adrenal insufficiency

Question 1

definition of adrenal insufficiency

Answer 1

deficiency of adrenal cortical hormones (eg mineralcorticoids, glucocorticoids and androgens)

adrenoleukodystrophy - X linked inherited disease - has adrenal atrophy and demyelination

Question 2

aetiology of adrenal insufficiency

Answer 2

primary (ADDISON’S) - autoimmune >70%

infection - TB, meningococcal septicaemia (waterhouse-friderichsen syndrome), CMV (HIV pts), histoplasmosis

infitration - met (lung, breast, myeloma), lymphomas, amyloidosis

infarction - secondary to thrombophilia

inherited - adrenoleukodystrophy, ACTH receptor mutation

surgical - bilateral adrenalectomy

secondary - pituitary or hypothalamic disease

iatrogenic - sudden cessation of long term steroid therapy

Question 3

epidemiology of adrenal insufficiency

Answer 3

most common cause - iatrogenic

primary rare

annual incidence of Addison’s is 8 in 1million

Question 4

chronic symptoms of adrenal insufficiency

Answer 4

non specific vague symptoms

dizzyness,

anorexia

weight loss

diarrhoea

vomiting

abdo pain

lethargy

weakness

depression

Question 5

symptoms of acute Addisonian crisis

Answer 5

acute adrenal insufficiency with major haemodynamic collapse - precipiated by stress eg infection or surgery

Question 6

signs of adrenal insufficiency

Answer 6

postural hypotension

increased pigmentation - more noticeable on buccal mucosa, scars, skin creases, nails, pressure points - from melanocytes being stimulated by increased ACTH

loss of body hair in women - androgen deficiency

vitiligo (and other autoimmune conditions)

Question 7

signs of addisonian crisis

Answer 7

hypotensive shock, tachycardia, pale, cold, clammy, oliguria

Question 8

investigations for adrenal insufficiency

Answer 8

cofirm dx

• 9am cortisol <100nmol/L - diagnostic
• if >550nmol/L - dx unlikely
• 100-550nmol/L - should have short synacthen test - IM 250ug tetracosactrin (synthetic ACTH) is given = <550nmol/L at 30min = dx

identify level of defect of ACTH

• high in primary
• low in secondary

long synacthen

• One milligram tetracosactrin is given and cortisol is measured at 0, 30, 60,90 and 120min then at 4, 6, 8, 12 and 24 h. Patients with primary adrenal insufficiency show no increase after 6.

identify cause

• auto-Ab against 21-hydroxylase
• abdo CT or MRI
• adrenal biopsy for microscopy, culture, PCR depending on suspected cause
• TFT

Question 9

investigation sin addisonion crisis

Answer 9

FBC - neutrophilia

UE - high urea, and K, low Na

ESR/CRP - high in acute infection

Ca may be high

low glucose

blood cultures

urinalysis - culture and sensitivity - UTI

CXR - TB carcinoma infection

Question 10

treatment of addisonion crisis

Answer 10

rapid IV fluid rehydration - 0.9% saline, 1L over 30-60min, 2-4L in 12-24hr

50ml of 50% dextrose - correct hypoglycaemia

IV 200mg hydrocortisone bolus followed by 100mg 6hrly until BP stable

treat cause - AB

monitor: temp, pulse, RR, BP, sats, UO

Question 11

management for chronic adrenal insufficiency

Answer 11

replace glucocorticoids with hydrocortisone 3x daily

replace mineralocorticoids with fludrocortisone

increase hydrocortisone with acute illness/stress

if associated with hypothyroidism - hydrocortisone before thyroxine - avoid precipitating an addisonian crisis

Question 12

advice for people with adrenal insufficiency

Answer 12

steroid warning card

medic alert bracelet

emergency hydrocortisone ampule

pt education

Question 13

complications of adrenal insufficiency

Answer 13

hyperkalaemia

death in a crisis

Question 14

prognosis of adrenal insufficiency

Answer 14

adrenal function rarely recovers

normal LE if treated

type 1 - (autosomal recessive disorder caused by mutations in the AIRE gene which encodes a nuclear transcription factor.): Addison’s disease, chronic mucocutaneous candidiasis, hypoparathyroidism.

Type II (Schmidt’s syndrome): Addison’s disease, diabetes mellitus Type 1, hypothyroidism, hypogonadism.