hllj/quesmed huggingface dataset:
A 19-year-old female presents to A&E with a severe headache and stiff neck. She is wearing sunglasses, as she reports the light makes her feel worse. On examination a non-blanching rash on her right lateral forearm is seen.


A lumbar puncture is taken to extract her CSF (cerebral spinal fluid) which is then sent for further analysis:


||||
|---------------------------------|:-------:|-------------------------|
|Total Protein|3.6 g/L|0.15 - 0.45|
|Glucose|1.9 mmol/L|3.2 - 4.4|
|Cell Count|/250 μL|< 5|

Cell Type: Neutrophils


Which of the following causative microorganisms and gram stain is most likely responsible for this patient's infection?

Options:
explanation: Neisseria meningitidis is gram-negative, not positive.
name: Neisseria meningitidis (gram-positive diplococcus)
label: b
answer: no
explanation: The CSF results with an increased white cell count consisting predominantly of neutrophils are consistent with bacterial meningitis. Neisseria meningitidis is one of the most common bacterial causes of meningitis and the rates are highest for young children, adolescents, and young adults. Neisseria meningitis is a gram-negative bacteria appearing in pairs on a gram stain, hence it is called a diplococcus.
name: Neisseria meningitidis (gram-negative diplococcus)
label: a
answer: yes
explanation: The CSF results with an increased white cell count consisting of neutrophils are consistent with bacterial meningitis. In the UK, Neisseria meningitidis is a more common cause than Streptococcus pneumoniae.
name: Streptococcus pneumoniae (gram-positive diplococcus)
label: c
answer: no
explanation: The CSF results with an increased white cell count consisting of neutrophils are consistent with bacterial meningitis. In the UK, Neisseria meningitidis is a more common cause than Streptococcus pneumoniae.
name: Streptococcus pneumoniae (gram-positive in clusters)
label: d
answer: no
explanation: The CSF results with an increased white cell count consisting of neutrophils are consistent with bacterial meningitis. In the UK, Neisseria meningitidis is a more common cause than Haemophilus influenzae.
name: Haemophilus influenzae (gram negative bacilli)
label: e
answer: no

Correct answer: a
A 45-year-old man presents to his GP surgery with jaundice. He is noted to have abnormal liver function tests (LFTs)


||||
|---------------------------|:-------:|--------------------|
|Bilirubin|74 µmol/L|< 17|
|Alanine Aminotransferase (ALT)|2835 IU/L|10 - 50|
|Alkaline Phosphatase (ALP)|155 IU/L|25 - 115|


The LFT results suggest hepatic causes of jaundice. After further serology testing, a diagnosis of hepatitis B is made.


Which of the following positive result on serology would suggest a highly infective state for this patient?

Options:
explanation: No, this is the incorrect answer. HBeAb is the antibody against the hepatitis B e-antigen. Make sure that you do not mistake Ab (antibody) for Ag (antigen).
name: HBeAb
label: c
answer: no
explanation: No, this is the incorrect answer. Immunoglobulin M (IgM) is an antibody produced by the body as part of the response to infection. It does not indicate the patient is highly infectious with regard to their hepatitis B.
name: IgM (core antibody)
label: e
answer: no
explanation: Yes, this is the correct answer. HBeAg is the e-antigen of the hepatitis B virus. It is produced by the hepatitis B virus, and indicates active viral replication. Thus, raised levels in the bloodstream indicate a highly infectious state.
name: HBeAg
label: a
answer: yes
explanation: No, this is the incorrect answer. HBsAg, or the hepatitis B virus surface antigen, is the first antigen to be elevated in the bloodstream following an infection. It is responsible for the initial rises in ALT levels but does not indicate a highly infective state.
name: HBsAg
label: b
answer: no
explanation: No, this is the incorrect answer. This is the hepatitis B surface antibody. This is one of the last antibodies to appear serologically, which indicates the clearance of the hepatitis B virus and hence recovery.
name: HBsAb
label: d
answer: no

Correct answer: a
A 58-year-old man presents to the endocrinology clinic complaining of severe fatigue and acute weight gain. On examination, the patient has increased abdominal obesity with purple striae and a dorso-cervical fat pad. It is suspected that he has a condition associated with an excess of cortisol. Investigation results show the following: Cortisol: 1535 nmol/L (reference range = 150-700 nmol/L) ACTH: 120 ng/L (reference range = 10-80 ng/L) High dose dexamethasone suppression test: Cortisol Suppression Present 

Which of the following is the most likely underlying diagnosis?

