj/quesmed huggingface dataset:
A 17-year-old boy is admitted to the haematology ward following an acute episode of pain in his abdomen. He has no other history of trauma or previous surgeries. However, he is known to have a chronic medical condition, for which he is prescribed hydroxycarbamide and penicillin.

A blood film shows the presence of erythrocytes with small DNA remnants.

Which of the following is the most likely cause of his blood film appearance?

Options:
explanation: No, this is not the correct answer. This patient has had no history of previous trauma or surgeries. This patient's drug history and presentation make a diagnosis of sickle cell disease the most likely.
name: Splenectomy
label: b
answer: no
explanation: No this is not the correct answer. This patient's drug history and presentation make a diagnosis of sickle cell disease the most likely. By contrast, a patient with Hashimoto's disease would be prescribed an anti-thyroid medication such as levothyroxine.
name: Hashimoto's disease
label: e
answer: no
explanation: No, this is not the correct answer. This patient's drug history and presentation make a diagnosis of sickle cell disease the most likely.
name: Systemic lupus erythematosus
label: d
answer: no
explanation: This presence of DNA remnants in erythrocytes in the blood film are called Howell-Jolly Bodies. This finding suggests hyposplenism. There are many causes of hyposplenism and as this patient is prescribed hydroxycarbamide and penicillin (both of which are treatments for sickle cell disease) the patient is most likely to have sickle disease.

The presence of sickle cells in the bloodstream can result in infarcts and fibrosis of the spleen resulting in a lack of functioning splenic tissue. This impairs the ability of the spleen to sequestrate defective red blood cells, with DNA remnants resulting in Howell-Jolly bodies.
name: Sickle Cell Disease
label: a
answer: yes
explanation: No, this is not the correct answer. This patient's drug history and presentation make a diagnosis of sickle cell disease the most likely.
name: Coeliac disease
label: c
answer: no

Correct answer: a
A 70-year-old man presents to the GP with shortness of breath, fatigue, and lethargy. He also reports having several recent episodes of fainting and chest pain.


A full blood count and blood film is taken.


||||
|--------------|:-------:|---------------|
|Haemoglobin|83 g/L|(M) 130 - 170, (F) 115 - 155|
|Mean Cell Volume (MCV)|93 fL|80 - 96|
|Platelets|312x10<sup>9</sup>/L|150 - 400|


The blood film is also shown below [lightgallery]


He smokes and drinks alcohol regularly, but is otherwise fit and well with no known medical conditions or regular medications.


Which of the following is the likely cause of the patient's blood film results?

Options:
explanation: No, this is not the correct answer. If this patient had disseminated intravascular coagulation, they would have a reduced platelet count, and would be systemically more unwell.
name: Microangiopathic haemolytic anaemic secondary to disseminated intravascular coagulation
label: b
answer: no
explanation: No, this is not the correct answer. Alpha thalassaemia is likely to cause microcytic anaemia, and this patient's MCV is normal.
name: Alpha thalassaemia
label: e
answer: no
explanation: No, this is not the correct answer. Schistocytes can formed due to osmotic damage, however this does not match the patient's symptoms or history.
name: Osmotic damage
label: d
answer: no
explanation: No, this is not the correct answer. Although sickle cell disease results in red cell fragments known as Howell-Jolly bodies, this is generally detected in childhood and presents with episodes of acute pain.
name: Sickle cell anaemia
label: c
answer: no
explanation: Yes, this is the correct answer. The blood film shows the presence of schistocytes. These are fragments of red blood cells that form due to mechanical damage to the red blood cells. This can be caused by aortic valve stenosis. As the patient has experienced episodes of syncope and angina (due to reduced perfusion of the aorta and coronary arteries), this is the most likely explanation. Schistocytes can be formed as the red blood cells pass through the stenosed aortic valve.
name: Microangiopathic haemolytic anaemia secondary to aortic valve stenosis
label: a
answer: yes

Correct answer: a
A 40-year-old man presents to the GP with severe jaundice and an enlarged abdomen. On examination he tests positive for a 'hepatic flap'.


A set of liver function tests (LFTs) are requested:


||||
|--------------|:-------:|---------------|
|Bilirubin|97 µmol/L|< 17|
|Alanine Aminotransferase (ALT)|678 IU/L|10 - 50|
|Alkaline Phosphatase (ALP)|89 IU/L|25 - 115|

On questioning, the patient reports that he drinks around 40 units of alcohol a week for the past 15 years.


