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.
Adrenal glands
The adrenal glands are two triangle-shaped glands. One gland is located on top of each kidney.
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
Disseminated intravascular coagulation
Disseminated intravascular coagulation is a serious disorder in which the proteins that control blood clotting become overactive.
- 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
Blood culture
A blood culture is a laboratory test to check for bacteria or other germs in a blood sample.
-
Complete blood count with differential
Complete blood count with differential
A complete blood count (CBC) test measures the following:The number of red blood cells (RBC count)The number of white blood cells (WBC count)The tota...
-
Blood clotting studies
Blood clotting studies
Prothrombin time (PT) is a blood test that measures the time it takes for the liquid portion (plasma) of your blood to clot. A related blood test is ...
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
Lumbar puncture
Cerebrospinal fluid (CSF) collection is a test to look at the fluid that surrounds the brain and spinal cord. CSF acts as a cushion, protecting the b...
-
Skin biopsy
and
Gram stain
Skin biopsy
A skin lesion biopsy is when a small amount of skin is removed so it can be examined. The skin is tested to look for skin conditions or diseases. A...
Gram stain
A gram stain of a skin lesion is a laboratory test that uses special stains to detect and identify bacteria in a sample from a skin sore. The gram s...
-
Urine analysis
Urine analysis
Urinalysis is the physical, chemical, and microscopic examination of urine. It involves a number of tests to detect and measure various compounds th...
Tests that may be ordered to help diagnose acute adrenal crisis include:
-
ACTH (cosyntropin) stimulation test
ACTH (cosyntropin) stimulation test
The ACTH stimulation test measures how well the adrenal glands respond to adrenocorticotropic hormone (ACTH). ACTH is a hormone produced in the pitu...
-
Cortisol blood test
Cortisol blood test
The cortisol blood test measures the level of cortisol in the blood. Cortisol is a steroid (glucocorticoid) hormone produced by the adrenal gland. C...
-
Blood sugar
Blood sugar
A blood glucose test measures the amount of a sugar called glucose in a sample of your blood. Glucose is a major source of energy for most cells of t...
-
Potassium blood test
Potassium blood test
This test measures the amount of potassium in the fluid portion (serum) of the blood. Potassium (K+) helps nerves and muscles communicate. It also ...
-
Sodium blood test
Sodium blood test
The sodium blood test measures the amount of sodium in the blood. Sodium can also be measured using a urine 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.
Vaccine
All content below is taken in its entirety from the CDC Meningococcal ACWY Vaccines - MenACWY and MPSV4 Vaccine Information Statement (VIS): www. cd...
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 - illustration
Neisseria meningitidis
Meningococcal lesions on the back
illustration
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Adrenal gland hormone secretion - illustration
Adrenal glands produce hormones such as estrogen, progesterone, steroids, cortisol, and cortisone, and chemicals such as adrenalin (epinephrine), norepinephrine, and dopamine. 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
Neisseria meningitidis
Meningococcal lesions on the back
illustration
-
Adrenal gland hormone secretion - illustration
Adrenal glands produce hormones such as estrogen, progesterone, steroids, cortisol, and cortisone, and chemicals such as adrenalin (epinephrine), norepinephrine, and dopamine. When the glands produce more or less hormones than required by the body, disease conditions may occur.
Adrenal gland hormone secretion
illustration
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.