Waterhouse Frederichsen syndrome

Waterhouse Frederichsen syndrome 

Updated: 06/21/2022

© Jun Wang, MD, PhD

General features
  • Adrenal insufficiency
  • Due to bilateral/unilateral adrenal hemorrhage
  • Associated with bacterial or viral infection 
  • Commonly associated with meningococcal infection 
  • Other associated pathogens include
    • Haemophilus influenzae
    • Pseudomonas aeruginosa
    • Escherichia coli
    • Streptococcus pneumoniae
Pathogenesis

Clinical presentations
  • Sudden onset
  • Nonspecific presentations: headache, fever, weakness, abdominal/flank pain, anorexia, etc
  • Signs of shock: Tachycardia, hypotension, etc
  • Abdominal rigidity or rebound tenderness
  • If associated with meningococcemia: petechial rash, purpura fulminans, other neurological abnormalities
Key Laboratory findings
  • Features of Disseminated intravascular coagulation
  • Leukocytosis
  • Hyponatremia and hyperkalemia: Mineralocorticoid deficiency
  • Hypoglycemia: glucocorticoid deficiency
  • Elevated: ACTH, renin
  • Decreased: Aldosterone, glucocorticoid
Radiological features
  • Adrenal hemorrhage
Pathological features
Management
  • Management for shock: Volume resuscitation, vasopressor, etc
  • Management for sepsis: Antibiotics based on blood microbiology study results
  • Other supportive managements: Treatment for hypoglycemia, hyponatremia, hyperkalemia, etc
Prognosis
  • High fatality rate, especially when diagnosis and treatment are delayed

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