Miss D, 36 year old female was admitted with a sudden collapse following a viral flu.

She had complained of weakness and lethargy over the last few days.

Immediately before collapsing she had complained of severe abdominal pain and had a bout of vomiting. On admission her blood pressure was 60/40mmHg, and the pulse was weak and rapid. CBS was 56mg/dl.

 On further questioning it was revealed that the patient had been taking 8 tablets of prednisolone daily without a prescription for joint pains. Recently, she had decided to go off the drugs as she was feeling well. The attending doctor requested an urgent serum electrolyte report.

What do you expect to find in the serum electrolyte report?

Moderate hyperkalaemia, normal or decreased sodium

What is your most likely diagnosis?

Adrenal insufficiency resulting in Addisonian crisis

What are the other possible differential diagnoses?

 Anorexia nervosa Acute abdomen Pregnancy related complications

What are your priorities on clinical suspicion of the disease?

It should be treated promptly without waiting for laboratory confirmation. Still, blood can be taken for essential investigations prior to treatment.

What are the problems this patient might have?

Hypoglycaemia Electrolyte imbalance Circulatory collapse Ongoing infection

How will you treat the patient?

Establish IV access with two wide bore cannulae Take blood at time of insertion of cannulae for FBC, BU/SE, blood culture, RBS Take 10ml of blood in a heparinized tube for later analysis of cortisol levels Order urine full report, urine culture, inward CXR and an ECG as other investigations Correct hypoglycaemia with 50% dextrose 50 ml IV and repeat if necessary

Correct fluid deficit by

o Correcting deficit

o Replacing ongoing losses

o Providing maintenance requirement until the patient takes orally

As the blood pressure is below 90mmHg in this patient, she needs one unit of colloids infused fast.
If the blood pressure is still low, 20ml/kg boluses can be given until it picks up.

If facilities are available, fluid replacement is best guided by insertion of a CVP line. When blood pressure is above 90 mmHg further fluids can be given at a rate of 500 ml every 4 to 6 hours judged by the clinical

When blood pressure is above 90 mmHg further fluids can be given at a rate of 500 ml every 4 to 6 hours judged by the clinical signs of overload or deficit. Replace ongoing losses: Ongoing losses as vomiting and diarrhea has to be replaced with normal saline or as oral fluids if the patient is taking orally Maintenance fluid requirement: Maintenance requirement also needs to be supplemented intravenously if the patient is not taking orally.

 Replacement of corticosteroids: Hydrocortisone IV is the drug of choice as it has both glucocorticoid and mineralocorticoid activities. It should be given as IV hydrocortisone 100mg stat and as an infusion of 100mg, 8 hourly for 24 48 hours. Then convert to 50mg 8 hourly for 48 hours and later 30mg total dose PO per day. (20mg mane and 10mg vesper). Fludrocortisone can be added 50300 g PO daily

 Assess need for antibiotics. The patient may have infection.

Look for features of infection, and start appropriate antibiotics, usually broad spectrum IV antibiotics until culture results are available.

What are the possible causes for adrenal insufficiency in this patient?

The most apparent cause is sudden withdrawal of steroid therapy Other causes to consider are, Autoimmune (polyglandular autoimmune diseases, antibodies against adrenal cortex) Neoplastic conditions (primary, metastatic) Infective conditions (tuberculosis, meningococcal sepsis) Metabolic disorders (amyloidosis) Vascular events (infarction, haemorrhage)

How will you localize the problem in the hypothalamo-pituitaryadrenal axis?

The defect can either be in the adrenal gland (primary) or in pituitary gland (secondary). First establish adrenal insufficiency by doing a morning cortisol level at 9.00 a.m. Values less than 3g/dl confirm the diagnosis while values above 19ug/dl exclude the diagnosis. If it is inconclusive, three tests are used to confirm adrenal insufficiency;

 A) Short synacthen test (measuring serum cortisol after synthetic corticotrophin dose, serum cortisol is measured after 30 and 60 minutes and values below 13ug/dl are diagnostic of adrenal insufficiency) B) Metyrapone test (read) C) Insulin tolerance test (read)

 Once adrenal insufficiency is established further tests are needed to find the site of malfunction in hypothalamo-- pituitary adrenal axis. A) A serum ACTH level > 100 pg/ml is diagnostic of primary adrenal insufficiencyB) Long synacthen test to confirm primary adrenal insufficiency in inconclusive situations C) CRH test to diagnose secondary (pituitary) from tertiary (hypothalamic) adrenal insufficiency D) CT / MRI, tissue culture and histological diagnosis would be useful in finding an aetiology for adrenal insufficiency.

What advice would you give this patient on discharge?

 Explain regarding the aetiology of the condition Advice regarding the importance of not stopping steroids suddenly. Give a time plan on tailing off of steroids No dietary restrictions, increase salt intake No activity restrictions Avoid unnecessary medication

Adrenal crisis