Waterhouse-Friderichsen syndrome
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2008-06-18T04:06:11Z
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{{Infobox_Disease |
Name = Waterhouse-Friderichsen syndrome |
Image = |
Caption = |
DiseasesDB = 29316 |
ICD10 = {{ICD10|A|39|1|a|30}}, {{ICD10|E|35|1|e|20}} |
ICD9 = {{ICD9|036.3}} |
ICDO = |
OMIM = |
MedlinePlus = |
eMedicineSubj = med |
eMedicineTopic = 3009 |
MeshID = D014884 |
}}
'''Waterhouse-Friderichsen syndrome''' (WFS) is massive, usually bilateral, [[hemorrhage]] into the [[adrenal gland]]s caused by [[Wiktionary:fulminant|fulminant]] [[meningococcemia]].<ref name=Robbins_2005>{{cite book | author = Kumar V, Abbas A, Fausto N | title = Robins and Coltran: Pathological Basis of Disease | edition = 7th | publisher = Elsevier | year = 2005 | pages = pp. 1214–5 | id = ISBN 978-0721601878}}</ref> WFS is characterised by overwhelming bacterial infection, rapidly progressive [[hypotension]] leading to [[Shock (medical)|shock]], [[disseminated intravascular coagulation]] (DIC) with widespread [[purpura]], particularly of the skin, and rapidly developing [[adrenal insufficiency|adrenocortical insufficiency]] associated with massive bilateral adrenal hemorrhage.
==Epidemiology==
''Meningococcus'' is another term for the [[bacteria]]l 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.{{Fact|date=February 2007}} 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. Finally, infants between 6 months and 2 years of age are also at a high risk, since they have lost maternal antibodies against the bacteria by the age of 6 months, and it will take them until the age of 2 years before they start producing their own antibodies.
WFS can also be caused by ''[[Streptococcus pneumoniae]]'' infections, a common bacterial pathogen typically associated with meningitis in the adult and elderly population.<ref name=Robbins_2005 /> ''[[Staphylococcus aureus]]'' has recently also been implicated in [[Pediatrics|pediatric]] WFS.<ref name=Adem_2005>{{cite journal |author=Adem P, Montgomery C, Husain A, Koogler T, Arangelovich V, Humilier M, Boyle-Vavra S, Daum R |title=Staphylococcus aureus sepsis and the Waterhouse-Friderichsen syndrome in children |journal=N Engl J Med |volume=353 |issue=12 |pages=1245–51 |year=2005 |pmid=16177250 |doi=10.1056/NEJMoa044194}}</ref>
Routine vaccination against meningococcus is recommended for people who have poor [[spleen|splenic]] function (who, for example, have had their spleen [[splenectomy|removed]] or who have [[Sickle-cell disease|sickle-cell anemia]] which damages the spleen), or who have certain immune disorders, such as [[Complement system|complement]] deficiency.<ref name=Rosa_2004>{{cite journal |author=Rosa D, Pasqualotto A, de Quadros M, Prezzi S |title=Deficiency of the eighth component of complement associated with recurrent meningococcal meningitis--case report and literature review |journal=Braz J Infect Dis |volume=8 |issue=4 |pages=328–30 |year=2004 |pmid=15565265 |doi=10.1590/S1413-86702004000400010}}</ref>
==Historical==
Waterhouse-Friderichsen syndrome is [[eponym|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.<ref name=Waterhouse_1911>{{cite journal | author = Waterhouse R | title = A case of suprarenal apoplexy | journal = [[The Lancet|Lancet]] | year = 1911 | volume = 1 |pages = 577–8 | doi = 10.1016/S0140-6736(01)60988-7 }}</ref><ref name=Friderichsen_1918>{{cite journal | author = Friderichsen C | title = Nebennierenapoplexie bei kleinen Kindern | journal = Jahrb Kinderheilk | year = 1918 | volume = 87 | pages = 109–25 }}</ref>
== Symptoms ==
Waterhouse-Friderichsen syndrome is the most severe form of meningococcal septicemia.
The onset of the illness is nonspecific with fever, rigors, vomiting and headache. Soon a rash appears; first macular, not much different from the rose spots of typhoid, and rapidly becoming petechial and purpuric with a dusky gray color. Hypotension is the rule and rapidly leads to septic shock. The cyanosis of extremities can be impressive and the patient is very prostrated or comatose. In this form of meningococcal disease, meningitis generally does not occur.
There is hypoglycemia with hyponatremia and hyperkalemia, and the Synachten test demonstrates the acute suprarenal failure. Leukocytosis need not to be extreme and in fact leukopenia may be seen and it is a very poor prognostic sign. CRP levels can be elevated or almost normal. Thrombocytopenia is sometimes extreme, with alteration in PT and PTT suggestive of DIC.
Acidosis and acute renal failure can be seen as in any severe sepsis.
Meningococci can be readily cultured from blood or CSF, and can sometimes be seen in smears of cutaneous lesions.
== Treatment ==
Fulminant meningococcemia is a medical emergency and need to be treated with adequate antibiotics as fast as possible. Benzylpenicillin is the drug of choice with chloramphenicol as a good alternative in allergic patients. Hydrocortisone can sometimes reverse the hypoadrenal shock. Sometimes plastic surgery and grafting is needed to deal with tissue necrosis
Ceftriaxone is an antibiotic commonly employed today.
==References==
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==External links==
* [http://www.whonamedit.com/synd.cfm/2187.html Whonamedit.com] page on the eponym
{{Bacterial diseases}}
[[Category:Bacterial diseases]]
[[Category:Syndromes]]
[[de:Waterhouse-Friderichsen-Syndrom]]
[[es:Síndrome de Waterhouse-Friderichsen]]
[[pl:Zespół Waterhouse'a-Friderichsena]]