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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
Q

Which hormones are impacted by 21-Hydroxylase enzyme deficiency

A

Cortisol

Aldosterone

26
Q

21-Hydroxylase enzyme deficiency can cause cortisol and aldosterone deficiences but EXCESS of what hormone?

A

adrenal androgens

  • b/c cortisol and aldosterone cannot be made, their precursors are going to shift towards androgen synthesis
27
Q

Treatment for 21-Hydroxylase Deficiency

A

Glucocorticoid

Mineralocorticoid

28
Q

Vascular collapse, vomiting, and death in infancy are clinical features of what hormone deficiency?

A

Cortisol

29
Q

Polyuria, volume depletion, and hyperkalemia in infancy are clinical features of what hormone deficiency?

A

Aldosterone

30
Q

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)?

A

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
Q

Treatment for 11-Hydroxylase deficiency

A

Glucocorticoid

Anti-hypertensives

32
Q

What enzyme deficiency (type of CAH) can lead to hypertension but without salt wasting and masculinization of external genitalia in female?

A

17-Hydroxylase deficiency

33
Q

Treatment for 17-Hydroxylase deficiency

A

Glucocorticoid

Estrogen/Progesterone for females

34
Q

What is the disorder than can lead to PAI (primary adrenal insufficiency) via spontaneous bilateral adrenal hemorrhage?

A

Waterhouse-Friederichsen Syndrome

aka. Spontaneous Bilateral Hemorrhage with Disseminated Infection

35
Q

Synthetic ACTH that is used to measure cortisol in diagnosis of adrenal insufficiency

A

Cosyntropin

Endocrine-Week 1/2 (18 decks)

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