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Waterhouse-Friderichsen syndrome

Fulminant meningococcemia - Waterhouse-Friderichsen syndrome; Fulminant meningococcal sepsis - Waterhouse-Friderichsen syndrome; Hemorrhagic adrenalitis

 

Waterhouse-Friderichsen syndrome (WFS) is a collection of symptoms resulting from the failure of the adrenal glands to function normally as a result of bleeding into the gland.

Causes

 

The adrenal glands are 2 triangle-shaped glands. One gland is located on top of each kidney. The adrenal glands produce and release different hormones that the body needs to function normally. The adrenal glands can be affected by many diseases, such as infections like WFS.

WFS is caused by severe infection with meningococcus bacteria or other severe infection from bacteria, such as:

  • Group B streptococcus
  • Pseudomonas aeruginosa
  • Streptococcus pneumoniae
  • Staphylococcus aureus

 

Symptoms

 

Symptoms and signs usually come on very suddenly. They are due to the bacteria growing (multiplying) inside the body. Symptoms include:

  • Fever and chills
  • Joint and muscle pain
  • Headache
  • Vomiting

Infection with bacteria causes bleeding throughout the body, which causes:

  • Bodywide rash
  • Disseminated intravascular coagulation in which small blood clots cut off blood supply to the organs
  • Septic shock

Bleeding into the adrenal glands causes adrenal crisis, in which not enough adrenal hormones are produced. This leads to symptoms such as:

  • Dizziness, weakness
  • Very low blood pressure
  • Very fast heart rate
  • Confusion or coma

 

Exams and Tests

 

The health care provider will perform a physical examination and ask about the person’s symptoms.

Blood tests will be done to help confirm if the infection is caused by bacteria. Tests may include:

  • Blood culture
  • Complete blood count with differential
  • Blood clotting studies

If the provider suspects the infection is caused by meningococcus bacteria, other tests that may be done include:

  • Lumbar puncture to get a sample of spinal fluid for culture
  • Skin biopsy and Gram stain
  • Urine analysis

Tests that may be ordered to help diagnose acute adrenal crisis include:

  • ACTH (cosyntropin) stimulation test
  • Cortisol blood test
  • Blood sugar
  • Potassium blood test
  • Sodium blood test
  • Blood pH test

 

Treatment

 

Treatment involves giving antibiotics as soon as possible to treat the bacterial infection. Glucocorticoid medicines will also be given to treat adrenal gland problem. Supportive treatments will be needed for other symptoms.

 

Outlook (Prognosis)

 

WFS is fatal unless treatment for the bacterial infection is started right away.

 

Prevention

 

To prevent WFS caused by meningococcal bacteria, a vaccine is available.

 

 

References

Stephens DS, Apicella MA. Neisseri meningitides. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 8th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 213.

Stewart PM. Krone NP. The adrenal cortex. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 12th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 15.

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    • Meningococcal lesions on the back

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    • Adrenal gland hormone secretion

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          Review Date: 11/30/2015

          Reviewed By: Jatin M. Vyas, MD, PhD, Assistant Professor in Medicine, Harvard Medical School; Assistant in Medicine, Division of Infectious Disease, Department of Medicine, Massachusetts General Hospital, Boston, MA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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