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質問 1：
A 26-year-old woman was urgently referred to clinic with a 6-week history of retroorbital headaches and deteriorating vision. Her past medical history was unremarkable, although on questioning she admitted that she had recently found it increasingly difficult to cope with her busy job.
On examination, her pulse was 60 beats per minute and regular, and her blood pressure was 110/75 mmHg lying and 90/60 mmHg standing. She was pale and had dry skin. Visual acuities were reduced (6/12 right; 6/24 left), and she had a bitemporal inferior quadrantanopia.
Investigations:
serum sodium132 mmol/L (137-144) serum potassium4.0 mmol/L (3.5-4.9)
short tetracosactide Synacthen@ test (250 micrograms): serum cortisol (30 min after tetracosactide)185 nmol/L (>550)
plasma follicle-stimulating hormone2.7 U/L plasma luteinising hormone3.5 U/L serum prolactin1050 mU/L (<360) serum thyroid-stimulating hormone0.3 mU/L (0.4-5.0) serum free T48.0 pmol/L (10.0-22.0)
serum insulin-like growth factor 14.7 nmol/L (7.5-37.3)
MR scan of brainsee image
What is the most likely diagnosis?
A. craniopharyngioma
B. prolactinoma
C. non-functioning pituitary adenoma
D. Rathke's cleft cyst
E. autoimmune hypophysitis
正解：A
質問 2：
A 20-year-old woman with Turner's syndrome had heard that there was a risk of ovarian cancer associated with Turner's syndrome. She asked to undergo a pre-emptive oophorectomy.
For what genotype is oophorectomy most likely to be recommended?
A. 45X/46XX
B. 45X/47XXX
C. 45XO
D. 45X/46XY
E. 45X/46Xxi (Xq)
正解：D
質問 3：
A 16-year-old boy was referred with concern about delayed puberty. His stature had been short as a child. He reported an increase in height at the age of 13, and had begun to develop pubic hair at the age of 14. He reported no further growth or development in the past year. His father recalled going through puberty at the age of 13.
On examination, his height was 1.60 m (between 0.4th and 2nd centile), weight was 56.4 kg (between 9th and 25th centile), genital development was Tanner stage 2 and testicular volume was 8 mL bilaterally. Pubic hair was Tanner stage 2, with no evidence of androgenic axillary hair. Bone age at the left wrist was 13.5 years.
Investigations:
serum testosterone2.9 nmol/L (9.0-35.0)
Which feature in his clinical presentation most strongly suggests a diagnosis other than constitutional delay?
A. being below the 2nd centile for height
B. failure to progress through puberty
C. absence of axillary hair in the presence of pubic hair
D. discordance between the height centile and the weight centile
E. 2.5-year delay in bone age
正解：B
質問 4：
A 17-year-old girl presented with primary amenorrhoea. She had grown and developed normally. There was no history of galactorrhoea or hirsutism.
On examination, her height was 1.69 m, her weight was 68.3 kg, and her body mass index was 23.9 kg/m2 (18-25). She had stage 5 breast development and stage 5 pubic hair. Her visual fields were full to confrontation.
Investigations:
serum cortisol (09.00 h)416 nmol/L (200-700) serum oestradiol51 pmol/L (200-400)
serum follicle-stimulating hormone0.8 U/L (2.5-10.0) serum luteinising hormone1.2 U/L (2.5-10.0) serum thyroid-stimulating hormone1.2 mU/L (0.4-5.0) serum free T415.6 pmol/L (10.0-22.0)
What is the most appropriate next investigation?
A. luteinising hormone-releasing hormone test
B. karyotyping
C. short tetracosactide (Synacthen@) test
D. serum prolactin
E. serum insulin-like growth factor 1
正解：D
質問 5：
A 48-year-old man presented with gynaecomastia. His serum oestradiol was increased and a CT scan of adrenal glands revealed a 13-cm tumour of the left adrenal gland. Further workup showed increased secretion of 17-hydroxyprogesterone, cortisol and androstenedione. A diagnosis of adrenocortical carcinoma was suspected.
Investigations:
staging CT scan of chest and abdomenno evidence of metastasis
What is the most appropriate next step in management?
A. adrenal fine-needle biopsy
B. left adrenalectomy followed by adjuvant mitotane treatment
C. MR scan of adrenal glands with chemical shift analysis
D. left adrenalectomy followed by adjuvant combination chemotherapy
E. iodocholesterol scan
正解：B
A 26-year-old woman was urgently referred to clinic with a 6-week history of retroorbital headaches and deteriorating vision. Her past medical history was unremarkable, although on questioning she admitted that she had recently found it increasingly difficult to cope with her busy job.
