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(OMS2) ENRP Exam 2 > Adrenal pathology > Flashcards

Adrenal pathology Flashcards

1
Q

What presents with Primary hyperaldosteronism (Conn’s syndrome)

A 
1. Hypertension
◦ Refractory hypertension
◦ Adrenal mass and hypertension
◦ Hypertension at a young age
◦ Severe hypertension (>160/100 mmHg)
  1. Hypokalemia
  2. Hypomagnesemia
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2
Q

Secondary hyperaldosteronism

edit

A 
Renin-Angiotensin-Aldosterone system
◦ Diuretic use
◦ Decreased renal perfusion
◦ Arterial hypovolemia
◦ Pregnancy
◦ Renin-secreting tumors
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3
Q

Describe an aldosterone-secreting adenoma

A
  1. May be very small (<2 cm)
  2. Young patients 30s-40s
  3. Spironolactone bodies (from treatment)
  4. High incidence of ischemic heart disease
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4
Q

What is the defective enzyme of Congenital Adrenal Hyperplasia (CAH) responsible for

A

steroidogenesis

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5
Q

What are 90-95% of CAH cases caused by

A

21-hydroxylase deficiency

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6
Q

What is a 21 hydroxylase deficiency known as

A

salt wasting syndrome

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7
Q

What is the presentation of a complete 21 hydroxylase deficiency in males and females?

A

◦ Salt wasting –> hyponatremia
◦ Hyperkalemia
◦ Hypotension

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8
Q

How does a complete 21 hydroxylase deficiency present in only females

A

virilization (seen at birth)

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9
Q

Describe Simple virilizing syndrome (without salt wasting)

A

Partial lack of enzyme (hydroxylase)
◦ Some mineralocorticoids
◦ Small amount of cortisol, not enough to prevent ACTH overproduction

Virilization

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10
Q

Describe Nonclassic/Late onset adrenal virilism

A

Most common

Partial lack of 21-hydroxylase
◦ Precocious puberty
◦ Acne and hirsutism at time of puberty

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11
Q

How do you diagnose CAH?

A

Serum 17-hydroxyprogesterone

increases with severity

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12
Q

What is extremely important for diagnosis of CAH

A

Heel stick

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13
Q

Therapy for CAH

A
  1. Glucocorticoids
    - Replenishes cortisol
    - Provides negative feedback for ACTH suppression
    - No further overstimulation of androgen production
  2. Mineralocorticoids as necessary
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14
Q

Causes of primary adrenocortical insufficiency

A
  • Loss of cortical cells

* Defect in hormonogenesis

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15
Q

Causes of secondary adrenocortical insufficiency

A
  • Hypothalamic-pituitary disease

* HPA suppression by extra-adrenal steroid source

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16
Q

Possible causes of primary acute adrenocortical insufficiency

A
  1. Adrenal crisis
  2. Rapid withdrawal of steroids
  3. Massive adrenal hemorrhage (Waterhouse-Friderichsen syndrome)
  4. Stress, infection, trauma, burns
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17
Q

How does adrenal crisis present

A
  • Hypotension
  • Hyponatremia
  • Hyperkalemia
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18
Q

What pathogen is associated with waterhouse friderichsen syndrome

A

neisseria meningitidis

19
Q

symptoms of acute adrenal insufficiency

A 
Hypotension (refractory to volume repletion)
Abdominal pain
Fever
Nausea/vomiting
Hyponatremia
Hypoglycemia
20
Q

Signs of Primary chronic adrenocortical insufficiency (Addison’s disease)

A
  • Long duration of malaise, fatigue
  • Anorexia and weight loss
  • Joint pain
  • Hyperpigmentation of skin
21
Q

What is Nelson disease

What is noticable symptom

A

Pituitary tumor caused by the removal of adrenal glands for cushing disease

Increased pigmentation from ACTH(?)

