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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 two 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:

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:

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

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

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 and glucocorticoid drugs are given.

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, Updated Edition. 8th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 213.

Stewart PM, Newell-Price JDC. The adrenal cortex. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 13th ed. Philadelphia, PA: Elsevier; 2016:chap 15.

BACK TO TOP Text only

  • Meningococcal lesions on the back - illustration

    Meningococcemia is a life-threatening infection that occurs when the bacteria Neisseria meningitidis invades the blood stream. Bleeding into the skin (petechiae and purpura) usually occurs and the tissue may die (become necrotic or gangrenous). If the patient survives, the areas heal with scarring.

    Meningococcal lesions on the back

    illustration

  • Adrenal gland hormone secretion - illustration

    The adrenal gland secretes steroid hormones such as cortisol and aldosterone. It also makes precursors that can be converted to sex steroids (androgen, estrogen). A different part of the adrenal gland makes adrenaline (epinephrine). When the glands produce more or less hormones than required by the body, disease conditions may occur.

    Adrenal gland hormone secretion

    illustration

  • Meningococcal lesions on the back - illustration

    Meningococcemia is a life-threatening infection that occurs when the bacteria Neisseria meningitidis invades the blood stream. Bleeding into the skin (petechiae and purpura) usually occurs and the tissue may die (become necrotic or gangrenous). If the patient survives, the areas heal with scarring.

    Meningococcal lesions on the back

    illustration

  • Adrenal gland hormone secretion - illustration

    The adrenal gland secretes steroid hormones such as cortisol and aldosterone. It also makes precursors that can be converted to sex steroids (androgen, estrogen). A different part of the adrenal gland makes adrenaline (epinephrine). When the glands produce more or less hormones than required by the body, disease conditions may occur.

    Adrenal gland hormone secretion

    illustration

 

Review Date: 9/27/2017

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, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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