Options:
explanation: No, this is not the correct answer. Cortisol is normally secreted from the zona fasiculata of the adrenal cortex, not from the pituitary gland. A pituitary adenoma would secrete ACTH, rather than cortisol.
name: Benign pituitary adenoma secreting cortisol
label: b
answer: no
explanation: The patient demonstrates symptoms of Cushing's syndrome. The investigation results are key in identifying the underlying diagnosis. A combination of raised ACTH and a normal (suppression present) dexamethasone suppression test points towards a pituitary pathology generating excess ACTH. This is also known as Cushing's disease.

The ACTH levels are increased due to increased ACTH secretion from the anterior pituitary gland, which leads to more cortisol secretion from the adrenal cortex. The high-dose dexamethasone suppression test shows cortisol suppression is present, which indicates that the increased ACTH levels are due to secretions from the pituitary gland rather than another ectopic source. This is because dexamethasone exerts negative feedback upon ACTH release from the pituitary gland, but not upon an ectopic source of ACTH.
name: Benign pituitary adenoma secreting ACTH
label: a
answer: yes
explanation: No, this is not the correct answer. In an adrenal adenoma, there are increased levels of cortisol secretion into the bloodstream from the adrenal cortex. The ACTH levels would be reduced due to the increased negative feedback that high levels of cortisol exert on the anterior pituitary gland to reduce the ACTH levels in the bloodstream.
name: Excess cortisol secreted by an adrenal adenoma
label: d
answer: no
explanation: No, this is not the correct answer. When performing a high dose dexamethasone suppression test, cortisol suppression would not be present in a paraneoplastic small cell lung cancer. This is because dexamethasone is a synthetic steroid which acts upon the anterior pituitary to reduce ACTH secretions and subsequent cortisol production through negative feedback mechanisms. By contrast, in ectopic secretion of ACTH from the cells of a lung tumour, there is no negative feedback system being initiated on the cells of the tumour, and so there would be no suppression by dexamethasone.
name: Paraneoplastic small cell lung cancer
label: c
answer: no
explanation: No, this is not the correct answer. This would lead to a reduced ACTH levels, due to increased negative feedback on the anterior pituitary gland.
name: Chronic exposure to prescribed corticosteroids
label: e
answer: no

Correct answer: a
A 54-year-old woman presents to the GP with lethargy and recent anorexia. She also reports that she has been feeling quite dizzy recently. On examination, she has patches of hyperpigmentation present on her palmar creases and gums.

After measuring her 9am cortisol and ACTH (adrenocorticotropic hormone) level, a diagnosis of addison's disease is suspected.

Which of the following describes the pathophysiology behind this patient's hyperpigmentation symptoms?

Options:
explanation: No, this is not the single best answer, as it is the MSH specifically resulting in the hyperpigmentation within the skin.
name: Decreased cortisol production so there is increased POMC (pro-opiomelanocortin) production
label: d
answer: no
explanation: This patient is exhibiting symptoms of Addison's disease. This is characterised by adrenal insufficiency resulting in reduced cortisol production, which in turn results in reduced negative feedback on the anterior hypothalamus so there are elevated levels of ACTH.
name: Reduced ACTH production
label: b
answer: no
explanation: This patient is exhibiting symptoms of Addison's disease. This is characterised by adrenal insufficiency resulting in reduced cortisol production, which in turn results in reduced negative feedback on the anterior hypothalamus so there are elevated levels of ACTH.
name: Reduced ACTH production so there is reduced POMC (pro-opiomelanocortin) and MSH (melanocyte-stimulating hormone) production
label: c
answer: no
explanation: No, this is not the single best answer, as there is increased POMC and MSH secretion that results from the increase in ACTH. It is the increased production of MSH which directly leads to hyperpigmentation of the skin.
name: Decreased cortisol production so there is increased ACTH production
label: e
answer: no
explanation: This patient is exhibiting symptoms of Addison's disease. This is characterised by adrenal insufficiency resulting in reduced cortisol production, which in turn results in reduced negative feedback on the anterior hypothalamus so there are elevated levels of ACTH. POMC (pro-opiomelanocortin) is required for the production of ACTH and MSH. So if there is increased ACTH production, there is an increased need to produce POMC. MSH is also a breakdown product of POMC, hence there will be increased MSH levels leading to increased stimulation of melanin by melanocytes within the epidermis.
name: Increased ACTH production causing increased POMC (pro-opiomelanocortin) and MSH (melanocyte-stimulating hormone) production
label: a
answer: yes