On examination, her pulse was 60 beats per minute and regular, and her blood pressure was 110/75 mmHg lying and 90/60 mmHg standing. She was pale and had dry skin. Visual acuities were reduced (6/12 right; 6/24 left), and she had a bitemporal inferior quadrantanopia.
Investigations:
serum sodium132 mmol/L (137-144) serum potassium4.0 mmol/L (3.5-4.9)
short tetracosactide Synacthen@ test (250 micrograms): serum cortisol (30 min after tetracosactide)185 nmol/L (>550)
plasma follicle-stimulating hormone2.7 U/L plasma luteinising hormone3.5 U/L serum prolactin1050 mU/L (<360) serum thyroid-stimulating hormone0.3 mU/L (0.4-5.0) serum free T48.0 pmol/L (10.0-22.0)
serum insulin-like growth factor 14.7 nmol/L (7.5-37.3)
MR scan of brainsee image
What is the most likely diagnosis?
A. craniopharyngioma
B. prolactinoma
C. non-functioning pituitary adenoma
D. Rathke's cleft cyst
E. autoimmune hypophysitis
正解：A
質問 2：
A 20-year-old woman with Turner's syndrome had heard that there was a risk of ovarian cancer associated with Turner's syndrome. She asked to undergo a pre-emptive oophorectomy.
For what genotype is oophorectomy most likely to be recommended?
A. 45X/46XX
B. 45X/47XXX
C. 45XO
D. 45X/46XY
E. 45X/46Xxi (Xq)
正解：D
質問 3：
A 16-year-old boy was referred with concern about delayed puberty. His stature had been short as a child. He reported an increase in height at the age of 13, and had begun to develop pubic hair at the age of 14. He reported no further growth or development in the past year. His father recalled going through puberty at the age of 13.
On examination, his height was 1.60 m (between 0.4th and 2nd centile), weight was 56.4 kg (between 9th and 25th centile), genital development was Tanner stage 2 and testicular volume was 8 mL bilaterally. Pubic hair was Tanner stage 2, with no evidence of androgenic axillary hair. Bone age at the left wrist was 13.5 years.
Investigations:
serum testosterone2.9 nmol/L (9.0-35.0)
Which feature in his clinical presentation most strongly suggests a diagnosis other than constitutional delay?
A. being below the 2nd centile for height
B. failure to progress through puberty
C. absence of axillary hair in the presence of pubic hair
D. discordance between the height centile and the weight centile
E. 2.5-year delay in bone age
正解：B
質問 4：
A 17-year-old girl presented with primary amenorrhoea. She had grown and developed normally. There was no history of galactorrhoea or hirsutism.
On examination, her height was 1.69 m, her weight was 68.3 kg, and her body mass index was 23.9 kg/m2 (18-25). She had stage 5 breast development and stage 5 pubic hair. Her visual fields were full to confrontation.
Investigations:
serum cortisol (09.00 h)416 nmol/L (200-700) serum oestradiol51 pmol/L (200-400)
serum follicle-stimulating hormone0.8 U/L (2.5-10.0) serum luteinising hormone1.2 U/L (2.5-10.0) serum thyroid-stimulating hormone1.2 mU/L (0.4-5.0) serum free T415.6 pmol/L (10.0-22.0)
What is the most appropriate next investigation?
A. luteinising hormone-releasing hormone test
B. karyotyping
C. short tetracosactide (Synacthen@) test
D. serum prolactin
E. serum insulin-like growth factor 1
正解：D
質問 5：
A 48-year-old man presented with gynaecomastia. His serum oestradiol was increased and a CT scan of adrenal glands revealed a 13-cm tumour of the left adrenal gland. Further workup showed increased secretion of 17-hydroxyprogesterone, cortisol and androstenedione. A diagnosis of adrenocortical carcinoma was suspected.
Investigations:
staging CT scan of chest and abdomenno evidence of metastasis
What is the most appropriate next step in management?
A. adrenal fine-needle biopsy
B. left adrenalectomy followed by adjuvant mitotane treatment
C. MR scan of adrenal glands with chemical shift analysis
D. left adrenalectomy followed by adjuvant combination chemotherapy
E. iodocholesterol scan
正解：B
Mahiro -
勉強はちょっと大変だと思います。
でも最後まで頑張りました。合格できるのは何よりです。