Introduction: Adrenal crisis is a life-threatening condition and an absolute medical emergency, requiring prompt diagnosis and treatment to prevent grave morbidity and mortality. We describe a case that posed significant diagnostic dilemma due to incongruity of history and clinical findings.
Case Report: A 9-year-old girl presented acutely with loss of consciousness, GCS of 5/15. This was preceded by a 3-day history of abdominal pain, vomiting and diarrhoea. She had PDA repair at 18 months of age. Examination findings demonstrated cardiovascular collapse. She was peripherally shut down, tachycardic with unrecordable blood pressure. Investigations showed severe metabolic acidosis (pH 6.97, lactate 8.6 mmol/l, base excess −19.4). Serum glucose level was 8.6 mmol/l, sodium 131 mmol/l, potassium 4.3 mmol/l. She received 40 ml/kg of 0.9% saline. Fluid resuscitation improved her clinical and biochemical states. She was subsequently managed for gastroenteritis and pneumonia (evident on chest radiograph). Inflammatory markers and blood cultures were negative. Further work up revealed a borderline Synacthen test (baseline cortisol: 274 nmol/l, 30 min: 395 nmol/l, 60 min: 479 nmol/l) with ACTH level of 5.6 pmol/l. She failed her insulin tolerance test in terms of cortisol response (basal glucose: 4.4 mmol/l, cortisol: 166 nmol/l, 15 min glucose: 1.3 mmol/l, cortisol: 168 nmol/L). Other endocrine panel were unremarkable. Magnetic resonance imaging of pituitary was normal. She had a good outcome after 1 week hospital stay. She was commenced on oral hydrocortisone at 10 mg/m2 per day and fludrocortisone at 100 μg/day. Adrenal insufficiency medic alert bracelet and emergency card were given. Subsequent outpatient reviews showed resolution of gastrointestinal symptoms and lethargy.
Conclusion: This case buttresses the point that it does not always tick the boxes. Adrenal insufficiency should be strongly considered in all cases of cardiovascular collapse in Emergency Paediatrics even in the absence of classical biochemical findings.
22 - 24 Nov 2017
British Society for Paediatric Endocrinology and Diabetes