Correct answer: a
A 53-year-old woman presents to the endocrinology clinic with fatigue, weakness, excessive sweating, and heat intolerance. On examination, she has a neck lump which elevates when swallowing.


A series of thyroid function tests are as follows:

||||
|---------------------------|:-------:|------------------------------|
|Thyroid Stimulating Hormone|<0.05 mU/L|0.3 - 4.2|
|Total T4|4.5 nmol/L|60 - 150|
|Total T3|202 pmol/L|3.1 - 6.8|


The patient also tests positive for thyroid stimulating immunoglobulin antibodies (TSI), hence a diagnosis of Grave's disease is made. She is prescribed carbimazole to manage her symptoms.


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

Options:
explanation: Yes, this is the correct mechanism of action. Carbimazole prevents the coupling and iodination of tyrosines on thyroglobulin, thus reducing the production of thyroid hormones. This in turn relieves the symptoms or hyperthyroidism.
name: Prevents the coupling and iodination of tyrosine on thyroglobulin which is catalysed by the enzyme thyroid peroxidase
label: a
answer: yes
explanation: No, this is not the correct mechanism of action.
name: Prevents the exit of T3 and T4 hormones out of the thyroid follicular cells by blocking the T3/T4 export protein
label: d
answer: no
explanation: No, this is not the correct mechanism of action.
name: Preventing the entry of iodine into the thyroid follicular cells by blocking the basolateral Na+/I- symporter
label: c
answer: no
explanation: No, this is not the correct mechanism of action. The coupling and iodination of tyrosine on thyroglobulin does not occur through apoptosis, but rather is catalysed by the enzyme thyroid peroxidase.
name: Prevents the coupling and iodination of tyrosine on thyroglobulin through apoptosis
label: b
answer: no
explanation: No, this is not the correct mechanism of action.
name: Inhibition of endocytosis of thyroglobulin from the colloid into the thyroid follicular cells
label: e
answer: no

Correct answer: a
A 23-year-old man presents to the GP with recent paraesthesia bilaterally on his upper and lower limbs. He has also been experiencing involuntary contraction of his muscles. He has recently undergone a partial thyroidectomy, but has no other past medical or surgical history.


Neurological examination is otherwise entirely normal.


The GP requests some investigations shown below:


||||
|---------------------------|:-------:|------------------------------|
|Calcium|0.9 mmol/L|2.2 - 2.6|
|Alkaline Phosphatase (ALP)|64 IU/L|25 - 115|
|Parathyroid Hormone|0.3 pmol/L|1.6 - 8.5|

His full blood count and renal function are normal.


Which of the following is the most likely underlying diagnosis?