In view of their concerns about the patient's alcohol consumption, the GP discusses the possibility of reducing his alcohol dependence by prescribing **disulfiram**.


Which of the following best describes the mechanism of action of disulfiram?

Options:
explanation: No, this is not the correct mechanism of action. Disulfiram works by inhibiting aldehyde dehydrogenase leading to the build-up of acetaldehyde, leading to unpleasant physical symptoms associated with alcohol consumption. By contrast, acamprosate is another medication used to treat alcohol dependence that works by stabilising NMDA and GABA receptors, that are otherwise disrupted during periods of acute alcohol withdrawal.
name: NMDA receptor antagonist
label: d
answer: no
explanation: No, this is not the correct mechanism of action. Disulfiram works by inhibiting aldehyde dehydrogenase leading to the build up of acetaldehyde, leading to unpleasant physical symptoms associated with alcohol consumption. By contrast, Acamprosate is another medication used to treat alcohol dependence that works by stabilising NMDA and GABA receptors, that are otherwise disrupted during periods of acute alcohol withdrawal.
name: GABA receptor agonist
label: e
answer: no
explanation: Yes, this is the correct mechanism of action. The acetaldehyde build-up causes the patient to feel a number of unpleasant side effects, similar to a hangover, including nausea and a headache. This acts as negative reinforcement, to make the prospect of consuming alcohol less appealing so the patient is better able to abstain.
name: Inhibition of aldehyde dehydrogenase leading to the build up of acetaldehyde
label: a
answer: yes
explanation: No, this is not the correct mechanism of action. Disulfiram works by inhibiting aldehyde dehydrogenase leading to the build up of acetaldehyde, leading to unpleasant physical symptoms associated with alcohol consumption.
name: Inhibition of aldehyde dehydrogenase leading to the build up of acetate
label: c
answer: no
explanation: No, this is not the correct mechanism of action. Disulfiram leads to a build up of acetaldehyde, leading to unpleasant physical side effects and making the experience of consuming alcohol less desirable.
name: Inhibition of alcohol dehydrogenase leading to reduced levels of acetaldehyde
label: b
answer: no

Correct answer: a
A 10-week-old male baby is brought to the GP by his mother, who expresses concerns around excessive vomiting and poor weight gain. On examination, the baby appears slightly jaundiced and there is an absent red light reflex indicative of cataracts.

The patient is diagnosed with an inherited condition known as galactosaemia which is characterised by an increase in plasma galactose and galactose-1-phosphate levels.

Which of the following enzymes is this infant most likely to be deficient in?

Options:
explanation: Yes, this is the correct answer. The GALT enzyme is most likely to be deficient in galactosaemia. There is an increase in the levels of galactose AND galactose-1-P, which directly indicates a transferase deficiency.
name: Galactose-1-phosphate uridylyl transferase (GALT)
label: a
answer: yes
explanation: No, this is not the correct answer. This would result in a very rare form of galactosaemia and would only result in the elevation of galactose within the bloodstream. It would not affect the galactose-1-P levels within the blood.
name: Galactokinase
label: b
answer: no
explanation: No, this is not the correct answer. This enzyme is responsible for the conversion of UDP-galactose to UDP-glucose in the galactose metabolic pathway. It does not cause galactosaemia.
name: UDP-galactose 4' epimerase
label: e
answer: no
explanation: No, this is not the correct enzyme. This enzyme is a key regulator in glycolysis. It does not cause galactosaemia.
name: Phosphofructokinase
label: d
answer: no
explanation: No, this is not the correct answer. This enzyme is responsible for the conversion of galactose to galactitol resulting in reduced NADPH levels and subsequent cataract formation. It does not cause galactosaemia.
name: Aldose reductase
label: c
answer: no

Correct answer: a
A genetic test is performed for a 3 day old neonate due to recent episodes of seizures and skin hypopigmentation. A diagnosis of phenylketonuria (PKU) is made.

The paediatrician explains PKU is associated with the deficiency of an enzyme known as phenylalanine hydroxylase, in turn leading to deficiency of an endogenous amino acid responsible for the synthesis of catecholamines and various other compounds.

Which of the following amino acids is the neonate most likely to be deficient in?

