[MCQ] Disorders of the Adrenal Cortex- Part 7

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Disorders of the Adrenal Cortex- Part 7

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Question 1

Which of the following is given in the treatment of adrenal insufficiency ?

A	Hydrocortisone
B	Fludrocortisone
C	DHEA
D	All of the above

Question 1 Explanation:

Replacement therapy in adrenal insufficiency aims to correct both glucocorticoid & mineralocorticoid deficiencies by hydrocortisone and fludrocortisone respectively. In females with adrenal insufficiency and low androgen levels, DHEA may improve quality of life & bone mineral density.

Question 2

What proportion of total glucocorticoid dose should be given in morning ?

A	One third
B	One half
C	Two third
D	Three fourth

Question 2 Explanation:

Dose of hydrocortisone for most adults is 20 - 30 mg/day. To simulate normal diurnal adrenal rhythm, two-thirds of the dose is taken in morning & remaining one-third is taken in late afternoon.

Question 3

In adrenal insufficiency, during fever, the dose of hydrocortisone should be ?

A	Kept the same
B	Doubled
C	Tripled
D	Quadrupled

Question 3 Explanation:

During periods of intercurrent illness (fever), dose of hydrocortisone should be doubled.

Question 4

In Addison’s disease, mineralocorticoid administration is unnecessary at hydrocortisone doses of ?

A	> 10 mg/day
B	> 40 mg/day
C	> 80 mg/day
D	> 100 mg/day

Question 4 Explanation:

In Addison’s disease, mineralocorticoid administration is unnecessary at hydrocortisone doses of >100 mg/day because of the mineralocorticoid effects of hydrocortisone at such dosages

Question 5

Which of the following favors a diagnosis of primary adrenocortical insufficiency ?

A	Dehydration
B	Hyponatremia
C	Hyperkalemia
D	All of the above

Question 5 Explanation:

Severe dehydration, hyponatremia, and hyperkalemia are characteristic of severe mineralocorticoid insufficiency and favor a diagnosis of primary adrenocortical insufficiency

Question 6

Which of the following is false about secondary adrenocortical insufficiency ?

A	No hyperpigmentation
B	Low ACTH level
C	Near-normal aldosterone secretion
D	None of the above

Question 6 Explanation:

Patients with secondary adrenocortical hypofunction are not hyperpigmented, have low ACTH levels, evidence of multiple hormone deficiencies, have near-normal level of aldosterone secretion

Question 7

Acute adrenocortical insufficiency may result from ?

A	Meningococcal septicemia
B	Anticoagulant therapy
C	Pregnancy
D	All of the above

Question 8

Acute adrenocortical insufficiency may result from ?

A	Pseudomonas septicemia
B	Coagulation disorder
C	Rapid withdrawal of steroids
D	All of the above

Question 8 Explanation:

Acute adrenocortical insufficiency may result from septicemia with Pseudomonas or meningococcemia (Waterhouse-Friderichsen syndrome), anticoagulant therapy or a coagulation disorder, bilateral adrenal hemorrhage in newborn due to birth trauma, during pregnancy, following idiopathic adrenal vein thrombosis, as a complication of venography, rapid withdrawal of steroids from those on chronic steroid therapy, congenital adrenal hyperplasia, those on mitotane, ketoconazole, phenytoin, rifampin

Question 9

During stress, plasma cortisol levels should be constantly maintained at ?

A	10 μg/dL
B	20 μg/dL
C	30 μg/dL
D	40 μg/dL

Question 9 Explanation:

Continuous hydrocortisone infusion maintains plasma cortisol constantly at stress levels of 30 μg/dL.

Question 10

Which of the following is false in acutely ill patients ?

A	Cortisol levels rise four- to sixfold
B	Diurnal variation is abolished
C	Unbound fractions of cortisol rises
D	None of the above

Question 10 Explanation:

During critical illnesses cortisol levels rise 4 - 6 fold, diurnal variation is abolished & unbound fractions of cortisol rise in circulation & target tissues.

Question 11

Functional or relative adrenal insufficiency is ?

A	Adrenal insufficiency on withdrawal of long term steroids
B	Subnormal cortisol production during acute severe illness
C	Failure to take replacement therapy in adrenal insufficiency
D	Ineffective replacement therapy in adrenal insufficiency

Question 11 Explanation:

Subnormal cortisol production during acute severe illness has been termed “functional” or “relative” adrenal insufficiency.

Question 12

Following cosystropin, increment of less than what between peak & baseline cortisol levels defines relative adrenal insufficiency ?

A	< 3 μg/dL
B	< 6 μg/dL
C	< 9 μg/dL
D	< 12 μg/dL

Question 12 Explanation:

Relative adrenal insufficiency is defined as a <9 μg/dL increment between peak & baseline cortisol levels following administration of 250 μg of cosystropin as an IV bolus

Question 13

What value of a random cortisol level is indicative of relative adrenal insufficiency ?

