European Board Examination in Endocrinology, Diabetes and … sample questions FINAL 2019.pdf ·...

European Board Examination in Endocrinology, Diabetes and Metabolism

Sample Questions

Updated January 2019

1. A 45-year-old man was referred with a 6-week history of polyuria, polydipsia and 7-kg weight loss. One week previously, his general practitioner had diagnosed type 2 diabetes mellitus and had started him on treatment with metformin. The patient had made appropriate changes to his diet, but his fasting plasma glucose readings remained above 15.0 mmol/L (3.0–6.0). He was feeling tired but was otherwise well. On examination, he was overweight with a BMI of 34.0 kg/m2 (18.0–25.0). Urinalysis showed glucose 3+, ketones 2+. Investigations: serum sodium 142 mmol/L (137–144) serum potassium 4.9 mmol/L (3.5–4.9) serum bicarbonate 23 mmol/L (20–28) serum creatinine 102 µmol/L (60–110) fasting plasma glucose 19.7 mmol/L (3.0–6.0) What is the most appropriate additional treatment? A exenatide B gliclazide C intravenous insulin infusion D pioglitazone E subcutaneous insulin

EXPLANATION: Patient has features consistent with type 1 diabetes mellitus, and insulin would be the most appropriate treatment.


2. A 55-year-old woman had previously been found to have a 4-cm papillary thyroid cancer. She had been treated with surgery and radioiodine and subsequently had an undetectable thyroglobulin but a high titre of anti-thyroglobulin antibody. What is the most appropriate investigation at annual follow-up? A anti-thyroid peroxidase antibodies B CT scan of neck C PET-CT scan of neck D ultrasound scan of neck E whole-body iodine uptake scan

EXPLANATION: Ultrasound scan is the most sensitive investigation for the detection of locally recurrent papillary carcinoma, other investigations should be considered if ultrasound scan is negative or distant metastases are suspected.


3. A 78-year-old woman was referred for bone density assessment after developing acute mid-thoracic bone pain. She had previously been found to have osteoporosis after fracturing her right wrist after tripping in the street. She had also experienced two previous episodes of severe back pain that were thought to have been caused by vertebral fractures. She had lost 12.5 cm in height. She had been taking alendronic acid and calcium and vitamin D tablets regularly for 3 years. Her mother had developed a severe kyphosis in her seventies. On examination, there was a mild thoracic kyphosis and tenderness over the patient’s T10 vertebra. Investigations: ESR 35 mm/1st h (<30) serum creatinine 105 µmol/L (60–110) serum corrected calcium 2.35 mmol/L (2.20–2.60) serum alkaline phosphatase 95 U/L (45–105) X-ray of thorax and lumbar spine new crush fracture of T10;

previously reported fractures of T8 and L1

DXA scan of spine (L2–L4) T score –3.8 DXA scan of total hip T score –3.4 What is the most appropriate treatment? A continue alendronic acid B HRT C raloxifene D strontium ranelate E teriparatide


EXPLANATION: Teriparatide is effective at reducing vertebral and non-vertebral fractures in post-menopausal women, and reduces both pain and disability due to spinal fractures. It is the most appropriate choice to control both the immediate symptoms and for long-term prevention. Teriparatide is the most effective drug in this age group, particularly for spinal fractures.


4. A 77-year-old woman sustained a low-trauma fracture of her right wrist. Two years previously, she had fractured her proximal humerus. Investigations for secondary causes of osteoporosis revealed no abnormalities. Investigations: DXA scan result: T-score L1–L4 –3.9 left hip –2.7 What cell type in bone primarily senses strain and microdamage? A adipocyte B fibroblast C osteoblast D osteoclast E osteocyte

EXPLANATION: Osteocytes derive from osteoblasts and have long cytoplasmic extensions that detect strain in bone.


5. A 36-year-old man of European descent was reviewed in the diabetes clinic. He had type 2 diabetes mellitus that had been diagnosed 6 months previously. He had been symptom free and had no family history of cardiovascular disease. He was a smoker. On examination, his BP was 138/76 mmHg. His weight was 90 kg and his BMI was 32.0 kg/m2 (18.0–25.0). Investigations: urinary albumin:creatinine ratio 0.6 mg/mmol (<2.5) serum cholesterol 5.3 mmol/L (<5.2) serum HDL cholesterol 0.90 mmol/L (>1.55) fasting serum triglycerides 2.20 mmol/L (0.45–2.30) What is the most appropriate next step in the management of his lipid profile? A assess the risk of cardiovascular disease using a risk calculator

B observe and repeat lipid profile in a few months C start a fibrate D start a statin E start nicotinic acid EXPLANATION: A risk calculator, eg: QRISK, is recommended to measure the probability of cardiovascular disease. Treatment should only be started if there is a >10% risk of developing cardiovascular disease in the next 10 years.


6. A 32-year-old woman presented to the outpatient clinic with a 1-year history of amenorrhoea that had started after stopping her oral contraceptive pill. She had had two successful pregnancies and was otherwise well. On examination, there was an upper outer quadrantanopia. Investigations: serum sodium 138 mmol/L (137–144) serum potassium 3.8 mmol/L (3.5–4.9) serum follicle-stimulating hormone 2.0 U/L (2.5–10.0) serum luteinising hormone 2.0 U/L (2.5–10.0) serum prolactin 8450 mU/L (100–360) MR scan of pituitary 2-cm adenoma with suprasellar

extension What is the most appropriate management? A cabergoline B conformal pituitary radiotherapy C octreotide D refer for pituitary surgery E stereotactic pituitary radiosurgery EXPLANATION: Dopamine agonist therapy is the first-line treatment for a macroprolactinoma.


7. A 26-year-old woman was admitted with diabetic ketoacidosis. After 24 hours of treatment with intravenous fluids, potassium and insulin, her normal subcutaneous insulin regimen was resumed. However, she felt nauseated, her blood glucose began to rise and there was a concomitant increase in urine ketones (3+). On examination, her pulse was 118 beats per minute and her BP was 106/66 mmHg. Investigations: serum sodium 136 mmol/L (137–144) serum potassium 4.4 mmol/L (3.5–4.9) serum bicarbonate 15 mmol/L (20–28) serum creatinine 78 µmol/L (60–110) random plasma glucose 7.3 mmol/L What is the most appropriate next step in management? A increase subcutaneous basal insulin at bedtime B increase subcutaneous bolus insulin with meals C start fixed-rate intravenous insulin infusion D start glucose 5% with fixed-rate intravenous insulin E start glucose 10% with fixed-rate intravenous insulin EXPLANATION: A fixed-rate insulin infusion is recommended for faster resolution of diabetic ketoacidosis. If the blood glucose is below 14 mmol/L, it is necessary to administer intravenous infusion of 10% glucose in order to avoid hypoglycaemia and permit the continuation of fixed-rate intravenous insulin.


8. An 18-year-old woman was referred by her general practitioner for further investigation of ‘funny turns’ during which she developed palpitations, sweating, tremor, hunger, anxiety and paraesthesiae. Her symptoms were relieved immediately after drinking a sugary drink. She was otherwise well and was not taking any regular medication. There was a family history of diabetes mellitus. A spontaneous hypoglycaemic episode had not been captured and she was admitted to the diabetes and endocrine ward for a prolonged supervised fast. Her renal function was normal. After a 12-hour fast she experienced her typical symptoms. Urinalysis showed no urinary ketones. Investigations (after 12-h fast): fasting plasma glucose 2.0 mmol/L (3.0–6.0) serum insulin (after hypoglycaemia) 56 pmol/L (<21) serum C-peptide 514 pmol/L (180–360) What is the most appropriate next step in management? A coeliac axis angiography B MR scan of abdomen and pelvis to localise a mesenchymal tumour producing IGF-2 C MR scan of pancreas to localise an insulinoma D obtain a careful history looking for access to exogenous insulin E request a plasma sulphonylurea screen

EXPLANATION: There is a raised insulin and C-peptide in the presence of hypoglycaemia. With a family history of diabetes, access to insulin/sulphonylurea should be thought about. In this case, the fact that both C-peptide and insulin are raised suggests that this is endogenous insulin secretion. Requesting a sulphonylurea screen is the most appropriate next step, and depending on the result of this, further investigations may be required.


9. A 30-year-old woman with type 2 diabetes mellitus reported that her younger brother had recently been found to have the same condition. Both her father and paternal grandfather had been treated with insulin. Her father had multiple kidney cysts on ultrasound. What gene mutation is most likely to be responsible for this pedigree? A GCK B HNF1α C HNF1β D HNF4α E KCNJ11 EXPLANATION: The family history of type 2 diabetes mellitus occurring at this young age suggests monogenic diabetes. The renal cysts point strongly to a gene deficit in HNF1β.


10. A 64-year-old woman with a 20-year history of type 2 diabetes mellitus presented following the sudden onset of a painful right eye. She had noticed drooping of her right eyelid but no double vision. There were no other neurological symptoms. On examination, there was a complete right-sided ptosis, with her eye position down and out. Her pupils were equal on both sides. Investigations: haemoglobin A1c 57 mmol/mol (20–42) MR scan of brain no evidence of any intracranial

pathology She was worried about her long-term prognosis and outcome. What is the most likely prognosis? A her condition is likely to deteriorate before it improves B her prognosis is uncertain and depends upon metabolic control C she is at risk of progression, and her other eye might also be affected D she is likely to be left with a permanent ptosis E she is likely to make a very good recovery EXPLANATION: Diabetes can be associated with a mononeuritis. In this scenario, although it is true that her prognosis is uncertain and her outlook is dependent on her metabolic control, a better answer is that she will make a good recovery - precisely because she does have good control.


