adrenal insufficiency

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بسم الله الرحمن الرحيم RIBAT UNIVERSITY HOSPITAL DEPARTMENT OF MEDICINE Unite (E) Adrenal Insufficiency Dr. Eyad Gadour (MBBS National Ribat University)

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Basics Description - Inadequate hydrocortisone secretion to meet body's stress requirement - Adrenal deficiency: 1- Inadequate cortisol 2- Unresponsive to stimulation with adrenocorticotropic hormone (ACTH) - Functional hypoadrenalism: 1- Inadequate cortisol 2- Partial responsive to stimulation with ACTH - Addisonian crisis (acute adrenal insufficiency): 1- Life-threatening emergency 2- Precipitated by intensification of: * Chronic adrenal insufficiency * Acute adrenal hemorrhage * Rapid steroid withdrawal * Treatment of hypothyroidism with unrecognized adrenal disease * Steroid-dependent patient under stress owing to pregnancy, surgery, trauma, infection, or dehydration

EtiologyPrimary Adrenal Failure : 

EtiologyPrimary Adrenal Failure 1- Adrenal dysgenesis/impaired steroidogene: Congenital hypoplasia Allgrove syndrome: ACTH resistance Achalasia Alacrima Glycerol kinase deficiency: Psychomotor retardation Hypogonadism Muscular dystrophy Congenital hyperplasia Aldosterone synthetase deficiency Mitochondrial disease

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2- Adrenal destruction: - Autoimmune: 1- Autoimmune polyglandular syndrome types 1 and 2 (alopecia universalis, chronic mucocutaneous candidiasis, hypoparathyroid, thyroid autoimmunity, diabetes, celiac disease, pernicious anemia) 2- Adrenoleukodystrophy - Infectious: 1- Granulomatous: tuberculosis 2- Protozoal and fungal: histoplasmosis, coccidioidomycosis, candidiasis 3- Viral: cytomegalovirus, herpes simplex virus, and HIV 4- Bacterial - Infiltration: 1- Sarcoid 2- Neoplasm 3- Hemochromatosis 4- Amyloidosis 5- Iron depletion

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3- Postadrenalectomy 4- Hemorrhage: 1- Sepsis: particularly meningococcemia, Pseudomonas infection 2- Birth trauma/anoxia 3- Pregnancy 4- Seizures 5- Anticoagulants 6- Rhabdomyolysis 5- Pharmacologic inhibition: Etomidate((Amidate), a nonbarbiturate, is used for induction of anesthesia). Herbal medications(الاعشاب) Ketoconazole ( gluoccorticoid antagonist ) Metyrapone Suramin

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Secondary Adrenal Failure Pituitary insufficiency: - Sepsis - Head trauma - Hemorrhage - Infarction (Sheehan syndrome) - Infiltration: neoplasm, amyloid, sarcoid, hemochromatosis - Adrenocorticotropic hormone deficiency - Pharmacologic: glucocorticoid administration, herbal medications Tertiary Adrenal Failure - Hypothalamus insufficiency - Sepsis - Infiltrative: neoplasm, amyloid, sarcoid, hemochromatosis - Head trauma

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Diagnosis Signs and Symptoms Symptoms: Depression Lethargy Malaise Myalgias Anorexia Abdominal pain Nausea Vomiting Dehydration (found in primary adrenal insufficiency only) Salt craving Signs: Fever or hypothermia Mental status changes Tachycardia Orthostatic blood pressure changes or frank shock Weight loss Goiter Hypogonadism Hyperkalemia Sodium depletion Eosinophilia Hyperpigmentation (found in primary adrenal insufficiency only) Vitiligo Addisonian crisis: Hypotension and shock Hyponatremia Hyperkalemia Hypoglycemia

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Essential Workup Laboratory confirmation of diagnosis not possible in emergency department Adrenal crisis: life-threatening condition: High degree of suspicion should prompt initiation of therapy before definitive diagnosis. Plasma cortisol level <20 آµg/dL accompanied by shock suggests adrenal insufficiency. Electrolytes: Potassium Sodium BUN, creatinine: Elevated owing to dehydration Serum glucose levels may be low

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Tests Lab CBC with differential: Anemia Eosinophilia Lymphocytosis Arterial blood gases: Hypoxemia Acidosis Cosyntropin stimulation test: Adrenal deficiency: Random serum cortisol <20 آµg/dL (while stressed) ACTH stimulation unresponsive Functional hypoadrenalism: Random serum cortisol = 20 آµg/dL (while stressed) Sixty minutes post ACTH stimulation <30 آµg/dL or delta cortisol (60 minutes - baseline) = 9 آµg/dL Search for underlying infection. Imaging ECG Chest radiograph

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Differential Diagnosis Sepsis Shock from any cause Acute abdominal emergency

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Treatment Initial Stabilization Airway, breathing, and circulation management (ABCs) Cardiac monitor Blood pressure support for hypotension: Normal saline (0.9%) IV fluids 500 mL–1 L (peds: 20 mL/kg) bolus Avoid pressors (if possible): May precipitate dysrhythmias Supplemental oxygen to meet metabolic needs Correct hyperthermia: Initiate cooling measures. ED Treatment Glucocorticoid replacement: Hydrocortisone: 100 mg or Dexamethasone: 4 mg Dexamethasone will not interfere with results of cosyntropin stimulation tests. Volume expansion: D5W 0.9% normal saline at rate of 500–1,000 mL/h for first 3–4 hours Care should be taken to note patient's age, volume, and cardiac and renal function. For hypoglycemia: D50W Treat life-threatening dysrhythmias secondary to hyperkalemia with calcium ,Sodium bicarbonate: 1–2 mEq/kg IV, and Insulin (regular): 10 units by IV push /glucose. Identification and correction of underlying precipitant

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Follow-Up Disposition Admission Criteria All patients with acute adrenal insufficiency ICU admission for patients with unstable or potentially unstable cases Discharge Criteria Normal laboratory evaluation with treated adrenal insufficiency

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