Waterhouse-Frideriksen Syndrome

What is Waterhouse-Frideriksen Syndrome?

Fulminant meningococcemia, or Waterhouse-Frideriksen syndrome, is a meningococcemia with symptoms of vasomotor collapse and shock. This is the most severe, prognostically extremely unfavorable form of meningococcal infection. In essence, it is an infectious toxic shock. It occurs in 10–20% of patients with generalized meningococcal infection and often ends in death.

Causes of Waterhouse-Frideriksen Syndrome

The causative agent of meningococcal infection is Gram-negative diplococcus Neisseria meningitidis, highly sensitive to environmental factors: it dies when the temperature, humidity, solar radiation intensity changes, and is whimsical to the conditions of cultivation. Outside the body, vitality is maintained for no more than 30 minutes. Known 13 serotypes of the pathogen. Epidemiological significance in Ukraine are serotypes A, B, C. Occasionally there are polyagglutinating strains. With direct microscopy of blood and cerebrospinal fluid, the pathogen is detected intra- and extracellularly in the form of co-located cocci in the form of coffee beans. In the cell membrane of the microbe there is a lipopolysaccharide, which is responsible for the endotoxin-like effect in meningococcal sepsis.

In most developed countries, the prevalence of meningococcal infection is 1-3 cases per 100 thousand population, occurs at any age, however, 70-80% are children up to three years. Children of the first three years, especially the first year of life, are most susceptible to the development of hypertoxic (over-acute) infections.

There are epidemiological increases in the incidence of disease every 10–20 years, which is associated with a change in the causative agent and a change in the immunological structure of the population (an increase in the susceptible stratum due to born children, reduced immunity in adults). There are also seasonal increases in the incidence (March-April-May), although patients with this infection are recorded throughout the year.

The source of the disease is a sick or bacillicarrier. The transmission mechanism is airborne. The most infectious patients with catarrhal phenomena from the nasopharynx. Healthy carriers also have epidemiological significance, since their number is hundreds of times higher than the number of patients. It is known that a harbinger of the next rise in incidence is the increase in the number of carriers of meningococcus. In children’s groups, close long-term contact indoors contributes to infecting children, especially in bedrooms. Susceptibility to meningococcus is quite high. But the peculiarity is that the characteristic clinical picture is observed only in 0.5% of those infected. Familial susceptibility to meningococcal disease is extremely rare. In these families, there were cases of meningococcemia and meningitis of different children at different times (with an interval of several years), as well as repeated cases of the disease in one of these children.

Mortality of children of the first year of life is the highest. The main reason is hyperacute meningococcal sepsis with the development of infectious toxic shock (ITS), as well as severe purulent meningitis, complicated by edema-swelling of the brain. Great impact on the outcome of the disease have the timeliness of diagnosis, timeliness and correctness of the initiated treatment.

Pathogenesis during Waterhouse-Frideriksen Syndrome

Waterhouse-Frideriksen syndrome, as well as the primary chronic insufficiency of the adrenal cortex, is associated with the destruction of the cortex. Since the destructive process in this case is more intensive (usually it is adrenal hemorrhage, rarely acute ischemia), adrenal insufficiency syndrome develops suddenly, without the previous stage of chronic corticosteroid deficiency. The pathogenesis of the classic Waterhouse-Friderikson syndrome, which occurs on the background of a severe, acutely current (usually septic) infection, has not been completely clarified. The opinion is expressed that this syndrome is a special case of the so-called general adaptation syndrome, i.e. a consequence of severe sepsis, causing overstrain and depletion of adaptive pituitary-adrenal mechanisms. This point of view is confirmed by the fact that in many cases of the typical (according to etiology and clinical picture) Waterhouse-Frideriksen syndrome in the adrenal glands there are no anatomical changes. Apparently, this is the same mechanism of development of acute adrenal insufficiency in newborns during complicated labor or in pregnant women with severe toxemia, but it is possible that in all the cases under consideration this syndrome is a consequence of some other mechanisms.