Options:
explanation: No, this is not the correct answer. Patient's with homocystinurias are deficient in cysteine. Make sure you do not confuse this with phenlyketonuria.
name: Cysteine
label: e
answer: no
explanation: No, this is not the correct answer.
name: Threonine
label: d
answer: no
explanation: No, this is not the correct answer. Phenylalanine is in excess within the bloodstream with patient with PKU.
name: Phenylalanine
label: b
answer: no
explanation: Yes, this is the correct answer. The enzyme deficiency in PKU prevents the conversion of phenylalanine to tyrosine. This leads to the excess of phenylalanine within the bloodstream and a deficiency of tyrosine. The excess of phenylalanine leads to the excess of phenylacetate and phenylacetate (phenylketones) within the urine. Tyrosine is vital in the synthetic pathway of adrenaline, noradrenaline, dopamine, melanin, and thyroid hormone synthesis (T3 & T3), hence it is relatively simple to predict the patient's signs and symptoms if they are deficient in tyrosine.
name: Tyrosine
label: a
answer: yes
explanation: No, this is not the correct answer.
name: Tryptophan
label: c
answer: no

Correct answer: a
A 67-year-old man visits the GP for a check-up after a recent hospital admission for an ST-segment elevation myocardial infarction (STEMI). On examination, there is evidence of xanthelasma, tendon xanthoma, and a corneal arcus. A set of investigations are requested: **Total serum cholesterol**: 12 mmol/L (3.5-6.5 mmol/L) **Serum Triacylglycerol**: 2.9 mmol/L (0.7-2.0 mmol/L) **Serum lipid profile**: Increased amount of LDL The patient was commenced on atorvastatin during his hospital admission, but has not continued taking it as he does not understand its purpose.

Which answer option best describes the mechanism of action of atorvastatin?

Options:
explanation: No, this is not the correct mechanism of action. Statins such as atorvastatin inhibit the HMG-CoA reductase enzyme, thus reducing serum cholesterol levels.
name: Sequesters bile acids within the gastrointestinal tract
label: e
answer: no
explanation: No, this is not the correct mechanism of action. Statins such as atorvastatin inhibit the HMG-CoA reductase enzyme, thus reducing serum cholesterol levels.
name: Increased activation of lipoprotein lipase
label: d
answer: no
explanation: Yes, this is the correct mechanism of action. Statins such as atorvastatin inhibit the HMG-CoA reductase enzyme, which usually drives cholesterol synthesis in the liver. Blocking this enzyme, therefore, helps to reduce serum cholesterol levels, and is an important part of the secondary prevention management following a myocardial infarction.
name: Inhibition of HMG-CoA reductase
label: a
answer: yes
explanation: No, this is not the correct mechanism of action. Statins such as atorvastatin inhibit the HMG-CoA reductase enzyme, thus reducing serum cholesterol levels.
name: Allosteric activation of HMG-CoA reductase
label: b
answer: no
explanation: No, this is not the correct mechanism of action. Statins such as atorvastatin inhibit the HMG-CoA reductase enzyme, thus reducing serum cholesterol levels.
name: Inhibition of lyase enzyme
label: c
answer: no

Correct answer: a
A 16-year-old boy present to A&E after taking more than 30 paracetamol tablets.

The A&E consultant explains that paracetamol overdose results in the build-up of a toxic metabolite leading to oxidative stress within hepatocytes. Fortunately, a treatment exists that can prevent such liver damage.

Which of the following toxic metabolites is seen in a paracetamol overdose, and which drug is used to prevent it causing hepatic damage?

Options:
explanation: Yes, this is the correct answer. When paracetamol is in excess within the bloodstream, it exceeds the conjugating ability of the liver. Hence this leads to the build-up of the toxic metabolite known as NAPQI. NAPQI leads to further oxidative damage of cells due to the depletion of glutathione levels. N-acetylcysteine is used for the treatment of paracetamol overdose by replenishing glutathione levels, which prevents further oxidative damage.
name: N-acetyl-p-benzo-quinone imine (NAPQI) and N-acetylcysteine
label: a
answer: yes
explanation: No, this is not the correct answer. Although N-acetylcysteine is used in the management of paracetamol overdose, acetaminophen is an alternative name for paracetamol.
name: Acetaminophen and N-acetylcysteine
label: b
answer: no
explanation: No, this is not the correct answer. Paracetamol overdose leads to an excess of toxic by-product N-acetyl-p-benzo-quinone imine (NAPQI), and is treated with N-acetylcysteine.
name: Acetaminophen and glutathione
label: d
answer: no
explanation: No, this is not the correct answer. Paracetamol overdose leads to an excess of toxic by-product N-acetyl-p-benzo-quinone imine (NAPQI), and is treated with N-acetylcysteine.
name: Paracetamol conjugated with glucuronide and N-acetylcysteine
label: e
answer: no
explanation: No, this is not the correct answer. Although NAPQI correctly refers to the toxic by-product of paracetamol overdose. Such overdoses are treated with N-acetylcysteine.
name: N-acetyl-p-benzo-quinone imine (NAPQI) and glutathione
label: c
answer: no

