Cushing's syndrome!

I saw this lady during my medical clinic follow up this morning.

I think all of you certainly can pick out what is the underlying diagnosis for this lady. You are right, it is Cushing’s syndrome. If you want to know more about Harvey William Cushing (1869-1939), you can find his story here!

There are always questions about Cushing’s syndrome in MRCP Part 1 and 2 simply because it is a common disease in clinical practice. Some of the questions I have about Cushing’s syndrome are as follow,

1) This 34 year old female( above picture) presents with a nine
month history of weight gain, weakness and
amenorrhoea. Examination reveals the appearances
as shown, a blood pressure of 180/110 mmHg and
proximal myopathy. Urinalysis shows ++ glucose
and a pregnancy test is negative.

Which of the following would be the most
appropriate diagnostic test?

1 )Random Cortisol concentration
2 )Oestradiol concentration
3 )Oral glucose tolerance test
4 )Pituitary MRI scan
5 )Urine free cortisol estimation

ASWER: 5

2) A 22 year old female presents with a one year
history of secondary amenorrhoea and a 5 year
history of facial hirsutism. Examination reveals
normal female secondary sexual characterisitcs
with mild facial hair and hair extending up to
the umbilicus and tops of thighs. Investigations
reveal an oestradiol concentration of 65 pmol/l
(NR 130 - 450), a LH of 3.2 mU/l (NR 3 -10
mU/l), a FSH of 3.5 (NR 3-10 mU/l), a prolactin
of 320 (NR less than 450 mU/l), a testosterone
of 3.4 pmol/l (NR less than 3). From the
following list, select two investigations that
may provide useful diagnostic information.

1 )17 hydroxyprogesterone (17 OHP) concentration
2 )Androstenedione concentration
3 )Dehydroepiandrostenedione sulphate (DHEAS)
concentration
4 )Karyotype
5 )Pregnancy test
6 )Sex Hormone Binding globulin (SHBG)
concentration
7 )Skin biopsy
8 )Thyroid function tests
9 )Transvaginal ovarian ultrasound scan
10 )Urine free cortisol concentration

ANSWER: 1,10

3) A 38 year old female presents with weight gain,
hirsutism and hypertension of 2 years duration. (above image)
She has also noted oligomenorrhoea over the last
2 years and over the last two months has had no
periods.
Examination reveals a BMI of 32.4, a reddish
complexion, with a blood pressure of 168/98 mmHg
and abdominal striae. She has difficulty rising
from a squatting position.
Investigations reveal the following: Normal
U+Es, FBC Normal, plasma glucose 12.1 mmol/l.
Thyroxine 12.4 nmol/l (NR 9.8 -23.1), TSH 0.85
mU/l (NR 0.5 - 4)
Oestradiol less than 80 pmol/l (NR 130 - 510),
LH 4.2 mU/l (NR 2-10 mU/l) FSH 2.1 mU/l (NR
2-10)
9 am Cortisol 550 nmol/l (NR 200 - 550 ) and
ACTH 45 (NR 8-50)
Midnight Cortisol 420 nmol/l (NR less than 180)
and ACTH 35 (8-20)
24hr Urine free cortisol 580 nmol/d (NR 90 -
290 nmol/d)
Normal chest X-ray, ECG shows LVH
Cortisol at end of low dose dexamethasone test
(48 hrs 0.5 mg qds) = 210 nmol/l
Cortisol at end of high dose dexamthasone test
(48 hrs 2mg qds) =150 nmol/l
MRI of pituitary - Normal
Which of the following apply to this patient?

1 )She is likely to have an adrenal adenoma and
should be confirmed with CT adrenals
2 )She is likely to have ectopic ACTH secretion
and requires CT chest.
3 )She is likely to have pituitary dependent
Cushing's disease and requires Inferior petrosal
sinus sampling with CRF stimulation
4 )She is likely to have ectopic Cushing's
syndrome and should have a labelled octreotide
scan
5 )The results suggest that she has
Pseudo-Cushing's due to polycystic ovarian
syndrome

ANSWER: 3

4) A 73 year old female is diagnosed with Cushing's disease. Which of the following is correct?
1) adrenalectomy would be the treatment of choice.
2 )op-DDD is a treatment if unfit for surgery
3 )Ketoconazole may be used as a treatment if unfit for surgery
4 )Recurrence of Cushing's disease after transphenoidal surgery is less than 5%
5 )yttrium implantation is an effective treatment


ANSWER: 3

Other common questions in the exam include,

1) Relative glucocortocoid effect of different steroid as below,

Cortisol/hyrocortisone
1
prednisolone
4
Dexamethasone
30
Fludrocortisone
10

2) Drugs causing hirsutism include
Minoxidil, pheytoin,cyclosporin and androgens.


Hope this post helps you all!