Waterhouse - Frideriksen syndrome: pathogenesis, emergency treatment, treatment

Adrenal glands in the body are an important endocrine organ. The hormones produced by them affect the pressure indices and the amount of circulating fluid, the level of mineral salts and individual trace elements, the rate of metabolism, even the work of the pituitary gland. And in situations where the function of these small but extremely important glands suffers, conditions that threaten life and health develop.

Definition and history

The Waterhouse-Frideriksen syndrome is an acute adrenal insufficiency that occurs after traumatizing an organ with a hemorrhage into its parenchyma. This causes extremely severe violations of the constancy of the internal environment of the body and can lead to death.

The description of this condition appeared for the first time in 1894, but it was not enough, and in 1911 Waterhouse identified fifteen cases of the disease, which helped him to collect all the details together. Not lagging behind him, seven years later, in 1918, Frederiksen also publishes an essay on this pathological process.

Causes

Scientists agree that the Waterhouse-Frideriksen syndrome is caused by a massive hemorrhage simultaneously in both adrenal glands. Potentially dangerous contingent are newborns, babies, children and young people. Sex in this case does not matter. This condition can be triggered by prolonged childbirth, oxygen starvation of the fetus or its progression through the birth canal using forceps or a vacuum extractor. In addition, severe congenital diseases, as well as the complication of pregnancy with late gestosis, can play an important role.

The syndrome of Waterhouse - Frideriksen in adults is most often manifested as a complication of an infectious disease. Usually, the causative agent is meningococcus, streptococcus or staphylococcus. But besides them, the causes of hemorrhage can be such nosologies as measles, scarlet fever, typhoid, diphtheria, as well as malignant tumors, tumors, peritonitis and DIC-syndrome with adrenal vein thrombosis.

Development of the disease

Many authors distinguish this syndrome as part of a general malfunction of the adaptive mechanism in the development of acute sepsis. But it is possible that in young children and pregnant women a completely different mechanism by which the syndrome of Waterhouse - Frideriksen develops. Its pathogenesis is manifested in numerous hemorrhagic necrosis in the adrenal cortex. There are so many of them that the whole body is soaked with blood, the capsule over-extends, and even its rupture.

As for the general pathogenesis, it manifests itself in the form of sepsis:

- dilated capillaries and arterioles;
- pronounced intoxication syndrome;
- the presence of concomitant meningitis or meningoencephalitis;
- an increase in the thymus and regional lymph nodes.

Symptomatics

The syndrome of Waterhouse - Frideriksen with meningococcal infection can develop suddenly against the background of complete well-being. He progresses so quickly that a person can die within a day.

It all begins with excessive excitability, irritability and headache. Then pain in the abdomen, nausea, vomiting and diarrhea join. If the pain syndrome is too intense, then the doctor may suspect the patient of acute surgical pathology. Initially, the temperature is low, but just a couple of hours from the onset of the disease, it will be 39-40 degrees. Because of vomiting and diarrhea, water and minerals leave the body, which leads to disruption of the heart, brain and other vital organs. Eventually a person loses consciousness and falls into a coma.

There is another form of this disease, manifested in a sharp drop in blood pressure or collapse. Other symptoms may not be at all. The person is at first in a stupor, but as hypoxia progresses, the cox and coma quickly come. Dies without regaining consciousness.

The Waterhouse-Frideriksen syndrome in children develops faster than in adults, regardless of the cause. Given that their compensatory capabilities of the body are rapidly depleted, and many systems and organs are still in the process of formation and development, the result comes earlier than the day after the onset of the disease.

Diagnostics

The syndrome of Waterhouse - Frideriksen develops so quickly that often doctors do not have time to conduct a full survey and understand what is happening to the person. If in the waiting room the doctor on duty suspects this pathology, then the treatment starts immediately, and the diagnosis joins in the process, because the account goes on for hours.

Laboratory to confirm the diagnosis should be found in a general blood test:

- leukocytosis and shift the formula to the left;
- Reduced blood glucose;
- Thrombocytopenia and low coagulation;
- reduction of electrolytes;
- Increase in nitrogen and urea blood.

However, these indicators are not specific and may indicate a wide range of surgical and somatic diseases. In order to be sure, it is necessary to carefully collect the anamnesis, as well as to conduct a lumbar puncture and describe the neurological status. But all this is done after the stabilization of the human condition.

Differential diagnosis

The Waterhouse-Frideriksen syndrome should be distinguished from surgical abdominal diseases such as acute appendicitis, acute pancreatitis, perforation of the stomach or duodenal ulcer. The doctor must check the presence of meningeal symptoms to exclude cerebral hemorrhage, cavernous sinus thrombosis. With a cardiac form, the ECG will help to understand whether a person has a heart attack or not.

Treatment

First of all, after the diagnosis of "Waterhouse-Frideriksen syndrome" it is necessary to compensate the patient for fluid flow, electrolytes and adrenal hormones insufficiency. To do this, through a venous access enter up to half a liter of "Hydrocortisone" or 120 milligrams of "Prednisolone", then three grams of five percent glucose and ten milliliters of a five percent solution of ascorbic acid. So we will act on the main links of pathogenesis, which cause the syndrome of Waterhouse - Frideriksen. Emergency care simultaneously improves blood rheology, dilutes it, increases the amount of systemic fluid and increases blood pressure. A substitution of steroid hormones helps maintain the pressure at the desired level.

After stabilizing the condition, hormones continue to be injected intramuscularly: "Hydrocortisone" is 50-75 milligrams every six hours, and deoxycorticosterone acetate is ten milliliters three times a day. Be sure to monitor the pressure indicators and, if necessary, puncture the "Epinephrine", "Mesaton", cardiac glycosides.

If the cause of the disease is an infection, for example, meningococcal, then, in addition to basic therapy, the patient is administered antibiotics. As soon as the patient's condition returns to normal, doses of hormones begin to gradually decrease. It is very important to properly prepare the body for the fact that it must independently produce glucocorticosteroids. It is impossible to abruptly cancel preparations, it can provoke a repeated crisis.

It is important to confirm that the patient had the Waterhouse-Friderichsen syndrome. Treatment alone can be dangerous, because excessive flooding of the body causes systemic edema, including cerebral edema, and large doses of hormones can provoke mental disorders. Therefore, it is extremely important to constantly monitor the patient's condition and adjust appointments according to his needs.

Forecast

The Waterhouse-Frideriksen syndrome is an extremely difficult pathology, which can not always be identified on time because of the variety of nonspecific symptoms. The outcome depends not only on how competently the doctor behaves in an emergency situation, but also on the degree of defeat of the adrenal glands and compensatory possibilities of the body. A frequent outcome of this pathology is a fatal outcome.

Prevention

Basically, these are anti-epidemic measures in the focus of infection, in which a patient with meningococcal infection is identified. The doctor who identified such a patient is required to notify the sanitary epidemiological service and isolate the patient. In the next three days, contact persons are checked for the presence of the disease and quarantine is introduced in the work collective or educational institution for a period of ten days. As a preventive measure, people who are in close contact with the patient are prescribed a short course of antibiotics.

Epidemiology

The syndrome of Waterhouse - Frideriksen, unfortunately, quite frequent pathology. In developed countries, the prevalence of meningococcal infection is 1-3 cases per 100 thousand of the population. And more than half of the sick are preschool children. In addition, every decade there is an increase in morbidity. This is due to the mutation of the pathogen and a decrease in the immunity of the population.

These statistics are likely not true, as many cases remain undiagnosed due to cross-over symptoms. The Waterhouse-Frideriksen syndrome develops so quickly that doctors do not have enough time to perform a sufficient number of tests.