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RECOGNITION AND MANAGEMENT OF ACUTE ADRENAL CRISES Dr Rohit Rajagopal Staff Specialist Endocrinologist August 2016 OUTLINE • Basic Physiology and Terminology • Clinical Presentation • Management OUTLINE • Basic Physiology and Terminology • Clinical Presentation • Management HYPOTHALAMIC-PITUITARY-ADRENAL AXIS Tertiary Adrenal Failure Secondary Adrenal Failure Hypothalamic/Pituitary mass Pituitary apoplexy Irradiation Trauma Long-term steroid use Primary Adrenal Failure Autoimmune adrenalitis (Addison’s disease) TB, HIV, Cryptococcus Bilateral adrenal haemorrhage/infarct Congenital Drugs – eg ketoconazole OTHER PITUITARY HORMONES • LH, FSH – sex hormones, reproductive function • GH – linear growth, metabolic effects, body composition • Prolactin – lactation • TSH – thyroid hormones • ADH – regulation of osmolality and water balance OTHER ADRENAL HORMONES • Aldosterone • Regulation of salt and water balance and hence volume and BP by acting on the renal distal tubule to facilitate K excretion and Na/H2O reabsorbtion • Main stimulus is serum K level and the Renin-Angiotensin system (not ACTH) • Sex steroids OUTLINE • Basic Physiology and Terminology • Clinical Presentation • Management ADRENAL CRISES • A life-threatening emergency related to acute adrenal insufficiency (usually primary) although may also occur with secondary/tertiary especially if acute or following sudden withdrawal of longterm steroid therapy • Majority of manifestations are due to aldosterone deficiency although cortisol is also important for BP maintenance ADRENAL CRISES • Incidence: 5-10 cases per 100 patient years annually and a recent Australian study suggests an increasing trend • 1 in 200 patients die from an adrenal crisis • Early recognition of the clinical features and prompt institution of treatment is vital! ADRENAL CRISES – CLINICAL FEATURES • Predominant manifestation is shock usually out of proportion to the severity of the presenting illness • Other symptoms include: • Nausea, vomiting and anorexia • Abdominal pain – may mimic an “acute abdomen” • Unexplained fever • Lethargy, fatigue, weakness, confusion, coma • May have a background history of long-standing lethargy, anorexia, weight loss and fatigue • May carry a “Sick day plan” or MedicAlert tag ADRENAL CRISES – CLINICAL FEATURES • Other symptoms/signs may also be present that may point to an aetiology of the underlying disease or precipitant: • Increased pigmentation – suggests chronic ACTH excess • Vitiligo and other autoimmune diseases • Headaches and visual field disturbances • Source of infection or history of missed steroid doses ADRENAL CRISES - LABORATORY FEATURES • Hyponatraemia • Hyperkalaemia (usually primary) • Hypoglycaemia (ACTH deficiency) • Increased urea • Anaemia, eosinophilia • Increased ESR • Mild hypercalcaemia • High TSH ADRENAL CRISES – OTHER INVESTIGATIONS • Should be directed at possible precipitant: • Blood and urine cultures, CXR, ECG etc • IF FEBRILE, PATIENT HAS AN INFECTION TILL PROVEN OTHERWISE • Collect blood for ACTH, Cortisol, Aldosterone and Renin BUT DO NOT WAIT FOR RESULTS PRIOR TO INITIATING TREATMENT! • 51 yo female with 7 yr history of known Addison’s disease • On Hydrocortisone 20 mg mane, 10 mg nocte and Fludrocortisone 0.1 mg bd • Had a MedicAlert bracelet and aware of sick day management • Developed viral gastroenteritis with a 24 hour history of vomiting and diarrhoea • Tripled usual hydrocortisone but unable to keep pills down • Called ambulance as directed • Found to have BP of 80/60 at scene; given 1L Hartmann’s and transferred to ED Gargya, IMJ - 2016 • On arrival, BP was 101/62. Labelled as ‘normotensive’ and triaged to be reviewed in 60 min • IV fluids and steroids not given although requested by family • 3 hours later, patient had a cardiorespiratory arrest and found to have a pH of 7.06 • Resuscitated, intubated and transferred to ICU • Treating endocrinologist informed 5 hours later • Total LOS 16 days and admission complicated by stress-induced cardiomyopathy and a broken tooth Gargya, IMJ - 2016 Gargya, IMJ - 2016 OUTLINE • Basic Physiology and Terminology • Clinical Presentation • Management ADRENAL CRISES – ACUTE MANAGEMENT • Establish IV access – preferably 2 large bore cannulas (collect bloods as per previously) • IV Fluids: • 1-3 litres of N/Saline as quickly as possible • 5% dextrose via second line if hypoglycaemic • Regular monitoring of haemodynamic status including urine output and electrolytes/glucose to prevent fluid overload • Glucocorticoid replacement: • 100 mg iv hydrocortisone bolus followed by 50 mg q 8 hourly ADRENAL CRISES – SUBACUTE MANAGEMENT • Contact Endocrinology service • Ongoing IV N/Saline for next 24-48 hours • Search for and treat possible precipitant eg infection • Taper IV steroids over next 24-48 hours and commence oral replacement • Oral fludrocortisone if required once IV fluids ceased ADRENAL CRISES – LONG-TERM MANAGEMENT • If no previous diagnosis, establish cause of adrenal insufficiency • A low cortisol (<100 nmol/L) with an elevated ACTH is diagnostic of primary adrenal failure • Borderline cortisol levels or inappropriately normal ACTH levels may require further dynamic testing • Lifelong hormone replacement • Hydrocortisone 18-30 mg daily in divided doses or Prednisolone 4.5-7.5 mg daily • Fludrocortisone or other pituitary hormone replacement as indicated • Sick day plans, script for IM Solu-Cortef and instructions on how to use, MedicAlert tags, Addison’s society SICK DAY PLANS Gargya, IMJ - 2016 WHAT DO AMBULANCES CARRY? Gargya, IMJ - 2016 ANY QUESTIONS?