Correct answer: a
A 60-year-old man presents to the GP with symptoms of heartburn and a mild cough due to gastro-oesophageal reflux disease (GORD). He has a BMI of 30.

Acid reflux can cause adaptive changes in the cells lining the oesophagus from one differentiated cell type to another. This cellular change, or metaplasia, is known as Barrett's oesophagus.

Which of the following best describes the metaplastic change that cells undergo in Barrett's oesophagus?

Options:
explanation: No, this is not the correct answer. In Barrett's oesophagus, the stratified squamous epithelial cells are converted to simple columnar epithelium.
name: Stratified squamous epithelium to transitional epithelium
label: e
answer: no
explanation: Yes, this is the correct answer. This is an example of metaplasia, where the cells of the oesophagus (stratified squamous epithelium) have adapted due to repeated exposure to stomach acid and have been converted to gastric epithelium (simple columnar epithelium).
name: Stratified squamous epithelium to simple columnar epithelium
label: a
answer: yes
explanation: No, this is not the correct answer. In Barrett's oesophagus, the stratified squamous epithelial cells are converted to simple columnar epithelium.
name: Simple squamous epithelium to simple cuboidal epithelium
label: d
answer: no
explanation: No, this is not the correct answer. In Barrett's oesophagus, the stratified squamous epithelial cells are converted to simple columnar epithelium.
name: Simple squamous epithelium to simple columnar epithelium
label: c
answer: no
explanation: No, this is not the correct answer. In Barrett's oesophagus, the stratified squamous epithelial cells are converted to simple columnar epithelium.
name: Stratified squamous epithelium to simple cuboidal epithelium
label: b
answer: no

Correct answer: a
A 50-year-old man presents to A&E with severe right leg pain. On examination, his right leg is visibly swollen in comparison to the left. His observations are all within normal range.

The patient's D-dimer is found to be elevated. He undergoes an ultrasound doppler, which identifies a right sided deep vein thrombosis (DVT).

The following day, he is noted to have a new unilateral facial droop and some slurred speech. An urgent CT head reveals an ischaemic stroke.

Which of the following is the most likely explanation for the patient's deterioration?

Options:
explanation: Embolisation to the lungs is a recognised complication of a DVT. This results in a pulmonary embolism, causing symptoms such as shortness of breath and pleuritic chest pain. However, this would not account for the patient's facial droop or slurred speech.
name: Embolism of the DVT to the lungs
label: e
answer: no
explanation: This is a possible cause of a haemorrhagic, rather than ischaemic stroke. Although similar symptoms may result, the CT findings rule this out as an underlying diagnosis.
name: A ruptured berry aneurysm in the brain
label: d
answer: no
explanation: No, this is not the correct answer. A DVT would not be expected to have this effect on venous return.
name: Reduced perfusion of the brain due to reduced venous return to the heart
label: c
answer: no
explanation: Yes, this is the most likely answer. The DVT has embolised to reach the right side of the heart. This emboli has then passed through a structural defect in this patient's atrial septum known as a patent foramen ovale (PFO). Essentially this emboli has bypassed the lungs to reach the systemic circulation and has occluded a vessel within the cerebral circulation, resulting in an ischaemic stroke. This process is known as a paradoxical emboli.

The PFO is an embryological defect whereby the foramen ovale of the atrial septum fails to fuse to become the fossa ovalis. They can be asymptomatic, and therefore pass undetected, in adults. It can lead to a paradoxical emboli following actions such as coughing or lifting heavy objects.
name: Embolism of the DVT which passed through a patent foramen ovale
label: a
answer: yes
explanation: No, this is not the correct answer. It is extremely uncommon for an arteriovenous anastomosis to exist that allows direct passage of a DVT to the brain.
name: Embolism of the DVT which bypassed the lungs via an arteriovenous anastomoses
label: b
answer: no

Correct answer: a
A 43-year-old man visits the GP with a persistent cough, that occasionally produces bloody sputum. He also has troubling night sweats and has found his clothes are now too big for him. A chest x-ray reveals bilateral upper lobe pulmonary infiltrates.

