Семейная средиземноморская лихорадка (периодическая болезнь): современный взгляд на проблему

The paper deals with the most common classical autoinflammatory disease familial Mediterra-nean fever (FMF)/periodic disease. This is a monogenic hereditary disease caused by mutations with an autosomal recessive pattern of inheritance. The most common types of mutations are given. Hyperactivation of innate (antigen-specific) immunity is a basic pathogenic mechanism of the disease and IL-1ß is a leading mediator. FMF prominently occurs in certain ethnic groups (Sephardic Jews, Armenians, Turks, and Arabs). In spite of the fact that there may be multiple organ failure, 12-72-hour febrile fever episodes accompanied by the symptoms of peritonitis and/or pleuropericarditis. AA amyloidosis is the most serious complication of FMF. Colchicine therapy is a basic treatment for preventing this complication. In case of colchicine inef-fi-cacy/intolerance, other agents, including genetically engineered biological drugs (IL-1ß inhibitors, etc.), may be used.Статья посвящена наиболее частому «классическому» аутовоспалительному заболеванию — семейной средиземноморской лихорадке/периодической болезни (Familial Mediterranean Fever — FMF). Это наследственное моногенное заболевание, обусловленное мутациями гена MEFVс аутосомно-рецессивным типом наследования. Приведены наиболее часто встречающиеся типы мутаций. Основным патогенетическим механизмом заболевания является гиперактивация естественного (антиген-неспецифического) иммунитета, а ведущим медиатором — ИЛ1ß. Заболевание встречается преимущественно в определенных этнических группах (евреи-сефарды, армяне, турки, арабы). Несмотря на возможность полиорганного поражения, типичным для FMF являются эпизоды фебрильной лихорадки длительностью 12—72 ч, сопровождающиеся симптомами перитонита и/или плевроперикардита. Самое серьезное осложнение FMF — развитие АА-амилоидоза. Основным методом лечения, предотвращающим это осложнение, является применение колхицина. В случае неэффективности/непереносимости колхицина, возможно применение других лекарственных средств, в том числе генно-инженерных биологических препаратов (ингибиторы ИЛ1ß и др.)

Hydromagnetic Waves in the Magnetosphere and the Ionosphere

The book deals with Ultra-Low-Frequency (ULF)-electromagnetic waves observed on Earth and in Space. These are so-called geomagnetic variations or pulsations. Alfvén's discovery related to the influence of the strong magnetic field on the conducting fluids (magnetohydrodynamics) led to development of the concept that the ULF-waves are magnetospheric magnetohydrodynamic (MHD)-waves. MHD-waves at their propagation gather information about the magnetosphere, ionosphere, and the ground. There are two applied aspects based on using the ULF electromagnetic oscillations. The first one is the ground-based diagnostics of the magnetosphere. This is an attempt to monitor in the real time the magnetosphere size, distance to the last closed field-lines, distribution of the cold plasma, etc. The second one is the deep electromagnetic sounding of the Earth. The basis for these studies is the capability of any electromagnetic wave to penetrate a conductor to a finite depth. The ULF-waves can reach the depth of a few hundred kilometers. Thus, geophysicists now have a unique tool that can be applied to study the solid Earth as well as its gas-plasma shells - the ionosphere and magnetosphere. This book integrates topics pertaining to all scales of the MHD-waves, emphasizing the linkages between the ULF-waves below the ionosphere on the ground and magnetospheric MHD-waves. It will be most helpful to graduate and post-graduate students, familiar with advanced calculus, who study the science of MHD-waves in the magnetosphere and ionosphere

Increasing the Interval of Canakinumab Administration Effectively Supports the Remission of Schnitzler’s Syndrome

Schnitzler’s syndrome (SchS) is a rare, disabling, autoinflammatory disorder characterized by recurrent urticarial rash and monoclonal IgM gammopathy. Interleukin-1 beta (IL-1β) plays an important role in the pathophysiology of SchS. Only anecdotal reports demonstrate the efficiency and safety of human monoclonal anti-human IL-1β antibody (canakinumab) use in SchS therapy. However, there are no generally accepted recommendations concerning the scheme (or frequency) of canakinumab use for this disease. Here, we report the effective long-term treatment of SchS in a 44-year-old male with a standard canakinumab dose (150 mg) but with an increased 4-month injection interval

Familial Mediterranean fever (a periodic disease): The present-day view of the problem