11. A 49-year-old woman presented with progressive loss of vision in both eyes. She was otherwise well and her only medication was HRT. Examination showed bitemporal lower quadrantanopia. Investigations: serum osmolality 296 mosmol/kg (278–300) urinary osmolality 102 mosmol/kg (100–1000) serum cortisol (09.00 h) 456 nmol/L (320–700) serum follicle-stimulating hormone 1.3 U/L (2.5–10.0) serum luteinising hormone 2.0 U/L (2.5–10.0) serum prolactin 1123 mU/L (100–360) serum thyroid-stimulating hormone 2.3 mU/L (0.4–5.0) serum free T4 18.1 pmol/L (10.0–22.0) MR scan of brain (pre-and post-contrast) see images a and b What is the most likely cause of her loss of vision? A craniopharyngioma B meningioma C non-functioning pituitary adenoma D prolactinoma E Rathke’s cleft cyst EXPLANATION: The MR scan shows a suprasellar mass that enhances with contrast. At 49, she is at the age you would expect to see a second peak in presentation. The prolactin is only slightly raised suggesting a stalk effect; it is not high enough for a prolactinoma.


12. A 35-year-old woman was referred with a left lower thyroid lesion. She was asymptomatic. Examination confirmed the presence of a 2 × 3-cm, firm, mobile, non-tender mass. Investigations: fine-needle aspiration biopsy Thy3f How is Thy3f defined? A abnormal; diagnostic of malignancy B abnormal; diagnostic of medullary thyroid cancer C abnormal; follicular neoplasm suspected D non diagnostic or inadequate E non-neoplastic (consistent with nodular goitre or thyroiditis) EXPLANATION: This refers to the classification of thyroid fine-needle aspirations. Thy3 indicates an abnormal result, and the f indicates that a follicular neoplasm is suspected. [


13. An 18-year-old man was referred with recently diagnosed hypertension. He had no previous history of note and was not taking any medication. His father had been found to have hypertension in his 20s after sustaining a brain haemorrhage. On examination, his BP was raised at 190/110 mmHg. Investigations: serum sodium 142 mmol/L (137–144) serum potassium 3.4 mmol/L (3.5–4.9) plasma renin activity (after 30 min supine) 0.2 pmol/mL/h (1.1–2.7) plasma aldosterone (after 30 min supine) 450 pmol/L (135–400) urinary oxocortisol 32.0 nmol/mmol creatinine (0.8–

6.5) What is the most appropriate investigation? A adrenal venous sampling B aldosterone suppression test C captopril test D CT scan of adrenal glands E genetic testing

EXPLANATION: The combination of low renin, high aldosterone and raised urinary oxocortisol suggests glucocorticoid remediable aldosteronism (GRA). In addition, the low potassium, hypertension and the family history, which includes a brain haemorrhage, is also in keeping. GRA is autosomal dominant, and, therefore, genetic testing is the most appropriate investigation.


14. A 24-year-old woman was admitted with confusion of 24 hours’ duration. She had been feverish with episodic vomiting for 48 hours. She had lost 10 kg in weight over the previous 4 months, and had a poor appetite. She had become progressively fatigued. She had been made redundant from her cleaning job 7 weeks previously. On examination, her pulse was 93 beats per minute and regular, and her BP was 93/64 mmHg. She was disorientated, with a Glasgow coma score of 11, but there were no focal neurological signs. She was thin with a BMI of 17.0 kg/m2 (18.0–25.0). Examination was otherwise normal. Investigations: serum sodium 119 mmol/L (137–144) serum potassium 5.0 mmol/L (3.5–4.9) serum bicarbonate 18 mmol/L (20–28) serum urea 9.7 mmol/L (2.5–7.0) serum creatinine 134 µmol/L (60–110) serum corrected calcium 3.20 mmol/L (2.20–2.60) fasting plasma glucose 3.3 mmol/L (3.0–6.0) serum cortisol (09.00 h) 192 nmol/L (320–700) serum thyroid-stimulating hormone 8.3 mU/L (0.4–5.0) serum free T4 13.6 pmol/L (10.0–22.0) What is the most appropriate intravenous therapy? A aciclovir B hydrocortisone C metoclopramide D pamidronate disodium E sodium chloride 0.9% EXPLANATION: Weight loss, low sodium, high potassium, vomiting, low BP, low bicarbonate and high urea, all in keeping with Addison’s disease. The 09.00 h cortisol is below the normal range. Given the clinical features, she requires hydrocortisone.


15. A 35-year-old woman presented with a 6-month history of diffuse bone pain. Hypophosphataemic rickets had been diagnosed in childhood and she had been taking oral phosphate supplements since. She was currently taking two Phosphate-Sandoz® tablets four times daily. On examination, a proximal myopathy was present. Investigations: serum creatinine into FAIM 105 µmol/L (60–110) serum corrected calcium 2.35 mmol/L (2.20–2.60) serum phosphate 0.60 mmol/L (0.80–1.45) serum alkaline phosphatase 344 U/L (45–105) plasma parathyroid hormone 9.5 pmol/L (0.9–5.4) serum 25-OH-cholecalciferol 85 nmol/L (>50) What is most appropriate treatment? A add calcitriol B add cinacalcet C increase oral phosphate D intravenous phosphate E parathyroidectomy EXPLANATION: 1,25 vitamin D levels tend to be low in this condition. Replacing with calcitriol will rectify this. The combination of phosphate and calcitriol is viewed as standard replacement therapy. The addition of calcitriol should improve the symptoms, and is monitored by the serum alkaline phosphatase level. Phosphate replacement is not monitored by serum phosphate levels, but by serum alkaline phosphatase and serum parathryoid hormone levels.


16. A 27-year-old woman presented 20 weeks into her first pregnancy with tremor, sweats, diarrhoea and no vomiting. On examination, there was a fine tremor. Her pulse was 110 beats per minute and regular, and her peripheries were hot and moist. She had a moderate diffuse goitre and there was no thyroid-associated ophthalmopathy. Investigations: serum thyroid-stimulating hormone <0.1 mU/L (0.4–5.0) serum free T4 37.7 pmol/L (10.0–22.0) What is the most appropriate treatment for her thyrotoxicosis? A carbimazole 20 mg once daily B carbimazole 20 mg once daily and levothyroxine 100 micrograms once

daily C propylthiouracil 100 mg twice daily D propylthiouracil 100 mg twice daily and levothyroxine 100 micrograms

once daily E propylthiouracil 200 mg twice daily and levothyroxine 200 micrograms

once daily EXPLANATION: This patient presents with new-onset thyrotoxicosis at 20 weeks of pregnancy, probably secondary to Graves’ disease. Block replace regimens (answers B, D and E) are not recommended during pregnancy since the placenta is more permeable to antithyroid drugs than to levothyroxine and the fetal thyroid is more sensitive to antithyroid drugs than the mother’s thyroid. Propylthiouracil would be the drug of choice before 16 weeks in view of potential teratogenicity associated with carbimazole. Carbimazole is the preferred antithyroid drug at this stage in pregnancy.


17. A 16-year-old girl was referred with primary amenorrhoea. She was otherwise healthy but reported significant weight gain over the past 2 years. On examination, she had minor facial hirsutism and a BP of 138/68 mmHg. Her BMI was 29.0 kg/m2 (18.0–25.0). Investigations: serum 17-hydroxyprogesterone 7 nmol/L (1–10) serum oestradiol 280 pmol/L (200–400) serum testosterone 2.9 nmol/L (0.5–3.0) serum sex hormone binding globulin 19 nmol/L (40–137) serum follicle-stimulating hormone 4.0 U/L (2.5–10.0) serum luteinising hormone 6.0 U/L (2.5–10.0) serum prolactin 600 mU/L (100–360) What is the most likely diagnosis? A 21-hydroxylase deficiency B Cushing’s syndrome C polycystic ovary syndrome D prolactinoma E Turner syndrome EXPLANATION: Amenorrhoea, hirsutism and weight gain are suggestive of a diagnosis of polycystic ovary syndrome, which is further supported by findings of raised free testosterone and increased LH/FSH ratio. A higher concentration of 17-hydroxypreogesterone would be expected in patients with 21-hydroxylase deficiency (answer A) and a higher prolactin concentration would be expected in those with prolactinoma (answer D). LH and FSH would be high and oestradiol low in patients with Turner syndrome. More striking clinical features including striae, moon facies and higher BP would be expected in patients with Cushing’s syndrome (answer B).


18. A 19-year-old woman presented to her general practitioner with secondary amenorrhoea of 3 months’ duration. There was no history of weight loss or hirsutism and there had been no previous menstrual disturbance. She was not taking any medication and was otherwise well. On examination, there was no galactorrhoea and no visual loss. Investigations: serum cortisol (09.00 h) 459 nmol/L (320–700) serum oestradiol 1500 pmol/L (200–400) serum testosterone 1.6 nmol/L (0.5–3.0) serum follicle-stimulating hormone <0.3 U/L (2.5–10.0) serum luteinising hormone <0.3 U/L (2.5–10.0) serum prolactin 1205 mU/L (100–360) serum thyroid-stimulating hormone 0.5 mU/L (0.4–5.0) serum free T4 14.1 pmol/L (10.0–22.0) What is the most likely diagnosis? A macroprolactinaemia B microprolactinoma C partial hypopituitarism D polycystic ovary syndrome E pregnancy EXPLANATION: Pregnancy (answer E) is the most likely diagnosis overall in this setting and a pregnancy test should be undertaken in patients presenting with amenorrhoea. The biochemical findings of raised oestradiol, low gonadotropins and mildly raised prolactin are consistent with the first trimester of pregnancy. The patient’s symptoms and signs are not suggestive of polycystic ovary syndrome (answer D), and the biochemical findings of suppressed gonadotropins and raised oestradiol are not consistent with this diagnosis. In patients with microprolactinoma (answer B) a low concentration of oestradiol would be expected. The absence of visual field defect, normal thyroid function and normal serum cortisol are not consistent with partial hypopituitarism (answer C). There is no suggestion that macroprolactinaemia (answer A) is present.