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

Question 14

What value of a random cortisol level excludes the diagnosis of relative adrenal insufficiency ?

A	> 24 μg/dL
B	> 34 μg/dL
C	> 44 μg/dL
D	> 54 μg/dL

Question 14 Explanation:

A random cortisol level of <=15 μg/dL is indicative of relative adrenal insufficiency and a random cortisol level >34 g/dL usually excludes the diagnosis of relative adrenal insufficiency

Question 15

Isolated aldosterone deficiency occurs in ?

A	Protracted heparin administration
B	Pretectal disease of nervous system
C	Severe postural hypotension
D	ALl of the above

Question 15 Explanation:

Isolated aldosterone deficiency with normal cortisol production occurs in hyporeninism, as an inherited biosynthetic defect, following removal of aldosterone-secreting adenomas, during protracted heparin therapy, in pretectal disease of CNS, and in severe postural hypotension.

Question 16

The most common form of CAH is due to impairment of ?

A	17-hydroxylase/17,20-lyase (CYP17)
B	21-hydroxylase (CYP21A2)
C	11β-hydroxylase (CYP11B1)
D	3β-HSD2

Question 16 Explanation:

The most common form of CAH (95% of cases) is a result of impairment of CYP21A2.

Question 17

CAH caused by deficiency of 21-hydroxylase is characterised by ?

A	Cortisol deficiency
B	With or without aldosterone deficiency
C	Androgen excess
D	All of the above

Question 17 Explanation:

CAH caused by deficiency of 21-hydroxylase is characterised by cortisol deficiency, with or without aldosterone deficiency, and androgen excess

Question 18

The highest rates of classic CAH occur in ?

A	Alaska
B	Brazil
C	Philippines
D	USA

Question 18 Explanation:

The highest rates of classic CAH occur in two geographically isolated populations - Yupic Eskimos of Alaska (one in 280) & French island of La Réunion (one in 2100). High rates have also been reported in Brazil (one in 7500) and the Philippines (one in 7000).

Question 19

The 21-hydroxylase gene is located on chromosome ?

A	6
B	8
C	10
D	12

Question 19 Explanation:

21-hydroxylase gene is located on chromosome 6p21·3 within HLA histocompatibility complex. There are two highly homologous 21-hydroxylase genes resulting from ancestral duplication : an active gene, CYP21A2 (CYP21B) & an inactive pseudogene CYP21A1P (CYP21A, CYP21P).

Question 20

A very high concentration of which of the following is diagnostic of classic 21-hydroxylase deficiency ?

A	Androstenedione
B	11-deoxycortisol
C	17-hydroxyprogesterone
D	Pregnenolone

Question 20 Explanation:

A very high concentration of 17-hydroxyprogesterone (>242 nmol/L; normal < 3 nmol/L at 3 days in full-term infant) in a randomly timed blood sample is diagnostic of classic 21-hydroxylase deficiency.

Question 21

What value of stimulated concentration of 17-hydroxyprogesterone is diagnostic of 21-hydroxylase deficiency ?

A	> 15 nmol/L
B	> 25 nmol/L
C	> 35 nmol/L
D	> 45 nmol/L

Question 21 Explanation:

A corticotropin stimulated concentration of 17-hydroxyprogesterone higher than 45 nmol/L is diagnostic of 21-hydroxylase deficiency.

Question 22

“Hypertensive” variant of Congenital adrenal hyperplasia (CAH) is due to deficiency of ?

A	21-hydroxylase (CYP21A2)
B	17-hydroxylase/17,20-lyase (CYP17)
C	11-hydroxylase (CYP11B1)
D	3β-HSD2

Question 22 Explanation:

CYP11B1 deficiency causes a “hypertensive” variant of CAH. Hypertension & hypokalemia occur because of impaired conversion of 11-deoxycorticosterone to corticosterone, resulting in the accumulation of 11-deoxycorticosterone, a potent mineralocorticoid

Question 23

CYP17 deficiency is characterized by ?

A	Hypogonadism
B	Hypokalemia
C	Hypertension
D	All of the above

Question 23 Explanation:

CYP17 deficiency is characterized by hypogonadism, hypokalemia, and hypertension. It causes decreased production of cortisol & shunting of precursors into the mineralocorticoid pathway with hypokalemic alkalosis, hypertension, and suppressed plasma renin activity

Question 24

In CAH, prednisone is the drug of choice in all except ?

A	Infants
B	Children
C	Adolescents
D	Adults

Question 25

Which of the following has the longest half-life ?