Options:
explanation: Yes, this is the correct answer. The patient has had a partial thyroidectomy, which essentially means partial removal of the thyroid gland. As the parathyroid glands are located on the posterior aspect of the thyroid gland, it is possible for them to be damaged iatrogenically during thyroid surgery. As the parathyroid glands secrete PTH into the bloodstream, the patient will have a reduced serum PTH concentration. PTH is responsible for the activation of osteoclasts to resorb bone and secrete calcium into the bloodstream. Hence if there is a deficiency of PTH, there will be a reduced calcium ion concentration and the patient will be experiencing symptoms of hypocalcaemia.
name: Hypoparathyroidism secondary to iatrogenic damage to the parathyroid glands during the partial thyroidectomy
label: a
answer: yes
explanation: No, this is not the correct answer. Whilst chronic kidney disease can cause hypocalcaemia due to a reduced vitamin D3 level, the PTH level would be expected to increase in compensation.  This would be known as secondary hyperparathyroidism due to hyperplasia of the parathyroid glands, in response to low serum calcium levels. By contrast this patient demonstrates low PTH levels. Their renal function is also normal, ruling out a diagnosis of CKD.
name: Chronic kidney disease resulting in the deficiency of active vitamin D3 production
label: e
answer: no
explanation: No, this is not the single best answer. This is possible in type 1 polyglandular syndrome, however given the patient's history of a previous partial thyroidectomy, iatrogenic to the parathyroid glands is far more likely.
name: Hypoparathyroidism secondary to autoimmune damage to the parathyroid glands resulting in atrophic changes
label: b
answer: no
explanation: No, this is not the correct answer. The patient has a serum PTH level below the normal range.
name: Primary hyperparathyroidism secondary to an adenoma of one or more of the parathyroid glands
label: d
answer: no
explanation: No, this is not the single best answer. This is a possible underlying explanation but not the most likely diagnosis considering the patient's history of a previous partial thyroidectomy.
name: Familial associated hypoparathyroidism
label: c
answer: no

Correct answer: a
A 19-year-old man presents to A&E with symptoms of nausea, vomiting, and abdominal pain. Her mother notes that he has been losing weight in recent months, despite eating and particularly drinking a great deal.


The clinician reports that the patient is breathing very quickly, and has fruity-smelling breath.


A series of investigations are requested:


 **Blood (BM stick)**


Plasma glucose: 20 mmol/L (normal <6.1 mmol/L))


 **Urine multistick**


Ketones: +++


Glucose: 9% (<5%)


Which of the following best explains the ketone levels in the patient's urine?

Options:
explanation: No, this is not the correct answer. Whilst this statement is true, it is not the inhibition of HMG-CoA reductase which directly results in the increased production of ketone bodies. This patient has diabetic ketoacidosis (DKA) secondary to type 1 diabetes mellitus (T1DM). They have a lower insulin/glucagon ratio, resulting in increased activation of the lyase enzyme and thus urinary ketones.
name: A lower insulin/glucagon ratio resulting in inhibition of the HMG-CoA reductase enzyme
label: c
answer: no
explanation: No, this is not the correct answer. This patient has diabetic ketoacidosis (DKA) secondary to type 1 diabetes mellitus (T1DM). The lyase enzyme is not inhibited in this hormonal state, it is activated.
name: A lower insulin/glucagon ratio resulting in inhibition of the lyase enzyme
label: d
answer: no
explanation: No, this is not the correct answer. HMG-CoA reductase is responsible for converting HMG-CoA to mevalonate and subsequently cholesterol. It does not lead to ketones in the urine.
name: A lower insulin/glucagon ratio resulting in an increased activation of the HMG-CoA reductase enzyme
label: b
answer: no
explanation: No, this is not the correct answer. This patient has diabetic ketoacidosis (DKA) secondary to type 1 diabetes mellitus (T1DM). They therefore have a lower insulin/glucagon ratio.
name: A higher insulin/glucagon ratio resulting in increased activation of the lyase enyzme
label: e
answer: no
explanation: Yes, this is the correct answer. This patient has diabetic ketoacidosis (DKA) secondary to type 1 diabetes mellitus (T1DM). In T1DM, there is autoimmune destruction of the beta cells of the pancreas responsible for producing insulin. Hence there is a lower insulin/glucagon ratio which results in increased activation of lyase.

Lyase is responsible for the conversion of HMG-CoA to acetoacetate, bringing about the synthesis of other ketone bodies (such as acetone and beta hydroxybutyrate)
name: A lower insulin/glucagon ratio resulting in an increased activation of the lyase enzyme
label: a
answer: yes

Correct answer: a
A 23-year-old woman with a previous medical history of Crohn's disease visits the GP for an annual check-up. She reports a sense of numbness and tingling in both her arms. In addition, she reports feeling quite fatigued which has been disrupting her work life. She denies any rectal bleeding.



A full blood count and blood film is requested



||||
|--------------|:-------:|---------------|
|Haemoglobin|78 g/L|(M) 130 - 170, (F) 115 - 155|
|Mean Cell Volume (MCV)|119 fL|80 - 96|
|Mean Cell Haemoglobin (MCH)|21 pg|27 - 33|
|Reticulocytes|19x10<sup>9</sup>/L|25 - 100|


A recent colonoscopy report notes that her Crohn's disease primarily affects her distal ileum.



