[MCQ] Disorders of the Adrenal Cortex- Part 5

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Disorders of the Adrenal Cortex- Part 5

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Question 1

Rapid ACTH stimulation test is performed ?

A	Early morning
B	Afternoon
C	Late evening
D	Any time of the day

Question 1 Explanation:

Rapid ACTH stimulation test can be performed at any time of the day. It involves administration of 25 units (0.25 mg) of cosyntropin IV or IM & measurement of plasma cortisol levels before administration, 30 & 60 minutes after administration

Question 2

Criterion for a normal response in “Rapid ACTH stimulation test” is a cortisol level of ?

A	>8 μg/dL above baseline
B	>12 μg/dL above baseline
C	>16 μg/dL above baseline
D	>18 μg/dL above baseline

Question 2 Explanation:

Criterion for a normal rapid ACTH stimulation test response is a stimulated cortisol level of >500 nmol/L (>18 μg/dL), and the minimal stimulated normal increment of cortisol is >200 nmol/L (>7 g/ dL) above baseline

Question 3

Normally, in screening ‘overnight dexamethasone suppression test’, plasma cortisol level at 8 AM should be ?

A	< 5 μg/dL
B	< 10 μg/dL
C	< 15 μg/dL
D	< 20 μg/dL

Question 3 Explanation:

The best screening procedure to test pituitary-adrenal suppressibility is the overnight dexamethasone suppression test. Measurement of plasma cortisol levels at 8 AM following oral administration of 1 mg dexamethasone the previous midnight is

Question 4

Cushing’s disease refers to ?

A	ACTH-producing pituitary tumor
B	Exogenous ACTH tumor
C	Pituitary ACTH-secreting tumor
D	All of the above

Question 5

Cushing’s syndrome refers to ?

A	Exogenous ACTH tumor
B	Adrenal tumor
C	Pituitary ACTH-secreting tumor
D	All of the above

Question 5 Explanation:

Traditionally, only basophilic ACTH-producing pituitary tumor is defined as Cushing’s disease. Cushing’s syndrome refers to all causes of excess cortisol like exogenous ACTH tumor, adrenal tumor, basophilic pituitary ACTH-secreting tumor, or excessive glucocorticoid treatment

Question 6

Which of the following is false about ‘Ectopic ACTH syndrome’ ?

A	Caused by nonpituitary tumors secreting ACTH /CRH
B	Bilateral adrenal hyperplasia
C	Typical s/s of Cushing’s syndrome present
D	Hypokalemic alkalosis

Question 6 Explanation:

Ectopic ACTH syndrome is caused by nonpituitary tumors that secrete either ACTH and/or CRH & cause bilateral adrenal hyperplasia. Ectopic production of CRH results in clinical, biochemical, and radiologic features indistinguishable from those caused by hypersecretion of pituitary ACTH. Typical signs & symptoms of Cushing’s syndrome may be absent or minimal with ectopic ACTH production & hypokalemic alkalosis is a prominent manifestation.

Question 7

Which of the following can cause ‘Ectopic ACTH syndrome’ ?

A	Oat cell bronchogenic carcinoma
B	Carcinoid tumors of thymus, pancreas, ovary
C	Medullary carcinoma of thyroid
D	All of the above

Question 7 Explanation:

Most cases of Ectopic ACTH syndrome are associated with primitive small cell (oat cell) bronchogenic carcinoma, carcinoid tumors of thymus, pancreas, ovary, medullary carcinoma of thyroid, or bronchial adenomas.

Question 8

Which of the following is a cause of ACTH-independent Cushing’s syndrome ?

A	ACTH-independent macronodular hyperplasia (AIMAH)
B	Primary pigmented nodular adrenal disease (PPNAD)
C	McCune-Albright syndrome
D	All of the above

Question 8 Explanation:

Mineralocorticoids are the major determinants of potassium metabolism mediated by the binding of aldosterone to MR in epithelial cells (principal cells in renal cortical collecting duct).

