Potential involvement of immune system in pathophysiology of congenital glaucoma, cataract, and retinopathy of prematurity (ROP) in infants has been suggested. However, its understanding still remains limited. T regulatory cells (CD4+CD25highFoxP3+CD127low), with their role in autoimmunity, are considered pivotal in this respect, although there is a scarcity of publications in this context. This study aims to address this gap. Our purpose was to compare the levels of blood cells with (CD4+CD25highFoxP3+CD127low) phenotype in infants with ROP, congenital glaucoma and cataract, and healthy full-term infants. This retrospective case-control study included 131 infants (262 eyes). Inclusion criteria were as follows: age under 12 months and a confirmed diagnosis of congenital cataract (20 eyes), congenital glaucoma (21 eyes), and ROP (158 eyes). The control group consisted of 27 full-term infants (54 eyes) with normal eye exam. Primary outcomes included study of Treg cells (CD4+CD25highFoxP3+CD127low) levels in all groups. Secondary outcomes involved the correlation between the CD4+CD25highFoxP3+CD127low subpopulation and weight at birth. According to our previous studies and when comparing the results in the present study of children under 1 year of age, we found significant differences in the number of T regulatory cells (CD4+CD25highFoxP3+CD127low) between premature and full-term children, as well as in the patient groups with cataract and glaucoma by their weight category. The authors were able to detect differences between stages of retinopathy of prematurity by the number of T regulatory cells (CD4+CD25highFoxP3+CD127low), distinguishing posterior aggressive retinopathy of prematurity as a special form of this pathology. The reduced levels of CD4+CD25highFoxP3+CD127low cells in infants with ROP type 1 suggests autoimmune reactions in its pathophysiology. The remarkable difference in (CD4+CD25highFoxP3+CD127low) Treg cells in patients with congenital glaucoma and cataract may indicate immune-mediated mechanisms in their development. The clinical significance of the revealed correlation between the level of studied T cells and birth weight in infants with cataract and glaucoma is not clear and requires further investigation with regard to the disease severity. The index can be used as a potential prognostic marker of the disease course and visual outcomes. Collectively, this study provided valuable insights on the potential targets for novel treatment strategies in infants with ROP, glaucoma, and cataracts.

Развитие инфекционных осложнений у больных с воспалительными заболеваниями определяется тем, насколько эффективную защитную реакцию нейтрофилы больного будут развивать при контакте с патогеном. Противоинфекционная устойчивость организма во многом определяется готовностью нейтрофилов формировать нейтрофильные сети, способные эффективно захватывать патогены. Цель исследования. Определение параметров нейтрофильных экстраклеточных ловушек (НЭЛ) у больных с воспалительными заболеваниями брюшной полости. Методика. Обследованы 28 прооперированных больных с различными формами абдоминального воспаления и 6 терапевтических больных с язвенным колитом, 6 пациентов обследованы при поступлении. Нейтрофилы выделяли, используя градиентное центрифугирование. Для подсчета НЭЛ использовали флюоресцентную микроскопию с красителем SYBR Green (Evrogen; Россия), специфично взаимодействующего с двухцепочечной ДНК. Функциональную активность НЭЛ определяли в тесте с захватом Klebsiella pneumoniae (ATCC 700603). Результаты. При исследовании больных в послеоперационном периоде обнаружили, что при остром аппендиците/абсцессе, остром холецистите и остром панкреатите/панкреонекрозе обнаруживаются близкие количества НЭЛ – 12,63±2,13%, 15,66±2,81% и 12,64±2,73%. В этих же группах больных регистрируются достоверные различия в размерах НЭЛ, которые достигают 46,82±6,70 мкм, 73,62±16,29 мкм и 96,84±27,17 мкм, соответственно. Увеличение размеров НЭЛ в этих группах больных объясняется присутствием, помимо сетевидных структур, дополнительно еще и нитевидных, волокнистых и вуалеобразных нейтрофильных внеклеточных структур. Группа больных с неспецифическим язвенным колитом, получающих консервативное лечение (группа сравнения), демонстрирует тенденцию к уменьшению размеров НЭЛ – до величины 31,96±14,75 мкм. Исследование функциональной активности НЭЛ показывает, что её снижение предшествует развитию септического состояния (на примере выявленного клинического случая). Заключение. Все, без исключения, исследованные нозологические формы воспаления в брюшной полости сопровождаются формированием НЭЛ в виде нейтрофильных сетей. Помимо нейтрофильных сетей воспаление индуцирует у части больных формирование НЭЛ в виде одиночных нитей, волокон и вуалей. Количественные параметры НЭЛ коррелируют с размерами и распространением очага воспаления на соседние анатомические области. На примере описанного клинического случая обнаружено резкое ослабление функциональной активности НЭЛ, которое предшествовало развитию септического осложнения. Прослеживается связь между исходно ослабленной функциональной активностью НЭЛ и развитием септического осложнения у пациента в послеоперационном периоде. The development of infectious complications in patients with inflammatory diseases is determined by how effective protective reaction the patient’s neutrophils will develop upon contact with the pathogen. The body’s anti-infective resistance is largely determined by the willingness of neutrophils to form neutrophilic networks capable of effectively capturing pathogens. Aim. Determination of parameters of neutrophil extracellular traps (NETs) in patients with inflammatory diseases of the abdominal cavity. Methods. 28 operated patients with various forms of abdominal inflammation and 6 therapeutic patients with ulcerative colitis were examined, 6 patients were examined upon admission. Neutrophils were isolated using gradient centrifugation. To calculate the NETs, fluorescence microscopy with the dye SYBR Green (Evrogen; Russia), which specifically interacts with double-stranded DNA, was used. The functional activity of NETs was determined in the Klebsiella pneumoniae capture test (ATCC 700603). Results. In the study of patients in the postoperative period, it was found that in acute appendicitis/abscess, acute cholecystitis and acute pancreatitis/pancreonecrosis, similar amounts of NETs are found – 12,63±2,13%, 15,66±2,81% and 12.64±2.73%. In the same groups of patients, significant differences in the size of the NETs are recorded, which reach 46.82±6.70 microns, 73.62±16.29 microns and 96.84±27.17 microns, respectively. The increase in the size of the NETs in these groups of patients is explained by the presence, in addition to network-like structures, additionally filamentous, fibrous and cloud-like neutrophil extracellular structures. A group of patients with nonspecific ulcerative colitis receiving conservative treatment (comparison group) shows a tendency to decrease the size of the NEL to a value of 31.96±14.75 microns. A study of the functional activity of NETs shows that its decrease precedes the development of a septic condition (using the example of an identified clinical case). Conclusion. All, without exception, the studied nosological forms of inflammation in the abdominal cavity are accompanied by the formation of NETs in the form of neutrophilic networks. In addition to neutrophilic networks, inflammation induces the formation of NETs in the form of single strands, fibers and clouds form in some patients. The quantitative parameters of the NETs correlate with the size and spread of the inflammatory focus to neighboring anatomical areas. On the example of the described clinical case, a sharp decrease in the functional activity of NETs was found, which preceded the development of a septic complication. There is a connection between the initially weakened functional activity of NETs and the development of septic complications in the patient in the postoperative period.

