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Introduction. Familial Mediterranean fever (FMF) is a non-infectious genetic disease caused by a mutation in the MEFV gene. This disease is more common in people of Mediterranean or Middle Eastern descent, but can occur in any ethnic group, including those living European countries. FMF has typical clinical symptoms. Its long term without appropriate treatment the disease can lead to a violation of the child's physical development and result in the development of amyloidosis. The aim is to focus the attention of the medical community of Ukraine and inform European medical specialists on the FMF cases due to migration changes in Ukraine. This article presents a clinical case of FMF in a 4-year-old child who was admitted to our hospital with complaints of fever, abdominal pain, vomiting, and stool retention. Additional examination included: laboratory tests (complete blood count, urinalysis, biochemical blood test with determination of liver and kidney tests); instrumental methods: ultrasound examination of the abdominal organs and urinary system, X-ray examination of the abdominal organs with contrast. The results of additional tests revealed the signs of inflammatory process and impaired kidney function. When clarifying the family medical history, it was revealed that the older brother suffers from FMF (a mutation of the MEFV genotype was determined) and takes specific therapy (colchicine). The final diagnosis was determined: Acute strangulated intestinal obstruction. Familial Mediterranean Fever (clinically). Peritonitis. Operative laparoscopic and conservative treatment was performed. The patient was discharged in a satisfactory condition with appropriate recommendations. Conclusions. The number of FMF cases in children have increased due to changes in the migration process in Ukraine. The relevance of this disease has increased. Taking into consideration a significant migration of population, the likelihood of an increase in the number of cases of FMF in European countries is increasing, which requires awareness among a wide range of doctors.In this report we have described a pediatric FMF case which was represented by recurrent episodes of fever, abdominal pain, impaired kidney function test results, and inflammatory markers. A determined mutation of the MEFV genotype in the brother of our patient makes the diagnosis of FMF unquestionable. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

Along with familial Mediterranean fever (FMF), it is now considered an autoinflammatory disease and gout. The commonality of the basic mechanisms of inflammation underlying the pathogenesis of FMF and gout predetermines the possibility of using similar therapies aimed at stopping and preventing seizures (colchicine and IL-1 inhibitors). A clinical case is presented describing the presence of a combination of FMF and gout in a patient. The patient was prescribed anakinra, which proved to be effective both as a treatment for FMF and gout. The appointment of an IL-1 inhibitor fully justified expectations: already after the first injection of anakinra, the intensity of swelling and pain in the joints decreased in the patient.

The aim of the review was to characterize the epidemiological situation on rickettsiosis of the tick-borne spotted fever (TSF) group in the constituent entities of the Russian Federation in 2012–2021 and provide a long-term forecast for 2022–2026. In 2020–2021, indicators of registered incidence of tick-borne rickettsiosis in Russia on the whole decreased relative to the average long-term pre-pandemic level in 2012–2019 by 2 fold for all forms of the TSF group on average (Siberian tick-borne typhus – STT, Astrakhan spotted fever and Mediterranean fever). A comparative analysis of the territories revealed a correlation between the incidence of STT and the number of medical facility visits due to “tick bites” (r=0.67, p=0.015), which, against the background of an increase in the frequency of contacts of the population with vectors in 2020–2021 in most regions and a decrease in the incidence in the same regions indicates the credible nature of the changes. The ranking of territories according to average long-term incidence rates made it possible to classify the Republic of Altai and the Altai Territory as regions of extremely high epidemic hazard as regards STT; high hazard – the Republic of Tuva; above average – the Republic of Khakassia, Khabarovsk Territory and the Jewish Autonomous Region. The established downward trend in the incidence of tick-borne rickettsiosis among the population will be sustained in most entities of the Russian Federation within the next 5 years, excluding the Republic of Altai, the Altai Territory, the Republic of Crimea and the city of Sevastopol, where current incidence or an increase should be expected. The results of the analysis indicate the need to put more emphasis on natural-focal infections at the time of COVID-19 pandemic.

Aim. To investigate an influence of the currently changed etiologic structure of AA-amyloidosis on the diagnosis and treatment tactics. Materials and methods. In 110 patients with АА-amyloidosis followed during full disease duration (1 month 29 years) etiology, clinical manyfestations and approaches to diagnose and treatment of AA-amyloidosis were evaluated. With ELISA levels of amyloid precursor acute phase inflammation reactant SAA and neutrophil activity marker S100A12 were measured. Results. Among the most common causes of AA-amyloidosis at the present stage, in addition to RA (40.3%), a significant place is occupied by a group of diseases with a predominantly autoinflammatory mechanism (53.73%). To confirm the autoinflammatory mechanism of the predisposing disease it is recommended to study a highly sensitive parameter serum protein S100A12. An effective marker of the risk of AA-amyloidosis progression, especially in patients with subclinical activity of inflammatory disease, is a high level of production of amyloidogenic protein-a precursor of SAA.

