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MSPA-Endocrinology > Adrenal diseases > Flashcards

Adrenal diseases Flashcards

1
Q

what are the drugs that can precipitate adrenal insufficiency ?

A
  • Glucocorticoids ( Mainly oral administration)
  • High dose of Fluticasone propionate inhalation.
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2
Q

What is the most common cause of adrenal insufficiency ?

A
  • Autoimmune adrenalitis or Addison’s disease.
  • Autoimmune polyglandular syndrome.
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3
Q

What is the most common cause of secondary adrenal insufficiency ?

A

Exogenous glucocorticoid use.

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4
Q

What is the acid base balance difference between primary vs secondary adrenal insufficiency?

A

In Addisons disease a mild metabolic acidosis is present. Whereas in secondary adrenal insufficiency the acid base balance is normal.

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5
Q

What are the symptoms of primary adrenal insufficiency ?

A
  • weight loss and fatigue
  • Salt craving
  • loss of appetite and GIT symptoms.
  • Myalgia and joint symptoms
  • Orthostatic instability
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6
Q

What are the metabolic findings in Addison’s disease ?

A
  • Hyponatremia
  • Hypovolemia
  • Hypoglycemia
  • Hyperkalemia
  • Hypercalcemia
  • variable TFT findings.
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7
Q

What is the most commonly used test for diagnosing Addison’s disease ?

A

250 mcg short of synthetic ACTH.

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8
Q

What is the procedure for 250 mcg synthetic ACTH test ?

A
  • A pre-sample is drone to measure the cortisol level.
  • Then 250 mcg ACTH is given
  • 30 min post intervention another sample is drawn to measure the cortisol level.
    *
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9
Q

What is the morning cortisol level indicating adrenal insufficeny ?

A

A morning cortisol level of < 100 ng.

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10
Q

What is the indication for insulin tolerance test ?

A

It is typically used in secondary adrenal insufficiency as it can screen pituitary dysfunction because insulin can suppress GH level too.

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11
Q

In primary adrenal insufficiency what are the hormones deficient ?

A

Cortisol and aldosteron

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12
Q

What is the cause of acute adrenal insufficiency?

A

Adrenal haemorrhage

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13
Q

What is the cause of chronic adrenal insufficiency?

A

Addison’s disease

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14
Q

What is the most common cause of Addison’s disease in developed world vs under developed world ?

A
  • In the developed world autoimmune adrenalitis.
  • In the under developed world it is tuberculosis, HIV, and disseminated fungal infection.
  • Bilateral adrenal metastasis can cause addison’s disease in both communities.
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15
Q

What is secondary adrenal insufficiency?

A

It is due to pituitary corticotrophs dysfunction leading to reduced ACTH production. As ACTH only regulates the production of cortisol by the Zona fasiculata. The zona glomerulosa produced aldosterone is normal in secondary adrenal insufficiency.

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16
Q

What is the most common cause of secondary adrenal insufficiency?

A

Pan hypopituitarism.

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17
Q

What is tertiary adrenal insufficiency?

A

It is caused by hypothalamic dysfunction that causes CRH secretion impairment leading to reduced ACTH and cortisol production. Similar to secondary adrenal insufficiency, the aldosterone levels are unaffected.

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18
Q

What are the causes of tertiary adrenal insufficiency?

A
  • Sudden withdrawal of chronic glucocorticoid therapy and resolution of Cushing’s syndrome.
  • Head trauma and CNS lesions affecting hypothalamus.
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19
Q

What is the cause an symptoms of Addison’s crisis ?

A

It occurs in patients with adrenal insufficiency or dysfunction and is precipitated by stress or trauma. The patients presents with
* Vomiting and abdominal pain
* Fever
*hypotension and shock .
* Altered mental status and coma.

20
Q

What are the specific signs of Addison’s disease ?

A
  • Hyperpigmentation of the oral mucosa, palmar creases, and knuckles.
21
Q

What is the cause of hyperpigmentation in Addison’s disease ?

A

It occurs due to increased production of melanocyte stimulating hormone which is derived from the similar precursor molecule for ACTH called Proopiomelanocortin.

22
Q

What is the cause of salt craving in Addison’s disease ?

A

Aldosteron deficiency which can also cause hyponatremia and hyponatremic volume contraction.

23
Q

What is the cause of hyperkalemia and metabolic acidosis in Addison’s disease ?

A

impaired aldosterone production by the zona glomerulosa impairs urinary excretion of K+ leading to hyerkalemia and metabolic acidosis.

24
Q

Addison’s disease can present in people with ____ diseases ?

A

Autoimmune disease.

25
Q

Why in central adrenal insufficiency patients do not present with hyperpigmentation or hyperkalemia ?

A

In central adrenal insufficiency there is no ACTH excess or aldosterone deficiency.

26
Q

What is the interpretation of morning cortisol and ACTH testing ?

A
  • In Addison’s disease there is low cortisol with high ACTH.
  • If both are low it suggests central adrenal insufficiency.
27
Q

What is the interpretation of Cosyntropin ( synthetic ACTH stimulation test ?

A

It’s purpose is to differentiate b/w primary vs central disease and is also useful when the morning glucose test is inconclusive.
* Low cortisol level pre and post Cosyntropin administration confirms Addison’s disease.
* An increase in cortisol level after Cosyntropin administration confirms central adrenal insufficency.

