Amatoxin syndrome is a life-threatening fungal poisoning that occurs in the majority of cases after ingestion of tuberous-leaf mushroom species. The toxins contained in these mushrooms can lead to liver and kidney failure, and in about 10 percent of cases, amatoxin syndrome is lethal despite emergency medical care.
What is amatoxin syndrome?
Amatoxin syndrome is a life-threatening mushroom poisoning that occurs in the majority of cases after ingestion of tuberous-leaf mushroom species. Amatoxin syndrome is the name given to a complex of symptoms that occur after poisoning by certain species of mushrooms that contain amanitins, which are highly toxic to the human organism. In Europe, the most common species of mushrooms that cause an amatoxin syndrome include the green buttonleaf mushroom (Amanita phalloides), the yellow buttonleaf mushroom (Amanita citrina), the cone-hooded buttonleaf mushroom (Amanita virosa), and the white buttonleaf mushroom (Amanita verna). After a latency period of about 6 to 24 hours, the first gastrointestinal symptoms manifest as colicky abdominal pain, vomiting, and nausea, as well as calf cramps and watery diarrhea that can lead to a dangerous loss of electrolytes and fluids (gastrointestinal phase). After about 24 hours, an apparent recovery phase initially occurs, but by this time irreversible damage to internal organs, especially the liver and kidneys, has already begun. This hepatorenal phase is manifested by icterus (jaundice), increase in liver-typical enzymes, internal bleeding due to the decreasing concentration of coagulation factors, an increase in urinary substances, coma hepaticum, and renal failure.
Causes
Amatoxin syndrome is caused by ingestion of certain types of mushrooms that contain amanitins. The toxic effect here is due to the fact that amanitin attaches to the transcriptase known as mRNA polymerase, inhibiting the rewriting of the DNA strand into an RNA strand. As a result of the missing RNA strand, no information reaches the cytoplasm from the nucleus (cell nucleus), where the ribosomes normally encode amino acid sequences with the help of the RNA strand. In cells affected by amatoxin syndrome, this process is suppressed. Since proteins (including hormones and enzymes) can no longer be synthesized and all processes involving these substances fail, the entire cell metabolism (cellular metabolism) collapses. As little as 0.1 mg/kg or 20 to 40 mg of the tuberous leaf fungus is sufficient in an adult human for an amatoxin syndrome to be lethal.
Symptoms, complaints, and signs
Typical signs of mushroom poisoning include nausea and vomiting, chills, fever, and impaired consciousness. The gastrointestinal tract may also experience colic, watery diarrhea, and cramps, which increase in intensity during the first three to four hours after ingestion of the toxin. In addition, dizziness, sweating, and serious respiratory problems such as shortness of breath may occur. Characteristically, the anatoxin syndrome subsides after about four six hours. Then there is apparent relief, but this is quickly interrupted by serious, life-threatening symptoms. Sufferers experience kidney failure relatively quickly, manifested by signs of jaundice: The skin turns yellow, the eye sockets collapse, and sweating occurs. This is accompanied by internal bleeding, which eventually leads to anemia. Such bleeding anemia is noticeable by dizziness, fatigue and pallor. Furthermore, as a result of fungal poisoning, there is a breakdown of the entire cell metabolism, which is noticeable by a rapid decrease in mental and physical performance. After six hours, the organs are usually already irreversibly damaged. Those affected then fall into a coma and die from mushroom poisoning.
Diagnosis and course
In addition to the symptoms characteristic of an amatoxin syndrome, the poisoning can be diagnosed laboratory-diagnostically by a marked decrease in antithrombin III. In addition, histologic evidence of periportal (located around the hepatic orifice) inflammatory infiltrates and centrolobular (located in the middle of the hepatic lobe) necrosis can be detected in the liver.In addition, a determination of the alpha-amanitin concentration in the urine and a so-called lignin test, in which the suspected fungus is tested for amatoxins (blue-green staining) on lignin-containing paper, may be indicated. Due to the comparatively long latency period of more than four hours, during which the toxic substances can spread in the organism, the amatoxin syndrome is one of the most dangerous fungal poisonings. Accordingly, about 10 percent of poisoning cases with amanitin have a lethal course despite emergency medical care.
Complications
Amatoxin syndrome is associated with life-threatening complications that, in the worst case, can lead to the death of the patient if timely countermeasures against tuberous mushroom poisoning are not initiated. The highly toxic amantines of tuberous mushrooms cause gastrointestinal symptoms such as colicky abdominal pain, vomiting, nausea, and watery diarrhea. These can lead to a life-threatening loss of fluids and electrolytes. Other accompanying symptoms include chills, palpitations, shortness of breath, dizziness, sweating, and impaired consciousness. The latency period is six to 24 hours. After that, there is an apparent relief that many patients equate with symptom resolution and recovery. However, irreversible damage to organs such as the liver and kidneys actually occurs during this phase. This part of the disease process is called the hepatorental phase, which is characterized by an increase in liver-typical enzymes, internal bleeding, jaundice, a decreased concentration of coagulation factors, an increase in urinary substances, coma hapaticum, and kidney failure. Tuber fungus poisoning leads to a breakdown of the entire cell metabolism (cellular metabolism), as proteins such as enzymes and hormones are no longer synthesized. Patients with amatoxin syndrome are a medical emergency because the comparatively long latency period of up to six hours during which the toxic substances can spread in the body means that the chances of survival are comparatively low. In about ten percent of patients, despite timely treatment, such a deterioration of health occurs that tuber fungus poisoning takes a lethal course.
