Newman HYHO Adrenal CIS Flashcards

1
Q

Where does cortisol exert negative feedback?

A

1) hypothalamus (decrease release of CRH)

2) anterior pituitary (decrease release of ACTH)

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2
Q

What does HPA axis control?

A

reactions to stress (digestion, immune system, mood and emotions, sexuality, energy storage and expenditure)

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3
Q

Function of HPA axis negative feedback system

A

helps regulate the concentration of hormones in the blood and thus prevents over or under correction

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4
Q

Zones of adrenal cortex + hormone they produce

A

1) glomerulosa = mineralocorticoids
2) fasciculata = glucocorticoids
3) reticularis = sex steroids

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5
Q

Short term stress response in terms of HPA axis and adrenal cortex

A

stimulation of the adrenal medulla resulting in release of catecholamines ([nor]epinephrine)

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6
Q

Long term stress response in terms of HPA axis and adrenal cortex

A
  • stimulation CRH stimulation of anterior pituitary
  • stimulation of adrenal cortex by ACTH
  • release of mineralocorticoids and glucocorticoids
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7
Q

Describe importance of RAAS and how angiotensin II stimulates secretion from adrenal cortex

A

1) RAAS is important for regulation of renal, cardiac, and vascular physiology
2) angiotensin II stimulates aldosterone secretion from the adrenal cortex

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8
Q

Most common cause of ambiguous genitalia in a genetically female infant

A

congenital adrenal hyperplasia (causing virilization of the genitalia)

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9
Q

Most common form of Congenital adrenal hyperplasia

A

21-hydroxylase deficiency (results in deficiency of aldosterone and cortisol, increase in testosterone)

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10
Q

signs and symptoms of CAH

A
  • failure to thrive
  • recurrent vomiting
  • dehydration
  • hypotension
  • hyponatremia
  • hyperkalemia
  • shock
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11
Q

21-hydroxylase effects on aldosterone, cortisol, and androgens

A
  • Aldosterone: low
  • cortisol: low
  • androgens: high
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12
Q

Mainstays of treatment in an infant in crisis d/t CAH

A

1) hydrocortisone (IV or IM)
2) fluids/glucose (IV)
3) management of hyperkalemia

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13
Q

Purpose of mandatory newborn screening

A

to detect potentially fatal or disabling conditions in newborns as early as possible, hopefully before they develop a serious illness

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14
Q

Three categories of adrenal gland defect responsible for primary adrenal insufficiency (Addison’s disease)

A

1) adrenal dysfunction
2) adrenal dysgenesis
3) impaired steroidogenesis

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15
Q

Signs and symptoms of primary adrenal insufficiency

A
  • fatigue
  • reduced stamina
  • weakness
  • anorexia
  • weight loss
  • skin hyperpigmentation
  • salt craving
  • musculoskeletal pain
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16
Q

lab findings seen with primary adrenal insufficiency

A
  • moderate neutropenia
  • low serum sodium
  • high serum potassium
  • fasting hypoglycemia
    • low 8 am plasma cortisol accompanied by simultaneous significant elevation of plasma ACTH
17
Q

laboratory findings seen in patients suffering from acute adrenal crisis

A
  • low serum cortisol
  • low blood sugar
  • low serum sodium
  • high serum potassium
    • metabolic acidosis
  • inadequate bump in cortisol level with ACTH stimulation test
18
Q

Clinical signs acute adrenal crisus

A
  • dehydration
  • dizziness
  • rapid heart rate
  • rapid respiratory rate
  • confusion
  • abdominal pain
19
Q

Main components of acute adrenal crisis treatment

A
  • *hydrocortisone (critical and emergent if in crisis)
  • fluids/glucose
  • fludrocortisone (after the hydro is given)
  • treat hyperkalemia
20
Q

significance of low serum cortisol with low serum ACTH

A

secondary adrenal insufficiency

21
Q

clinical signs and symptoms of cushing syndrome

A
  • truncal obesity
  • moon facies
  • buffalo hump
  • hirsutism
  • thirst
  • HTN
22
Q

Abnormal labs/screening test associated with cushing syndrome

A
  • *elevated midnight cortisol levels

- dexamethasone stimulation test

23
Q

Explain dexamethasone suppression test findings associated with Cushing disease

A

ACTH: normal to mildly elevated
Cortisol: not suppressed by low doses, but suppressed by high doses

24
Q

Explain dexamethasone suppression test findings associated with Primary hypercortisolism

A

ACTH: undetectable or low
cortisol: not suppressed by high or low doses

25
Q

Explain dexamethasone suppression test findings associated with ectopic ACTH syndrome

A

ACTH: elevated in the hundreds

cortisol: not suppressed by high or low doses
* *likely associated with small cell carcinoma of the lung

26
Q

Explain why most patients with primary aldosteronism are hypertensive

A

excessive aldosterone production increases sodium retention, which increases water retention, which increases blood pressure

27
Q

Signs and symptoms of primary aldosteronism

A

1) low renin HTN
2) hypokalemia (muscle weakness/polyuria/polydipsia)
3) metabolic alkalosis

28
Q

Describe Conn Syndrome

A
  • initially used to describe the condition in which a unilateral aldosterone producing adrenal adenoma resulted in HTN
  • now used interchangeably with primary hyperaldosteronism (with or without adenoma)
29
Q

location/secretion of pheochromocytoma

A

adrenal medulla

- secrete epi/norepi

30
Q

location/secretion of paraganglioma

A

outside of adrenal gland

- secrete epi/norepi or are non-secreting

31
Q

symptoms of a pheochromocytoma and paraganglioma (7)

A
  • paroxysmal in timing
  • pressure elevated (HTN)
  • pounding pain (HA)
  • perspiration
  • panic (impending doom)
  • palpitations
  • pallor
32
Q

most sensitive test for diagnosing secretory pheochromocytomas and paragangliomas

A

plasma fractionated free metanephrines

33
Q

Describe von hippel-lindau disease type 2 and describe inheritance

A
  • autosomal dominant inheritance
  • 20% develop pheochromocytomas
  • Patients have: retinal capillary hemangiomas, hemagioblastomas of CNS, increased risk of renal cysts that transform into RCC
34
Q

Treatment and order of treatment for patients with pheochromocytomas

A
  • treat with alpha-blocker first (phenoxybenxamine)
  • after alpha-blockade established, start beta blockers
  • *ALWAYS alpha before beta
35
Q

When are adrenal gland incidentalomas found?

A

found incidentally on abdominal CTs or MRIs done for other purposes

36
Q

Prognosis adrenal gland incidentalomas

A
  • most are benign

- can also be malignant tumors of several types

37
Q

Treatment adrenal gland incidentalomas

A
  • if >4cm in diameter, and not obviously benign, it should be resected
  • if h/o malignancy exists in the patient, it must be resected
38
Q

Assessments required for patients with adrenal gland incidentalomas (3)

A

1) assessment for Cushing syndrome
2) assessment for hyperaldosteronism
3) testing for pheochromocytoma

39
Q

General cause of MEN syndromes

A

caused by gene mutations and therefore tend to run in families