Clinical Guideline. SPEG MCN Protocols Sub Group SPEG Steering Group

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1 Clinical Guideline SECONDARY CARE MANAGEMENT OF SUSPECTED ADRENAL CRISIS IN CHILDREN AND YOUNG PEOPLE Date of First Issue 24/01/2015 Approved 28/09/2017 Current Issue Date 16/06/2017 Review Date 01/09/2019 Version 2.0 Author / Contact Scott Williamson (scott.williamson@nhs.net); John Schulga Group Committee Final Approval (jschulga@nhs.net) SPEG MCN Protocols Sub Group SPEG Steering Group SPEG Guideline Management of Adrenal Crisis in Children 2017 Page 1 of 8

2 SECONDARY CARE MANAGEMENT OF ADRENAL CRISIS IN CHILDREN AND YOUNG PEOPLE Contents Purpose of Guideline... 2 Causes of Adrenal Insufficiency... 3 Primary... 3 Secondary... 3 Diagnosis of Adrenal Crisis... 3 History and examination... 3 Investigations... 4 Interpretation of Results... 5 Features of Adrenocortical Failure Caused by Adrenal or CNS Disease... 5 Initial Treatment of Adrenal Crisis... 6 Ongoing Management of Adrenal Crisis... 7 Maintenance Steroid Replacement... 7 Hydrocortisone... 7 Fludrocortisone... 8 Purpose of Guideline Guidance for the management of suspected adrenal crisis in children and young people under 16 years who have been referred to secondary care paediatrics in Scotland. The following guidelines are applicable for general use. Individual situations may require deviation from these guidelines. Always inform the paediatric endocrine team as soon as possible of any suspected case of adrenal crisis. Who should use this document? Paediatricians, Emergency Medicine clinicians, Paediatric Endocrinologists this guideline is not intended for use in primary care. Patients to whom this document applies. Children and young people under 16 years who have been referred to secondary care or have self-referred acutely. SPEG Guideline Management of Adrenal Crisis in Children 2017 Page 2 of 8

3 Causes of Adrenal Insufficiency Primary Secondary Exogenous steroid suppression Adrenal haemorrhage Autoimmune (Addison s disease) Infection (e.g. TB adrenal glands) Adrenal tumour Adrenoleukodystrophy, Congenital adrenal hypoplasia Congenital adrenal hyperplasia (e.g. 21-Hydroxylase deficiency) Congenital panhypopituitarism ACTH deficiency Radiotherapy for cancer treatment Diagnosis of Adrenal Crisis History and examination History can give added weight to diagnosing adrenal insufficiency: History of chronic fatigue, weakness, abdominal pain, anorexia, weight loss, vomiting, diarrhoea, salt craving, skin pigmentation. The patient may have a history of adrenal insufficiency or exogenous glucocorticoid use (e.g. high dose inhaled steroids, prolonged oral prednisolone). Other autoimmune conditions may be a clue to the presence of Addison s disease. Undiagnosed ACTH deficiency may be associated with headache, visual changes, seizures, focal neurological signs. Examination findings may include: Presence of fever and other signs of intercurrent illness. Hypotension and shock Volume depletion. Hypoglycaemia. Seizures. SPEG Guideline Management of Adrenal Crisis in Children 2017 Page 3 of 8

4 Investigations It is vital that blood investigations are performed BEFORE administering exogenous steroids. DO NOT WAIT for results to come back before starting treatment if you suspect adrenal crisis. Initial investigations may include: Serum urea, electrolytes, creatinine. Glucose. Urinary electrolytes. Full blood count Urinalysis Cortisol ACTH (EDTA sample rapidly transported to laboratory). INVESTIGATIONS REQUIRED IN THE ASSESSMENT OF THE CAUSE OF ADRENAL INSUFFICIENCY Cause Cortisol ACTH 17-OHP Aldosterone Renin Adrenal antibodies Primary Secondary 21-Hydroxylase Deficiency Other biochemical tests may be performed if necessary (e.g. thyroid function, gonadotrophins, prolactin, etc., if other abnormalities are suspected. SPEG Guideline Management of Adrenal Crisis in Children 2017 Page 4 of 8

