Haff disease is an acute food poisoning associated with toxic products accumulating in the insides of fish. Pathognomonic symptoms of nosology are recurrent attacks of muscle pain and symptoms of kidney failure. Fever and signs of intoxication are moderately pronounced. Diagnosis is based on the detection of laboratory criteria for organ dysfunction, the results of a biological test. There is no etiotropic treatment, therapy includes pathogenetic, symptomatic methods; the main role is given to detoxification measures of the body.
ICD 10
T61 Toxic effect of toxic substances contained in eaten seafood
General information
Haff disease (paroxysmal-toxic alimentary myoglobinuria, Yuksovskaya or sartlanskaya disease) is a toxicosis of food origin. Occurs in predatory fish, carnivorous animals and birds, has been described in humans since 1924, was first identified among fishermen of the Frisches-Gaffa Bay.
Causes
The etiological agent, the toxin that causes Haff disease in humans, has not been precisely established. Ergot alkaloids of cane seeds, reeds, industrial pollution, river horsetail are called as the reason. The most likely theory is about the pathogenic effects of cyanobacteria. Their toxins accumulate inside the tissues and organs of fish living in reservoirs, provoking the appearance of symptoms of the disease, the death of some of the affected individuals. Infection of people occurs in an alimentary way.
The main risk factors are viral hepatitis C, diabetes mellitus, coronary heart disease, vitamin B deficiency, consumption of large amounts of fish, seasons with a significant rise in the water level in reservoirs that have replaced the low-water period, deterioration of the environmental parameters of the reservoir, especially the flowering of blue-green algae, sewage discharge. Accidental or deliberate consumption of fish fat, as well as their insides, is dangerous.
Pathogenesis
The pathogenesis of Haff disease has not been fully studied. It is believed that the toxin disrupts the electrolyte balance of skeletal muscle myofibrils, which causes rhabdomyolysis, lactic acid accumulates, and myoglobin is released. Muscle tissue protein passes through the renal tubules, causing blockage of the ascending knee of the Henle loop. Concentrating in the distal part of the nephron, metabolic products destroy the epithelium, provoking acute tubular necrosis.
In addition to the defeat of the urinary system, pathological processes occur in the spinal ganglia, lateral horns of the spinal cord, striated musculature. It is characterized by the presence of pathohistological changes in the central nervous system (more often edema of the brain and its membranes, multiple point hemorrhages and degenerative processes of the cytoplasm of nerve cells), the structure of parenchymal organs.
Symptoms of Haff disease
The incubation period takes several hours, sometimes days after consuming the infected product. Haff disease manifests acutely, during or 2-4 hours after physical exertion, alcoholism. The first symptoms are muscle pains throughout the body: in the lumbar region, limbs, chest. The intensity of painful sensations varies widely from slight discomfort to complete immobility.
The defeat of the intercostal muscles and the musculature of the shoulder girdle is dangerous by the appearance of symptoms of respiratory insufficiency (shortness of breath, cyanosis of the skin), which without timely treatment leads to death. Other manifestations of Haff disease include a decrease in the amount of urine, a change in its color from red-brick to black, the occurrence of fever no more than 38 ° C, diarrhea, nausea, vomiting. Often a person has a moderate increase in blood pressure.
The duration of the attack does not exceed 3-4 days. During the intercalary period, body temperature normalizes, muscle soreness gradually decreases, urine acquires a normal color, well-being improves. A similar new attack of Haff disease occurs with hypothermia, heavy physical labor, and alcohol intake. In severe cases, diuresis does not recover in a person between attacks.
Complications
The most common complications of Haff disease are acute renal failure, leading to uremic coma, cardiac arrhythmias and conduction disorders in the form of bradycardia, extrasystole, tachyarrhythmia. During the attack, acute cardiac and respiratory failure often occurs. The appearance of toxic hepatitis, symptoms of hypercoagulation, edema-swelling of the brain is possible; cases of acute gastric ulcer have been described.
When the respiratory muscles are affected, hypostatic, sometimes aspiration pneumonia may be detected. Treatment with excessive fluid intake with the development of oliguria often leads to pulmonary edema. A small proportion of patients develop nephrosclerosis, chronic renal failure, and renal hypertension. When the secondary flora is attached, chronic pyelonephritis is detected.
Diagnostics
Confirmation of the diagnosis is made by an infectious disease specialist. Usually, a neurologist’s consultation is required, other specialists are involved according to indications. It is important to carefully collect an epidemiological history with details about the place of residence, work activity, and the nature of nutrition. The basic clinical, laboratory and instrumental signs of Haff disease are:
- Physical data. An objective examination of a person reveals muscle hypertonus, sharp soreness and swelling of the muscles during palpation, often forced posture, sometimes shortness of breath. Hepatomegaly, enlargement of the boundaries of the heart, rhythm disturbances, and extrasystoles may be detected during auscultation. Symptoms of an acute abdomen are possible. An assessment of the color and amount of urine, daily diuresis is mandatory.
- Laboratory tests. Characteristic changes in the hemogram include leukocytosis, neutrophilosis, shift of the formula to the left, aneosinophilia, acceleration of ESR. The concentration of AST, ALT, potassium, creatinine, myoglobin, lactate dehydrogenase, creatine phosphokinase increases many times during biochemical screening. Pathology of the general analysis of urine – hematuria, proteinuria, leukocyturia, granular and hyaline cylinders.