19. A 48-year-old bus driver attended a diabetes clinic for review. He was taking metformin and gliclazide and had been intolerant of other oral medications. There were no complications of diabetes. He had never experienced hypoglycaemia. He had held a vocational (Group 2) driving licence for 5 years. He was advised to start insulin. What advice should he be given about driving once he starts insulin? A may be able to hold a vocational licence, can continue driving a bus but

must inform the agency as soon as possible B may be able to hold a vocational licence, must stop driving a bus immediately and inform the agency C may wish to inform the agency, does not need by law to do so unless he

develops complications of diabetes D will not be able to hold a vocational licence and must stop driving a bus

immediately E will not be able to hold a vocational licence, may apply for an alternative

licence to allow him to continue driving a bus EXPLANATION: Since this patient will be started on insulin treatment he must stop driving and inform the licensing agency immediately. He may be able to hold a vocational (Group 2) licence if a number of qualifying criteria are met and this involves an application to the licensing agency. This includes at least 3 months of monitoring blood sugars with a meter with a memory before the licence can be renewed.


20. A 38-year-old woman presented with a goitre. It had been growing steadily for the past 4 months. She described a 3-month history of worsening dysphagia and had experienced choking on lying down. She had unintentionally lost 3 kg in weight. On examination, she was clinically euthyroid and had a large, smooth, non-tender goitre with retrosternal extension. Investigations: serum thyroid-stimulating hormone 3.2 mU/L (0.4–5.0) serum free T4 19.8 pmol/L (10.0–22.0) anti-thyroid peroxidase antibodies 20 IU/mL (<50) fine-needle aspiration cytology Thy2 CT scan of neck see image What is the most appropriate next step in management? A high-dose radioactive iodine therapy B levothyroxine therapy C pulmonary flow loop D referral for thyroidectomy E technetium-99m scan of thyroid (20-min uptake)

EXPLANATION: This patient has significant compressive symptoms from an enlarging goitre, which is causing tracheal narrowing on a CT scan. It is likely that the weight loss is related to dysphagia. The most appropriate treatment is with thyroidectomy (answer D). Further investigation with a pulmonary flow loop (answer C) will not be of additional diagnostic benefit. Thyroid function is normal and a technetium scan (answer E) will not provide further information regarding the goitre over and above the CT scan. Suppressive levothyroxine therapy (answer B) is not effective in shrinking goitres of this size and is not indicated. Radioactive iodine therapy (answer A) may cause some goitre shrinkage but is less effective than surgery and does not represent the treatment of choice in a 38-year-old patient with no significant comorbidities.


21. A 27-year-old woman presented with a history of altered menstrual habit. Since her menarche at the age of 12, she had had a monthly period, but for the previous 8 months, she had menstruated on alternate months. She was not taking any medication. On examination, her BMI was 26.0 kg/m2 (18.0–25.0) and she had normal postpubertal secondary sexual characteristics. Investigations (by general practitioner): serum testosterone 3.1 nmol/L (0.5–3.0) serum prolactin 620 mU/L (100–360) serum thyroid-stimulating hormone 2.2 mU/L (0.4–5.0) serum free T4 14.0 pmol/L (10.0–22.0) Investigations (at clinic): serum oestradiol 1398 pmol/L (200–400) What is the most appropriate next investigation? A MR scan of pituitary B serum 17-hydroxyprogesterone C serum β-HCG D short tetracosactide (Synacthen®) test (250 micrograms) E ultrasound scan of pelvis EXPLANATION: Pregnancy is the most likely diagnosis in this scenario and determination of serum β-HCG (answer C) is the most appropriate initial investigation. Serum oestradiol is raised consistent with pregnancy and this is not a feature of PCOS, which may warrant a pelvic ultrasound (answer E). The history is not suggestive of a diagnosis of 21-hydroxylase deficiency, which may warrant measurement of 17-hydroxyprogesterone (answer B). The history and biochemical findings do not indicate Addison’s disease or pituitary dysfunction, which may require evaluation by a Synacthen® test (answer D) or pituitary MR scan (answer A) respectively.


22. A 68-year-old woman without known type 2 diabetes mellitus was admitted after a collapse. On examination, she was drowsy and tachycardic with a BP of 98/62 mmHg. Investigations: serum sodium 110 mmol/L (137–144) serum potassium 3.0 mmol/L (3.5–4.9) serum chloride 56 mmol/L (95–107) serum bicarbonate 25 mmol/L (20–28) serum urea 22.0 mmol/L (2.5–7.0) serum creatinine 301 µmol/L (60–110) random plasma glucose 117.0 mmol/L What is her calculated anion gap? A 26 B 28 C 30 D 32 E 34 EXPLANATION: The calculated anion gap is (Na+K) – (HCO3+Cl) = (110+3) – (56+25) = 32


23. A 44-year-old woman with a 30-year history of type 1 diabetes mellitus presented to the emergency department after becoming acutely unwell. Investigations: serum sodium 126 mmol/L (137–144) serum potassium 4.1 mmol/L (3.5–4.9) serum chloride 90 mmol/L (95–107) serum bicarbonate 17 mmol/L (20–28) serum urea 7.5 mmol/L (2.5–7.0) serum creatinine 136 µmol/L (60–110) random plasma glucose 27.5 mmol/L arterial blood gases, breathing air: PO2 18.9 kPa (11.3–12.6) PCO2 3.8 kPa (4.7–6.0) pH 7.26 (7.35–7.45) H+ 55 nmol/L (35–45) bicarbonate 16 mmol/L (21–29) base excess –8.3 mmol/L (±2.0) chest X-ray no consolidation ECG sinus tachycardia She was treated with a diabetic ketoacidosis integrated care pathway, with sodium chloride 0.9%, intravenous insulin and potassium. Over the next 6 hours, her acidosis and clinical state failed to improve. What investigation should be urgently performed? A CT pulmonary angiography B plasma lactate C plasma paracetamol D serum amylase E serum salicylate


EXPLANATION: This patient presents with a metabolic acidosis presumed to be secondary to diabetic ketoacidosis. The non-improvement of the patient’s clinical status and biochemical findings suggest that the metabolic acidosis is due to another reason, such as sepsis. The finding of a raised lactate concentration (answer B) will provide further insights. There is no suggestion that the patient has ingested excess paracetamol or salicylate and answers C and E are, therefore, incorrect. There are no clinical features suggesting pancreatitis to warrant measurement of serum amylase (answer D). If a pulmonary embolus warranting a CT pulmonary angiogram (answer A) was suspected, the arterial blood gas would show a low PO2 concentration, which is not seen.


24. An 18-year-old man was referred with a 3-week history of exertional angina. He was found to have enlarged, orange tonsils, clouding of the cornea and hepatosplenomegaly. Investigations: serum cholesterol 4.67 mmol/L (<5.2) serum non-HDL cholesterol 2.89 mmol/L (<3.36) serum HDL cholesterol 0.23 mmol/L (>1.55) fasting serum triglycerides 2.98 mmol/L (0.45–2.30) What is the most likely diagnosis? A abetalipoproteinaemia B apolipoprotein A1 deficiency C apolipoprotein B deficiency D lecithin-cholesterol acyltransferase deficiency E Tangier disease EXPLANATION: Tangier’s disease, also known as familial alpha-lipoprotein deficiency or hypoalphalipoproteinemia is a rare inherited disorder characterised by a severe reduction in the amount of HDL, as is seen in this patient. Patients develop various cholesterol ester depositions especially in tonsils, which may appear yellow or orange as well as in liver and spleen resulting in hepatosplenomegaly.


25. A 24-year-old man was seen in the diabetes clinic. On examination, he had lost 7 kg in weight since the diagnosis of diabetes mellitus 3 months previously. His BMI was 33.0 kg/m2 (18.0–25.0). His father and grandfather had developed diabetes mellitus during their twenties. Investigations (at diagnosis): random plasma glucose 18.0 mmol/L haemoglobin A1c 57 mmol/mol (20–42) He had been treated with gliclazide, and metformin had been added later. Investigations (on presentation): haemoglobin A1c 73 mmol/mol (20–42) serum C-peptide 20 pmol/L (180–360) anti-glutamic acid decarboxylase (GAD) antibodies positive What is the most likely diagnosis? A chronic pancreatitis B latent autoimmune diabetes of adulthood C maturity-onset diabetes of youth D type 1 diabetes mellitus E type 2 diabetes mellitus EXPLANATION: The low C-peptide level makes significant insulin resistance highly unlikely. Latent autoimmune diabetes of adulthood (LADA) is usually diagnosed in patients over the age of 30, many of who will have been treated for some time with oral agents. The relatively rapid deterioration in haemoglobin A1c in this case is also somewhat contrary to that seen commonly in LADA. The lack of response to a sulfonylurea makes maturity-onset diabetes of youth (MODY) (HNF1a) unlikely. Positive anti-glutamic acid decarboxylase antibodies are also unusual in MODY.