Question 9

In ACTH-independent macronodular hyperplasia (AIMAH), which of the following receptor is ectopically expressed in the adrenal gland ?

A	Luteinizing hormone
B	Vasopressin
C	Serotonin
D	All of the above

Question 9 Explanation:

In ACTH-independent macronodular hyperplasia (AIMAH), adrenal cortisol excess occurs due to ectopic expression of receptors not usually found in adrenals. These include receptors for luteinizing hormone, vasopressin, serotonin, interleukin-1, or gastric inhibitory peptide (GIP). Activation of these receptors results in upregulation of PKA signaling with a subsequent increase in cortisol production

Question 10

Primary pigmented nodular adrenal disease (PPNAD) is best related to which of the following ?

A	Crow-Fukase Syndrome
B	Hirschsprung disease
C	Carney’s complex
D	Von Hippel–Lindau syndrome

Question 10 Explanation:

Mutations in a regulatory subunit of PKA (PRKAR1A) are found in primary pigmented nodular adrenal disease (PPNAD) as part of Carney’s complex.

Question 11

Which of the following is a feature of Carney’s complex ?

A	Cardiac myxomas
B	Hyperlentiginosis
C	Sertoli’s cell tumors
D	All of the above

Question 11 Explanation:

Carney’s complex is an autosomal dominant multiple neoplasia condition associated with cardiac myxomas, hyperlentiginosis, Sertoli’s cell tumors, and PPNAD.

Question 12

McCune-Albright syndrome is caused by activating mutations in which of the following ?

A	GNAS-1
B	PKA
C	CREB
D	StAR

Question 12 Explanation:

McCune-Albright syndrome can cause ACTH-independent Cushing’s syndrome. It features polyostotic fibrous dysplasia, unilateral café-au-lait spots, and precocious puberty. McCune-Albright syndrome is caused by activating mutations in GNAS-1 (guanine nucleotide binding protein alpha stimulating activity polypeptide 1).

Question 13

Which of the following is the commonest feature of ‘Cushing’s syndrome’ ?

A	Centripetal obesity
B	Hypertension
C	Hirsutism
D	Proximal myopathy

Question 14

Which of the following is suggestive of Cushing’s syndrome ?

A	Urine free cortisol > 50 μg/day
B	Plasma cortisol > 5 μg/dL after standard low-dose dexamethasone suppression test
C	Absence of normal fall of plasma cortisol at midnight
D	All of the above

Question 15

Most common cause of Cushing’s syndrome is ?

A	Iatrogenic administration of steroids
B	Adrenal macronodular hyperplasia
C	Adrenal micronodular dysplasia
D	Adrenal neoplasia

Question 15 Explanation:

The most common cause of Cushing’s syndrome is iatrogenic administration of steroids.

Question 16

Decreased bone mineralization due to use of steroids is particularly pronounced in ?

A	Children
B	Adults
C	Elderly
D	All of the above

Question 16 Explanation:

Decreased bone mineralization is particularly pronounced in children.

Question 17

Which of the following is typical of Cushing’s syndrome ?

A	Moon facies
B	Buffalo hump
C	Truncal obesity
D	All of the above

Question 17 Explanation:

Hypercortisolism promotes deposition of adipose tissue in upper face (moon facies), interscapular area (buffalo hump), supraclavicular fat pads & mesenteric bed (truncal obesity). Rarely, episternal fatty tumors and mediastinal widening secondary to fat accumulation occur.

Question 18

Which of the following features of Cushing’s syndrome is considered more specific ?

A	Hypertension
B	Osteoporosis
C	Broad violaceous cutaneous striae
D	Obesity

Question 18 Explanation:

Signs & symptoms of hypercortisolism (obesity, hypertension, osteoporosis & diabetes) are nonspecific. Easy bruising, typical striae, myopathy & virilizing signs are more suggestive of Cushing’s syndrome.

Question 19

What value of a 24-hour urine free cortisol is suggestive of Cushing’s syndrome ?

