Synonyms
Adrenal apoplexy (failure of the adrenal glands due to bleeding or vascular occlusion), suprarenal apoplexy
Definition and introduction
The Waterhouse Friedrichsen Syndrome is a state of shock caused by toxins (bacterial toxins) produced by the bacteria. There is an extreme consumption of coagulation factors (consumption coagulopathy) and death of tissue (haemorrhagic necrosis) of the adrenal cortices, mostly as a result of bacterial blood poisoning (meningococcal sepsis). Waterhouse Friedrichsen syndrome occurs in about 10% to 20% of patients with meningococcal sepsis.
Mortality (lethality) is very high, up to 90%, despite treatment. Untreated, Waterhouse Friedrichsen syndrome is always fatal. At the moment, the peak age is still in infancy, but in the meantime more often adolescents are affected, which indicates that the number of new cases (incidence) is slowly shifting towards adulthood.
The Waterhouse Friedrichsen Syndrome is caused by the massive release of toxins from the causing bacteria. These are mostly meningococci. Pneumococci and Haemophilus influenzea can also trigger this severe disease pattern.
The release of toxins leads to a massive activation of the coagulation factors in the blood. As a result, a large number of thrombi are formed, which lead to the occlusion of the blood vessels. On the other hand, the high consumption of coagulation factors in other areas leads to massive bleeding.
These occur in the skin, mucous membrane and internal organs. This puts patients in a shock situation and they bleed to death. Endotoxin shock also occurs, which leads to functional impairment of the liver, kidneys, adrenal glands and lungs.
Clinically, the Waterhouse Friedrichsen Syndrome leads to massive bleeding into the skin, so-called petechiae. In addition, mucous membrane bleeding and intravital death spots occur. These are cold, livid skin areas where the blood stagnates.
Furthermore, the Waterhouse Friedrichsen Syndrome shows various signs of shock. The kidneys stop working, i.e. there is very little or no urine excretion. In addition, there is respiratory distress, due to the shock state of the lungs, and a yellow coloration of the patient, due to a shock liver.
If the brain vessels are blocked by thrombi, neurological abnormalities occur. These include cramps and somnolence. Typically, these symptoms develop within a few hours.
The symptomatology (clinical picture) is considered an important indication, since Waterhouse Friedrichsen Syndrome must be treated as quickly as possible. Therefore, a combination of a large number of small bleedings on the skin (petechiae), fever and diarrhoea should already make one think of a Waterhouse Friedrichsen syndrome. In the blood the different coagulation and fibrinolysis tests are conspicuous, since all coagulation factors are massively consumed.
In addition, the number of white blood cells (leukocytes) and thrombocytes is reduced. It is important to start the antibiotic therapy as early as possible. Penicillin G and cefotaxime are usually used for this.
In addition, the shock state shock of the patient must be combated. This means that fluid must be supplied via the veins to compensate for a lack of volume. In addition, the patient has to be ventilated, the acid-base and electrolyte balance should be balanced.
If massive bleeding is the main concern, platelet concentrates and fresh plasma can be administered. There is no vaccination against the group of meningocococci, which are the most common cause of the Waterhouse Friedrichsen Syndrome and which occur in our country. However, vaccination against pneumococci and haemophilus influenzea is possible.
These are contained in the 6-fold vaccine, which can be given from the 3rd month of life. Nevertheless, antibodies against meningococci are formed naturally in the course of life, which then protect against an invasion and thus make the disease really rare. The Waterhouse Friedrichsen Syndrome is despite its rarity a very dangerous clinical picture, which is very often fatal even under intensive medical therapy. Most important for the survival of the patient is a diagnosis as early as possible, which must be followed by immediate intensive care and antibiotic treatment.
