Waterhouse-Friderichsen syndrome

Infobox_Disease
Name = Waterhouse-Friderichsen syndrome


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 a disease of the adrenal glands mostly commonly caused by the bacterium "Neisseria meningitidis". The infection leads to massive hemorrhage into one or (usually) both adrenal glands.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] It is characterized by overwhelming bacterial infection meningococcemia, low blood pressure and shock, disseminated intravascular coagulation (DIC) with widespread purpura, and rapidly developing adrenocortical insufficiency.

Epidemiology

"Meningococcus" is another term for the bacterial species "Neisseria meningitidis", blood infection with which usually underlies WFS.

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

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 disease which damages the spleen), or who have certain immune disorders, such as a complement deficiency.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]

igns and symptoms

WFS 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. Low blood pressure (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 ACTH stimulation test demonstrates the acute adrenal failure. Leukocytosis need not to be extreme and in fact leukopenia may be seen and it is a very poor prognostic sign. C-reactive protein levels can be elevated or almost normal. Thrombocytopenia is sometimes extreme, with alteration in prothrombin time (PT) and partial thromboplastin time (PTT) suggestive of diffuse intravascular coagulation (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 was once the drug of choice with chloramphenicol as a good alternative in allergic patients. Ceftriaxone is an antibiotic commonly employed today. Hydrocortisone can sometimes reverse the hypoadrenal shock. Sometimes plastic surgery and grafting is needed to deal with tissue necrosis.

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.cite journal | author = Waterhouse R | title = A case of suprarenal apoplexy | journal = Lancet | year = 1911 | volume = 1 |pages = 577–8 | doi = 10.1016/S0140-6736(01)60988-7 ] cite journal | author = Friderichsen C | title = Nebennierenapoplexie bei kleinen Kindern | journal = Jahrb Kinderheilk | year = 1918 | volume = 87 | pages = 109–25 ]

References

External links

* [http://www.whonamedit.com/synd.cfm/2187.html Whonamedit.com] page on the eponym