Hyperadrenalism

Introduction

• Cushing’s disease
  • Hyperadrenalism due to pituitary adenoma
• Cushing’s syndrome
  • Hyperadrenalism for exposure to excess glucocorticoids over prolonged period
  • Endogenous
    • Primary adrenal adenoma, carcinoma or hyperplasia
    • Secondary to ACTH or CRH release
    • Ectopic ACTH from bronchogenic carcinoma or carcinoid tumors
  • Exogenous (iatrogenic) by far most common

Clinical features

• Cushing’s syndrome
  • Classically increased body weight with central obesity, rounded face, hypertension, fatigue, weakness and proximal myopathy, hirsutism, striae, bruising, reduced libido, amenorrhoea, depression and/or personality changes
  • Easy bruising
  • Plethora
  • Resistant hypertension
  • Osteopaenia or early-onset osteoporosis/fracture
  • Proximal weakness is useful to differentiate pure obesity from Cushing’s syndrome (relative weakness for patient size)

Investigations

• FBC – Polycythaemia, neutrophilia and oesinophilia
• UEC – Hyperglycaemia, hypokalaemia and metabolic alkalosis
• 24 hr urinary cortisol >4x ULN is diagnostic
• Outpatient dexamethasone suppression test
  • Day 2 cortisol level should be <50% of baseline level indicating normal suppression and excluding Cushing’s syndrome
• Long dexamethasone suppression test
  • Inpatient increasing dexamethasone dosing to see at what level suppression occurs
  • Cushing’s disease only suppresses at high doses
• CXR – To rule out bronchogenic carcinoma as cause
• MRI adrenals to identify tumors

Treatment

• Depends on cause

Carcinoid crisis

• Due to release of vasoactive peptides and biogenic amines from neuroendocrine tumors arising from enterochromaffin cells
• Commonly from GIT, bronchopulmonary, ovarian, biliary or neck tissue
• Associated with MEN I
• Stress, eating, alcohol, emotional events, liver palpation and exogenous catecholamine delivery can all induce carcinoid crisis
• Get excessive production of serotonin, histamine, dopamine, bradykinin, kallikrein, somatostatin, VIP, ACTH and prostaglandins
• Presentation
  • Cutaneous flushing of face, neck, upper chest
  • Abdominal cramps, profuse diarrhoea
  • Bronchospasm
  • Hypotension
  • Mimics anaphylaxis, severe asthma, severe gastroenteritis and can cause cardiac arrest
• Shock state/arrest may be refractory to adrenaline/noradrenaline
• Investigation
  • 24hr urinary HIAA
  • Serum chomogranin-A
• Treatment
  • Octreotide inhibit release of serotonin and acts as a vasoactive agent
    • 100-500mcg bolus then 100-200mcg/hr in shock state
  • Thiamine 500mg
  • Nicain
  • Aminophylline for bronchospasm

Last Updated on October 6, 2021 by Andrew Crofton