26. A 24-year-old man was seen in the diabetes clinic for routine review. He had type 1 diabetes mellitus that had been treated for 20 years. He had a group 1 driving licence (ordinary car, motorcycle). He gave a history of an episode of disorientation and confusion 5 months previously while at work. The episode resolved after he was given glucose gel 40%. He also reported an episode of sweating and confusion at approximately 02.00 h 2 months previously. His wife woke up and gave him glucose gel 40%, after which he regained consciousness. He had one other episode of confusion while walking home from work 1 month previously. He had felt hungry and sweaty beforehand. The episode resolved after eating sweets. What is the most appropriate advice about informing the licensing agency and driving? A he need not inform the licensing agency unless severe hypoglycaemia

recurs within 1 year of the first episode B he need not inform the licensing agency unless severe hypoglycaemia

recurs within 1 year of the second episode C he should inform the licensing agency and should not drive for 1 year

after the first episode D he should inform the licensing agency and should not drive for 3 months

after the second episode E he should inform the licensing agency and should not drive for 3 months

after the third episode EXPLANATION: This question really only needs an understanding of the most recent licensing guidelines regarding driving and hypoglycemia. This man is demonstrating hypoglycemia unawareness.


27. A 47-year-old woman was referred with abnormal thyroid function. She had a long history of seizure disorder and mental health problems. She had felt tired for several years. Her menstrual cycle was regular, with no galactorrhoea. Her medication included daily phenytoin, lofepramine, trazodone and lansoprazole. On examination, her pulse was 76 beats per minute and regular, and her BP was 132/84 mmHg with no postural drop. She appeared euthyroid with no goitre. Her visual fields were full. Investigations: serum cortisol (09.00 h) 546 nmol/L (320–700) serum luteinising hormone 12.2 U/L (2.5–10.0) serum prolactin 444 mU/L (100–360) serum thyroid-stimulating hormone 2.1 mU/L (0.4–5.0) serum free T4 8.3 pmol/L (10.0–22.0) What is the most likely explanation for her abnormal thyroid function? A assay interference B Cushing’s syndrome C effect of phenytoin D non-functioning pituitary adenoma E non-thyroidal illness (sick euthyroid syndrome)

EXPLANATION: Phenytoin in therapeutic doses displaces thyroxine and, to a lesser extent, tri-iodothyronine from binding proteins in serum, and thus increases peripheral clearance of thyroid hormones.


28. A 20-year-old woman, who was 11 weeks pregnant, complained of nausea, abdominal pains and occasional vomiting, associated with poor appetite. She had failed to gain any weight over the past 2 weeks. She underwent blood tests at her general practitioner’s surgery. On examination, her BP was 120/84 mmHg with no postural drop, and her thyroid was just palpable. Investigations: serum urea normal serum creatinine normal liver function tests normal serum thyroid-stimulating hormone 0.1 mU/L (0.4–5.0) serum free T4 19.0 pmol/L (10.0–22.0) serum free T3 6.0 pmol/L (3.0–7.0) What is the most likely diagnosis? A choriocarcinoma B Graves’ thyrotoxicosis C mild hyperemesis gravidarum D thyroid-test derangement in non-thyroidal illness E toxic multinodular goitre EXPLANATION: The most likely diagnosis by far would be mild hyperemesis gravidarum. The patient is coming to the end of the first trimester and has a suppressed TSH probably caused by high levels of HCG and receptor promiscuity. In non-thyroidal illness TSH is usually normal and occasionally mildly elevated. There is no clinical suspicion of a toxic multinodular goiter and the free T4 is normal. Graves’ cannot be totally excluded (a measurement of TSH receptor antibody, or to a lesser extent anti-thyroid peroxidase antibodies might be helpful) but hyperemesis is far more likely. Choriocarcinoma can produce these changes in thyroid function but is extremely rare (1:70,000 pregnancies per year) and, therefore, is not the most likely diagnosis in this case.


29. A 32-year-old woman presented to the emergency assessment unit feeling unwell. Although confused, she was able to confirm that she had previously had no serious known illnesses. There was no record of her on the hospital computer system. On examination, she was thin, unwell and tachypnoeic. Her temperature was 34.8°C, her pulse was 120 beats per minute and her BP was 79/42 mmHg. Her oxygen saturation was 98% (94–98) breathing air. Urinalysis showed ketones 3+. Investigations: serum sodium 151 mmol/L (137–144) serum potassium 5.9 mmol/L (3.5–4.9) serum bicarbonate <5 mmol/L (20–28) serum urea 13.2 mmol/L (2.5–7.0) serum creatinine 148 µmol/L (60–110) random plasma glucose 31.2 mmol/L ECG slightly peaked T waves


In addition to insulin, what is the most appropriate infusion? A calcium carbonate B colloid C sodium bicarbonate 8.4% D sodium chloride 0.45% E sodium chloride 0.9%

EXPLANATION: Fluid resuscitation is a critical part of treating patients with diabetic ketoacidosis. Intravenous solutions replace extravascular and intravascular fluids and electrolyte losses. They also dilute both the glucose level and the levels of circulating counter-regulatory hormones. Insulin is needed to help switch from a catabolic state to an anabolic state, with uptake of glucose in tissues and the reduction of gluconeogenesis as well as free fatty acid and ketone production. Initial correction of fluid loss is by sodium chloride 0.9% solution. The recommended schedule for restoring fluids is as follows:

1. Administer 1–3 L during the first hour. 2. Administer 1 L during the second hour. 3. Administer 1 L during the following 2 hours. 4. Administer 1 L every 4 hours, depending on the degree of dehydration and central venous pressure readings.

When the patient becomes euvolemic, the physician may switch to half the sodium chloride 0.9% solution, particularly if hypernatremia exists. Sodium chloride 0.9% should be administered at a rate appropriate to maintain adequate BP and pulse, urinary output, and mental status.


30. A 53-year-old woman was admitted for a routine inguinal hernia repair. She had a 10-year history of type 2 diabetes mellitus controlled with metformin 850 mg three times daily and gliclazide 160 mg twice daily. On the first postoperative day, she felt nauseated and declined breakfast. However, she had been given her usual medication. Two hours later, she was found drowsy and confused in her bed, and was unable to cooperate with the nurses. Her bedside capillary blood glucose concentration was 2.4 mmol/L. An assessment showed that it was safe for her to swallow. What is the most appropriate action to take as a result of this finding? A glucagon 2 mg intramuscularly B glucose 20% 200 mL intravenously over 10 min C orange juice 250 mL D two biscuits followed by milk 250 mL E two tubes of 25 g glucose gel orally

EXPLANATION: This question tests the most appropriate method to treat hypoglycaemia in a conscious patient who is drowsy, confused and unable to cooperate but who is able to swallow. In this situation, giving gluco gel orally is the quickest and safest non-invasive way to reverse the hyoglycaemia.


31. A 52-year-old man presented with a 5-day history of a flu-like illness. During the previous 24 hours he had become increasingly drowsy and confused. He had been otherwise well before this episode and was not taking any medication. On examination, his temperature was 38.8°C, his pulse was 98 beats per minute and his BP was 110/64 mmHg. He had a Glasgow coma score of 13 and there was no focal neurological deficit. Investigations: serum sodium 134 mmol/L (137–144) serum potassium 4.5 mmol/L (3.5–4.9) serum urea 7.4 mmol/L (2.5–7.0) serum creatinine 108 µmol/L (60–110) CT scan of head see image What is the most likely diagnosis? A meningioma B pituitary macroadenoma C posterior fossa tumour D subarachnoid haemorrhage E subdural haematoma EXPLANATION: This is an image of a pituitary macroadenoma. There are no features on the CT scan of a subdural or subarachnoid haemorrhage. The presentation is of sepsis.


32. A 57-year-old woman was referred to the outpatient clinic from the eye hospital having presented with visual disturbance. She was otherwise asymptomatic. Investigations: serum follicle-stimulating hormone 45.0 U/L (>30.0) serum luteinising hormone 33.0 U/L (>30.0) serum prolactin 300 mU/L (100–360) serum thyroid-stimulating hormone 1.5 mU/L (0.4–5.0) serum free T4 15.0 pmol/L (10.0–22.0) MR scan of pituitary 2-mm-diameter hypodense lesion in left lobe of pituitary; no other findings What is the most appropriate next step in management? A arrange further MR scan of pituitary after 6 months B arrange overnight dexamethasone suppression test C measure GH 2 h after glucose tolerance test D reassure and discharge from clinic E refer to pituitary multidisciplinary team for consideration of selective

microadenomectomy EXPLANATION: This is a pituitary incidentaloma (presumably an innocuous non-functioning pituitary adenoma). It is not likely to be the cause of any visual symptoms. She has preserved pituitary function evidenced by postmenopausal gonadotropins. In the absence of features of cortisol or growth hormone excess, there is no indication to investigate for Cushing’s or acromegaly. There is no indication to repeat MR scan for a 2 mm lesion .


