Adrenal crisis

Published on 23/06/2015 by admin

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10.4 Adrenal crisis

Yuresh Naidoo

Essentials

1 The prompt recognition of the possibility of adrenal crisis or the risk of adrenal insufficiency is paramount to early and appropriate management.
2 Adrenal crisis should be considered as a possible contributor in any child with acute severe cardiovascular collapse.
3 In children, the majority of cases are due to primary adrenal failure, with congenital adrenal hyperplasia the most common cause.
4 A crisis can be precipitated in a child with known adrenal insufficiency who develops an intercurrent illness or other physiological stress.
5 Signs of glucocorticoid deficiency include hypoglycaemia, hypotension (absolute and postural) and refractory shock.
6 Signs of mineralocorticoid deficiency include dehydration (often out of proportion to estimated fluid losses), hyperkalaemia, hyponatraemia, acidosis, and pre-renal failure.
7 Patients are at risk of hypoglycaemia.
8 The management of adrenal crisis involves immediate fluid resuscitation, replacement of corticosteroid and treating hypoglycaemia.
9 The differential diagnosis of a collapsed neonate (particularly male) in the first 14 days includes adrenal insufficiency.
10 Prevention of adrenal crisis in susceptible children may be possible by following a predetermined action plan during intercurrent illnesses.

Introduction

Adrenal crisis is a life-threatening emergency caused by acute insufficiency of the adrenal hormones cortisol and aldosterone. This can occur in situations of stress where the adrenal gland would normally respond by an increase in glucocorticoid secretion. A crisis can be precipitated in a child with known adrenal insufficiency, who develops an inter-current illness or other physiological stress (e.g. burns, surgery, trauma, and sepsis). In this situation, the increased cortisol requirements of the stress or the altered oral intake of normal replacement therapy results in a relative insufficiency and rapid clinical deterioration. Alternatively, the emergency department (ED) visit may represent a new presentation of adrenal insufficiency in a child previously unrecognised to have the subtle, often non-specific symptoms of lack of adrenal hormones or a child who is at risk due to suppression by prolonged steroid therapy. The prompt recognition of the possibility of adrenal crisis or the risk of adrenal insufficiency is paramount to early and appropriate management. These children are at risk of hypoglycaemia and this needs to be anticipated and managed accordingly.

Adrenal insufficiency can be primary, due to a failure of secretion of the adrenal cortex, or secondary to hypothalamic or pituitary dysfunction. In children, the majority of cases are due to primary adrenal failure, with congenital adrenal hyperplasia the most common cause. The incidence of neonatal congenital adrenal hyperplasia in Australia is estimated at 5.9 cases per 100 000 births.

Primary causes include:

• congenital adrenal hyperplasia;
• Addison’s disease (autoimmune);
• adrenal aplasia/hypoplasia;
• adrenal infarction secondary to haemorrhage/sepsis;
• other – trauma, tumour, post-surgical.

Secondary causes include:

• central nervous system (CNS) tumour or trauma;
• idiopathic;
• exogenous steroid therapy – including inhaled corticosteroids.

Clinical presentation

Clinical features may be subtle; however, the diagnosis should be considered in all children with cardiovascular collapse. Features to look for in history, examination and investigation findings are listed below, with differential diagnosis.

History

• Prior steroid use, including inhaled steroids for asthma or other condition.
• Known congenital adrenal hyperplasia or other adrenal insufficiency on replacement therapy.
• Severe physiological stress (sepsis, trauma, burns, surgery).
• On anticoagulants, haemorrhagic diathesis.
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