Disorders Of The Adrenal Cortex

Front 1

adrenal cortex divided into 3 zones

Back 1

1. glomerulosa
2. fasiculata
3. reticularis

Front 2

cortisol levels peak

Back 2

-in the morning
-gradually decrease throughout the day
(if we averaged everyone)

Front 3

Cushing's syndrome

Back 3

-the disease state resulting from a chronic excess of cortisol

Front 4

causes of cushings syndrome

Back 4

1. Cushing's disease- 65% (ACTH-secreting pituitary tumor)
2. adrenal adenoma
3. adrenal carcinoma
4. ectopic ACTH- ex. small cell lung
5. Ectopic CRG
6. ectopic adrenal receptors

Front 5

clinical features of cushings syndrome

Back 5

1. truncal obesity
2. generalized obesity
3. plethora
4. moon face
5. HTN
6. bruising
7. purplish striae
8. muscle weakness
9. irreg menses
10. hirsutism
11. backache, osteoporosis

Front 6

pathogenesis of clinical features of cushings syndrome

Back 6

1. Immunosuppression - glococorticoids suppress lymphocytes, decrease AB synthesis, dec PMN chemotaxis
2. mineralcorticoid effects: due to either or to concurrent overproduction of mineralcorticoids; can cause sodium retention, hypokalemia, HTN

Front 7

pathogenesis of clinical features of cushings syndrome- cont

Back 7

Androgenic effects of byproducts of cortisol synthesis: hirsutism and virilization seen in some female patients
Miscellaneous: decreased intestinal calcium absorption, hypercalciuria and kidney stones, increased appetite, insomnia, alterations in mood, psychosis.

Front 8

cushings syndrome screening tests

Back 8

1. overnight dexamethasone suppression test: 1 mg at bedtime. Serum cortisol at 8AM the next morning is normally <3.6 ug/dL
2. 24 hour urine free cortisol. Normal is <45-100
3. Salivary cortisol at midnight (should be very low; nml values depend on assay)

Front 9

DDx for cushings syndrome

Back 9

1. plasma ACTH
2/ Serum potassium - often low in ectopic ACTH
3. 24-hr urine 17-ketosteroids may be very high (>40 mg/24 hr) in adrenal carcinoma

Front 10

Cushing's syndrome anatomic localization

Back 10

-MRI of sella turcia
-CT scan of adrenals
-Petrosal sinus sampling for plasma ACTH measurements in selected cases
-octreotide nuclear scan to visualize somatostatin receptors found on carcnioid tumors, islet cell tumors and other sources of ectopic ACTH

Front 11

Treatment of cushing's syndrome

Back 11

1. Surgery
2. Radiation therapy directed at tumor
3. Cabergoline- may suppress ACTH secretion from some pituitary tumors
4. adrenal enzyme blockade with metyrapone aminogluthethimide or ketoconazole
4. Anti-tumor chemo

Front 12

OTHER CAUSES OF CORTISOL NON-SUPPRESSIBILITY IN 1-MG OVERNIGHT TEST

Back 12

1. stress or illness
2. active alcohol abuse
3. major depression
4. phenytoin therapy
5. massive obesity

Front 13

Nelson's Syndrome

Back 13

-an invasive, ACTH-secreting tumor of the pituitary occuring after bilateral adrenalectomy for pituitary-dependent cushing's disease

Front 14

Nelson's syndrome features

Back 14

-post-adrenalectomy, pt on replacement doses of glucocorticoid
-avg time from adrenalectomy to dx: 3 yrs
-intense hyperpigmentation
-temporal visual field cuts, rapidly progressive
-extra-ocular palsies
-pituitary apoplexy
-extremely high plasma ACTH concentration

Front 15

mgmt of Nelson;s syndrome

Back 15

Prevention:
-primary tx of cushings disease directed at pituitary rather than adrenals
-pituitary radiation at the time of adrenalectomy
Treatment:
-surgical hypophysectomy
-radiotherapy
-bromocriptine

Front 16

Primary adrenal insufficiency

Back 16

Etiology: autoimmune, tbc, fungal, CMV, hemorrhage (increased by anticoagulants)
Symptoms: anorexia, weight loss, nausea, vomiting, hyperpigmentation, orthostasis
Laboratory: hyponatremia, hyperkalemia, low cortisol, low aldosterone, high ACTH
Poor cortisol response to IV cosyntropin (0.25 mg); normal peak cortisol >20 ug/dL

Front 17

Autoimmune Disorders Associated with Addison’s Disease

Back 17

Hashimoto’s thyroiditis
Graves’ disease
Type 1 diabetes mellitus
Hypoparathyroidism
Autoimmune gonadal failure
Vitiligo
Alopecia
Pernicious anemia/atrophic gastritis

Front 18

treatment of primary adrenal insuffiency

Back 18

1. glucocorticoids
2. mineralcorticoids
3. medic-alert or other id
4. increase dose of glucocorticoid for stress;give parenterally if needed
5. ? DHEA in some women

Front 19

secondary adrenal insufficiency

Back 19

-due to hypothalamic/pituitary disease
-ssx: same as primary, except no hyperpigmentation
-lab: hyponatremia, normokalemia, low cortisol, low ACTH, nl. aldosterone
-blunt cortisol response to cosyntropin

Front 20

treatment of secondary adrenal insufficiency

Back 20

-usually just glucocorticoids required
-aldosterone secretion is maintained by the renin-angiotensin system, so Florinef is not needed
-? DHEA in some women
-medic-alert or other ID
-increase dose of glucocorticoid for stress; give parenterally if needed

Front 21

Adrenal incindentalomas

Back 21

-an adrenal mass is coincidentally found in about 1-2% of abd CT scans
-most such masses are benign and do not secrete any hormonal product
-determine which masses need to be surgically removed:
1. those which are endocrinologically active
2. those which as at high risk for malignancy

Front 22

pathology of adrenal incidentalomas

Back 22

-non-functioning cortical adenoma
-cortisol-secreting adenoma
-aldosterone-secreting adenoma
-adrenocortical carcinoma
-pheochryomocytoma
-adrenal metastases from- non-adrenal tumor

Front 23

to r/o adrenaltumor

Back 23

1.24-hr urine or plasma free metanephrines to R/O pheochromocytoma
2. 1-mg overnight dexamethasone supp. test to R/O cortisol-producing adenoma or carcinoma
3. High basal serum DHEA-S suggests androgen-producing tumor
4. Serum potassium and plasma aldosterone/PRA ratio to R/O hyperaldosteronism
5. May need to repeat evaluation yearly for 2-4 years to confidently exclude mild hyperfunction.

Front 24

if adrenal mass is non-functioning

Back 24

1. Immediate surgical removal if mass > 4 cm in diameter (some suggest 5 or 6 cm)
2. If < 4 cm diameter, follow size with repeat CT scans at 4 and 12 months; remove if growing (25% of benign lesions grow slightly over 1 yr)
3. Masses in oncology patients may be adrenal metastases (esp. from lung, breast). CT-guided fine needle aspiration biopsy is useful in the diagnosis of metastases.