When should one go to the doctor?
Amatoxin syndrome must be treated by a doctor in any case. If the syndrome is not treated promptly, in most cases the patient will die directly due to the severe poisoning. Therefore, if the affected person suffers from the symptoms of amatoxin syndrome after eating mushrooms, treatment by a doctor must be urgent. As a rule, either the emergency doctor must be called or a hospital must be visited directly. Most of those affected suffer from vomiting, nausea and diarrhea. The abdomen and stomach are extremely painful and dizziness occurs. Furthermore, disturbances of consciousness or shortness of breath may also be indicative of the complaint. It is not uncommon for strong heart palpitations and panic attacks to occur. If these complaints occur, a doctor must be consulted immediately and treatment started. However, even with treatment, in some cases the patient dies. In many cases, the patient’s condition improves in a few hours in amatoxin syndrome. However, organs are still damaged, so a visit to the doctor is essential.
Treatment and therapy
Amatoxin syndrome is a life-threatening fungal poisoning and is a medical emergency. Here, primary toxin elimination or gastric emptying by mechanical irritation may be indicated in the early stages of amatoxin syndrome. Sustained application of medicated charcoal (activated charcoal) can reduce enterohepatic (affecting the intestinal-liver circulation) toxin circulation and stimulate excretion of the toxin from the organism. In addition, to prevent exsiccosis as well as hypovolemic shock due to the persistent watery diarrhea, compensation for electrolyte and fluid loss is indicated. In addition, silibinin, an active ingredient contained in milk thistle, should be infused as soon as possible to inhibit the infiltration of amanitin into the liver cells. Furthermore, in the context of a substitution therapy or administration of Fresh Frozen Plasma, the decreasing antithrombin III concentration can be compensated. To remove the toxins causing the amatoxin syndrome extracorporeally from the blood, hemoperfusion can be used.The blood of the patient is passed through a column containing activated carbon, for example, which binds the toxins and removes them from the bloodstream. This procedure is generally used when hemodialysis (extracorporeal blood washing) has not been able to eliminate the toxins to a sufficient extent. In the presence of marked liver damage, liver transplantation is indicated for amatoxin syndrome.
Outlook and prognosis
Due to amatoxin syndrome, the patient may die in the worst case. Urgent treatment by a physician is necessary to prevent kidney and liver failure. Those affected by amatoxin syndrome suffer from the usual symptoms of poisoning. Diarrhea and vomiting occur, and most patients also suffer from severe pain in the stomach and abdomen. Furthermore, sweating may also occur and those affected feel ill and fatigued. There are chills and palpitations. Furthermore, the patient may also lose consciousness. If the amatoxin syndrome occurs, the emergency doctor must usually be called directly. In this case, detoxification is performed in order to save the affected person. Likewise, the patient continues to be dependent on bed rest and recuperation, suffering from a greatly reduced ability to cope with stress. In most cases, the symptoms of this poisoning can be recovered relatively easily if treatment is initiated early. If the amatoxin syndrome has severely damaged the liver or kidneys, transplantation may also be necessary.
Prevention
Amatoxin syndrome can be prevented by avoiding ingestion of mushroom varieties that contain the toxin amanitin. Accordingly, inexperienced mushroom pickers should refrain from picking and eating unknown mushroom varieties altogether to rule out mushroom poisoning and, correspondingly, life-threatening amatoxin syndrome.
Follow-up
Special options for aftercare are not available to patients with amatoxin syndrome in most cases. The primary focus is to treat the disease to prevent a fatal outcome. In some cases, the patient’s life expectancy is also significantly reduced by the syndrome. In the case of amatoxin syndrome, the affected person is dependent on taking medication. These should be taken regularly, and attention should also be paid to their interaction with other medications. Early diagnosis and treatment of the syndrome have a very positive effect on the further course and can prevent complications or even a fatal course. However, the poisoning can damage the liver of the affected person to such an extent that a transplantation is necessary to keep the affected person alive. Therefore, if the first signs of amatoxin syndrome appear, an emergency physician must be called immediately or direct hospitalization must be sought. After successful treatment, the patient must rest and take care of his body. Physical exertion or sports activities should be avoided. Likewise, only light food should be taken at the beginning in order not to overload the digestion. With successful treatment of amatoxin syndrome, the patient’s life expectancy is usually not reduced.
What you can do yourself
Amatoxin syndrome is usually caused by eating the highly toxic tuberous-leaf mushroom. If poisoning is suspected, the stomach contents should be vomited first and an emergency physician should be consulted immediately. The emergency service must be informed about the circumstances of the poisoning, appearing symptoms and the constitution of the patient by means of the W-questions. In addition, if possible, a piece of the mushroom should be kept on hand to enable a rapid diagnosis. The affected person should be placed in the recovery position and warmed with blankets until the arrival of the rescue service. If necessary, resuscitative measures must be performed. In the meantime, sufferers can identify the syndrome by its clear symptoms. For example, severe colicky abdominal pain and other gastrointestinal symptoms indicate amatoxin syndrome. The most effective self-medication, in addition to alerting emergency services, is to vomit the consumed mushroom. Even if this is successful, the inevitable second phase of the disease (hepatorenal phase) must be cured in a hospital.After recovery, physical rest and bed rest are recommended. Depending on whether and which organs have been damaged by the amatoxin syndrome, further therapeutic measures must be worked out together with the responsible physician.
