Name
ADDISONIAN CRISIS
DESCRIPTION
DETAIL
CAUSES : - IDIOPATHIC AUTOIMMUNE ADRENOCORTICAL INSUFFICIENCY DUE * AUTOIMMUNE ATROPHY * FIBROSIS * LYMPHOCYTIC INFILTRATION OF ADRENAL CORTEX * TUBERCULOSIS - HEREDITARY PREDISPOSITION TO AUTOIMMUNE ADDISON DISEASE - IDIOPATHIC AUTOIMMUNE ADDISON DISEASE IN ASSOCIATION WITH OTHER AUTOIMMUNE PHENOMENA LIKE * SCHMIDT SYNDROME * POLYGLANDULAR AUTOIMMUNE DISEASE 1 & 2 * IDIOPATHIC HYPOPARATHYROIDISM * MUCOCUTANEOUS CANDIDIASIS * TYPE I DIABETES MELLITUS * HASHIMOTO THYROIDITIS * GRAVES DISEASE * VITILIGO * ALOPECIA AREATA, TOTALIS & UNIVERSALIS * PREMATURE OVARIAN OR TESTICULAR FAILURE * PERNICIOUS ANEMIA * MYASTHENIA GRAVIS * IDIOPATHIC HYPOPHYSITIS * CHR ACTIVE HEPATITIS * PRIMARY BILIARY CIRRHOSIS - CHR GRANULOMATOUS DISEASES * TUBERCULOSIS * SARCOIDOSIS * HISTOPLASMOSIS * BLASTOMYCOSIS * CRYPTOCOCCOSIS - HEMATOLOGIC MALIGNANCIES * MALIGNANT INFILTRATION IN HODGKINS & NON HODGKINS LYMPHOMA, LEUKEMIA * METASTATIC DISEASE INVOLVEMENT - INFILTRATIVE METABOLIC DISORDERS * AMYLOIDOSIS * HEMOCHROMATOSIS - ACQUIRED IMMUNE DEFICIENCY SYNDROME * AIDS WITH OPPORTUNISTIC BACTERIAL INFECTION * GLUCOCORTICOID RESISTANCE SYNDROME * AIDS PATIENT WITH MEGESTROL USE AS APPETITE STIMULANT - ALLGROVE SYNDROME - ABNORMALITIES OF BETA OXIDATION OF VERY LONG CHAIN FATTY ACIDS LIKE * ADRENOLEUKODYSTROPHY * ADRENOMYELONEUROPATHY - CONGENITAL ADRENAL HYPERPLASIA DUE TO * 20,22 DESMOLASE ENZYME DEFICIENCY * 3-BETA HYDROXYSTEROID DEHYDROGENASE DEFICIENCY * 21-HYDROXYLASE ENZYME DEFICIENCY - DRUG INDUCED * KETOCONAZOLE * AMINOGLUTETHIMIDE * MITOTANE * BUSULPHAN, ETOMIDATE, TRILOSTANE * METHADONE - IRRADIATION -------------------------------------------------------------------------- CAUSES OF ACUTE ADDISON DISEASE - STRESS LIKE INFECTION, TRAUMA, SURGERY, EMOTIONAL TURMOIL - FAILURE TO INCREASE STEROID DOSE IN REPLACEMENT THERAPY - BILATERAL ADRENAL HAEMORRHAGE - BILATERAL ADRENAL ARTRY EMBOLI & BILATERAL VEIN THROMBOSIS - BILATERAL ADRENALECTOMY FOR ANY REASONOTHER TESTS - * ACTH STIMULATION TEST - POSITIVE ( NORMAL ADRENALS RELEASE 2 - 5 TIMES ITS BASAL PLASMA CORTISOL OUTPUT * SERUM SOD., CHLORIDE & BICARBONATES - DECREASED * SERUM POTASSIUM - INCREASED * PLASMA CORTISOL & ALDOSTERONE - DECREASED & FAIL TO INCREASE ON ACTH STIMULATION * PLASMA ACTH LEVELS * SERUM CATECHOLAMINES * RAPID ACTH TEST - PATIENT WITH CONGENITAL ADRENAL HYPERPLASIA RESPOND WITH MARKED INCREASE IN 17-HYDROXY PROGESTERONE LEVELS & SUBNORMAL CORTISOL RESPONSE * RANDOM PLASMA CORTISOL LEVELS - >25 MCG/DL EXCLUDES ADRENAL INSUFFICIENCY * URINARY & SWEAT SODIUM - MAY BE ELEVATED * BUN & SERUM CREATININE - ELEVATED DUE TO HYPOVOLEMIA * ADRENAL AUTOANTIBODIES - MAY BE PRESENT
TYPENOTES
Administration of glucocorticoids in supraphysiologic or stress doses is the only definitive therapy. Dexamethasone does not interfere with serum cortisol assay and, thus, may be the initial drug of choice. However, because dexamethasone has little mineralocorticoid activity, fluid and electrolyte replacement is essential. A short ACTH stimulation test may be performed during resuscitation. Once complete, hydrocortisone 100 mg IV every 6 hours is the preferred treatment to provide mineralocorticoid support. Delaying glucocorticoid replacement therapy while awaiting the results of the ACTH stimulation test is inappropriate and dangerous. In addition to corticosteroid replacement, aggressive fluid replacement with 5 or 10% intravenous dextrose and saline solutions and treatment of hyperkalemia is mandatory. Fludrocortisone, a mineralocorticoid, may also be given. A thorough search for a precipitating cause and administration of empiric antibiotics is indicated. Reversal of coagulopathy should be attempted with fresh frozen plasma. Pressors (eg, dopamine, norepinephrine) may be necessary to combat hypotension.
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
SERUM SODIUM, SERUM POTASSIUM, SERUM CHLORIDE, MONTOUX TEST, ACTH STIMULATION TEST, X-RAY CHEST P.A. VIEW( NORMAL ), ULTRA SOUND WHOLE ABDOMEN - FEMALE, BUN, COMPLETE BLOOD COUNT, MRI