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ENDOCRINOLOGY > Adrenal > Flashcards

Adrenal Flashcards

1
Q

What gene is associated with congenital adrenal hypoplasia?

A

DAX1

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2
Q

Name the 3 zones of the adrenal cortex and what they produce

A
  1. Zona glomerulosa: mineralocorticoid (aldosteron)
  2. Zona fasciculata: glucocorticoid (cortisol) and androgens, thickest layer (75%)
  3. Zona reticularis: glucocorticoid (cortisol) and androgens (possibly basal glucocorticoid production only rather than ACTH-dependent)
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3
Q

Draw a picture of the adrenal gland and its layers

A
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4
Q

Draw steroidogenesis

A
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5
Q

When do ACTH and glucocorticoid production peak?

A

In the morning just before awakening (6th-8th hourse of sleep)

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6
Q

List 5 things that may impact the timing and height of 8:00 am cortisol test

A
  1. Physical stress (major illness, surgery, trauma or starvation)
  2. Psychological stress (anxiety, depression, mania or psychosis)
  3. CNS and pituitary disorders
  4. Cushing syndrome
  5. Liver disease
  6. Chronic renal failure
  7. Alcoholism
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7
Q

What is the advantage of measuring salivary cortisol?

A

No binding proteins in saliva, so it reflects free cortisol

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8
Q

What percentage of cortisol in circulation is bound?

A
  • 10% free
  • 75% bound to CBG
  • 15% bound to albumin
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9
Q
A
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10
Q

What is the difference between low dose and high dose dexamethasone suppression tests?

A
  • Low dose (1 mg): screening test for Cushing syndrome, negative if <50 nmol/L
    • False positives in hospitalized/chronically ill patients, acute illness, depression/anxiety, alcoholism, high estrogen states, uremia
  • High dose (8 mg): historically used to differentiate Cushing disease vs ectopic ACTH and adrenal tumors as Cushing disease was thought to be suppressible to <50% of baseline (HOWEVER it is not a great test and excptions are common)
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11
Q

List 1 advantage and 2 disadvantages of low-dose ACTH stimulation test over high-dose

A
  • Advantage: more sensitive/specific in secondary adrenal insufficiency
  • Disadvantage:
    • Logistics (must be done in AM, with IV ACTH)
    • Technical limitations (ACTH is only available in 250 mcg vials, unstable in solution, adheres to glas and tubing)
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12
Q

What is the metyrapone test used for?

A
  • To diagnose suspected pituitary ACTH deficiency
  • Metyrapone inhibits 11-beta hydroxylase (11-deoxycortisol –> cortisol), which should stimulate ACTH secretion
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13
Q

Describe the insulin induced hypoglycemia test

A
  • Hypoglycemia –> CNS stress response
  • CRH –> ACTH –> cortisol
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14
Q

List 4 causes of hypoglycemia and low cortisol in a 4 week old female

A
  1. Congenital adrenal hyperplasia (StAR mutation, 3-beta-hydroxysteroid dehydrogenase, 21-hydroxylase)
  2. Adrenal hypoplasia congenita (DAX1 mutation)
  3. Hypopituitarism (PROP1, POU1F1, POMC mutation, SOD)
  4. ACTH resistance syndromes/hypoplasia (familial glucocorticoid deficiencies, triple A/Algrove syndrome)
  5. Peroxisome biogenesis disorders (ex. Zellweger syndrome, neonatal adrenaleukodystrophy)
  6. Birth trauma
  7. Infection/sepsis
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15
Q

List 5 causes of primary adrenal insufficiency

A
  • Autoimmune (Addisions)
  • Metastatic malignancy or lymphoma
  • Adrenal hemorrhage
  • Coagulopathy
  • Antiphospholipid antibody syndrome
  • Infectious (sepsis, TB, CMV, fungi, HIV)
  • Adrenaleukodystrophy
  • Peroxisome disorders (Zellweger)
  • Infiltrative (amyloidosis, hemochromatosis)
  • Congenital adrenal hyperplasia/hypoplasia
  • Familial glucocorticoid deficiency and hypoplasia
  • Triple A (Algrove) syndrome
  • Drugs (ketoconazole, metyrapone)
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16
Q

What conditions are seen in APS type 1?

What is the gene associated?

A
  • AIRE (autoimmune regulator) gene
  • Addisons
  • Hypoparathyroidism
  • Mucocutaneous candidiasis
  • Less common: autoimmune hepatitis, dystrophy of dental enamel and nails, alopecia, vitiligo, keratopathy, dysfunction of gonads, thyroid, pancreatic beta cells, gastric parietal cells)
17
Q

What conditions are seen in APS type 2?

