Features of the course and outcomes of Haff disease in the territory of the Tyumen region

Purpose is to identify the features of the clinical picture, course and outcomes of Haff disease in the Tyumen region. Materials and methods. a retrospective study was conducted. There were included nine patients (7 men and 2 women) with a diagnosis of “Alimentary-toxic paroxysmal myoglobinuria (Haff disease) (T62.8)”. Were collected original demographics (age, gender), symptoms, time to onset of initial symptoms, time to hospitalization, and laboratory tests (general blood analysis, biochemical analysis of blood, analysis of blood coagulation functions and of arterial blood gases). Results. 4 (44.4%) patients had stage 3 severity, in 3 (33.3%) — stage 2 severity and in 2 (22.3%) — stage 1 severity of alimentary-toxic paroxysmal myoglobinuria. The main complaints presented by patients at the moment admissions were pain in the leg muscles (n = 6; 66.7%) and lower back (n = 6; 66.7%), dark urine (n = 5; 55.5%) and weakness (n = 4; 44.4%). Myoglobin, ALT, AST, LDH, CPK and CPK-MB were increased in all patients, the average level of these indicators was 4691.4 ± 2831.5 ng/ml, 1572.0 ± 547.5 U/L, 547.3 ± 158.2 U/L, 1555.7 ± 420.6 U/L, 33662.4 ± 15195.7 U/L and 1848.8 ± 692.6 U/L, respectively. The main complications developed in patients with stage 3 severity include acute renal and hepatic insufficiency and cardiomyopathy. Milder degrees of the disease (1–2 stages) were characterized by such complications as hepatitis and myocarditis. Also 1 death was observed, the analysis of this clinical case is analyzed in detail. Limitations. The main limitation of the study is the small group of patients due to the rare occurrence of Huff disease. In addition, there are no data on the amount of eaten fish, while there is evidence of a direct relationship between the severity of the disease and the amount of eaten food. The results are obtained in this region, and may differ from the results of other territories. Conclusion. with the development of myalgia or weakness of unknown etiology, it is necessary to ask patients if they have eaten fish in the previous 24 hours.