A	> 5 μg/day
B	> 25 μg/day
C	> 50 μg/day
D	> 150 μg/day

Question 19 Explanation:

A 24-hour urine free cortisol level of >140 nmol/day (50 μg/day) is suggestive of Cushing’s syndrome

Question 20

In Cushing’s syndrome, after a standard low-dose dexamethasone suppression test, urinary cortisol to fall to ?

A	< 10 μg/day
B	< 20 μg/day
C	< 30 μg/day
D	< 40 μg/day

Question 21

In Cushing’s syndrome, after a standard low-dose dexamethasone suppression test, plasma cortisol to fall to ?

A	< 5 μg/dL
B	< 8 μg/dL
C	< 10 μg/dL
D	< 16 μg/dL

Question 21 Explanation:

In Cushing’s syndrome, definitive diagnosis is established by failure of urinary cortisol to fall to <25 nmol/day (10 μg/day) or of plasma cortisol to fall to <140 nmol/L (5 μg/dL) after a standard lowdose dexamethasone suppression test (0.5 mg every 6 hour for 48 hours). High-dose dexamethasone administration means 2 mg every 6 hour for 48 hours or 8-mg overnight.

Question 22

Normal plasma ACTH level is ?

A	< 60 pg/mL
B	< 80 pg/mL
C	< 100 pg/mL
D	< 120 pg/mL

Question 23

Features suggestive of adrenocortical carcinoma (ACC) include all except ?

A	Size > 4 - 6 cm
B	Soft tissue calcifications visible on CT
C	Tumor inhomogeneity
D	Low unenhanced CT values (<10 HU)

Question 23 Explanation:

Features suggestive of malignancy include large size (>4 - 6 cm), irregular margins & tumor inhomogeneity, soft tissue calcifications visible on CT & high unenhanced CT attenuation values (>10 HU).

Question 24

Principal drug for the treatment of adrenocortical carcinoma (ACC) is ?

A	Mitotane
B	Ketoconazole
C	Mifepristone
D	Metyrapone

Question 24 Explanation:

Principal drug for treatment of ACC is mitotane (o,p’-DDD), isomer of DDT.

Question 25

Chemical adrenalectomy can be done by ?

A	Ketoconazole
B	Mitotane
C	Metyrapone
D	All of the above

Question 25 Explanation:

Chemical adrenalectomy can be done with ketoconazole or mitotane or aminoglutethimide or metyrapone or Mifepristone. These are not curative but effective as long as chronically administered in selected patients.

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See all quizzes of Disorders of the Adrenal Cortex at here:

1.Rapid ACTH stimulation test is performed ?
A. Early morning
B. Afternoon
C. Late evening
D. Any time of the day

2.Criterion for a normal response in “Rapid ACTH stimulation test”
is a cortisol level of ?
A. >8 μg/dL above baseline
B. >12 μg/dL above baseline
C. >16 μg/dL above baseline
D. >18 μg/dL above baseline
3. Normally, in screening ‘overnight dexamethasone suppression
test’, plasma cortisol level at 8 AM should be ?
A. < 5 μg/dL
B. < 10 μg/dL
C. < 15 μg/dL
D. < 20 μg/dL

4. Cushing’s disease refers to ?
A. ACTH-producing pituitary tumor
B. Exogenous ACTH tumor
C. Pituitary ACTH-secreting tumor
D. All of the above
5. Cushing’s syndrome refers to ?
A. Exogenous ACTH tumor
B. Adrenal tumor
C. Pituitary ACTH-secreting tumor
562 MCQ’s FOR MEDICAL PROFESSIONALS BY PROF. AJAY MATHUR Endocrinology
D. All of the above

6. Which of the following is false about ‘Ectopic ACTH syndrome’ ?
A. Caused by nonpituitary tumors secreting ACTH /CRH
B. Bilateral adrenal hyperplasia
C. Typical s/s of Cushing’s syndrome present
D. Hypokalemic alkalosis