33. A 63-year-old man attended the endocrinology clinic. His general practitioner had found evidence of hypogonadism. Investigations: baseline cortisol 98 nmol/L short tetracosactide (Synacthen®) test (250 micrograms): serum cortisol (30 min after tetracosactide) 237 nmol/L (>450) serum testosterone 1.2 nmol/L (9.0–35.0) serum follicle-stimulating hormone 1.3 U/L (1.0–7.0) serum luteinising hormone <1.0 U/L (1.0–10.0) serum prolactin 1384 mU/L (100–360) serum thyroid-stimulating hormone <0.1 mU/L (0.4–5.0) serum free T4 2.4 pmol/L (10.0–22.0) serum IGF-1 2.4 nmol/L (3.3–23.3) What is the most appropriate initial treatment? A cabergoline B desmopressin C hydrocortisone D levothyroxine E testosterone

EXPLANATION: This question tests clinical skills. The diagnosis is pan-hypopituitarism. Hydrocortisone should be started immediately. Other replacement therapies should not be started until cortisol has been replaced, to avoid precipitating an adrenal crisis. The prolactin level is only modestly raised and there is no indication to start cabergoline immediately, and testosterone/thyroid hormone replacement can wait. There is no evidence of diabetes insipidus.


34. A 55-year-old woman was referred with a 12-month history of progressive facial hirsutism. She had undergone normal menopause 3 years previously. On examination, her BP was 146/88 mmHg. Her weight was 74 kg and her BMI was 26.0 kg/m2 (18.0–25.0). She had mild untreated facial hair growth on her chin and above her upper lip. There was no other evidence of hirsutism. Investigations: serum cortisol (09.00 h) 456 nmol/L (320–700) overnight dexamethasone suppression test (1 mg): serum cortisol 48 nmol/L (<50) serum testosterone 6.2 nmol/L (0.5–3.0) plasma adrenocorticotropic hormone (09.00 h) 12.0 pmol/L (3.3–15.4) serum follicle-stimulating hormone 77.0 U/L (>30.0) serum luteinising hormone 48.0 U/L (>30.0) serum prolactin 225 mU/L (100–360) non-contrast CT scan of abdomen and pelvis no evidence of an adrenal or

ovarian mass What is the most likely diagnosis? A androgen-secreting adrenal adenoma B hypertrichosis C idiopathic hirsutism D ovarian hyperthecosis E pituitary-dependent Cushing’s disease EXPLANATION: Ovarian hyperthecosis is the most likely diagnosis. Cushing’s is excluded (other than cyclicalwhich is less likely. Hypertrichosis and idiopathic hirsutism are not associated with elevations of testosterone to this level. Imaging has shown no abnormalities.


35. A 30-year-old woman was referred with hypercalcaemia. She had been found to have primary hyperparathyroidism 3 months previously. When she attended the clinic, she stated that she was now 8 weeks pregnant and complained of nausea and thirst. She was not taking any medication. Investigations: serum corrected calcium 3.20 mmol/L (2.20–2.60) serum alkaline phosphatase 450 U/L (45–105) plasma parathyroid hormone 14.6 pmol/L (0.9–5.4) serum 25-OH-cholecalciferol 38 nmol/L (>50) What is the most appropriate time for a parathyroidectomy? A after delivery B as soon as possible after confirmation of hyperparathyroidism C first trimester of pregnancy D second trimester of pregnancy E third trimester of pregnancy EXPLANATION: This patient has significant and symptomatic hypercalcaemia that needs treating. The safest time to perform such surgery (and other surgery) is usually during the second trimester.


36. A 26-year-old woman was referred after she had been found to have a random plasma glucose concentration of 12.5 mmol/L. During an elective admission for an appendicectomy at the age of 10, it had been noted that she had a slightly raised plasma glucose. During her last pregnancy, gestational diabetes had required treatment with insulin from 35 weeks’ gestation. Her father had had diabetes mellitus for 20 years without complications. She had no symptoms, appeared well and had no complications. On examination, her BMI was 34.0 kg/m2 (18.0–25.0). Investigations: fasting plasma glucose 6.9 mmol/L (3.0–6.0) oral glucose tolerance test (75 g): 2-h plasma glucose 11.9 mmol/L (<7.8) What is the most likely cause of her diabetes? A GCK mutation B HNF1β mutation C HNF4α mutation D latent autoimmune diabetes of adults E mitochondrial diabetes

EXPLANATION: T2DM is still the most likely cause of her diabetes. She is obese, with a strong family history of diabetes. She does meet the clinical criteria to screen for rare monogenetic causes of diabetes, but T2DM is still the most likely diagnosis in this scenario. If she had monogenetic diabetes, it would be most likely to be GCK.


37. A 19-year-old man presented to his general practitioner with a 1-year history of fatigue and leg weakness. He had no significant past medical history and was not taking any regular medication. There was a significant family history of hypertension. On examination, his pulse was 88 beats per minute and his BP was 178/99 mmHg, supine. Neurological examination revealed grade 4/5 power in all muscle groups of his lower limbs but was otherwise normal. Investigations: serum sodium 135 mmol/L (137–144) serum potassium 2.1 mmol/L (3.5–4.9) serum chloride 95 mmol/L (95–107) serum bicarbonate 32 mmol/L (20–28) serum urea 3.2 mmol/L (2.5–7.0) serum creatinine 78 μmol/L (60–110) plasma renin activity (after 30 min supine) 0.2 pmol/mL/h (1.1–2.7) plasma aldosterone (after 30 min supine) 25 pmol/L (135–400) What is the most likely diagnosis? A apparent mineralocorticoid excess B Bartter syndrome C Gitelman syndrome D Gordon syndrome (pseudohypoaldosteronism type 2) E Liddle syndrome

EXPLANATION: This is based on the differences between Bartter’s, Gitelman’s and Liddle’s syndromes. This is Liddle’s because of the hypertension, the hypokalaemia and the metabolic alkalosis.


38. A 26-year-old woman who was 28 weeks pregnant complained of headaches. She had a history of a pituitary macroprolactinoma and had stopped taking a dopamine agonist following conception. The ophthalmologist reported that there was some limitation in her temporal fields of vision. An MR scan of pituitary showed the lesion had grown from 12 to 16 mm since the start of pregnancy, and was abutting the chiasm. What is the most appropriate advice? A monitor visual fields and repeat MR scan at 36 weeks’ gestation B refer for consideration of pituitary surgery C start cabergoline D start quinagolide E suggest induction of labour

EXPLANATION: In this scenario, given that she has a prolactin-secreting tumour the treatment would be to put her back on cabergoline as soon as possible, given the ophthalmological findings. The adverse effects of this would be an inability to breastfeed, but little else, given that the baby is beyond the stage of organogenesis.


39. A 16-year-old boy presented complaining of short stature. He had noticed that, since the age of 13, he had been lagging behind his peers in height. He had also gained weight over the past 2 years. He was not taking any medication. On examination, he was obese with a height of 154.7 cm and weight of 90 kg; his BMI was 37.0 kg/m2 (18.0–25.0). His BP was 138/89 mmHg. His genitalia were Tanner stage 3, with testicular volumes of 10 mL bilaterally. Investigations: serum sodium 142 mmol/L (137–144) serum potassium 3.8 mmol/L (3.5–4.9) serum urea 5.6 mmol/L (2.5–7.0) serum creatinine 84 µmol/L (60–110) haemoglobin A1c 49 mmol/mol (20–42) What is the most appropriate next step in management? A genetic screening for Prader–Willi syndrome B insulin tolerance test C MR scan of pituitary D overnight dexamethasone suppression test E reassure and discharge EXPLANATION: The clinical features and the slightly low potassium and the borderline raised haemoglobin A1c should alert you to the possibility of hypercortisolism. The screening test for Cushing’s syndrome would be the correct answer here.


40. A 19-year-old woman presented with a 12-week history of a left-sided neck swelling. There was no past medical or family history of note. On examination, there was a firm, 2.5-cm left thyroid swelling. Investigations: serum thyroid-stimulating hormone 2.1 mU/L (0.4–5.0) serum free T4 14.0 pmol/L(10.0–22.0) ultrasound scan of thyroid 2.5-cm thyroid nodule (U2) with no significant lymphadenopathy What is the most appropriate next step in management? A fine-needle aspiration B interval ultrasound scan of thyroid C left thyroid lobectomy D measure serum thyroglobulin E reassure and discharge

EXPLANATION: The ultrasound appearances that are indicative of a benign nodule (U1-2) should be regarded as reassuring not requiring fine-needle aspiration cytology (FNAC), unless the patient has a statistically high risk of malignancy. In this case, the patient is a young woman with low malignancy risk.


41. A 34-year-old woman was referred for investigation of resistant hypertension. She had a history of depression. Her medication comprised ramipril 10 mg daily, doxazosin 8 mg daily, mirtazapine 15 mg daily, methyldopa 250 mg three times daily, and the oral contraceptive pill. Which medication is most likely to give a false negative result for an aldosterone:renin ratio? A doxazosin B methyldopa C mirtazapine D oral contraceptive E ramipril EXPLANATION: The drugs with the biggest impact on the renin–angiotensin system are beta blockers (which reduce renin and thus increase the aldosterone/renin ratio) and aldosterone antagonists (which increase aldosterone). Doxazosin has minimal effect on the ratio. Mirtazapine will tend to increase the aldosterone level and hence the ratio and the oral contraceptive pill may likewise lead to an apparent increase in the level of steroid hormones on assays, owing to the increase in binding globulins. Ramipril is an ACE inhibitor, which reduces the production of aldosterone and will therefore artificially reduce the aldosterone:renin ratio, potentially giving a false negative result.


