Waterhouse-Friderichsen syndrome

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Waterhouse-Friderichsen syndrome
Classification & external resources
ICD-10 A39.1, E35.1
ICD-9 036.3
DiseasesDB 29316
eMedicine med/3009 
MeSH D014884

Waterhouse-Friderichsen syndrome (WFS) is massive, usually bilateral, hemorrhage into the adrenal glands caused by fulminant meningococcemia.[1] WFS is characterised by overwhelming bacterial infection, rapidly progressive hypotension leading to shock, disseminated intravascular coagulation (DIC) with widespread purpura, particularly of the skin, and rapidly developing adrenocortical insufficiency associated with massive bilateral adrenal hemorrhage.

Contents

[edit] Epidemiology

Meningococcus is another term for the bacterial species Neisseria meningitidis, which causes the type of meningitis which usually underlies this syndrome. Meningococcal meningitis occurs most commonly in children and young adults, and can occur in epidemics. In the United States it is the cause of about 20% of meningitis cases.[citation needed] At one time it was common among military recruits, but administration of the preventive meningococcal vaccine has greatly reduced this number. Freshman college students living in dormitory housing who have not been vaccinated are another risk group.

WFS can also be caused by Streptococcus pneumoniae infections, a common bacterial pathogen typically associated with meningitis in the adult and elderly population.[1] Staphylococcus aureus has recently also been implicated in pediatric WFS.[2]

Routine vaccination against meningococcus is recommended for people who have poor splenic function (who, for example, have had their spleen removed or who have sickle-cell anemia which damages the spleen), or who have certain immune disorders, such as complement deficiency.[3]

[edit] Historical

Waterhouse-Friderichsen syndrome is named after Rupert Waterhouse (1873–1958), an English physician, and Carl Friderichsen (1886–1979), a Danish pediatrician, who wrote papers on the syndrome, which had been previously described.[4][5]

[edit] References

  1. ^ a b Kumar V, Abbas A, Fausto N (2005). Robins and Coltran: Pathological Basis of Disease, 7th, Elsevier, pp. 1214–5. ISBN 978-0721601878. 
  2. ^ Adem P, Montgomery C, Husain A, Koogler T, Arangelovich V, Humilier M, Boyle-Vavra S, Daum R (2005). "Staphylococcus aureus sepsis and the Waterhouse-Friderichsen syndrome in children". N Engl J Med 353 (12): 1245-51. PMID 16177250. 
  3. ^ Rosa D, Pasqualotto A, de Quadros M, Prezzi S (2004). "Deficiency of the eighth component of complement associated with recurrent meningococcal meningitis--case report and literature review". Braz J Infect Dis 8 (4): 328-30. PMID 15565265. 
  4. ^ Waterhouse R (1911). "A case of suprarenal apoplexy". Lancet 1: 577–8. DOI:10.1016/S0140-6736(01)60988-7. 
  5. ^ Friderichsen C (1918). "Nebennierenapoplexie bei kleinen Kindern". Jahrb Kinderheilk 87: 109–25. 

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