He is referred for a lung biopsy.

The histological image of a section of this patient's lung is shown below [lightgallery]

Which of the following types of giant cell is seen in this histological image?

Options:
explanation: Yes, this is the correct answer. From this patient's signs and symptoms, he is likely to have either lung cancer or tuberculosis. However, the x-ray findings are more suggestive of tuberculosis. Langhans giant cells, which surround caseating granulomas, are common histological findings in patients with tuberculosis.
name: Langhans giant cell
label: a
answer: yes
explanation: No, this is not the correct answer.
name: Caseating giant cell
label: e
answer: no
explanation: No, this is not the correct answer.
name: Foreign body giant cell
label: c
answer: no
explanation: No, this is not the correct answer. Although easily confused, Langerhans giant cells are immune cells of the skin. They would not be seen in lung histology.
name: Langerhans giant cell
label: b
answer: no
explanation: No, this is not the correct answer. This giant cell is associate with fat necrosis.
name: Touton giant cell
label: d
answer: no

Correct answer: a
A 36-month-old boy is attends the GP surgery with his mother, who is concerned that his skin bruises very easily. Her father also has this problem and was diagnosed with a haematological condition as a child, but she cannot remember what it is called.


The child is otherwise fit and well, with no known medical conditions and no regular medications.


Investigation results are as follows:


||||
|---------------------------|:-------:|--------------------|
|Platelets|197x10<sup>9</sup>/L|150 - 400|
|Activated Partial Thromboplastin Time (APTT)|59  seconds|22 - 41|
|International Normalised Ratio (INR)|1.0|1.0|
|Bleeding Time|5 minutes|< 10|

Which of the followings is the most likely underlying diagnosis?

Options:
explanation: Yes, this is the correct answer. Haemophilia A is the deficiency of factor VIII in the clotting cascade. This condition is associated with a prolonged aPTT, while other coagulation tests are within the normal range. Symptoms include easy bruising and bleeding.
name: Haemophilia A
label: a
answer: yes
explanation: No, this is not the correct answer. Factor V Leiden conveys thrombophilia, where there is an increased predisposition to thrombosis, rather than easy bruising.
name: Factor V Leiden
label: e
answer: no
explanation: No, this is not the correct answer. Haemolytic uraemic syndrome is associated with a low platelet count. Individuals also present with renal failure.
name: Haemolytic uraemic syndrome
label: d
answer: no
explanation: No, this is not the correct answer. Antiphospholipid syndrome conveys thrombophilia, where there is an increased predisposition to thrombosis, rather than easy bruising.
name: Antiphospholipid syndrome
label: b
answer: no
explanation: No, this is not the correct answer. Von Willebrand's disease is associated with a prolonged aPTT and a prolonged bleeding time.
name: von Willebrand's disease
label: c
answer: no

Correct answer: a
A 56-year-old man with colorectal cancer undergoes surgery for resection of his tumour. The tumour has invaded through the bowel wall, and that there is some lymph node involvement.

Which of the following stages of the Duke's staging system best describes this tumour?

Options:
explanation: Yes, this is the correct answer. Duke's staging system is used to classify bowel cancers, which can be helpful in making treatment decisions and prognosticating. In Duke's stage C bowel cancer, the disease has invaded local lymph nodes but not metastasized.
name: Stage C
label: a
answer: yes
explanation: No, this is not the correct answer. A Duke's stage A is given when the tumour is confined to the colonic mucosa.
name: Stage A
label: b
answer: no
explanation: No, this is not the correct answer. A Duke's stage D is given when there is evidence of distant metastases. It is the most advanced form of disease.
name: Stage D
label: d
answer: no
explanation: No, this is not the correct answer. A Duke's stage B is given when the tumour has invaded through the bowel wall.
name: Stage B
label: c
answer: no
explanation: No, this is not the correct answer. This stage does not exist in the Duke's staging system, which classifies cancers from A to D.
name: Stage E
label: e
answer: no

Correct answer: a