42. A 20-year-old woman was found to have gestational diabetes mellitus. In a 75-g oral glucose tolerance test, her fasting plasma glucose was 7.5 mmol/L (3.0–6.0) and the measurement after 2 hours was 9.6 mmol/L (<7.8). She had been treated with insulin from 28 weeks’ gestation. At 32 weeks’ gestation, she reported that she felt unwell, hungry, and sweaty with palpitations whenever her fasting blood glucose was under 7.0 mmol/L. The symptoms were relieved by food. Her mother and maternal grandmother had both developed diabetes mellitus in their twenties; both had had a normal BMI. The patient’s BMI was 24.0 kg/m2 (18.0–25.0). What is the most likely cause of her diabetes? A glucokinase mutation B HNF1α mutation C HNF4α mutation D mitochondrial RNA mutation E type 2 diabetes mellitus EXPLANATION: This woman evidently has a strong history on her maternal side of type 2 diabetes developing at a relatively young age. This would be consistent with the group of conditions called maturity-onset diabetes of youth (MODY). There are various genetic causes. Glucokinase is a gene that acts as the ‘glucose sensor’ for the pancreas, so that when the blood glucose rises, the amount of insulin produced also increases. A malfunction leads to elevated glucose level and diabetes, although this increase in blood glucose is mild and usually does not need treatment. The pattern of diabetes in this patient is typical for a glucokinase mutation.


43. A 24-year-old woman with a 10-year history of type 1 diabetes mellitus was currently at 35 weeks’ gestation. She had no family history of diabetes and the child’s father did not have diabetes. What is her child’s risk of developing type 1 diabetes mellitus by the age of 20? A 0.2% B 2% C 10% D 20% E 40%

EXPLANATION: This question requires the knowledge of the approximate background risk of type 1 diabetes in the population (around 1%). The risk to a child with an affected parent is increased. The risk is more marked if the parent developed the condition before the age of 11, or if the affected parent is the father. An affected father increases the background risk approximately 10-fold. However, if the mother has type 1 diabetes and is 25 years or younger when the child is born, as in this case, the risk is lower than this, although higher than background. Estimates vary from 2%–4%. Once the mother is over 25 at the time of birth the risk drops to almost background level.


44. A 78-year-old man was admitted with a left hemiparesis. He had type 2 diabetes mellitus. A nasogastric tube was inserted and enteral feeding was started over 18 hours, with premixed insulin given at the start and midpoint of the feed. The nasogastric tube became blocked 2 hours into the feed and the feed was stopped. Three hours later, he became unrousable. Investigations: capillary blood glucose 2.1 mmol/L What is the most appropriate acute management? A buccal administration of 1 tube of Glucogel®

B intramuscular administration of 1 mL glucagon C intravenous administration of 50 mL of glucose 50% D nasogastric administration of 100 mL of Fortisip®

E nasogastric administration of 100 mL of Lucozade®

EXPLANATION: Here the options all seem plausible; however, on closer reading, it is clear than only one of them is the ‘most appropriate’. In someone who is unrousable, then buccal glucose is not advised because of the risk of aspiration. 50% glucose is no longer advised because of the risk of extravasation. Fortijuice®, not Fortisip® is more advisable and the volume is usually 110–140 mL, and the recent change in the formulation of Lucozade® means that 100 mL will provide insufficient carbohydrate.


45. A 56-year-old woman was admitted from the emergency department with confusion. Investigations: serum sodium 145 mmol/L (137–144) serum potassium 4.3 mmol/L (3.5–4.9) serum bicarbonate 16 mmol/L (20–28) serum creatinine 273 µmol/L (60–110) random blood glucose 42 mmol/L capillary blood ketones 0.1 mmol/L (<0.3) venous blood gases, breathing air: pH 7.30 (7.35–7.45) What is the most appropriate initial treatment regimen? A bolus intravenous dose of 10 units B fixed-rate intravenous infusion at 0.1 units/kg/h C fixed-rate intravenous infusion at 1 unit/h D no insulin initially E variable-rate intravenous infusion EXPLANATION: This patient fulfils the criteria for a diagnosis of hyperosmolar hyperglycaemic state and the guidelines state that initial treatment should be fluids only, with crystalloid. No need for additional insulin, particularly at this stage.


46. A 55-year-old man presented with disturbance in vision. On examination, there was a symmetrical bitemporal hemianopia affecting the upper quadrants of the visual fields. There was no ptosis, and ocular movements were full. He also had partial hypopituitarism. Investigations: enhanced T1 sagittal MR scan of brain and pituitary region see image What is the most likely diagnosis? A craniopharyngioma B meningioma C pituitary macroadenoma D pituitary metastasis E Rathke’s cleft cyst EXPLANATION: All of the possible pituitary lesions listed may present with hypopituitarism and visual disturbance but the radiological appearance of the lesion in this setting is characteristic of meningioma.


47. A 17-year-old boy was referred by his general practitioner because of concern about his development. He had been the shortest in his class for the past 4 years. His voice had not changed and he did not shave. He had begun to have migraine headaches 10 months previously. On examination, he had scanty pubic hair, Tanner stage 2 genitalia and 4-mL testes bilaterally. His height was below the fourth centile. Investigations: serum testosterone 1.6 nmol/L (9.0–35.0) serum follicle-stimulating hormone 1.3 U/L (1.0–7.0) serum luteinising hormone <0.5 U/L (1.0–10.0) serum IGF-1 15.0 nmol/L (9.3–56.0) What is the most appropriate next investigation? A coeliac screen B GnRH (gonadorelin) test C insulin stress test D karyotype analysis E MR scan of pituitary

EXPLANATION: Explanation: This 17-year-old boy has failed to progress through puberty – he appears to have entered early puberty as judged by the findings suggestive of Tanner stage 2. He has low gonadotropins and low testosterone, with a normal IGF-1 but at the bottom of the reference range. First and foremost, he needs to have a sellar/suprasellar space-occupying lesion excluded before further tests.


48. A 31-year-old woman was referred to the endocrinology clinic because of an incidental finding of hypercalcaemia. She had no relevant symptoms and was not taking any regular medication. Investigations: serum creatinine 60 µmol/L (60–110) eGFR (MDRD) 70 ml/min/1.73 m2 (>60) serum corrected calcium 2.70 mmol/L (2.20–2.60) 24-h urinary creatinine 6 mmol (9–18) 24-h urinary calcium 2.6 mmol (2.5–7.5) plasma parathyroid hormone (PTH) 6.7 pmol/L (0.9–5.4) serum 25-OH-cholecalciferol 64 nmol/L (>50) What is the most likely diagnosis? A familial hypocalciuric hypercalcaemia B non-PTH-mediated hypercalcaemia C primary hyperparathyroidism D pseudohypoparathyroidism E secondary hyperparathyroidism EXPLANATION: The low 24-h urinary calcium excretion should raise suspicion of familial hypocalciuric hypercalcaemia. Further investigation includes determination of the calcium/creatinine clearance ratio and mutation screening of the CASR gene.


49. A 44-year-old woman with a history of bipolar affective disorder was admitted to the medical ward following a seizure. No drug history was available from the patient. On examination, her Glasgow coma score was 14. Investigations: serum sodium 127 mmol/L (137–144) serum osmolality 266 mosmol/kg (278–300) urinary osmolality 50 mosmol/kg (100–1000) What is the most likely diagnosis? A central diabetes insipidus B cerebral salt wasting C dipsogenic polydipsia D lithium-induced nephrogenic diabetes insipidus E syndrome of inappropriate antidiuretic hormone EXPLANATION: Although limited investigations are provided, the finding of hypo-osmolar hyponatraemia with very dilute urine is strongly suggestion of excessive fluid intake.


50. A 49-year-old man with a 24-year history of type 1 diabetes mellitus attended for retinal screening. He had missed his appointment 1 year previously and had failed to attend any diabetes clinics in the past 12 months. Investigations: haemoglobin A1c 86 mmol/mol (20–42) What feature is most likely to indicate early referral to an ophthalmologist? A 10 microaneurysms and two sites of venous beading in the retina B 20 microaneurysms spread across the retina with two cotton-wool spots C two blot haemorrhages and three hard exudates, all more than 2 disc

diameters from the fovea D two hard exudates in the periphery E two microaneurysms within 1 disc diameter of the fovea

EXPLANATION: Venous beading is a feature of pre-proliferative retinopathy. Although careful examination of the retina should be performed in any patient with cotton wool spots, the presence of cotton wool spots themselves does not define pre-proliferative retinopathy.


51. A 42-year-old man with a 19-year history of type 1 diabetes mellitus revealed that he had been troubled with recurrent hypoglycaemia, and was concerned because there had been a reduction in his hypoglycaemia warning symptoms. He had needed third-party assistance to treat his hypoglycaemia several times, and had had paramedic assistance twice over the previous 3 months. He was advised to inform the driving agency of this change in his diabetes control. What is the most likely restriction that the driving agency will give him regarding driving? A no restriction on driving B stop driving for 3 months pending satisfactory medical review C stop driving for 6 months pending satisfactory medical review D stop driving for 1 year pending satisfactory medical review E stop driving permanently

EXPLANATION: The patient has required third-party assistance to rescue him from hypoglycaemia on more than one occasion in the last year. He is, therefore, required to abstain from driving for 3 months.


