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Endocrine-Week 1/2 > Adrenal 1 (AI & CAH) > Flashcards

Adrenal 1 (AI & CAH) Flashcards

1
Q

ACTH

A

Adrenocorticotropic Hormone

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2
Q

ACTH is derived from the peptide…

A

POMC (Proopiomelanocortin)

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3
Q

POMC produces what hormones/proteins

A

ACTH
a-MSH
B-endorphin

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4
Q

The synthesis of ACTH from POMC also produces Melanocyte-Stimulating Hormone (MSH) and b-endorphin (True or False)

A

True

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5
Q

ACTH stimulates the uptake of _________________ into cells of the Adrenal Cortex

A

Cholesterol

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6
Q

Even though Aldosterone is made from Cholesterol, Aldosterone Synthase is regulated by _______________ and not ACTH

A

Angiotensin II (RAAS)

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7
Q

Effects of Cortisol

A 
Dec. LH, FSH, TSH and GH
Depression/Psychosis
Anti-inflammatory
Na/H2O retention
Hypertension
Dec. bone mass
Inc. adipose tissue
Skin thinning/muscular atrophy
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8
Q

Laboratory findings of PRIMARY Adrenal Insufficiency

A 
High CRH (Hypothalamus)
High ACTH (Pituitary)
Low Cortisol/Aldosterone (Adrenal)
High Renin and Angiotensin (Kidney)

*loss of negative feedback

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9
Q

Causes of Primary Adrenal Insufficiency

A

Autoimmune (Addison’s)*
Infections (TB, HIV)
Fungal infections (Histoplasma)*
Infiltration (Metastasis, Amyloid, Sarcoid)
Hemorrhage (infections or anticoagulants)
Drugs (Ketoconazole, Rifampin)
Inherited (Adrenal Leukodystrophy/CAH)

*most common

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10
Q

Most common cause of Primary Adrenal Insufficiency

A

Autoimmune destruction of Adrenal Glands (Addison’s disease)

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11
Q

Signs/Symptoms of PRIMARY Adrenal Insufficiency

A

Hyperpigmentation (due to inc. ACTH–> inc. MSH)
Anorexia (dec. cortisol)
Hypotension (dec. cortisol)
Hyponatremia (dec. cortisol & dec. aldosterone)
Hyperkalemia (loss of aldosterone)
Dec. sexual/axillary hair (dec. androgen)

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12
Q

Why do Primary Adrenal Insufficiency result in dec. sexual hair in females but LESS so in males?

A

Males can partially compensate with testicles making androgens

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13
Q

Laboratory findings for SECONDARY Adrenal Insufficiency

A

Dec. CRH (Hypothalamus)
Dec. ACTH (Pituitary)
Dec. Cortisol (Adrenal)

*problem with Hypothalamus or Pituitary

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14
Q

Causes of SECONDARY Adrenal Insufficiency

A

Hypothalamic/Pituitary Tumors*
Drugs (glucocorticoids)*
Pituitary Infarcts (Sheehan’s syndrome)
Infetions/Diseases affecting pituitary/hypothalamus

*most common

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15
Q

MOST COMMON causes of Secondary Adrenal Insufficiency

A

Hypothalamic/Pituitary tumors (#1 non-iatrogenic cause)

Drugs (long-term glucocorticoids –> dec. ACTH –>adrenal cortex atrophy) (#1 overall cause)

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16
Q

Why is aldosterone secretion preserved in SECONDARY Adrenal Insufficiency (problem with Hypothalamus or Pituitary)

A

Reg. by Renin-Angiotensin system, which activates aldosterone synthase in response to hypovolemia. Thus, there should not be any impairment in aldosterone secretion in secondary Adrenal Insufficiency

17
Q

Why might patients with Secondary Adrenal Insufficiency fail to respond to a dose of ACTH (you would think that would solve the issue)?

A

People with chronic Secondary AI will not respond to a ACTH stimulus b/c of adrenal cortex atrophy

18
Q

In what situation would a patient with Secondary Adrenal Insufficiency have a falsely “normal” cortisol in response to a dose of ACTH?

A

If it is a RECENT/ACUTE case of Secondary AI (will still respond to ACTH, unlike CHRONIC cases where they are essentially “numb” and don’t respond anymore)

19
Q

If BOTH Primary and Secondary Adrenal Insufficiency have low Cortisol (measured after cosyntropin test), how can you diagnose one or the other?

A

Primary: high ACTH (due to issue being with Adrenal gland)
Secondary: low ACTH (due to issue being with Hypothalamus or Pituitary)

20
Q

Treatments for PRIMARY Adrenal Insufficiency

A 
Glucocorticoids (Hydrocortisone, Prednisone)
Mineralcorticoid replacement (Fludrocortisone)*

*remember that aldosterone also affected

21
Q

Treatments for SECONDARY Adrenal Insufficiency

A

Glucocorticoids (Hydrocortisone, Prednisone)

*Mineralcorticoids NOT required as Aldosterone not affected

22
Q

Primary Adrenal Insufficiency; autosomal recessive disorder, resulting in mutation of Adrenal Steroid Synthesis enzymes; causes chronically high ACTH (from Pituitary), leading to enlarged Adrenal glands; usually due to deficiency in 21-Hydroxylase enzyme (90-95% of cases)

A

Congenital Adrenal Hyperplasia

  • affects adrenal cortex (thickening of cortex only!)
23
Q

How can a deficiency in the Adrenal steroid synthesis pathway lead to Congenital Adrenal Hyperplasia

A

Low cortisol leads to chronically high ACTH production trying to stimulate adrenals, leading to enlarged adrenal glands

24
Q

MOST common enzyme deficiency responsible for Congenital Adrenal Hyperplasia

A

21-Hydroxylase enzyme (90-95%)

25
Which hormones are impacted by 21-Hydroxylase enzyme deficiency
Cortisol | Aldosterone
26
21-Hydroxylase enzyme deficiency can cause cortisol and aldosterone deficiences but EXCESS of what hormone?
adrenal androgens * b/c cortisol and aldosterone cannot be made, their precursors are going to shift towards androgen synthesis
27
Treatment for 21-Hydroxylase Deficiency
Glucocorticoid | Mineralocorticoid
28
Vascular collapse, vomiting, and death in infancy are clinical features of what hormone deficiency?
Cortisol
29
Polyuria, volume depletion, and hyperkalemia in infancy are clinical features of what hormone deficiency?
Aldosterone
30
How can a 11-Hydroxylase deficiency (type of Primary Adrenal Insufficiency) result in HYPERtension (where as 21-hydroxylase deficiency leads to hypotension due to hyperkalemia)?
11-DOC (Deoxycorticosterone, the substrate of 11-Hydroxylase) is a weak mineralcorticoid, but can exert a marked mineralcoticoid effect (K+ excretion) when it accumulates --> hypertension due to hypokalemia
31
Treatment for 11-Hydroxylase deficiency
Glucocorticoid | Anti-hypertensives
32
What enzyme deficiency (type of CAH) can lead to hypertension but without salt wasting and masculinization of external genitalia in female?
17-Hydroxylase deficiency
33
Treatment for 17-Hydroxylase deficiency
Glucocorticoid | Estrogen/Progesterone for females
34
What is the disorder than can lead to PAI (primary adrenal insufficiency) via spontaneous bilateral adrenal hemorrhage?
Waterhouse-Friederichsen Syndrome aka. Spontaneous Bilateral Hemorrhage with Disseminated Infection
35
Synthetic ACTH that is used to measure cortisol in diagnosis of adrenal insufficiency
Cosyntropin

Endocrine-Week 1/2 (18 decks)

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