Learn with MRCPUK : SEND training material for 100% pass

A 50-year-old woman with acromegaly presented with persistent sweating and headaches
despite having undergone trans-sphenoidal surgery and pituitary radiotherapy 2 years
previously. She had been intolerant of treatment with octreotide.
Investigations:
serum growth hormone11.1 ?g/L (<0.4)
serum insulin-like growth factor 186.2 nmol/L (5.6-23.3)
Following imaging, it was judged that there was no role for repeat surgery. She was treated
with pegvisomant 10 mg. Six months into treatment, her symptoms had improved.
Investigations (6 months later):
serum growth hormone20.3 ?g/L (<0.4)
serum insulin-like growth factor 115.2 nmol/L (5.6-23.3)
What is the most appropriate next step in management?

• A. arrange another full course of pituitary radiotherapy
• B. increase dosage of pegvisomant
• C. add cabergoline
• D. continue present dosage of pegvisomant
• E. stop pegvisomant

A 55-year-old dental nurse was referred for bone densitometry after sustaining a non-displaced fracture of the femur after falling down a step. She had experienced no other fractures. Her only medical problem was long-standing anaemia of unknown cause. The only family history was of persistent dental abscesses affecting her father. She had three children who were well. She was taking no medication.
Examination was normal.
Investigations:
haemoglobin102 g/L (115-165)
MCV85 fL (80-96)
white cell count6.0 ? 109/L (4.0-11.0)
platelet count245 ? 109/L (150-400)
erythrocyte sedimentation rate25 mm/1st h (<30)
serum creatinine85 umol/L (60-110)
serum corrected calcium2.40 mmol/L (2.20-2.60)
serum alkaline phosphatase56 U/L (45-105)
DXA scan spine (L2-L4)T score +5.8
DXA scan total hipT score +5.4
What is the most likely diagnosis?

• A. osteopetrosis
• B. Paget's disease
• C. high bone mass phenotype
• D. myelodysplasia
• E. fluorosis

A 67-year-old woman presented to her general practitioner with a swelling in her neck. It had been present for 4-5 years and had not changed in size during that time. She was completely asymptomatic and remained well.
On examination, there was a nodular goitre and no lymphadenopathy.
Investigations:
serum thyroid-stimulating hormone1.1 mU/L (0.4-5.0)
A subsequent ultrasound scan demonstrated seven nodules bilaterally (ranging in size from 5 mm to 15 mm), which had no suspicious features.
What is the most appropriate next step in management according to British Thyroid Association 2014 Guidelines for the Management of Thyroid Cancer?

• A. reassure and discharge
• B. radioactive iodine
• C. subtotal thyroidectomy
• D. levothyroxine 100 micrograms daily
• E. fine-needle aspiration of largest nodule

A 32-year-old woman, with a 22-year history of type 1 diabetes mellitus, was seen in a pre-pregnancy diabetes clinic. She was a primigravida.
On examination, she had early background retinopathy, her blood pressure was 128/68 mmHg, and her body mass index was 29.7 kg/m2 (18-25).
Investigations:
haemoglobin A1c56 mmol/mol (20-42)
urinary albumin:creatinine ratio1.2 mg/mmol (<3.5)
Over the years her haemoglobin A1c concentration had varied between 58 and 69 mmol/mol. She had impaired awareness of hypoglycaemia and experienced approximately two severe hypoglycaemic events per year. She was worried about the risk of severe congenital malformations in her baby.
To what extent will the average risk of severe congenital malformation be increased in infants born to this mother with pregestational diabetes?

• A. eight-fold
• B. four-fold
• C. two-fold
• D. six-fold
• E. ten-fold

A 17-year-old boy, with short stature, obesity and neurobehavioural problems, was referred because of cold intolerance.
On examination, he and his mother had similar body habitus and short fingers (brachydactyly).
Investigations (before attending clinic):
serum sodium143 mmol/L (137-144) serum potassium4.4 mmol/L (3.5-4.9) serum creatinine93 umol/L (60-110) serum corrected calcium2.02 mmol/L (2.20-2.60) serum phosphate1.7 mmol/L (0.8-1.4)
serum thyroid-stimulating hormone16.0 mU/L (0.4-5.0) serum free T410.0 pmol/L (10.0-22.0) plasma parathyroid hormone27.0 pmol/L (0.9-5.4)
His mother's blood tests were all normal.
What is the most likely diagnosis in this boy?

• A. McCune-Albright syndrome
• B. pseudohypoparathyroidism
• C. polyglandular autoimmune syndrome type 1
• D. pseudopseudohypoparathyroidism
• E. DiGeorge syndrome