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
Explain dexamethasone suppression test findings associated with ectopic ACTH syndrome
ACTH: elevated in the hundreds cortisol: not suppressed by high or low doses * *likely associated with small cell carcinoma of the lung
26
Explain why most patients with primary aldosteronism are hypertensive
excessive aldosterone production increases sodium retention, which increases water retention, which increases blood pressure
27
Signs and symptoms of primary aldosteronism
1) low renin HTN 2) hypokalemia (muscle weakness/polyuria/polydipsia) 3) metabolic alkalosis
28
Describe Conn Syndrome
- 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
location/secretion of pheochromocytoma
adrenal medulla | - secrete epi/norepi
30
location/secretion of paraganglioma
outside of adrenal gland | - secrete epi/norepi or are non-secreting
31
symptoms of a pheochromocytoma and paraganglioma (7)
- paroxysmal in timing - pressure elevated (HTN) - pounding pain (HA) - perspiration - panic (impending doom) - palpitations - pallor
32
most sensitive test for diagnosing secretory pheochromocytomas and paragangliomas
plasma fractionated free metanephrines
33
Describe von hippel-lindau disease type 2 and describe inheritance
- autosomal dominant inheritance - 20% develop pheochromocytomas - Patients have: retinal capillary hemangiomas, hemagioblastomas of CNS, increased risk of renal cysts that transform into RCC
34
Treatment and order of treatment for patients with pheochromocytomas
- treat with alpha-blocker first (phenoxybenxamine) - after alpha-blockade established, start beta blockers * *ALWAYS alpha before beta
35
When are adrenal gland incidentalomas found?
found incidentally on abdominal CTs or MRIs done for other purposes
36
Prognosis adrenal gland incidentalomas
- most are benign | - can also be malignant tumors of several types
37
Treatment adrenal gland incidentalomas
- 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
Assessments required for patients with adrenal gland incidentalomas (3)
1) assessment for Cushing syndrome 2) assessment for hyperaldosteronism 3) testing for pheochromocytoma
39
General cause of MEN syndromes
caused by gene mutations and therefore tend to run in families