Waterhouse-Friderichsen syndrome


Waterhouse-Friderichsen syndrome

Description, Causes and Risk Factors:

Abbreviation: WFS.

A condition occurring mainly in children under 10 years of age, characterized by vomiting, diarrhea, extensive purpura, cyanosis, tonic-clonic convulsions, and circulatory collapse, usually with meningitis and hemorrhage into the adrenal glands.

The Waterhouse-Friderichsen syndrome is a clinical entity first described in 1911 by Waterhouse in an eight-month-old child. In 1918, Friderichsen reviewed the literature and described a similar case with a fulminating course, characterized clinically by stupor, cyanosis, pallor, vomiting and a rapidly spreading purpuric eruption, and at autopsy by massive bilateral adrenal hemorrhages. This syndrome is associated with a bacteremia, the etiologic agent in the majority of cases being the meningococcus. Although the pneumococcus, hemolytic streptococcus and staphylococcus have also been inculpated, considerable doubt has been cast on the role of any of these organisms as the offending agent.

Waterhouse-Friderichsen syndrome

Most of the cases have occurred in children, but with the recent and widespread increase in meningococcal infections there have been many additional reports of this syndrome in adults. Although in the majority of reported cases the patients succumbed within twenty-four hours of onset, a few survived for as long as forty-eight hours. Recently, 2 cases with survival periods of eighty and eighty-eight hours both proved by autopsy, were described. Evidently this syndrome is not universally fatal, for there are 6 cases of recovery on record.

Causes and Risk Factors:

Multiple species of bacteria can be associated with the condition:

    Meningococcus is another term for the bacterial species Neisseria meningitidis; blood infection with said species usually underlies WFS. While many infectious agents can infect the adrenals, an acute, selective infection is usually Meningococcus.

  • WFS can also be caused by Streptococcus pneumoniae infections, a common bacterial pathogen typically associated with meningitis in the adult and elderly population.

  • Mycobacterium tuberculosis could also cause WFS. Tubercular invasion of the adrenal glands could cause hemorrhagic destruction of the glands and cause mineralocorticoid deficiency.

  • Staphylococcus aureus has recently also been implicated in pediatric WFS.

  • It can also be associated with Haemophilus influenzae.

  • Cytomegalovirus can cause adrenal insufficiency,[6] especially in the immunocompromised.

Rarely, Waterhouse-Friderichsen syndrome can be caused by the use of medications that promote blood clotting. Other causes include:

Waterhouse-Friderichsen syndrome is more common in males, probably reflecting a male predilection for several of the underlying conditions associated with adrenal hemorrhage.

Symptoms:

People affected with Waterhouse-Friderichsen syndrome experience dehydration and dizziness on a frequent manner. Bleeding is a common scene. In addition, there is a blue tinge on the mouth and the complexion starts becoming pale. At this stage you experience high fever, chills, headache, backache and weight-loss due to loss of appetite etc. These symptoms can be followed by a kind of shock and coma. This can lead to damage of brain and needs immediate treatment.

Diagnosis:

It is appropriate at this point to consider the diagnostic problem posed by the Waterhouse-Friderichsen syndrome. Since the incidence of this entity is constantly rising, the necessity for uniform criteria can hardly be disputed. From a survey of the foregoing material, there have been gathered clinical and laboratory data on which a diagnosis of this syndrome can be based. The following points are regarded to be of diagnostic significance: petechial eruption rapidly becoming purpuric; severe shock; facial edema; anuria of twenty-four to thirty-six hours' duration, followed by marked oliguria; urinalysis showing fixation of the specific gravity, albuminuria, hematuria or cylindruria; marked leukocytosis; isolation of the meningococcus by culture and, in some cases, by peripheral blood smear; renal failure, as evidenced by elevation of the blood nonprotein nitrogen and creatinin; and elevation of the blood sodium and diminution of the blood potassium.

A diagnosis of this syndrome cannot be based solely on any isolated factor mentioned above. On the basis of these studies it is concluded that the clinical signs per se are sufficient to warrant the diagnosis in acute, fulminating cases. With a more prolonged course, however, additional factors involving the alterations in blood chemistry, as well as the isolation of the meningococcus, must be consistently present to confirm the diagnosis.

Synacthen test may be used in order to demonstrate the acute suprarenal failure of the person affected with the Waterhouse-Friderichsen syndrome.

Treatment:

Waterhouse-Friderichsen syndrome is a medical emergency and needs 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.

The therapeutic approach in the Waterhouse-Friderichsen syndrome resolves itself into the treatment of shock, toxemia and bacteremia and the administration of adrenocortical hormone substitution therapy.

Shock is combated in the usual manner. The employment of heat and stimulants and the antishock position are advocated. Parenteral fluids in quantities of 3000 to 4000 cc should be given cautiously over a twenty-four-hour period. The intravenous administration of 500 cc of plasma, repeated in twelve hours, is recommended because of its osmotic effect, mirrored in a noticeable elevation of the blood pressure.

Toxemia is the gravest problem encountered in this entity, and the pathologic changes are directly attributable to it. Although at the present time there is no clear evidence that serotherapy (treatment of an infectious disease by injection of an antitoxin or serum containing specific antibody) is of value in meningococcal infections, it seems advisable to administer antimeningococcus serum in adequate amounts — 60,000 to 120,000 units — intravenously within the first twenty-four hours.

The control of meningococcemia has been made possible by the advent of chemotherapy. Of all the sulfonamide drugs, sulfadiazine is the derivative of choice. The necessity for massive doses of this drug, parenterally and orally, is apparent when one considers the overwhelming character of the bacteremia. The initial dose of sulfadiazine in this series was 5 gm intravenously and 8 gm orally. This was followed by large oral doses until a total of 25 to 30 gm had been given within the first twenty-four hours. The blood-sulfadiazine concentration was used as a guide for determining the size and frequency of subsequent doses. The optimal blood level was considered to be 15to 20 mg. per 100 cc.

The rationale of adrenocortical hormone substitution therapy in the Waterhouse-Friderichsen syndrome has been directed toward combating adrenal dysfunction resulting from the extensive hemorrhages and necrosis. The hope that this form of organotherapy (treatment of disease by preparations made from animal organs; now frequently by synthetic preparations instead of extracts of a gland) would succeed in tiding the patient over his critical period has met with disappointment. Experience indicates that its value is questionable. No resultant clinical improvement could be detected in the 4 cases in which it was used. Nevertheless, it may have contributed to the prolongation of the clinical course.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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