Gaffa disease is called alimentary-toxic paroxysmal myoglobinuria( ATPM): a severe disease characterized by the appearance in the urine of the pigment oxygen-binding protein myoglobin due to the pathological decomposition of muscle proteins.
This medical term was put into circulation during the outbreak in 1984 in the Novosibirsk region and killed six people. In total, 120 people were injured.
Pathologies are susceptible to humans, waterfowl and domestic( including ruminant) animals. The most difficult this disease cats suffer. Cases of defeat of foxes have been registered. The disease, occurring in severe form, leads to the irreversible destruction of skeletal muscles of virtually all vital organs.
The concept of
The first outbreak of the disease occurred in 1924: it affected fishermen and people living in the vicinity of Koenigsberg( in 1946 it was renamed Kaliningrad) on the shore of the Vistula( Frisches-Gaff) Gulf. The German word "Haff", meaning "bay", gave the name to this dangerous ailment.
Over the next fifteen years, numerous( no less than a thousand episodes) cases of human, cats and birds eating fish were recorded. All episodes were observed mainly in the summer and autumn months.
Over time, the incidence rate declined, and reports of single cases of Gaffian disease were reported mainly from Germany and the Soviet Union.
The Gaffa disease is often referred to as the Yuksovsky or Sartlan disease( in some sources - the Gaff-Yuksovsky) - in exact accordance with the names of the lakes in the vicinity of which outbreaks of pathology were noted:
- In 1934-36 she struck a population inhabiting the shores of Yuksovsky Lake located inLeningrad region. Of the four hundred people who fell ill then eight people died.
- The coast of Lake Sartlan, located in the Novosibirsk region, the disease was visited twice: in 1947-48 and in 1984.
In 2011, a real ecological disaster took place on the coast of Lake Kotokel, located in the Baikal region of Buryatia( just two kilometers from Baikal).
Poorly treated domestic waste water provoked mass death of waterfowl and fish. During the Kotokelian outburst of Gaffian disease, several dozen people were injured, one of whom died. The lake is stricken to this day.
Over a period of seven decades, over a dozen outbreaks of ATPM have been recorded on the globe. In 1997, six Americans suffered from the disease.
Researchers have been able to identify common features common to all outbreaks:
- The complete identity of the clinical manifestations of the disease has been established.
- All the diseased lived on the shore of large bodies of water and used to eat fish caught in them, at some point suddenly become toxic due to the accumulation in its tissues of toxic substances that penetrated the water from some plants and soil. It has been established that the toxicity of fish is temporary.
- Simultaneously with people, the cats that lived next to them were also amazed.
Toxic substances that enter the human body and animals together with the eaten fish lead to dystrophic changes:
- in the ganglionic nerve cells of the lateral horns of the spinal cord and the cerebral cortex;
- in epithelial tissues of convoluted renal tubules;
- in structures of striated muscle fibers.
Symptoms of Gaff disease
As medical statistics show, the disease affects mostly adults, since the content of myoglobin in the skeletal muscles of children is negligible.
Without any precursors, the disease develops a few hours or days after consuming poisoned fish. As a rule, this happens either at the time of intense physical activity( physical labor or a long walk), or two to four hours later.
The patient experiences sudden sharp pains in the chest area, in the lower back and in the muscles of the arms and legs, which can be strengthened even with insignificant movement( especially the muscle groups involved in recent physical exertion are especially painful).Within a short time, the pain syndrome encompasses all skeletal musculature.
Caught sudden pain, the victims very often fall in the same place and lose the ability to get up, because they have numb muscles. After being completely helpless, the patients are forced to lie motionless until they are found and taken to the nearest medical institution.
At the initial examination of the diseased the doctor notes:
- the difficulty of breathing due to the defeat of the respiratory muscles;
- tension and sharp soreness of all muscle groups and nerve trunks upon palpation;
- Conservation of consciousness and deep sensitivity;
- increased sweating;
- marked cyanosis of the extremities.
Body temperature in patients, as a rule, remains normal or keeps within subfebrile( up to 38 degrees) values.
Urinalysis indicates the presence of protein( erythrocytes), creatine, myoglobin, hyaline and granular cylinders( microscopic impression of the cavity of the renal tubules indicating insufficient filtration activity of the kidneys).
The arterial pressure of the affected during the first two days of the disease tends to moderate increase.
The occurrence of diffuse or focal lesions of the myocardium can be provoked by hyperkalemia( a condition characterized by a potassium concentration in the blood plasma exceeding 5 mmol / l).
Patients may develop tachyarrhythmia( a pathologically fast heart rhythm) or an extrasystole( a kind of arrhythmia accompanied by an extraordinary contraction of the heart muscle), and an electrocardiogram( ECG) will indicate the presence of signs of impaired conduction of the heart.
On the digestive function, the Gaffian disease does not have a significant effect. The combination of lesion of intercostal muscles and general hypothermia can trigger the onset of pneumonia.
Alimentary-toxic paroxysmal myoglobinuria can occur in three forms:
- Light, accompanied by lesion of exclusively skeletal muscles.