7. Which of the following can cause ‘Ectopic ACTH syndrome’ ?
A. Oat cell bronchogenic carcinoma
B. Carcinoid tumors of thymus, pancreas, ovary
C. Medullary carcinoma of thyroid
D. All of the above

8. Which of the following is a cause of ACTH-independent Cushing’s
syndrome ?
A. ACTH-independent macronodular hyperplasia (AIMAH)
B. Primary pigmented nodular adrenal disease (PPNAD)
C. McCune-Albright syndrome
D. All of the above
9. In ACTH-independent macronodular hyperplasia (AIMAH), which
of the following receptor is ectopically expressed in the adrenal
gland ?
A. Luteinizing hormone
B. Vasopressin
C. Serotonin
D. All of the above

10.Primary pigmented nodular adrenal disease (PPNAD) is best related
to which of the following ?
A. Crow-Fukase Syndrome
B. Hirschsprung disease
C. Carney’s complex
D. Von Hippel–Lindau syndrome

11. Which of the following is a feature of Carney’s complex ?
A. Cardiac myxomas
B. Hyperlentiginosis
C. Sertoli’s cell tumors
D. All of the above

12. McCune-Albright syndrome is caused by activating mutations in
which of the following ?
A. GNAS-1
B. PKA
C. CREB
D. StAR

13. Which of the following is the commonest feature of ‘Cushing’s
syndrome’ ?
A. Centripetal obesity
B. Hypertension
C. Hirsutism
D. Proximal myopathy
14. Which of the following is suggestive of Cushing’s syndrome ?
A. Urine free cortisol > 50 μg/day
B. Plasma cortisol > 5 μg/dL after standard low-dose
dexamethasone suppression test
C. Absence of normal fall of plasma cortisol at midnight
D. All of the above
15. Most common cause of Cushing’s syndrome is ?
A. Iatrogenic administration of steroids
B. Adrenal macronodular hyperplasia
C. Adrenal micronodular dysplasia
D. Adrenal neoplasia

16. Decreased bone mineralization due to use of steroids is
particularly pronounced in ?
A. Children
B. Adults
C. Elderly
D. All of the above

17. Which of the following is typical of Cushing’s syndrome ?
A. Moon facies
B. Buffalo hump
C. Truncal obesity
D. All of the above

18. Which of the following features of Cushing’s syndrome is
considered more specific ?
A. Hypertension
B. Osteoporosis
C. Broad violaceous cutaneous striae
D. Obesity

19. What value of a 24-hour urine free cortisol is suggestive of
Cushing’s syndrome ?
A. > 5 μg/day
B. > 25 μg/day
C. > 50 μg/day
D. > 150 μg/day

20. In Cushing’s syndrome, after a standard low-dose dexamethasone
suppression test, urinary cortisol to fall to ?
A. < 10 μg/day
B. < 20 μg/day
C. < 30 μg/day
D. < 40 μg/day
21. In Cushing’s syndrome, after a standard low-dose dexamethasone
suppression test, plasma cortisol to fall to ?
A. < 5 μg/dL
B. < 8 μg/dL
C. < 10 μg/dL
D. < 16 μg/dL

22. Normal plasma ACTH level is ?
A. < 60 pg/mL
B. < 80 pg/mL
C. < 100 pg/mL
D. < 120 pg/mL

23. Features suggestive of adrenocortical carcinoma (ACC) include
all except ?
A. Size > 4 – 6 cm
B. Soft tissue calcifications visible on CT
C. Tumor inhomogeneity
D. Low unenhanced CT values (<10 HU)

24. Principal drug for the treatment of adrenocortical carcinoma
(ACC) is ?
A. Mitotane
B. Ketoconazole
C. Mifepristone
D. Metyrapone

25. Chemical adrenalectomy can be done by ?
A. Ketoconazole
B. Mitotane
C. Metyrapone
D. All of the above

Disorders of the Adrenal Cortex

[MCQ] Disorders of the Adrenal Cortex- Part 5

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Disorders of the Adrenal Cortex- Part 5

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