A
  • HLA-related disorders
  • Type 1 diabetes
  • Autoimmune thyroid disease
  • Alopecia areata
  • vitiligo
  • Primary hypogonadism
  • Pernicious anemia
  • Celiac disease
18
Q

Describe familial glucocorticoid deficiency

A
  • Hereditary adrenocortical unresponsiveness to ACTH (high ACTH, low cortisol)
  • Aldosterone secretion is preserved
  • 2 subtypes:
    • ACTH receptor mutation
    • Allgrove/ Triple A syndrome with achalasia and alacrima
      *
19
Q

List 3 drugs that can cause primary adrenal insufficiency

A
  • Antifungals (ketoconazole)
  • Etomidate
  • Antiparasitic agent suramin
  • Metyrapone
20
Q

Differential diagnosis for hypoglycemia and low cortisol in a 4 week old female infant

A
  • Hypopituitarism
    • PROP1, PIT1, POMC, POU1F1
  • ACTH resistance syndrome & hypoplasia
    • Familial glucocorticoid deficiencies
    • Triple A (Algrove) syndrome
  • Congenital adrenal hypoplasia (DAX1)
  • CAH
    • StAR mutation, 3 beta hydroxysteroid dehydrogenase, 21 hydroxylase (but expect virilization)
  • Sepsis, infection
  • Peroxisome disorders (Zellweger)
  • Birth trauma
21
Q

Where should you look for hyperpigmentation in a patient with Addisons?

A
  • Buccal mucosa
  • Palmar creases
  • Nipples/areolae
  • Perivaginal and perianal mucosa
  • Sun-exposed areas
  • Scars (will be hyperpigmented)
  • Pressure areas (knuckles, toes, elbows, knees)
22
Q

What is the differential diagnosis for Cushing syndrome?

A
  • ACTH dependent: Cushing disease, nonpituitary neoplasm (ectopic ACTH)
  • ACTH independent
    • Iatrogenic (glucocorticoids)
    • Adrenal carcinoma
    • Adrenal neoplasm/adenoma
    • Macronodular adrenal hyperplasia
    • McCune albright syndrome
    • Primary pigmented nodular adrenal disease
    • Isolated micronodular adrenocortical disease
23
Q

What tumors are associated with ectopic ACTH secretion?

A

Mostly thoracic origin

  • Bronchial carcinoid
  • Small and non-small cell lung cancer
24
Q

What tests can you do to diagnose Cushing syndrome

A
  • 24 hour urinary free cortisol
  • Overnight 1 mg Dex suppression test
  • Late night salivary cortisol
25
Q

List the clinical features of Cushing syndrome

A
  • Obesity
  • Hypertension
  • Skin: plethora, hirsutism, striae, acne, bruising
  • MSK: osteopenia, weakness
  • Neuropsychiatric: emotional lability, euphoria, depression, psychosis
  • Gonadal: menstrual disorders, impotence, decreased libido
  • Metabolic: glucose intolerance, diabetes, hyperlipidemia, polyuria, kidney stones
26
Q

List 4 long-term complications of CAH 21-hydroxylase deficiency

A
  • Short stature
  • Infertility
  • Sexual dysfunction
  • Metabolic syndrome & increased CVD risk
  • Hypertension if over-treated
  • Decreased BMD
  • Hyperandrogenism if poorly controlled
  • Reduced quality of life
27
Q

4 issues related to CAH to discuss during pregnancy

A
  • Risk of transmission (offer genetic counselling)
  • Consider testing partner. If carrier, consider risks/benefits of dexamethasone treatment
  • Continue treatment with glucocorticoid that does not cross the placenta
  • Increased risk for pregnancy loss
  • Need stress dosing during pregnancy (hyperemesis, delivery)
  • May need c-section if any genital surgery
28
Q

What 3 lab tests are the best indicators of adequacy of CG treatment in CAH?

A
  • 17 OHP
  • Androstenedione
  • Testosterone
29
Q

List 8 lab findings seen in Addisons disease

A
  • Low cortisol
  • High ACTH
  • Hypoglycemia
  • Hyponatremia
  • Hyperkalemia
  • High renin
  • Low aldosterone
  • Positive adrenal antibodies
  • Mild acidosis
  • Increased Cr and urea (dehydration)
  • Hypercalcemia
  • Hematologic: normocytic normochromic anemia, neutropenia, eosinophilia, relative lymphocytosis

ENDOCRINOLOGY (21 decks)

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