14 year old female with life threatening illness, in shock
- initial steps:
Resusciation bay, assemble team, allocate roles, consider early ICU consult
Attention to A, B, C
Oxygen by non rebreather
ivi/ io access
check BSL early
Address immediate life threats – fluid bolus, treat immediate life threats, prepare for intubation
2. Results show:
High anion gap metabolic acidosis (metabolic acidosis can be inferred from low HCO3, AG= 18)
Elevated delta ratio – additional non anion gap metabolic acidosis (ratio=0.6)
Severe life threatening hypoglycaemia, hyperkalaemia
moderate to severe hyponatraemia
Moderate acute renal failure – likely pre renal but consider renal insult/ obstructive cause
Causes of acid base disorder – Renal failure, Addison’s disease/ crisis ppt by acute gastric illness, consider other causes. Has patient had a seizure (ICH/ meningitis/ low Na)?
3. Clinical Features of Addisonian crisis:
- Extreme weakness
- Mental confusion
- Extreme drowsiness, in advanced cases slipping towards a coma
- Pronounced dizziness
- Nausea and/or vomiting
- Severe headache
- Abnormal heart rate – either too fast or too slow
- Abnormally low blood pressure
- Feeling extremely cold
- Possibly a fever
- Possibly abdominal tenderness
Features of Addison’s disease:
Hyperpigmentation – which was present in this patient
Other skin findings include vitiligo, which most often is seen in association with hyperpigmentation in idiopathic autoimmune Addison disease. It is due to the autoimmune destruction of melanocytes.
Almost all patients complain of progressive weakness, fatigue, poor appetite, and weight loss.
Prominent gastrointestinal symptoms may include nausea, vomiting, and occasional diarrhea. Glucocorticoid-responsive steatorrhea has been reported.
Dizziness with orthostasis due to hypotension occasionally may lead to syncope. This is due to the combined effects of volume depletion, loss of the mineralocorticoid effect of aldosterone, and loss of the permissive effect of cortisol in enhancing the vasopressor effect of the catecholamines.
Myalgias and flaccid muscle paralysis may occur due to hyperkalemia.
Patients may have a history of using medications known to affect adrenocortical function or to increase cortisol metabolism.
Other reported symptoms include muscle and joint pains; a heightened sense of smell, taste, and hearing; and salt craving.
Patients with diabetes that previously was well-controlled may suddenly develop a marked decrease in insulin requirements and hypoglycemic episodes due to an increase in insulin sensitivity.[
Impotence and decreased libido may occur in male patients, especially in those with compromised or borderline testicular function.
Female patients may have a history of amenorrhea due to the combined effect of weight loss and chronic ill health or secondary to premature autoimmune ovarian failure. This patient had lost 10 kg in the past year.
Steroid-responsive hyperprolactinemia may contribute to the impairment of gonadal function and to the amenorrhea.
4. Treatment Priorities:
Resuscitation – ivi dextrose, treat hyperkalaemia, ivi fluids +/- inotropes, control airway
Specific treatment – ivi hydrocortisone, ?antibiotics
ICU -? dialysis
CT head when stable