The patient is otherwise fit and well, eating a balanced diet with minimal alcohol intake. She does not smoke.



Which of the following is the most likely cause of this patients symptoms?

Options:
explanation: No this is not the correct answer. Methotrexate inhibits the enzyme dihydrofolate reductase, leading to folate deficiency. Whilst this is a possible cause of macrocytic anaemia, this is not the single best answer as this patient is experiencing these symptoms due to Crohn's disease of the ileum.
name: Methotrexate use
label: e
answer: no
explanation: No, this is not the correct answer. Although folate deficiency is another cause of macrocytic anaemia, it is absorbed from the duodenum and jejunum of the GI tract and thus is less likely to be impaired in crohn's disease.
name: Folate deficiency
label: b
answer: no
explanation: No, this is not the correct answer. Although folate deficiency is another cause of macrocytic anaemia, it is absorbed from the duodenum and jejunum of the GI tract and thus is less likely to be impaired in crohn's disease.
name: Alcohol toxicity
label: d
answer: no
explanation: Yes, this is the correct answer. The patient has an MCV > 100 fL which suggests macrocytic/megaloblastic anaemia. In terms of her symptoms, she has presented with subacute degeneration of the spinal cord secondary to B12 or folate deficienct. The way to differentiate between these two causes is by looking at the patient history. First of all there is no mention of her diet, hence it is already difficult to differentiate in terms of dietary intake. However, the stem mentions that she has crohn's disease particularly affecting the ileum area. We know that vitamin B12 is absorbed from the ileum (terminal) and that crohn's disease can result in malabsorption in the area of the GI tract affected, making this the most likely explanation for her symptoms.
name: Vitamin B12 deficiency
label: a
answer: yes
explanation: No, this is not the correct answer as iron is absorbed from the upper jejunum and duodenum. If this patient was iron deficient, they would also have microcytic anaemia, rather than macrocytic.
name: Iron deficiency
label: c
answer: no

Correct answer: a

Vitamin B12 deficiency
A 60-year-old male presents to the GP with generalised itchy skin, a burning sensation in his hands, and severe headaches. On examination, the GP notices the presence of small haemorrhagic lesions on both his forearms and an enlarged spleen.

The patients haematocrit is measured:

Haematocrit (Hct): 0.71 (normal range 0.41-0.52)

Which of the following is the most appropriate first line management of the patient's condition?

Options:
explanation: No, this is not the correct answer. Steroids can be used for the treatment of immune thrombocytopenic purpura. However, this patient presents with symptoms of erythromelalgia, skin pruritus, and headaches which are indicative of polycythaemia vera. The first-line treatment for this is phlebotomy.
name: Corticosteroids
label: d
answer: no
explanation: Yes, this is the correct answer. The patient presents with symptoms of erythromelalgia, skin pruritus and headaches which are indicative of polycythaemia vera. This is further supported by the patient's elevated haematocrit (Hct). Polycythaemia vera is a myeloproliferative neoplasm associated with an increase in the proportion of red blood cells in the plasma. The main treatment for this condition is phlebotomy to reduce the haematocrit to <0.45.
name: Phlebotomy
label: a
answer: yes
explanation: No, this is not the correct answer. The patient presents with symptoms of erythromelalgia, skin pruritus, and headaches which are indicative of polycythaemia vera. The first-line treatment for this is phlebotomy.
name: Folic acid
label: c
answer: no
explanation: No, this is not the correct answer, this can be used for the treatment of immune thrombocytopenic purpura. However, this patient presents with symptoms of erythromelalgia, skin pruritus, and headaches which are indicative of polycythaemia vera. The first-line treatment for this is phlebotomy.
name: Intravenous immunoglobulin
label: e
answer: no
explanation: No, this is not the correct answer. Imatinib is the 1st line treatment for chronic myeloid leukaemia (CML). The patient presents with symptoms of erythromelalgia, skin pruritus, and headaches which are indicative of polycythaemia vera. The first-line treatment for this is phlebotomy.
name: Imatinib
label: b
answer: no

Correct answer: a