28
Q

What is Metyrapone stimulation test ?

A

Metyropone inhibits the conversion of 11-deoxycortisol to cortisol which should increase ACTH if the pituitary and hypothalamus are normal.
*f ACTH and 11 doxycortisol levels are low that confirms central adrenal insufficiency.

*If the ACTH is high and 11-deoxycortisol level or cortisol levels are low that confirms Addison’s disease.

29
Q

What is the utility of Rennin and Aldosterone level testing in the work up of adrenal insufficiency?

A

Physiologically Rennin is responsible for aldosterone secretion from the zona glomerulosa. If the aldosterone levels are low due to primary adrenal insufficiency. The rennin levels will be high due to compensatory reasons.

30
Q

What is the utility of CRH stimulation test in adrenal insufficiency?

A

It can help to differentiate secondary from Tertiary adrenal insufficiency. After CRH administration if ACTH levels increase that confirms tertiary adrenal insufficiency. On the other hand, if ACTH level dose not increase following CRH administration that confirms secondary adrenal insufficient.

31
Q

What is the management of adrenal insufficiency ?

A
  • In Addison’s disease glucocorticoid and aldosterone or minerellocorticodid replacement therapy.
  • In secondary and tertiary adrenal insufficiency glucocorticoid replacement only.
  • Patients should wear a wrist band or bracelet indicating that the condition to prevent adrenal crisis.
32
Q

What is the acute management of adrenal crisis ?

A

IV fluids and IV hydrocortisone.

33
Q

What is polyglandular autoimmune disease type 01?

A

It is a triad of Addison’s disease, hypoparathyroidism and chronic mucocutaneous candidiasis. They may also have primary hypogonadism.

34
Q

What is Schimdt syndrome ?

A

It is the polyglandular autoimmune disease type 02 in which, there is Addison’s disease and autoimmune thyroiditis. There may also be hypogonadism and T1 DM.

35
Q

What is the diagnostic marker of autoimmune adrenalitis ?

A

Anti-21 hydroxylase which is an anti-adrenal antibody.

36
Q

What is the pathophysiology of Adrenoleukodystrophy ?

A

It is caused by X-Linked recessive mutation of the ABCD1 gene leading to very long chain fatty acid accumulation in brain white matter, adrenal cortex and testis. It predominantly affects young males. The patients presents with neurological dysfunction, Addison’s disease, and signs of testicular dysfunction. Plasma VLCFA is elevated in this condition that point to the diagnosis.

37
Q

What is the cause of congenital adrenal hyperpasia ?

A

It is an autosomal recessive group of disorders in which defects in enzymes responsible for the synthesis of cortisol and aldosterone.

38
Q

In congenital adrenal hyperpasia if the enzyme starts with 1 it causes ____ and if it ends with 1 it causes ____

A
  • Starts with 1 causes HTN
  • Ends with 1 causes virulization in females.
39
Q

What is the effect of 21 hydroxylase deficiency ( subtype of congenital adrenal hyperpasia )?

A

It is an enzyme that converts progesterone to 11-deoxycorticosterone which is an aldosterone precursor. It is also responsible for converting 17- hydroxyprogesterone to 11deoxycortisole which is the precursor of cortisol.
The deficiency leads to ACTH mediated adrenal hyperplasia and increased production of androgen precursors causing.

40
Q

What are the phenotypes of 21-Hydroxylase deficiency ?

A
  • Classic salt wasting type - Caused by severe 21-hydroxylase deficiency in which females presents with ambiguous genitila at brith due to excess androgen precursors. Males presents with failure to thrive during the first two weeks. The children can have dehydration, vomiting, and hypotension due to aldosterone deficiency.
  • Classic non salt wasting type- It is due to moderate 21-hydroxylase deficiency in which females have ambiguous genitila at birth and male present with early virulization including large phallus at 2 to 4 years.

*Non-classic delayed type- It occurs due to mild 21-hydroxylase deficiency females presents with virulization and menstrual irregularities, males presents with early onset of puberty and sexual pre-maturity.

41
Q

What are the diagnostic finidngs in 21-hydroxylase deficiency?

A

*elevated levels of serum 17-hydroxyprogesterone.
* Due to aldosterone deficiency hyponatremia and hyperkalemia maybe seen in the salt wasting variant.
* Decreased levels of mineralocorticoids and cortisol
* Increased sex hormones and decreased renin activity.

42
Q

What is the Tx of 21-hydroxylase deficiency?

A

Hormone replacement with gluco and mnieralocorticoids to suppress pituitary secretion of ACTH. Which will also normalize androgen levels.

43
Q

what is the function of 11 beta hydroxylase ?

A

It converts 11- deoxycorticosterone to corticosterone and 11-deoxycortisol to cortisol.

44
Q

What is the pathophysiology of 11 beta hydroxylase deficiency ?

A

Accumulation of 11-deoxycorticosterone causing mild mineralocorticoid deficiency leading to hyokalemia and HTN. The rennin and aldosterone levels will be low.

45
Q

What is waterhouse frederichsen syndrome ?

A

It is characterised by bilateral adrenal haemorrhage due to severe bacterial infection.The most common organism is N. meningitidis. The patient can present with Endotoxic shock, DIC, Petechial rashes, and meningitis.

MSPA-Endocrinology (10 decks)

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