If we consider the syndrome of Waterhouse-Frideriksen as a stage of exhaustion of the general adaptation syndrome, then, obviously, addisonic crisis should also be included here, which sometimes develops during severe somatic diseases, extensive injuries or complex surgical interventions. The adaptive role of the pituitary-adrenal system in the formation of responses to the action of exogenous factors has been established and generally recognized. In accordance with the theory of general adaptation syndrome with prolonged exposure to adverse (stressful) factors, a depletion stage develops, which is accompanied by a decrease in adrenal function and morphological changes in their cortical layer (Selye, 1960). Numerous clinical studies have confirmed that the function of the adrenal cortex is reduced in severe suppurative diseases, pulmonary tuberculosis, gastric ulcer and duodenal ulcer, malignant tumors, heart defects, etc.

Symptoms of Waterhouse-Friedexen Syndrome

The disease begins unexpectedly and often within a few hours the patient is in deep prostration. The temperature rises quickly, chills are noted. Within a few hours, numerous petechiae and bruises appear on the body, and in some places the skin acquires a color resembling dead spots. Petechiae and purpura elements increase rapidly, with extensive subcutaneous hemorrhages likely. At the beginning of the disease, before the onset of shock, a generalized vasospasm is noted; the patient’s consciousness is light, he is pale, his lips are bluish, his hands and feet are cold to the touch. Meningeal syndrome, as a rule, is expressed even in cases where the cerebrospinal fluid remains unchanged. There is high hyperleukocytosis in the blood with a sharp neutrophilic shift. Blood pressure progressively drops. Pulse frequent, barely perceptible, soon ceases to be palpable. Increased cyanosis, dizziness, vomiting (often bloody), convulsions. Shortness of breath increases. Patients fall into a rapidly progressing prostration with loss of consciousness. Disturbance of salt and carbohydrate metabolism, excretion of 17-ketosteroids and other symptoms that could clarify the pathogenesis of the disease, with acute adrenal insufficiency do not have time to develop. In the absence of active hormone replacement therapy, coma develops and death occurs within 16-30 hours from the onset of the disease. Sudden death is the result of acutely developed adrenal insufficiency. At the autopsy find a sharp plethora of the adrenal glands and hemorrhages in them. Hemorrhage in the adrenal glands has in recent years been the most frequent direct cause of death in meningococcal sepsis. Rarely observed atrophy of the adrenal glands, also leading to the development of acute failure.
With the onset of shock, the patient falls into a coma, his heart output decreases and his blood pressure decreases. If the incipient shock is not recognized and a suitable treatment is urgently prescribed, death is almost inevitable as a result of cardiac and / or respiratory failure. During the recovery period, the patient can observe abundant peeling in places of eruption or loss of fingers as a result of gangrene.

In the absence of rational therapy, patients die in the first hours and days of the disease from acute cardiovascular or acute renal failure.

Diagnosis of Waterhouse-Frideriksen Syndrome

The diagnosis of all forms of meningococcal infection is based on a set of data obtained by epidemiological, anamnestic and clinical methods, and is finally determined using laboratory tests. Separate methods have different diagnostic value in various clinical forms of meningococcal infection. Thus, the diagnosis of meningococcal secretion is possible only when using the bacteriological method, when the material for the study is mucus from the proximal upper respiratory tract.

In the diagnosis of meningococcal nasopharyngitis, the main place is occupied by epidemiological and bacteriological methods, since it is impossible or extremely difficult to clinically distinguish meningococcal nasopharyngitis from another genesis.

In recognition of generalized forms, anamnestic and clinical diagnostic methods are of real diagnostic value, especially with a combination of meningococcemia and meningitis. In the diagnosis of meningitis is of great importance the study of cerebrospinal fluid.

In generalized forms of meningococcal infection, the final diagnosis is verified by the bacteriological method.

Of the immunological methods are the most sensitive and informative RNGA, ELISA.

Waterhouse-Frideriksen Syndrome Treatment

Therapeutic tactics for meningococcal infection depends on the clinical form, severity of the disease, the presence of complications, premorbid background.

Prevention of Waterhouse-Frideriksen Syndrome

When a diagnosis of meningococcal infection is established in each case, an emergency notification is sent to the district or city SES. In the children’s institution, quarantine is established for 10 days from the moment of isolation of the last patient. A bacteriological examination (nasopharyngeal smear) of contact with the carrier at the place of residence and in the children’s institution is carried out. Contact children in an institution are under observation: daily examination of the skin, pharyngeal mucosa, thermometry. They are assigned rifampicin for 2 days at an age dosage.