The paper deals with the most common classical autoinflammatory disease familial Mediterra-nean fever (FMF)/periodic disease. This is a monogenic hereditary disease caused by mutations with an autosomal recessive pattern of inheritance. The most common types of mutations are given. Hyperactivation of innate (antigen-specific) immunity is a basic pathogenic mechanism of the disease and IL-1ß is a leading mediator. FMF prominently occurs in certain ethnic groups (Sephardic Jews, Armenians, Turks, and Arabs). In spite of the fact that there may be multiple organ failure, 12-72-hour febrile fever episodes accompanied by the symptoms of peritonitis and/or pleuropericarditis. AA amyloidosis is the most serious complication of FMF. Colchicine therapy is a basic treatment for preventing this complication. In case of colchicine inef-fi-cacy/intolerance, other agents, including genetically engineered biological drugs (IL-1ß inhibitors, etc.), may be used

The formation of the cutting tool microgeometry by pulsed laser ablation

Laser ablation is considered as an alternative to other methods that allow precise processing of various tool materials and work with both the base material and thin films of wear-resistant coatings. This article presents an investigation of the possibility of setting the microgeometry of the surface of cutting tool made of high-speed steel M2 and hard alloy WCCo3 using ablation with nanosecond infrared marking Nd:YVO4 laser. Dependencies of the width and depth of the resulting tracks on the specified laser power are obtained. It was revealed that their depth depends on a set of several specified factors: power, scanning speed, pulse frequency rate, and a number of passes. The modes with a radiation power of up to 70% are of interest for the treatment of the tool surface using a laser because they have a more predictable profile and more efficient energy distribution. The most uniform and smooth surface were obtained after laser treatment with the following parameters: power 60%, frequency 10 kHz, scanning speed 200 mm/s irrespective of the material being processed. The greater flexibility of the coating material for laser processing was observed. The depth of tracks on the coated (TiAl)N samples is larger than on the uncoated samples. The possibility to obtain a chamfer with the specified width and angle parameters by laser processing based on the obtained dependencies is demonstrated

The first data on the concentration and emission of methane and carbon dioxide in the underground sources of the Peshernaya beam of Lake Baskunchak

For the first time, full-scale measurements of methane and carbon dioxide emissions by the chamber method were carried out in summer for groundwater sources discharged in the valley of the creek of the Peshernaya beam, located in the north-west of Lake Baskunchak. The values of pH, temperature, concentration of basic ions and methane were determined in the water of the sources. A detailed description of the identified groundwater sources was carried out, their primary morphometric and morphological characteristics were measured, as well as the flow rate. It has been established that more than ten ascending karst groundwater sources are discharged in the Peshernaya gully, five of which were active during the research period and were mainly confined to the left branch of the gully. The active sources of the left side of the beam are characterized by a small flow rate and are sodium chloride brines with a mineralization of 93.9–107.1 g/l. The concentration of methane in the water of active sources varied between 26.3–38.4 µl/l and was on average more than 2 times higher than in the waters of the stream fed by them and an inactive source. The specific flow of methane from the water surface of active sources varied in the range of 0.6–1.5 mg/(m2·hour), which is 2 orders of magnitude lower than the specific flow of CO2, which is 46.4–106.1 mg/(m2·hour)

Aptamer-conjugated superparamagnetic ferroarabinogalactan nanoparticles for targeted magnetodynamic therapy of cancer

Nanotechnologies involving physical methods of tumor destruction using functional oligonucleotides are promising for targeted cancer therapy. Our study presents magnetodynamic therapy for selective elimination of tumor cells in vivo using DNA aptamer-functionalized magnetic nanoparticles exposed to a low frequency alternating magnetic field. We developed an enhanced targeting approach of cancer cells with aptamers and arabinogalactan. Aptamers to fibronectin (AS-14) and heat shock cognate 71 kDa protein (AS-42) facilitated the delivery of the nanoparticles to Ehrlich carcinoma cells, and arabinogalactan (AG) promoted internalization through asialoglycoprotein receptors. Specific delivery of the aptamer-modified FeAG nanoparticles to the tumor site was confirmed by magnetic resonance imaging (MRI). After the following treatment with a low frequency alternating magnetic field, AS-FeAG caused cancer cell death in vitro and tumor reduction in vivo. Histological analyses showed mechanical disruption of tumor tissues, total necrosis, cell lysis, and disruption of the extracellular matrix. The enhanced targeted magnetic theranostics with the aptamer conjugated superparamagnetic ferroarabinogalactans opens up a new venue for making biocompatible contrasting agents for MRI imaging and performing non-invasive anti-cancer therapies with a deep penetrated magnetic field