A	Prednisone
B	Prednisolone
C	Methylprednisolone
D	Triamcinolone

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

1.Which of the following is given in the treatment of adrenal
insufficiency ?
A. Hydrocortisone
B. Fludrocortisone
C. DHEA
D. All of the above

2. What proportion of total glucocorticoid dose should be given in
morning ?
A. One third
B. One half
C. Two third
D. Three fourth

3. In adrenal insufficiency, during fever, the dose of hydrocortisone
should be ?
A. Kept the same
B. Doubled
C. Tripled
D. Quadrupled

4. In Addison’s disease, mineralocorticoid administration is
unnecessary at hydrocortisone doses of ?
A. > 10 mg/day
B. > 40 mg/day
C. > 80 mg/day
D. > 100 mg/day

5. Which of the following favors a diagnosis of primary adrenocortical
insufficiency ?
A. Dehydration
B. Hyponatremia
C. Hyperkalemia
D. All of the above

6. Which of the following is false about secondary adrenocortical
insufficiency ?
A. No hyperpigmentation
B. Low ACTH level
C. Near-normal aldosterone secretion
D. None of the above

7. Acute adrenocortical insufficiency may result from ?
A. Meningococcal septicemia
B. Anticoagulant therapy
C. Pregnancy
D. All of the above
8. Acute adrenocortical insufficiency may result from ?
A. Pseudomonas septicemia
B. Coagulation disorder
C. Rapid withdrawal of steroids
D. All of the above

9. During stress, plasma cortisol levels should be constantly
maintained at ?
A. 10 μg/dL
B. 20 μg/dL
C. 30 μg/dL
D. 40 μg/dL

10. Which of the following is false in acutely ill patients ?
A. Cortisol levels rise four- to sixfold
B. Diurnal variation is abolished
C. Unbound fractions of cortisol rises
D. None of the above

11. Functional or relative adrenal insufficiency is ?
A. Adrenal insufficiency on withdrawal of long term steroids
B. Subnormal cortisol production during acute severe illness
C. Failure to take replacement therapy in adrenal insufficiency
D. Ineffective replacement therapy in adrenal insufficiency

12. Following cosystropin, increment of less than what between
peak & baseline cortisol levels defines relative adrenal
insufficiency ?
A. < 3 μg/dL
B. < 6 μg/dL
C. < 9 μg/dL
D. < 12 μg/dL

13. What value of a random cortisol level is indicative of relative
adrenal insufficiency ?
A. <= 5 μg/dL
B. <= 10 μg/dL
C. <= 15 μg/dL
D. <= 20 μg/dL
14. What value of a random cortisol level excludes the diagnosis of
relative adrenal insufficiency ?
A. > 24 μg/dL
B. > 34 μg/dL
C. > 44 μg/dL
D. > 54 μg/dL

15. Isolated aldosterone deficiency occurs in ?
A. Protracted heparin administration
B. Pretectal disease of nervous system
C. Severe postural hypotension
D. ALl of the above

16. The most common form of CAH is due to impairment of ?
A. 17-hydroxylase/17,20-lyase (CYP17)
B. 21-hydroxylase (CYP21A2)
C. 11-hydroxylase (CYP11B1)
D. 3-HSD2

17. CAH caused by deficiency of 21-hydroxylase is characterised by ?
A. Cortisol deficiency
B. With or without aldosterone deficiency
C. Androgen excess
D. All of the above

18. The highest rates of classic CAH occur in ?
A. Alaska
B. Brazil
C. Philippines
D. USA

19. The 21-hydroxylase gene is located on chromosome ?
A. 6
B. 8
C. 10
D. 12

20. A very high concentration of which of the following is diagnostic
of classic 21-hydroxylase deficiency ?
A. Androstenedione
B. 11-deoxycortisol
C. 17-hydroxyprogesterone
D. Pregnenolone

24. What value of stimulated concentration of 17-hydroxyprogesterone
is diagnostic of 21-hydroxylase deficiency ?
A. > 15 nmol/L
B. > 25 nmol/L
C. > 35 nmol/L
D. > 45 nmol/L

25. “Hypertensive” variant of Congenital adrenal hyperplasia (CAH)
is due to deficiency of ?
A. 21-hydroxylase (CYP21A2)
B. 17-hydroxylase/17,20-lyase (CYP17)
C. 11-hydroxylase (CYP11B1)
D. 3-HSD2

26. CYP17 deficiency is characterized by ?
A. Hypogonadism
B. Hypokalemia
C. Hypertension
D. All of the above

27. In CAH, prednisone is the drug of choice in all except ?
A. Infants
B. Children
C. Adolescents
D. Adults

28. Which of the following has the longest half-life ?
A. Prednisone
B. Prednisolone
C. Methylprednisolone
D. Triamcinolone

Disorders of the Adrenal Cortex

[MCQ] Disorders of the Adrenal Cortex- Part 7

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Disorders of the Adrenal Cortex- Part 7

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