- Identification of infectious agents. The detection of the toxin is possible with a biological test on mammals (mice and cats). It is recommended to conduct a bioassay to exclude tetanus, agglutination reaction or blood PCR if leptospirosis is suspected, in some cases – PCR examination of smears-prints of the cornea, saliva, lacrimal fluid, cerebrospinal fluid (diagnosis of rabies).
- Instrumental methods. Chest x-ray is performed, less often CT or MRI of the spine, abdominal cavity, retroperitoneal space. Ultrasound imaging of the kidneys and liver, performing electromyography is necessary. ECG reveals myocardial conduction disorders that are common in Haff disease.
Differential diagnosis is carried out with leptospirosis, important manifestations of which are jaundice, hemorrhagic symptoms, localization of pain in the calf muscles; rabies, if there is a history of traumatic contact with an animal, hydrophobia; hemorrhagic fever with renal syndrome, characterized by typical rashes, alternating periods of oliguria and polyuria, visual impairment.
The exclusion of tetanus is necessary for trauma, abortion and childbirth outside the walls of medical institutions, a condition with a trism, a sardonic smile, tetanus. With myositis, there are no urinary disorders, with osteochondrosis, there is a violation of sensitivity at the level of the lesion. Myocardial infarction occurs with the irradiation of pain, mainly localization behind the sternum. Urolithiasis is manifested by renal colic, periodic hematuria, the release of stones during urination.
Treatment of Haff disease
Patients with this pathology should be hospitalized. Bed rest is recommended throughout the disease, especially during an attack. It is important to avoid physical fatigue, hypothermia – a comfortable temperature and humidity are maintained in the ward, clothes should correspond to the season. Patients with damage to the muscles of the shoulder girdle, intercostal muscles are placed in intensive care wards.
A diet rich in vitamins of group B (especially B1), C, E is prescribed, while products containing potassium, as well as a high-protein diet for the period of oligoanuria are temporarily excluded. Spicy, fatty, pickled, smoked, salted, coffee, alcohol are prohibited. Fluid intake is controlled, the amount of water and rehydration solutions, depending on the severity of dysuria, is correlated with the filtration capacity of the kidneys.
Conservative therapy
Assistance should begin from the first hours of the disease in order to stop the absorption of the toxin from the gastrointestinal tract, minimizing the impact on the body. Standards for the treatment of Haff disease have not been developed. It is important to ensure detoxification measures, including methods of extracorporeal detoxification: plasmapheresis, hemosorption, hemodialysis, hemo- and ultrafiltration. Currently , nosology treatment is carried out by the following methods:
- Etiotropic therapy. Since the role of a specific toxin has not been proven, the antidote is unknown. Partial removal of the pathological agent from the body in the first hours of illness is possible by gastric lavage, setting up a siphon enema, taking oral enterosorbents (activated charcoal, smectite) and copious warm drinking.
- Pathogenetic treatment. Intravenous administration of solutions of sodium chloride, rheopolyglucine, succinate-containing, glucose is necessary for detoxification. The use of diuretics is justified. Correction of the acid-base balance is carried out with the help of sodium bicarbonate. To reduce coagulopathy, heparin, antiplatelet agents, pentoxifylline are recommended.
- Symptomatic treatment. The appointment of opium analgesics, antihistamines, anticonvulsants, microcirculation-improving drugs, vitamins B, C, E, as well as antibacterial drugs with proven addition of pyogenic bacteria is shown.
The severe course of Haff disease is accompanied by organ ischemia, venous stagnation, plasma loss, reaching up to a third of the volume of circulating blood in humans. To improve survival, it is recommended to treat with hyperbaric oxygenation sessions, transfusion of freshly frozen plasma, in case of an increase in anemia, coagulopathic symptoms – erythrocyte and platelet mass, respectively.
Surgical treatment
Massive rhabdomyolysis can lead to necrotic changes in skeletal muscle cells (often in the lower or upper extremities). Surgical treatment involves fasciotomy, which helps to eliminate pronounced tissue compression while maintaining muscle viability, but against the background of impaired local blood flow and intense subfascial edema. The operation includes revision and excision of necrotic muscle bundles.
In the absence of pus, the wound is sutured within 3-4 days after the swelling subsides and the patient’s well-being improves. For ischemia resistant to conservative decongestant treatment, the only way out is amputation of the limb; excision of dead tissue with reconstruction of viable muscles is performed less often. Evaluation of viability is possible only during surgery: the preserved muscle has a normal color, is able to bleed and contract.
Prognosis and prevention
With timely detection of symptoms of Haff disease in humans, adequate treatment, the prognosis is favorable. The mortality rate is no more than 5%. The vast majority of cases of the disease do not exceed 1-7 days in duration, with a severe course, recovery takes about 6-8 weeks. After clinical recovery, patients are registered with a nephrologist, neurologist, less often a gastroenterologist for six months.
Specific prevention (vaccine) has not been developed. Non–specific measures are the prohibition of catching, eating and feeding fish to animals for the entire period of flowering of reservoirs, if cases of pathology are detected on the territory. It is recommended to abandon all types of culinary preparation of such toxin reservoirs as pike perch, pike, perch, salmon, carp, burbot during this period; there is data on the safety of low-fat, as well as fish products stored for more than six months.