Objective is to analyze the incidence of Siberian tick typhus (STT) in the Russian Federation over the period of 2010–2020, Astrakhan spotted fever (ASF) and Mediterranean fever (MF) since the official registration, and to forecast the development of the epidemic process for endemic rickettsioses for 2021. The analysis of the incidence of STT, ASF and MF in Russia for the period of 2010–2020, 2013–2020 and 2014–2020, respectively, has been carried out. The forecast of endemic rickettsioses morbidity in the European and Asian parts of Russia for 2021 has been made. The average long-term incidence of STT for 2010–2020 in the Russian Federation as a whole was 1.04 (CI95 1.02÷1.05) 0/0000, with no tendency to change. The maximum relative incidence of STT is typical for the Siberian Federal District (SFD), where the average long-term incidence rate for 2010–2020 was 6.20 (CI95 6.08÷6.31) per 100 thousand of the population. The Far Eastern Federal District (FEFD) – 4.70 (CI95 4.53÷4.87) 0/0000 came in second place, the third place was taken by the Ural Federal District (UFD) – 0.08 (CI95 0.07÷0.10) 0/0000. When assessing the 11-year dynamics of the relative incidence of STT by the Federal Districts, we have detected a tendency to its stabilization in the SFD and the FEFD. In the UFD, a significant downward trend was revealed. The upward trend in the incidence of STT remained in the Altai Republic. Major decline in STT incidence was observed in the Kurgan Region, Trans-Baikal Territory, Krasnoyarsk Territory, and the Republic of Khakassia. There was a declining trend in the incidence of Astrakhan spotted fever in the Astrakhan Region and the Republic of Kalmykia. In the Republic of Crimea and the city of Sevastopol there is no trend to increase the incidence of Mediterranean fever.

Objective of the study was to analyze the incidence of Siberian tick-borne typhus (STBT) in the Russian Federation between 2010 and 2019, Astrakhan spotted fever (ASF) and Mediterranean fever (MF) since official registration, and forecast the development of the epidemic process for endemic rickettsioses for 2020. Materials and methods. The analysis of the incidence of STBT, ASF and MF in the Russian Federation over the period of 2010–2019, 2013–2019 and 2014–2019, respectively, in relation to the results of zoological-entomological monitoring. Results and discussion. The average long-term incidence rate of STBT for 2010–2019 in the Russian Federation on the whole was 1.1 0 /0000 (DI95 1.05÷1.08) without trends towards change. The maximum relative incidence of STBT is typical for the Siberian Federal District (SFD) where the average long-term incidence rate for 2010–2019 amounted to 6.28 per 100 thousand of the population. In the second place is the Far Eastern Federal District (FEFD) – 5.17 0 /0000, in third – the Ural Federal District (UFD) – 0.1 0 /0000. When assessing the 10‑year dynamics of the relative incidence of STBT, we have revealed a significant tendency to increase in the FEFD, a tendency to stabilize in the SFD and a significant downward trend – in the UFD. A reliable increasing trend in STBT incidence was detected in the Altai Republic and in the Khabarovsk Territory. Major decline in STBT incidence was observed in the Trans-Baikal territory, Krasnoyarsk territory, Kurgan Region and the Republic of Khakassia. There is a marked tendency to increase in the incidence of MF in the Republic of Crimea. In the Astrakhan Region and the Republic of Kalmykia, there is an expressed tendency towards the reduction in the ASF morbidity rates.

The paper demonstrates current opportunities of effective management of periodic disease as an autoinflammatory disorder based on a clinical case of colchicine-resistant Mediterranean fever. We present modern approaches to the autoinflammation hypothesis and its molecular mechanisms in periodic disease. Based on literature and our own research results, we consider approaches to evaluate autoinflammation activity and to monitor treatment efficacy. Clinical value of S100A12, a novel marker of the activity of neutrophilic autoinflammation, is demonstrated.

The Crimean Peninsula is located in the Northern part of the Black sea, from the East it is washed by the Sea of Azov, to the South and West by the Black Sea. The unique geographical and climatic conditions facilitate leptospirosis, tularemia, tick-borne encephalitis, Lyme disease, intestinal yersiniosis, pseudotuberculosis, hemorrhagic fever with renal syndrome, Crimean-Congo hemorrhagic fever, Mediterranean fever, Q-fever and other infectious diseases natural foci formation on the territory of Crimea Republic. Tick-borne natural focal infections have the most significance due to favorable epidemiologic conditions especially on the background of high raid ticks attacks on people. A leading role in the epizootology and epidemiology of tick-borne natural-focal infections of the Crimea are playing Ixodidae that occur in different landscape-climatic zones, with the greatest their species diversity is observed in mountain-foothill, forest and forest-steppe regions. There are about 30 species in Ixodidae fauna of the Crimean Peninsula. Ticks species composition identification shows that over 50% of people attacks episodes in the Crimea on recent years is caused by Ixodes ricinus ticks species, the remaining are associated with Haemophisalis punctata, Rhipicephalus sanguineus, Hyalomma marginatum, Dermacentor marginatus and other. Refusal of treatment in medical institutions of the people affected by tick bites, and the possibility of an attack on people subtle phases of mites are lubricates the real picture of the frequency of contacts of the population with ticks and complicates the forecasting of the epidemiological situation. This review summarizes the available information about spreading of tick-borne encephalitis, Lyme disease, Mediterranean and Crimean-Congo haemorrhagic fevers on the territory of Crimea Republic and demonstrates the modern trends and manifestations of epidemic process of these nosological forms. The results obtained in the analysis of our investigations and literature data, showed that the conditions of globalization lead to an increase in the frequency of contacts of the population with natural foci, and one of the most common tick-borne natural focal infections on the territory of the Peninsula are Lyme disease and the Mediterranean fever. Epidemiological analysis of the prevalence of tick-borne encephalitis and Crimean-Congo haemorrhagic fever in Crimea revealed a decrease in the activity of natural foci of these infections at the present stage. The problem of tick-borne natural focal infections in Crimea Republic requires further careful study.