The adrenal glands in the body are an important endocrine organ. The hormones produced by them affect pressure indicators and the amount of circulating fluid, the level of mineral salts and individual trace elements, the metabolic rate, and even 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
Waterhouse-Friederiksen syndrome is an acute adrenal insufficiency that occurs after an organ is traumatized with a hemorrhage in 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 insufficient, and in 1911 Waterhouse identified fifteen cases of the disease that helped him put all the pieces together. Keeping up with him, seven years later, in 1918, Frederiksen also publishes an essay on this pathological process.
Causes
Scientists agree that Waterhouse-Friedericksen syndrome is caused by massive hemorrhage simultaneously in both adrenal glands. Potentially dangerous contingents are newborns, infants, 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 promotion through the birth canal using forceps or a vacuum extractor. In addition, severe congenital diseases, as well as complication of pregnancy with late gestosis, can play an important role.
Waterhouse-Friedericksen syndrome in adults most often manifests itself as a complication of an infectious disease. Usually the causative agent is meningococcus, streptococcus or staphylococcus. But besides them, the causes of hemorrhages can be such nosologies as measles, scarlet fever, typhoid, diphtheria, as well as malignant neoplasms, tumors, peritonitis, and DIC with adrenal vein thrombosis.
Disease development
Many authors identify this syndrome as part of a general failure of the adaptive mechanism in the development of acute sepsis. But it is possible that young children and pregnant women have a completely different mechanism by which Waterhouse-Friedericksen syndrome develops. Its pathogenesis is manifested in numerous hemorrhagic necrosis in the adrenal cortex. There are so many of them that the whole organ is soaked in blood, the capsule is overstretched and even ruptured.
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.
Symptomatology
Waterhouse-Friedericksen syndrome with meningococcal infection can develop suddenly against the background of complete well-being. It progresses so fast that a person can die within 24 hours.
It all starts with excessive irritability, irritability and headache. Then abdominal pain, nausea, vomiting and diarrhea join. If the pain syndrome is too intense, then the doctor may suspect acute surgical pathology in the patient. Initially, the temperature is low, but in just a couple of hours from the onset of the disease, it will be 39-40 degrees. Due to vomiting and diarrhea, water and mineral substances leave the body, which leads to disruption of the heart, brain and other vital organs. In the end, 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. There may be no other symptoms at all. At first the person is in a stupor, but as hypoxia progresses, stupor and coma quickly ensue. He dies without regaining consciousness.
Waterhouse-Friedericksen 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 a day from the onset of the disease.
Diagnostics
Waterhouse-Friedericksen syndrome is developing so fast that often doctors do not have time to conduct a full examination and understand what is happening to the person. If the doctor on duty suspected this pathology in the
emergency room , then the treatment begins immediately, and the diagnosis is joined in the process, because the count goes on the clock.
Laboratory confirmation of the diagnosis must be detected in a general blood test:
- leukocytosis and shift of the formula to the left;
- decrease in blood glucose;
- thrombocytopenia and low coagulability;
- decrease in electrolytes;
- increase in blood nitrogen and urea.
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 conduct a lumbar puncture and describe the neurological status. But all this is done after stabilization of the human condition.
Differential diagnosis
Waterhouse-Friedericksen syndrome should be distinguished from surgical diseases of the abdomen, such as acute appendicitis, acute pancreatitis, perforation of a stomach ulcer or duodenal ulcer. Be sure the doctor must check for meningeal symptoms to rule out brain hemorrhage, cavernous sinus thrombosis. With a cardiac form, an ECG will help to understand whether a person has a heart attack or not.
Treatment
First of all, after the diagnosis of Waterhouse-Friedericksen Syndrome is diagnosed, it is necessary to compensate the patient for sweating fluid, electrolytes and insufficiency of adrenal hormones. To do this, up to half a liter of Hydrocortisone or 120 milligrams of Prednisolone is administered via venous access, 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 in the pathogenesis that cause Waterhouse-Friedericksen syndrome. Emergency care simultaneously improves blood rheology, dilutes it, increases the amount of systemic fluid and increases pressure. And the replacement of steroid hormones helps to maintain pressure at the right level.
After stabilization, hormones continue to be administered intramuscularly: "Hydrocortisone" at 50-75 milligrams every six hours, and deoxycorticosterone acetate at ten milliliters three times a day. Be sure to monitor the pressure and, if necessary, pin up "Epinephrine", "Mesatone", cardiac glycosides.
If the cause of the disease is an infection, for example, meningococcal, then, in addition to the main therapy, antibiotics are administered to the patient. As soon as the patient's condition returns to normal, hormone doses begin to gradually decrease. It is very important to properly prepare the body for the fact that it must independently produce glucocorticosteroids. You can not abruptly cancel the drugs, this can provoke a second crisis.
It is important to confirm that the patient had exactly Waterhouse-Friedericksen syndrome. Treatment itself can be dangerous, since excessive flooding of the body causes systemic edema, including brain edema, and large doses of hormones can provoke mental disorders. Therefore, it is extremely important to constantly monitor the patient's condition and adjust the appointment according to his needs.
Forecast
Waterhouse-Friedericksen syndrome is an extremely severe pathology, which is not always possible to identify on time due to a variety of nonspecific symptoms. The outcome depends not only on how competently the doctor will behave in an emergency, but also on the degree of damage to the adrenal glands and the compensatory capabilities of the body. A frequent finale of this pathology is death.
Prevention
Basically, these are anti-epidemic measures in the foci of infection, in which a patient with meningococcal infection was identified. The doctor who has identified such a patient is required to notify the Sanitary and Epidemiological Service and isolate the patient. In the next three days, the 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 prophylaxis, people who have been in close contact with the patient are prescribed a short course of antibiotics.
Epidemiology
Waterhouse-Friedericksen syndrome, unfortunately, is a fairly common pathology. In developed countries, the prevalence of meningococcal infection is 1-3 cases per 100 thousand population. And more than half of the cases are pre-school children. In addition, every decade there is an increase in incidence. This is due to a mutation of the pathogen and a decrease in the immunity of the population.
These statistics are most likely not true, as many cases remain undiagnosed due to cross-symptoms. Waterhouse-Friedericksen syndrome is developing so fast that doctors do not have time to conduct a sufficient number of tests.