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Unit 5: Path - The Endocrine System > Adrenal Cortex > Flashcards

Adrenal Cortex Flashcards

1
Q

What are the three zones of the adrenal cortex?

A

Zona glomerulosa, zona fasciculata, zona reticularis

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2
Q

What do cells of the fasciculata and reticularis secrete?

A

Glucocorticoids and Sex hormones

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3
Q

Where is aldosterone produced?

A

Zona glomerulosa

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4
Q

Describe the pathogenesis of adrenal hyperplasia

A

Children are born with cortisol synthesis deficiency. ACTH is unopposed and upregulated causing adrenal hyperplasia.

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5
Q

What deficiency is a major cause of congenital adrenal hyperplasia?

A

21-Hydroxylase (P450C21) Deficiency

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6
Q

What is the function of 21-Hydroxylase? What occurs in its deficiency?

A

To convert 17-hydroxyprogesterone to 11-deoxycortisol; Impairment of cortisol synthesis and increased synthesis of androgens

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7
Q

What physical and sexual abnormalities are seen in a child with simple virilizing congenital adrenal hyperplasia?

A

Males: No abnormalities; Precocious puberty, potentially short stature Females Pseudohermaphroditism, fused labia, enlarged clitoris; Infertility

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8
Q

What is the cause of salt-wasting congenital adrenal hyperplasia?

A

Impaired aldosterone synthesis due to 21-hydroxylase deficiency

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9
Q

What does 11Beta-Hydroxylase Deficiency cause?

A

Congenital Adrenal Hyperplasia

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10
Q

What are three causes of adrenal cortical insufficiency?

A

Adrenal gland destruction, pituitary/hypothalamic dysfn or chronic corticosteroid therapy

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11
Q

What is another name for primary chronic adrenal insufficiency?

A

Addison Disease

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12
Q

What is the typical cause of Addison Disease?

A

Autoimmune destruction of the adrenal

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13
Q

What are the two types of polyglandular endocrinopathies?

A

Type I and Type II

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14
Q

What mutation is associated with Type I polyglandular disease?

A

AIRE gene (autoimmune regulator)

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15
Q

Another name for Type II polyglandular disease?

A

Schmidt Syndrome

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16
Q

What condition is associated with disorders in HLA-DR3?

A

Type II polyglandular autoimmune syndrome

17
Q

A section shows an adrenal gland with crhonic inflammation and fibrosis in the cortex and an intact medulla. Dx?

A

Addison disease

18
Q

What are characteristic signs of a person with primary adrenal insufficiency?

A

Hyperpigmentation and hypotension

19
Q

What are the three major precipitating factors of acute adrenal crisis?

A
  1. Abrupt withdrawal of corticosteroid Tx
  2. Stressful precipitating factors such as infection or surgery
  3. Waterhouse-Friderichsen syndrome
20
Q

What is the cause of secondary adrenal insufficiency?

A

Any condition that interferes with ACTH or CRH action

21
Q

What are the two major syndromes seen in adrenal hyperfunction?

A
  1. Cushing syndrome (hypercortisolism)
  2. Conn Syndrome (hyperaldosteronism)
22
Q

What is the difference betwixt Cushing syndrome and disease?

A
  • Syndrome: Hypercortisolism from any cause
  • Disease: Excess release of ACTH by corticotrope tumors
23
Q

What is the most common cause of Cushing Sydrome in the US?

A

Chronic Corticosteroid Administration

24
Q

What are the three major causes of ACTH-dependent adrenal hyperfunction?

A
  1. Ectopic ACTH production by a nonpituitary tumor
  2. Primary hypersecretion of ACTH by the pituitary (Cushing Disease)
  3. Inappropriate secretion of CRH by tumor
25
Q

What type of cancer typically is responsible for ectopic ACTH production?

A

SCLC

26
Q

What causes ACTH-independent Adrenal Hyperfunction?

A

Adrenal tumors

27
Q

Clear lipid laden cells arranged in nests are shown. What is the Dx?

A

Adrenal Adenoma

28
Q

Marked anisocytosis and nuclear pleomorphism is shown. What is the Dx?

A

Adrenal Cortical Carcinoma

29
Q

In iatrogenic Cushing Syndrome, what Syx are absent that are typically seen in Cushing Syndrome?

A

Hypertension and Hirsutism

30
Q

What are typical clinical features of Cushing syndrome?

A
  • Obesity - Moon Face, Buffalo Hump, Trunkal and Abdominal obesity
  • Atrophic skin, Purple Striae
  • MSK - osteoporosis, muscle wasting
  • HTN (due to mineralocorticoid excess)
  • Female: Virilization, Facial Hair, Oligomenorrhea
  • Men: ED, decreased libido
  • Psychological disturbances
31
Q

What causes glucose intolerance in cushing syndrome?

A

Cortisol -> Gluconeogenesis -> Glucose intolerance -> DIabetes Mellitus

32
Q

What is increased in all forms of cushing syndrome?

A

Increased glucocorticoids

33
Q

What causes inappropriate secretion of aldosterone?

A

Adrenal adenomas orhyperplasia

34
Q

What does Conn Syndrome lead to?

A

Hypernatermia and Hypokalemia

35
Q

What is type I familial hyperadlosteronism?

A

(Glucocorticoid suppressible) - caused by an autosomal dominant fusion of ACTH-responsive regulatory elements of the 11Beta Hydroxlase gene to the aldosterone synthase gene results in a hybrid gene that is ectopically and constitutively activated

36
Q

What is type II familial hyperaldosteronism?

A

Adrenal cortical adenoma

Unit 5: Path - The Endocrine System (6 decks)

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