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Potentially life-threatening emergency
Waterhouse-Friderichsen syndrome
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Other Resources UpToDate PubMed
Potentially life-threatening emergency

Waterhouse-Friderichsen syndrome

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Other Resources UpToDate PubMed

Synopsis

Life-threatening adrenal insufficiency due to adrenal hemorrhage or necrosis, more commonly occurring in children, and characterized by shock, hypotonia, hypotension, and disseminated intravascular coagulation. Associated infections include meningococcemia, Pseudomonas aeruginosa, and sepsis from Streptococcus pneumoniae, Escherichia coli, Haemophilus influenzae, Staphylococcus aureus, and Neisseria gonorrhoeae. Noninfectious causes are antiphospholipid syndrome, anticoagulant treatment, trauma, and adrenal hemorrhage following surgery. Common signs and symptoms are high fever, purpuric eruption, headache, and vomiting. As disease progresses, septic shock, cyanosis, and coma follow. If left untreated, prognosis is poor.

Management requires immediate treatment of shock and sepsis. Treatment with antibiotics and steroids may help avoid fatal outcome in fulminant infection. Adrenocorticosteroid and norepinephrine use is controversial, as corticosteroids may cause a Shwartzman reaction in fatal infections, but may be helpful in cases of circulatory collapse and hypotension.

Codes

ICD10CM:
A39.1 – Waterhouse-Friderichsen syndrome

SNOMEDCT:
36102002 – Waterhouse-Friderichsen syndrome

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Last Updated: 02/10/2016
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Potentially life-threatening emergency
Waterhouse-Friderichsen syndrome
Print 1 Images
Waterhouse-Friderichsen syndrome : Fever, BP decreased
Copyright © 2019 VisualDx®. All rights reserved.