22
Q

What causes most cases of primary hypoadrenalism in the US

A

Autoimmune adrenalitis

23
Q

Mutation responsible for AUTOIMMUNE POLYENDOCRINE

SYNDROME TYPE 1

A

Mutation in AIRE gene

24
Q

Symptoms of AUTOIMMUNE POLYENDOCRINE

SYNDROME TYPE 1

A 
◦ Adrenalitis
◦ Parathyroiditis
◦ Hypogonadism
◦ Pernicious anemia
◦ Mucocutaneous candidiasis
(Ab against IL-17 and IL-22)
◦ Ectodermal dystrophy

APECED

25
Q

Symptoms of AUTOIMMUNE POLYENDOCRINE

SYNDROME TYPE 2

A

Adrenalitis
Thyroiditis
Type 1 diabetes mellitus

26
Q

Presentation of Adrenocortical Insufficiency

A 
Lack of corticosteroids:
◦ Vague malaise
◦ Nausea/vomiting
◦ Hypoglycemia
◦ Refractory hypotension

Lack of mineralocorticoids
◦ Hyperkalemia
◦ Hyponatremia

27
Q

How do you test for adrenocortical insufficiency

A
  • Random cortisol

* ACTH-stimulation test

28
Q

What is the most important distinction between an adenoma and a carcinoma

A

Size

adrenal cortical carcinoma weighs >200g

29
Q

What is unique to the presentations of carcinomas (in comparison to an adenoma)

A

Compression/invasion of adjacent structures

virilizing

30
Q

What is the adrenal medulla composed of?

what is it responsible for?

A

Comprised of Chromaffin cells deriving from the neural crest

Responsible for catecholamine secretion

31
Q

How do pts present with pheochromocytoma

A

profound hypertension

32
Q

What do pheochromocytomas secrete

A

Catecholamines

33
Q

What rules are associated with pheochromocytoma?

A 
10% rule
◦ 10% are extra-adrenal (paraganglioma)
◦ 10% are bilateral
◦ 10% in kids
◦ 10% are malignant
◦ Can only tell by metastasis
◦ 10% are not associated with hypertension

25% rule: 25% are familial!

34
Q

What is the classic triad of pheochromocytoma

A

◦ Headache
◦ Palpitations
◦ Diaphoresis

35
Q

What are compliactions of acute and chronic pheochromocytoma

A

◦ Acute – related to catecholamine surges

◦ Chronic – cardiomyopathy

36
Q

How to diagnose a pheochromocytoma

A

Urine and plasma metanephrines

37
Q

Describe a myelolipoma

A

Benign
◦ Fat
◦ Bone marrow

Vary in size

Can present with hemorrhage

38
Q

How do you decide what to do with an adrenal incidentaloma

A

Proper management depends on many factors:

> 4 cm

Positive functional assays
◦ Dexamethasone suppression test for hypercortisolism
◦ Pheochromocytoma

CT enhancement characteristics

39
Q

Germline mutation in the MEN1 tumor suppressor gene is associated with what issues?

A 
Primary hyperparathyroidism
Pancreatic endocrine tumors
Pituitary adenomas
◦ Lactotroph
◦ Somatotroph
(“3 P’s)

Gastrinomas - duodena

40
Q

Germline gain-of-function mutation
in the RET proto-oncogene is associated with what issues?

MEN 2A

A

Pheochromocytoma
Medullary thyroid carcinoma
Parathyroid hyperplasia

41
Q

Germline gain-of-function mutation
in the RET proto-oncogene
(specific point mutation)

MEN 2B

A

Medullary thyroid carcinoma
Pheochromocytoma
Mucosal neuromas

42
Q

Medulary thyroid carcinoma is associated with what mutation

A

Germline gain-of-function mutation
in the RET proto-oncogene
(specific mutations)

43
Q

Generalities of MEN syndromes

A

Patients develop tumors at younger age
Tumors are more likely to be bilateral/multiple
Preceding hyperplasia is often seen
Tumors tend to be aggressive and recurrent

(OMS2) ENRP Exam 2 (9 decks)

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