HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Peracute bacterial sepsis with microcirculation disorders, disseminated intravascular coagulation and shock (adrenal insufficiency due to hemorrhagic infarction) as well as skin and adrenal bleeding. The incubation period is 3-4 days. Untreated mortality is 80%.
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EtiopathogenesisThis section has been translated automatically.
Neisseria meningitidis is the most frequent trigger; however, septic pneumococcal or staphylococcal infections with endotoxin release and resulting excessive consumption of protein C, consecutive consumption coagulopathy and microthrombosis (see below disseminated intravascular coagulopathy) also occur. Especially occurring in splenectomized patients.
In rare cases, the syndrome has been observed after varicella infection (Heitz AFN et al. 2017
Clinical featuresThis section has been translated automatically.
DiagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
Lethality 15-20%. Untreated, the mortality rate is 80%. Prognosis is rather unfavourable in case of extensive skin infestation.
ProphylaxisThis section has been translated automatically.
For splenectomized patients, the STIKO recommends vaccination with a conjugated meningococcal c vaccine for all children from the age of 12 months.
Note(s)This section has been translated automatically.
There is an obligation to report suspected illness, pathogen detection, illness or death.
LiteratureThis section has been translated automatically.
- Friderichsen C (1918) Adrenal apoplexy in young children. Jb Pediatrics 87: 109-125
- Heitz AFN et al (2017) A rare case of Waterhouse- Friderichsen syndrome during primary Varicella zoster
infection.Neth J Med 75:351-353.
- Karakousis PC et al (2001) Waterhouse-Friderichsen syndrome after infection with group A streptococcus. Mayo Clin Proc 76: 1167-1670
- Lewis LS (1979) Prognostic factors in acute meningococcaemia. Arch Dis Child 54: 44-48
- Little EGG (1901) Cases of purpura, ending fatally, associated with hemorrage into the suprarenal capsules. Brit J Derm 13: 445
- Marchand F (1880) About a peculiar disease of the sympathicus, the adrenal glands, the peripheral nerves without bronchial skin. Virchows Arch path Anat 81: 477-502
- Marinescu G (1976) On the clinical pathology of Waterhouse-Friderichsen syndrome. Münch med Wschr 118: 31-34
- Tsokos M (2003) Fatal Waterhouse-Friderichsen syndrome due to Ewingella americana infection. On J Forensic Med Pathol 24: 41-44
- Waterhouse R (1911) A case of suprarenal apoplexia. Lancet II: 577-578
Incoming links (17)Addison's disease; Adrenal apoplexy; Adrenal cortex apoplexy; Adrenal hemorrhage syndrome; Adrenocortical insufficiency, acute; Friderichsen-waterhouse syndrome; Fulminating purple meningococcemia; Meningococcal sepsis, fulminant; Meningococcal sepsis, fulminant or peracute; Meningococcic adrenal syndrome; ... Show all
Outgoing links (14)Antibiotics; Disseminated intravascular coagulation; Ecchymoses; Heparins systemic; Neisseria meningitidis ; Penicillin g; Protein c; Purpura fulminans; Purpura (overview); Shock; ... Show all
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