52. A 34-year-old woman, with type 2 diabetes mellitus controlled by a subcutaneous basal bolus insulin regimen, underwent onset of labour in the antenatal ward. Her midwife monitored her capillary blood glucose hourly. Over the previous 3 hours, the blood glucose levels recorded sequentially were 7.5, 8.2 and 9.0 mmol/L. Her cervix was 6-cm dilated. The midwife contacted the diabetes team to seek advice on the optimal management of her blood glucose control throughout labour. What is the most appropriate next step in management? A continue only with 1-hourly monitoring of blood glucose levels B subcutaneous rapid-acting analogue insulin bolus dose C variable-rate intravenous insulin infusion and intravenous glucose 10% D variable-rate intravenous insulin infusion and intravenous sodium chloride

0.9% E variable-rate intravenous insulin infusion only

EXPLANATION: A variable-rate intravenous insulin infusion with concomitant glucose 10% provides the greatest likelihood of glycaemic stability in this clinical context.


53. A 78-year-old man was brought unconscious to the emergency department, having collapsed in the hospital car park on his way to the diabetes renal clinic. He had longstanding type 2 diabetes mellitus, chronic kidney disease treated with ambulatory peritoneal dialysis, and COPD. On examination, he was pale and sweaty. His pulse was 112 beats per minute and his BP was 100/72 mmHg supine. He also had peripheral cyanosis. His capillary blood glucose was 5.9 mmol/L. Investigations (at time of collapse): random plasma glucose 1.9 mmol/L haemoglobin A1c 42 mmol/mol (20–42) What is the most likely cause of the discrepancy between his capillary blood glucose and plasma glucose? A acrocyanosis B darbepoetin C dehydration D haemolysis E icodextrin EXPLANATION: Icodextrin (corn-starch-derived glucose polymers) used in continuous ambulatory peritoneal dialysis can interfere with blood glucose measurement in certain capillary glucose analyzers, leading to over-estimation of blood glucose. The random plasma glucose measured in the laboratory is consistent with the patient’s hypoglycaemic presentation.


54. A 74-year-old man was admitted with a severe chest infection on a background of COPD. He was unwell and admitted to the high dependency unit. His blood glucose on admission was 12.1 mmol/L. He had no previous history of diabetes mellitus. He was started on intravenous antibiotic therapy and prednisolone 30 mg once daily. What is the most appropriate time to start a variable-rate insulin infusion in this patient? A he does not require a variable-rate insulin infusion B if his blood glucose level exceeds 12.0 mmol/L on one further occasion C if there is evidence of severe sepsis and his blood glucose level remains

above 6.0 mmol/L D if there is evidence of severe sepsis and his blood glucose level remains

above 17.0 mmol/L E on admission

EXPLANATION: Of the options given, a variable rate insulin infusion should only be started if the high glucose concentration is maintained. Management follows guidelines.


55. A 50-year-old man with type 1 diabetes mellitus of 14 years’ duration presented with erectile dysfunction, poor libido and fatigue. He mentioned recent problems in his marital relationship. His daily medication comprised simvastatin 40 mg, ramipril 10 mg and bendroflumethiazide 2.5 mg. Examination was normal. Investigations: serum sodium 139 mmol/L (137–144) serum potassium 4.4 mmol/L (3.5–4.9) serum urea 6.0 mmol/L (2.5–7.0) serum creatinine 100 µmol/L (60–110) eGFR (MDRD) 62 mL/min/1.73 m2 (>60) haemoglobin A1c 69 mmol/mol (20–42) serum cholesterol 5.4 mmol/L (<5.2) serum testosterone (09.00 h) 5.9 nmol/L (9.0–35.0) serum sex hormone binding globulin 58 nmol/L (10–62) serum follicle-stimulating hormone 4 U/L (1.0–7.0) serum luteinising hormone 6 U/L (1.0–10.0) serum prolactin 245 mU/L (100–360) MR scan of pituitary normal What is the most appropriate treatment? A epoprostenol injection B psychosexual counselling C sildenafil D stop ramipril and simvastatin E testosterone replacement


EXPLANATION: In the presence of a high normal sex hormone binding globulin level, the serum (total) testosterone level of just 5.9 nmol/L is consistent with a genuinely low free androgen index. A central structural abnormality has been excluded (required in the presence of ‘inappropriately normal’ gonadotropin levels). The patient, therefore, has clinical and biochemical evidence of hypogonadism and should be considered for testosterone replacement therapy. In this context, it is also appropriate to ensure that other diagnoses (e.g. Cushing’s syndrome) have been excluded if there are any clinical concerns.


56. A 17-year-old girl complained of primary amenorrhoea and lack of breast development. She had recently arrived in the UK from Uganda. On examination, she was 136 cm in height and her BMI was 23.0 kg/m2 (18.0–25.0). She had a low posterior hairline and an increased forearm-carrying angle. She had a broad upper chest with Tanner stage 1 breast development and no pubic or underarm hair. Investigations: serum 17-hydroxyprogesterone 2 nmol/L (1–10) serum oestradiol <60 pmol/L (200–400) serum luteinising hormone 39.0 U/L (2.5–10.0) serum prolactin 145 mU/L (100–360) serum thyroid-stimulating hormone 8.9 mU/L (0.4–5.0) cytogenetic analysis 45XO mosaic with 10% 46XY cells She was referred for gonadectomy. What is the most important preoperative investigation? A echocardiography B MR scan of heart C MR scan of pelvis D thrombophilia screen E ultrasound scan of renal tract EXPLANATION: Turner syndrome is associated with structural cardiovascular anomalies, including bicuspid aortic valve, aortic root dilatation and coarctation of the aorta. This is the most relevant issue in the context of general anaesthesia. Echocardiography is less effective than cardiac MR at detecting aortic disease. Recommended screening includes echocardiography for infants and young girls, and cardiac MR for older girls and adults.


57. A 19-year-old woman attended the endocrine clinic with a history of medulloblastoma that had been treated 5 years previously with surgery, chemotherapy and radiotherapy. She had recovered well from her disease, and wished to discuss her fertility. Menarche had occurred at the age of 13, although she had been amenorrhoeic for the past 4 years. Investigations: serum oestradiol <70 pmol/L (200–400) serum follicle-stimulating hormone 1.8 U/L (2.5–10.0) serum luteinising hormone 1.0 U/L (2.5–10.0) serum prolactin 980 mU/L (100–360) What investigation will best predict her potential for future fertility? A anti-Müllerian hormone B day 21 serum progesterone C gonadotropin-releasing hormone test D MR scan of pituitary E transabdominal ultrasound scan of ovaries

EXPLANATION: The radiotherapy and the biochemical pattern of central hypogonadism suggest radiation-induced hypogonadotropic hypogonadism. However, the chemotherapy may have adversely affected ovarian function. The serum anti-Müllerian hormone level is an indicator of follicle reserve, and predicts the response of the ovaries to ovarian stimulation used in IVF.


58. A 17-year-old boy was referred by a gastroenterologist because of concern about his development. He had been the shortest in his class for the past 4 years. His voice had not changed and he did not shave. His inflammatory bowel disease had been quiescent for the past 4 months and he had not required any prednisolone for 2 months. On examination, he had scanty pubic hair, Tanner stage 2 genitalia and 8-mL testes bilaterally. His height was below the fourth centile. His bone age was estimated to be 12 years. Investigations: serum testosterone 6.9 nmol/L (9.0–35.0) serum follicle-stimulating hormone 5.7 U/L (1.0–7.0) serum luteinising hormone 4.8 U/L (1.0–10.0) serum thyroid-stimulating hormone 1.7 mU/L (0.4–5.0) serum free T4 16.9 pmol/L (10.0–22.0) serum IGF-1 37.4 nmol/L (9.3–56.0) What is the most appropriate next step in management? A insulin tolerance test B MR scan of pituitary C review in 4 months D start intramuscular testosterone replacement at a dosage of 50 mg every

month E start intramuscular testosterone replacement at a dosage of 250 mg every

month EXPLANATION: Puberty is underway. From the clinical summary it is most likely that pubertal delay has arisen as a result of inflammatory bowel disease. There are no biochemical findings to suggest structural pituitary disease. Androgen therapy is not required with this serum testosterone level; moreover, such treatment may limit final height. It is likely that growth and development will progress with remission of the inflammatory bowel disease.


59. A 28-year-old woman with epilepsy was admitted with diarrhoea, vomiting, muscle cramps and paraesthesiae. Her medication included lamotrigine and lansoprazole. Investigations: serum urea 6.5 mmol/L (2.5–7.0) serum creatinine 74 µmol/L (60–110) serum corrected calcium 1.72 mmol/L (2.20–2.60) serum phosphate 1.7 mmol/L (0.8–1.4) serum albumin 38 g/L (37–49) serum alkaline phosphatase 101 U/L (45–105) serum magnesium 0.65 mmol/L (0.75–1.05) plasma parathyroid hormone 32.2 pmol/L (0.9–5.4) serum 25-OH-cholecalciferol 30 nmol/L (>50) What is the most likely cause of the hypocalcaemia? A anticonvulsant therapy B hypomagnesaemia C pseudohypoparathyroidism D pseudopseudohypoparathyroidism E vitamin D deficiency

EXPLANATION: The degree of elevation of plasma parathyroid hormone (PTH), with a poor calcium response, indicates a degree of PTH resistance. The high phosphate makes hypomagnesaemia less likely as the cause of the low calcium. Pseudohypoparathyroidism is caused by PTH resistance.