- of moderate severity, characterized by the attachment of lesions of the heart muscle.
- Heavy, adding to the above pathologies of kidney damage.
In the overwhelming majority of cases, the disease lasting from one to four( in rare cases up to seven) days, is characterized by a benign course. The recovery of a patient suffering from a severe form of pathology can take from 1.5 to 2 months.
The duration of attacks of acute muscle pain, completely immobilizing the patient, can range from three hours to three days. The occurrence of repeated painful attacks( they can be from five to sixteen) is often provoked by either physical exertion or the use of fish dishes.
In the most severe cases for three days, the victims have a typical clinical picture of food poisoning, followed by the appearance of seizures and failure of the liver and kidneys.
Reasons for the emergence of
For half a century, physicians, hydrochemists, bacteriologists and ichthyologists, who confirmed the connection of intoxication with eating fish( mostly predatory species represented by pike perch, burbot, pike, perch, etc.) were engaged in the study of the Gaffian disease, although cases of illness,provoked by the use of salmon and carp.
Nowadays it is finally established that the Gaffian-Yuksovsky disease belongs to the number of rare specific diseases affecting both humans and animals systematically feeding on poisonous fish( in a rather large amount).
No less certain is the fact that fish meat is less toxic than their fat and entrails.
Studies have shown that:
- The toxin has an extremely high heat resistance, allowing it to withstand autoclaving at a temperature of 120 to 150 degrees for an entire hour. That is why no type of culinary processing of fish: roasting, cooking, smoking and drying can not make the product completely safe, however, when the fish is degreased and with its long( more than half a year) storage, the toxicity of the poison is somewhat reduced.
- Fish of the same species can be completely harmless, and toxic( depending on the year and a certain section of the reservoir).
- It is noticed that on all without exception reservoirs all known outbreaks of pathology occurred against the background of a significant rise in the water level, replacing the periods of low water availability.
- The appearance of the Gaffian disease, as a rule, is preceded by a significant deterioration of the hydrobiological, hydrological and hydrochemical regimes in the reservoir.
German researchers, initially engaged in the study of the Gaffian disease, put forward several hypotheses at once, designed to explain the reasons for its occurrence. In very short time, assumptions about the parasitic, infectious-bacterial and viral nature of this pathology, without confirmation, were swept aside as insolvent.
Currently, the ailment is classified as a toxicosis that has an alimentary etiology and appears due to the consumption of fish, which at some point has become toxic.
According to the researchers, this happens for several reasons:
- Fish can acquire toxicity after eating plankton poisoned with extremely toxic substances that are washed out of the ground and enter the body of water with sewage.
- Some toxic components of ergot, swallowed by fish, along with high-standing waters that have flooded the coast, can become the culprits of fish poisoning.
- Inflorescences and seeds of some poisonous plants, in the first place - the throat( a frankincense pikulnik belonging to the family of labial flowers), possess a complex of toxins( for example, omega-6-unsaturated arachidonic acid), similar to the poisonous substance isolated from the organism affected by the disease of the fish,.Growing on the shores of lakes, they are in large quantities falling into the water and can cause a massive poisoning of the fish( pike, carass, peled, carp, roach, perch) inhabiting them.
- One of the most popular versions to this day remains the hypothesis put forward by the domestic researcher Leshchenko. He suggested that the appearance of a dangerous toxin may be due to the flowering of blue-green algae( cyanobacteria).It has long been noted that their active development coincides with the mass death of the lake fish. Penetrating into the body and making fish dangerous for animals and humans, toxins of cyanobacteria are used to accumulate( accumulate) in its tissues and organs, provoking the appearance of the Gaffian disease and often leading to death. In the pathoanatomical autopsy of the affected individuals, the researchers showed pronounced visual changes, represented by edema of the brain, a change in the color of the membranes and the injection( redness) of the vessels of the sclera, the presence of hemorrhages and the accumulation of exudate. In all cases, morphological changes were found in the tissues of the liver, kidneys and intestines. The results of histological studies showed the presence of pathological changes in the central nervous system( various types of edema of the brain and its membranes, the presence of multiple point hemorrhages and degenerative processes in the cytoplasm of nerve cells) and in the structures of all parenchymatous organs. Irreversible changes also affected the gill tissue and heart muscle. With a significant deterioration in the hydrochemical conditions in the reservoir, the main culprit in the loss of fish is vitamin B1 vitamin deficiency resulting from its decomposition by thiaminase, an enzyme contained in the Pseudomonas aeruginosa, which is a type of freshwater blue-green algae. Some researchers suggest that it is thiaminase and is the main culprit of the Gaffian disease, which is a pronounced avitaminosis B1.Its extreme stage can lead to a fatal outcome, provoked by a paralysis of the respiratory center. With short-term and mildly expressed B1-avitaminosis, there is a disruption of the digestive tract and, sometimes, the development of an allergic reaction.
Thus, in views on the etiology of the origin of alimentary-toxic paroxysmal myoglobinuria, there is still no unity. Biological experiments that can uncover the riddle of the origin of this disease continue to be actively pursued to this day.