5 Interpretation of Results Emergency Lab Results Supporting Diagnosis of Adrenal Insufficiency: Serum Urine Na+ K+ HCO3 Glucose Features of Adrenocortical Failure Caused by Adrenal or CNS Disease Adrenocortical Disease Hypothalamo-Pituitary Disease Biosynthetic Defect (CAH 21-hydroxylase) Deficient Hormone(s) Cortisol Aldosterone ACTH/CRF Cortisol Cortisol ± Aldosterone Hyperpigmentation Yes No Yes Serum Na + Low Serum K + High Normal Mildly low when patient well (unable to excrete free water load) Low if salt waster High if salt waster Plasma ACTH High Low High Cortisol response to Synacthen None May Increase (may require Insulin tolerance test to define) May Increase 17-OH Progesterone response to Synacthen test None Increase Exaggerated increase SPEG Guideline Management of Adrenal Crisis in Children 2017 Page 5 of 8

6 Initial Treatment of Adrenal Crisis Assure airway and breathing are adequate. Monitor the patient using a cardiac monitor. Patients will virtually always have significant volume depletion. Replace fluid losses with 20 ml/kg 0.9% Sodium Chloride bolus if necessary Treat hypoglycaemia (blood glucose below 2.6mmol/L) using 2mL/kg 10% dextrose. Give IV hydrocortisone if possible, AFTER diagnostic blood tests drawn The following doses are from the Children s BNF: o Neonate Initially 10mg iv, then either: Continuous intravenous infusion 100mg/m 2 daily 100mg/m 2 daily in divided doses every 6 to 8 hours Adjust the dose according to the response. o Child age 1 month to 11 years Initial dose of 2 to 4 mg/kg iv, then 2 to 4 mg/kg every 6 hours Adjust the dose according to the response o Child age 12 to 17 years 100mg iv every 6 to 8 hours o Hydrocortisone may also be given by continuous intravenous infusion. Please see local guidelines if this method is used. SPEG Guideline Management of Adrenal Crisis in Children 2017 Page 6 of 8

7 Ongoing Management of Adrenal Crisis Check electrolytes 2 to 4 hourly depending on the degree of illness. Inform the local paediatric endocrine team. Continue with i.v. dose until clinically improved. Switch to high dose oral hydrocortisone when able to tolerate oral fluids When stable, reduce over 4 to 5 days to oral maintenance dose (as per cbnf guidance). Commence fludrocortisone once stable. This is not needed in secondary adrenal insufficiency. Prescribe injectable (i.m) hydrocortisone AND TEACH PARENTS for home emergency use. Give patient/parents a completed steroid therapy card. Maintenance Steroid Replacement Hydrocortisone When the child has recovered from the acute episode, oral hydrocortisone should be commenced. The physiological replacement dose of Hydrocortisone for children is 10mg/m 2 /day. This is given three times daily, with the largest dose given in the morning. Some children may require hydrocortisone replacement four times daily. E.g. if a child s body surface area is calculated as 1.0m 2, their total replacement dose of Hydrocortisone is 10mg/day. This would be given as 5mg morning, 2.5mg afternoon, 2.5mg evening. Sick day dose of hydrocortisone: Families should know how to increase the dose of hydrocortisone during illness. The normal sick day dose is double the daytime dose given three times daily. Emergency treatment of adrenal insufficiency: If the child is unwell, and unable to take the oral hydrocortisone, the family should be shown how to administer intramuscular hydrocortisone. SPEG Guideline Management of Adrenal Crisis in Children 2017 Page 7 of 8

8 The dose of i.m hydrocortisone is age dependant: EMERGENCY HYDROCORTISONE DOSES Age Birth to 1 year Hydrocortisone Dose 25mg 1 years to 5 years 50mg Over 5 years 100mg Fludrocortisone Fludrocortisone is required in Primary Adrenal insufficiency. The dose of Fludrocortisone is 100 g/m 2 /day, given once daily (usually in the morning). Initial dose g once daily, maintenance g once daily. SPEG Guideline Management of Adrenal Crisis in Children 2017 Page 8 of 8