60. A 45-year-old obese man presented with intense loin pain and haematuria. He had a previous history of renal stones and longstanding hypertension. He was taking doxazosin 4 mg daily. Investigations: serum sodium 143 mmol/L (137–144) serum potassium 3.9 mmol/L (3.5–4.9) serum urea 6.8 mmol/L (2.5–7.0) serum creatinine 101 µmol/L (60–110) serum corrected calcium 2.48 mmol/L (2.20–2.60) serum phosphate 1.10 mmol/L (0.80–1.45) serum albumin 42 g/L (37–49) serum urate 0.41 mmol/L (0.23–0.46) 24-h urinary calcium 11.8 mmol (2.5–7.5) 24-h urinary urate 3.2 mmol (<3.6) 24-h urinary oxalate 0.36 mmol (0.14–0.46) plasma parathyroid hormone 4.2 pmol/L (0.9–5.4) What intervention is most likely to reduce the risk of further renal stones? A dietary calcium restriction B indapamide C oral allopurinol D urine acidification E urine alkalinisation EXPLANATION: This man has hypercalciuric renal stone disease. Indapamide, like a thiazide, is effective in decreasing calciuria in patients with urinary stone disease with hypercalciuria.


61. A 56-year-old woman was discussed at a thyroid cancer multidisciplinary team meeting. She had a recent diagnosis of follicular carcinoma of the thyroid (stage pT3, N1a) following a right thyroid lobectomy. Her past medical history included breast cancer, endometrial cancer and gangliocytoma of the cerebellum. The referrer had noted visible papules on her face and neck. A defect of which gene is associated with the most likely diagnosis? A PRKAR1A B PTEN C RET D SDHB E VHL EXPLANATION: The combination of thyroid, breast and endometrial cancer suggests Cowden syndrome (multiple hamartoma syndrome); skin fibromas may also be part of the condition. It is caused by PTEN mutation. Cerebellar gangliocytoma is also associated with PTEN mutation.


62. A 64-year-old man presented with a painless, rapidly enlarging, right-sided neck mass. He reported a feeling of pressure on swallowing, and his voice had become quieter over the previous 3 weeks. On examination, a 6 × 5-cm right-sided neck mass was palpable, contiguous with the right thyroid lobe. There was no palpable lymphadenopathy. A diagnosis of anaplastic carcinoma, consistent with a primary origin in the thyroid (Thy5) was made. Subsequently, a CT scan of neck, chest and abdomen was performed, which showed a 5 × 6 × 4-cm right-sided thyroid nodule invading strap muscles anterolaterally and merging into tracheal cartilage medially; there was no cervical lymphadenopathy and lungs, liver and bones were normal. What percentage of patients with this stage of disease survive for 12 months? A <5 B 10–20 C 25–35 D 40–50 E >60 EXPLANATION: Anaplastic carcinoma of thyroid has a poor prognosis; 12-month survival is unlikely but may occur.


63. A 26-year-old man presented with longstanding intermittent burning, tingling and numbness in his hands and feet, associated with bouts of fever with temperatures of up to 40.0°C. He had recurrent abdominal pain associated with nausea and diarrhoea. He had also noticed hearing difficulties, heat intolerance and decreased sweating. On examination, he was afebrile, his pulse was 72 beats per minute and his BP was 125/60 mmHg. Temperature sensation was absent over the dorsal surfaces of his hands and forearms, as well as below his knees. Sensorineural hearing loss was noted. Fundoscopic examination showed tortuous retinal blood vessels. Urinalysis showed protein 3+. Investigations showed normal haematology and biochemistry. Chest X-ray, electromyography and nerve conduction velocity studies were normal. An ECG showed left ventricular hypertrophy. Which investigation is most likely to confirm the diagnosis? A CSF for oligoclonal band B renal biopsy C serum angiotensin-converting enzyme D serum homocysteine E white cell α-galactosidase A EXPLANATION: The curriculum covers inborn errors of metabolism and, as such, you need to know the more common of these rare conditions. Usually, the clinical data will be ‘classical’ and are not trying to catch you out. This particular question is a two-step question – first make the diagnosis (here it is Fabry disease) and then identify the diagnostic test. [


64. A 29-year-old woman with an 18-year history of type 1 diabetes mellitus complained of frequent nausea and vomiting. A nuclear medicine gastric emptying study demonstrated delayed gastric emptying. What is the most appropriate treatment? A alternating erythromycin and cyclizine B aprepitant C erythromycin alone D gastric pacing E ondansetron

EXPLANATION: This is a two-step question. Firstly, you need to make the diagnosis, and here it is gastroparesis. Then you need to consider the most likely best treatment. All of the options given are plausible and have been used in treating the condition, but the key term here is ‘most appropriate’. Of the options given, alternating erythromycin and cyclizine is the best initial treatment.


65. A 76-year-old woman was admitted with a 3-day history of fever, rigors, increased urinary frequency and dysuria. She had type 2 diabetes mellitus with previous good glycaemic control with dietary control alone. On examination, her temperature was 38.6°C, her pulse was 114 beats per minute and her BP was 104/52 mmHg lying down. Urinalysis showed protein 1+, leukocytes 2+ and ketones 1+. She was administered sodium chloride 0.9% 1 L intravenously over 1 hour. Investigations (on admission): serum sodium 162 mmol/L (137–144) serum potassium 3.6 mmol/L (3.5–4.9) serum bicarbonate 22 mmol/L (20–28) serum urea 13 mmol/L (2.5–7.0) serum creatinine 150 µmol/L (60–110) eGFR (MDRD) 45 mL/min/1.73 m2 (>60) random plasma glucose 40 mmol/L capillary blood ketones 0.9 mmol/L (<0.3) What is the most appropriate next step in management? A insulin infusion 0.05 units/kg/h with intravenous sodium chloride 0.9% B insulin infusion 0.1 units/kg/h with intravenous sodium chloride 0.9% C insulin infusion 0.1 units/kg/h with intravenous glucose 10% D intravenous sodium chloride 0.45% without insulin infusion E intravenous sodium chloride 0.9% without insulin infusion EXPLANATION: Here, the diagnosis is hyperosmolar hyperglycaemic state meaning that the patient should be treated with intravenous sodium chloride 0.9% on its own until the glucose stops falling, and only then add fixed-rate intravenous insulin (at 0.5 units/kg/h). There are many ‘guideline-based questions’ in the examination, and candidates should check the exam website to see a list of appropriate reading.


66. A 55-year-old woman underwent a CT scan of abdomen and was incidentally noted to have a 2.7-cm smooth round mass in her right adrenal gland. She had been treated for hypertension since the age of 52. Arrangements were made for measurement of plasma aldosterone and plasma renin activity. Her antihypertensive therapy was reviewed to avoid interference in the investigation of the adrenal mass. Which therapy is most likely to allow reliable assessment of her renin and aldosterone concentrations? A amiloride B atenolol C bendroflumethiazide D doxazosin E lisinopril

EXPLANATION: The purpose of this question is to be familiar with the different classes of antihypertensive agents that can interfere with renin or aldosterone concentrations. There are other questions that ask this but not about antihypertensive agents.


67. A 65-year-old man with a 47-year history of type 1 diabetes mellitus was seen in the foot clinic. He gave a 3-week history of a dull ache in his right foot. He had sustained no trauma to his foot. At his most recent annual review, he had admitted to a 6-year history of worsening gustatory sweating and symptoms of postural hypotension. On examination, the right foot was 3.2°C warmer than the left foot. Investigations: haemoglobin A1c 90 mmol/mol (20–42) urinary albumin:creatinine ratio 18.7 mg/mmol (<2.5) X-ray of right foot calcified blood vessels only; no bony abnormality MR scan of right foot changes consistent with early Charcot’s joint What is the most appropriate next step in management? A ‘backslab’ plaster cast B removable ‘aircast’ boot C total contact plaster cast D urgent assessment by orthotist E urgent referral to orthopaedic surgeon EXPLANATION: Here, the purpose is to test your knowledge of ‘best practice’. The diagnosis is given to you, and although this treatment may not be available at your centre, the question looks for the understanding that immobilisation is the best treatment in this condition, and that the best way to achieve this is using a total contact cast.


68. A 56-year-old woman presented with a swelling in her neck, which she had noticed by chance 6 weeks previously. It had not changed in size since she had first noticed it, and she was completely asymptomatic. On examination, there was a smooth nodule in the thyroid that moved up on swallowing. There was no lymphadenopathy. Investigations: serum thyroid-stimulating hormone 2.8 mU/L (0.4–5.0) ultrasound scan of thyroid single 2.7-cm nodule in upper left lobe

(U2 classification) What is the most appropriate next step in management? A CT scan of neck B hemithyroidectomy C perform fine-needle aspiration biopsy D reassure and return to primary care E repeat ultrasound scan in 3 months EXPLANATION: The purpose of this question is to test your knowledge of the current ultrasound classification for the management of thyroid nodules. http://onlinelibrary.wiley.com/doi/10.1111/cen.12515/full


69. A 17-year-old girl with idiopathic isolated GH deficiency was referred from the paediatric endocrinologist to the adult endocrinology clinic. She had no other chronic diseases. In the past 12 months, her growth velocity had been less than 2 cm per year. She had had her first menstrual period at the age of 15. She was taking somatropin 1.5 mg daily. On examination, her height was 169 cm. What is the most appropriate next step in management? A continue somatropin at the same dose B increase somatropin dose C measure serum IGF-1 D MR scan of pituitary E reassessment of GH status

EXPLANATION: The purpose of this question is to be familiar with the national and international guidelines on the management of GH deficiency in childhood and how it should be reassessed. https://academic.oup.com/jcem/article-lookup/doi/10.1210/jc.2011-0179


European Board Examination in Endocrinology, Diabetes and … sample questions FINAL 2019.pdf ·...

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