Waterhouse-Friderichsen syndrome

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.

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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]

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]

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.

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