Adrenal Diseases

Pathophysiology Adrenal Insufficiency Adrenal Excess Adrenal Growths Congenital Adrenal Hyperplasia

Adrenal Gland

Derived from neuroendocrine tissue in the urogenital ridge Bilateral and adjacent to the kidneys (ad-renal) 2 major divisions (medulla and cortex) The inner medulla makes catecholamines (mostly epinephrine) Cortex divided into 3 zones Glomereolosa- Aldosterone FasiculataCortiosone ReticularisAndrogens

Adrenal Gland

Regulation of Cortisol

Adrenal Insufficiency

Causes: Acute Pituitary dysfunction Shock- septic. Presents with fever and abdominal pain Hemorrhage Hypotension or shock (more than 90 percent) Abdominal or flank pain (86 percent); Fever (66 percent) Anorexia, nausea, or vomiting (47 percent) Neuropsychiatric symptoms such as confusion or disorientation (42 percent)

Adrenal Hemmorage

Hypotension or shock (more than 90 percent) Abdominal or flank pain (86 percent); Fever (66 percent) Anorexia, nausea, or vomiting (47 percent) Neuropsychiatric symptoms such as confusion or disorientation (42 percent) Rebound tenderness (22 percent) Usually on Anticoagulants Associated with Pseudomonas/ meningococcemia (Waterhouse-Friederichsen syndrome)

Chronic Adrenal Insuffiency

Usually Autoimmune (Addisons Disease)

Symptoms (%)

Weakness, tiredness, fatigue 100 Anorexia 100 Gastrointestinal symptoms 92 Nausea 86 Vomiting 75 Constipation 33 Abdominal pain 31 Diarrhea 16 Salt craving 16 Postural dizziness 12 Muscle or joint pains 6-13

Signs of Adrenal Insuffiency (%)

Weight loss 100 Hyperpigmentation 94 Hypotension (systolic BP <110 mmHg) 88-94 Vitiligo 10-20 Auricular calcification 5 Laboratory abnormality Electrolyte disturbances 92 Hyponatremia 88 Hyperkalemia 64 Hypercalcemia 6 Azotemia 55

Hyperpigmintation

Hyperpigmentation

Diagnosis

Cortisol levels low ACTH levels high Difficulty with random blood tests

Pulsitile release

ACTH Stimulation Test

Draw baseline cortisol Administer 0.25 mg ACTH IV or IM Repeat cortisol 30 and 60 minutes later Normal if levels increase by 10 or any level >20 mcg/dl

Treatment of Adrenal Insuffiecy

Corticosteroids Hydrocortisone preferred because of it's short action Ususal dose 20-30 mg/day Florinef 0.5- 1.0 mg/ day if needed

Adrenal Excess

Centripetal obesity 79-97 Facial plethora 50-94 Glucose intolerance 39-90 Weakness, proximal myopathy 29-90 Hypertension 74-87 Psychological changes 31-86 Easy bruisability 23-84 Hirsutism 64-81 Oligomenorrhea or amenorrhea 55-80 Acne, oily skin 26-80 Abdominal striae 51-71

Cushings Syndrome

Elevation of Cortisol Usual cause is Pituitary growth Other causes of cortisol excess: Adrenal Tumor Ectopic ACTH production ( usually pulmonary tumors) Exogenous corticosteroids

Moon FACIES

Buffalo Hump

Striae

Cushings Disease- Diagnosis

Elevated cortisol Elevated ACTH level Dexamthasone Supression test 1 mg Dexamethasone given at midnight should suppress am cortisol to < 5 ug/dl 24 hour urine cortisol elevated

Cushings Disease Treatment

Remove source of ACTH Pituiatry tumor Cancer Remove source of Cortisol Production Adrenal Glands Medications Mititane, Aminoglutehamide

Pheochromocytoma

VERY RARE! (<0.2 % of patients with hypertension) Associated with MEN 2 ( Multiple Endocrine Neoplasia Syndrome) Result from excess catecholamine production from the adrenal medulla Classic symptoms are: Headache, Sweats, and Palpitations. Hypertension is usually present Elevation of plasma or urinary catecholamines needed to establish the diagnosis

Hyperaldosteronism

Elevation of Aldosterone Present with Hypokalemia Many also have hypertension Diagnosis elevated Aldosterone, low Potassoium and low Renin level Treatment Surgery or Aldosterone antagonist ( Ameleoride)

Adrenal Masses

Not an uncommon finding Most are not cancer, but some are hormonally active Benign characteristics < 4 cm Low Density on CT scanning No calcifications Low levels of DHEA- Sulfate Assess values of catecholamines, cortisol, adlosterone and DHEA- sulfate

Adrenal adenoma

Congenital Adrenal Hyperplasia

Multiple defects possible, but 21-OH most common Can present as complete or partial Complete block presents as salt wasting and ambiguous genetalia in females Partial block ( late onset) can present as hirsutism and oligomennorhea/infertility in woman Treatment is corticosteroids

Congenital Adrenal Hyperplasia