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

Siguiente

Adrenal cortical insufficiency

Descripción

Resumen del Recurso

Similar

	Creado por v.djabatey hace más de 10 años