Video shows the flowering of toxicogenic cyanobacteria in Lake Kotokel during the outbreak of the Gaffian disease:
Diagnosis
Diagnosis of the Gaffe-Yux disease in the first hours of the pathology development is usually difficult.
The basis for the accurate diagnosis is the data of laboratory studies, epidemiological analysis and a complex of clinical manifestations of pathology.
- The most reliable information is given by the results of a biochemical blood test, by which the doctor learns about the level of myoglobin and specific enzymes in blood serum, presented by alanine( ALT) and asparagine aminotransferase( ACT), creatine phosphokinase( CK) and lactate dehydrogenase( LDH).
Since the first hours of the appearance of pathology in the peripheral blood of the overwhelming( more than 85%) number of cases, neutrophilic leukocytosis has been observed: a condition characterized by an increased content of young stab neutrophil cells and normalizing to the end of the second to third day.
The rate of erythrocyte sedimentation( ESR) in the blood of patients slows down to one to four mm / h. The content of myoglobin in the blood serum increases from 200 to 800 ng / ml, and the level of the above plasma enzymes( ALT, CK, AST, LLDG) can increase by a factor of 100( and even more).
- No less valuable are the information obtained by performing an electrocardiogram that tracks the work of the cardiac muscle in dynamics.
- If suspected of alimentary-toxic paroxysmal myoglobinuria, it is also necessary to conduct electromyography - a technique that examines the bioelectric potentials that arise in the skeletal muscles of animals and humans in response to the stimulation of muscle fibers with the mandatory registration of electrical muscle activity.
Treatment of
In the first hours of illness, the injured person needs emergency medical care consisting in the introduction into his body:
- Analgesics - medications designed to relieve severe pain.
- of Vitamins belonging to group B and E( tocopherol acetate).
- Antihistamines , helping to cope with the manifestations of allergic reactions.
- Medicaments that are capable of rapid and effective sorption purification of the patient's body from toxic substances that have entered it. This task is performed by oral intake of activated carbon and preparations "Enterodesis" and "Enterosorb".
After this, the patient is warmed, laid in a warm bed and provided with an abundant drink. All these actions can be performed at home, even before sending the patient to a hospital in a medical institution.
In hospital conditions, the patient undergoes intensive filtration and detoxification therapy, which provides:
- Intravenous management of 400 ml of plasma-substituting preparation "Polyglucin"( depending on the patient's condition, it is injected or drip-wise).
- Bleaching of the blood plasma by introducing a 4% solution of sodium hydrogen carbonate( from 500 to 1500 ml).This procedure is carried out to eliminate acidosis - a condition characterized by a violation of the acid-base balance and leading to an excessive content of acids in the human body.
- Introduction of drugs that help improve microcirculation and normalization of coagulopathy - a pathological condition caused by all kinds of blood clotting disorders. To this end, drugs are injected into the patient's body: "Heparin"( from 20,000 to 40,000 units per day), "Reopoliglyukin"( from 400 to 800 ml), and antiaggregants - drugs that can reduce thrombogenesis, slowing down the adhesion( aggregation) of platelets. With this task can cope with 2% solution of the drug "Trental"( 5 ml) or 0.5% solution of the drug "Curantil"( 1-2 ml).
- For the implementation of forced diuresis - a detoxification procedure consisting of artificial stimulation of urine formation by simultaneous injection into the human body of diuretics and fluids. With the help of forced diuresis, they achieve accelerated elimination of toxins from the patient's body along with urine. As a drug that accelerates the process of urination, use the drug "Lasix"( it is administered by 40 mg at least four times a day, if necessary, the frequency of reception can be increased).In this case, the doctor should strictly monitor the state of hydration of the patient's body.
Patients suffering from a disease that occurs in a moderate or severe form need to be carried out:
- Hemosorbtion - a procedure for the adrenal cleansing of blood by contacting it with a sorbent outside the patient's body.
- Hemodialysis, carried out with the help of a device called an "artificial kidney."
- Hemodiafiltration - procedures for extracorporeal blood purification performed in relation to patients in critical condition.
- Hemofiltration is a technique for purifying blood by filtering it through a system of highly permeable membranes, which involves the simultaneous replacement of the removed filtrate with a special solution.
- Ultrafiltration - a technique designed to correct water homeostasis( carried out with excess fluid in the body) by removing from the blood protein-free liquid through a series of natural or artificial membranes designed to fulfill the role of an ultrafilter.
- Plasmapheresis is a procedure consisting in taking blood, cleaning it and returning it to the bloodstream( sometimes only a part of the blood taken is returned).
The above therapeutic manipulations are carried out until the complete disappearance of clinical manifestations of intoxication and failure of kidney function.
For immediate and qualified treatment, the victims should be hospitalized:
- in centers that specialize in poisoning therapy;
- in intensive care units;
- in ambulance hospitals, since they are all equipped with specialized equipment designed for plasmapheresis and hemodialysis.