Adrenal cortical insufficiency

congenital adrenal hyperplasia
1. the commonest non-iatrogenic cause of not enough cortisol & mineralocorticoid secretion
primary ACI (Addison's disease)
1. rare in children
2. may result from
  1. an autoimmune process
    1. sometimes in assoc w/ other autoimmune endocrine disorders
      1. diabetes mellitus
      2. hypothyroidism
      3. hypoparathyroidism
  2. haemorrhage/infarction
    1. neonatal
    2. meningococcal septicaemia
  3. X-linked adrenoleucodystrophy
    1. rare neurodegen metabolic disorder
  4. TB (now rare)
secondary to
1. hypopituitarism from hypothalmic-pituitary disease
2. hypothalamic-pituitary-adrenal suppression
  1. ff long term corticosteroid therapy
presentation
1. infants
  1. acute presentation
    1. salt-losing crisis
    2. hypotension
      1. and/or hypoglycaemia
2. dehydration ff a gastroenteritis-like illness
  1. child recovers until next episode
3. older children
  1. chronic ill health
  2. pigmentation
    1. e.g. buccal
  3. salt-craving
Diagnosis
1. U&E
  1. low Na+
  2. high K+
2. blood gas
  1. metabolic acidosis
3. blood glucose
  1. hypoglycaemia
4. low plasma cortisol
5. high plasma [ACTH]
  1. except in hypopituitarism
6. ACTH (Synacthen) test
  1. plasma [cortisol] remain low in
    1. primary adrenal failure
    2. long-standing pituitary/hypothalamic Addison's disease
  2. normal response exclude adrenal cortical insufficiency
Mx
1. adrenal crisis
  1. urgent Rx needed
    1. iv saline w/ glucose
    2. iv hydrocortisone
  2. all at risk of this should wear a MedicAlert bracelet
2. long-term replacement
  1. glucocorticoid & mineralocorticoid replacement
    1. increase glucocorticoid dose by 3x during illness or for an op
3. teach pt to inject im hydrocortisone in an emergency

Nächster

Adrenal cortical insufficiency

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