Waterhouse-Friderichsen syndrome
Fulminant meningococcemia - Waterhouse-Friderichsen syndrome; Fulminant meningococcal sepsis - Waterhouse-Friderichsen syndrome; Hemorrhagic adrenalitisWaterhouse-Friderichsen syndrome (WFS) is a group of symptoms caused when the adrenal glands fail to function normally. This occurs as a result of bleeding into the glands.
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.
Adrenal glands
The adrenal glands are two small triangle-shaped glands in the upper abdomen. One gland is located on top of each kidney.
Read Article Now Book Mark ArticleWFS is caused by severe infection with meningococcus bacteria or other bacteria such as:
- Group B streptococcus
- Pseudomonas aeruginosa
- Streptococcus pneumoniae
- Staphylococcus aureus
Symptoms
Symptoms occur suddenly. They are due to the bacteria growing in large numbers inside the body. Symptoms include:
- Fever and chills
- Joint and muscle pain
- Headache
- Vomiting
The infection with bacteria causes bleeding throughout the body, which causes:
- A rash throughout the body
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Disseminated intravascular coagulation in which small blood clots cut off blood supply to the organs
Disseminated intravascular coagulation
Disseminated intravascular coagulation (DIC) is a serious disorder in which the proteins that control blood clotting become overactive.
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Septic shock
Septic shock
Septic shock is a serious condition that occurs when a body-wide infection leads to dangerously low blood pressure.
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Bleeding into the adrenal glands makes the glands unable to produce enough adrenal hormones. This is called adrenal crisis, and it leads to symptoms such as:
- Dizziness, weakness
- Very low blood pressure
- Very fast heart rate
- Confusion or coma
Exams and Tests
Your health care provider will perform a physical exam and ask about your symptoms.
Blood tests will be done to confirm a bacterial infection. Tests may include:
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Blood culture
Blood culture
A blood culture is a laboratory test to check for bacteria or other germs in a blood sample.
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Complete blood count with differential
Complete blood count
A complete blood count (CBC) test measures the following:The number of white blood cells (WBC count)The number of red blood cells (RBC count)The numb...
Read Article Now Book Mark ArticleDifferential
The blood differential test measures the percentage of each type of white blood cell (WBC) that you have in your blood. It also reveals if there are...
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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. It measures the functio...
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If your provider suspects the infection is caused by meningococcus bacteria, other tests that may be done include:
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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...
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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 under a microscope. The skin is tested to look for skin conditi...
Read Article Now Book Mark ArticleGram 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 the skin. The Gram stai...
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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...
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Tests that may be ordered to help diagnose acute adrenal crisis include:
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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...
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Cortisol blood test
Cortisol blood test
The cortisol blood test measures the level of cortisol in the blood. Cortisol is a steroid (glucocorticoid or corticosteroid) hormone produced by th...
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Blood sugar
Blood sugar
A blood sugar 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 the...
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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 ...
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Sodium blood test
Sodium blood test
The sodium blood test measures the concentration of sodium in the blood. Sodium can also be measured using a urine test.
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Treatment
Antibiotics are started right away to treat the bacterial infection. Glucocorticoid medicines will also be given to treat adrenal gland insufficiency. 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.
Vaccine
All content below is taken in its entirety from the CDC Meningococcal ACWY Vaccine Information Statement (VIS): www. cdc. gov/vaccines/hcp/vis/vis-st...
Read Article Now Book Mark ArticleReferences
Newell-Price JDC, Auchus RJ. The adrenal cortex. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 15.
Stephens DS. Neisseria meningitides. In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 9th ed. Philadelphia, PA: Elsevier; 2020:chap 211.
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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
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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: 8/26/2023
Reviewed By: Jatin M. Vyas, MD, PhD, Associate Professor in Medicine, Harvard Medical School; Associate in Medicine, Division of Infectious Disease, Department of Medicine, Massachusetts General Hospital, Boston, MA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.