To date, the problem of preserving symptoms after recovery from a new coronavirus infection is urgent in the world. This condition is called postcovid syndrome. The clinical picture of postcovid syndrome has multiple manifestations: general, respiratory, cardiovascular, gastrointestinal, skin and other symptoms. At the moment, there are no laboratory criteria for the diagnosis of this condition, but the great role of neutrophils in the development of both acute disease and postcovid syndrome has been proven. The formation of neutrophil extracellular traps (not toz) is one of the pathophysiological mechanisms of the course of a new coronavirus infection. In addition, the effect of the ketosis process on the development of complications in the postcovid period has been proven. The article discusses the history of the term, various clinical manifestations of the postcovid period, as well as the role of innate immunity mechanisms at all stages of the course of a new coronavirus infection.

We studied the neutrophils and monocytes obtained from 37 patients with various inflammatory diseases such as psoriasis, acute infectious process in the abdominal cavity (acute appendicitis/abscess of the abdominal cavity, and acute cholecystitis), acute pancreatitis, and post-COVID syndrome after mild COVID infection. The number and the morphological structure of neutrophil extracellular traps (NET) as well as the effect of IgG on NET were examined. NET were visualized and counted by fluorescence microscopy with fluorescent dye SYBR Green. All the studied types of inflammation were accompanied by spontaneous formation of NET. After application of IgG, the number of NET doubled, their size increased, and transformation of net-like traps into the cloud forms was observed. The clouds form structure of the network is not capable of capturing pathogens with subsequent retraction, the products of its enzymatic degradation can be the factors of secondary alteration. The study results demonstrate a previously unknown mechanism of infection resistance.

Цель исследования - определение числа нейтрофильных экстраклеточных ловушек и уровня внеклеточных пуриновых азотистых оснований в периферической крови больных с постковидным синдромом. Методика. Обследован 41 амбулаторный пациент в возрасте от 18 до 59 лет (21 после перенесенной коронавирусной инфекции, 20 лиц, не перенесших инфекцию, составили группу сравнения). Для визуализации и подсчета НЭЛ использовали метод флуоресцентной микроскопии. Выявление нейтрофильных экстраклеточных ловушек (НЭЛ) проводили с помощью флюоресцентного красителя для двухцепочечной ДНК SYBR Green I (Evrogen). Пуриновые азотистые основания определяли методом цветной реакции, основанной на взаимодействии их с азотнокислым серебром с образованием окрашенного соединения. Результаты. НЭЛ нитевидной формы в крови у пациентов с постковидным синдромом наблюдались на протяжении 3 и более месяцев. Концентрация внеклеточных пуриновых азотистых оснований в крови больных возрастала пропорционально тяжести перенесенного заболевания. Заключение. Постковидный синдром сопровождается формированием в крови больных нейтрофильных экстраклеточных ловушек в нитевидной форме. В периферической крови больных с посткоронавирусным синдромом выявляются внеклеточные пуриновые азотистые основания в концентрациях, способных вызвать вторичную альтерацию клеток. Aim. The aim of the study was to determine the number of neutrophil extracellular traps (NETs) and the concentration of extracellular purine nitrogenous bases in the peripheral blood of patients with post-COVID syndrome. Methods. 41 outpatient patients aged 18 to 59 years were examined (21 after coronavirus infection and 20 without previous infection, the comparison group). Fluorescence microscopy was used to visualize and count NETs. NETs were detected using a fluorescent dye for double-stranded DNA, SYBR Green I (Evrogen). Purine nitrogenous bases were determined by a color reaction based on their interaction with silver nitrate to form a colored compound. Results. The filamentous NETs in the blood of patients with post-COVID syndrome were present for 3 months or longer. The concentration of extracellular purine nitrogenous bases in the patients’ blood increased proportionally to the disease severity. Conclusion. Post-COVID syndrome is accompanied by the formation of filamentous NETs in the blood of patients. Extracellular purine nitrogenous bases are found in the peripheral blood of patients with post-COVID syndrome at concentrations that are capable of causing secondary alterations of cell.

Нейтрофильные экстраклеточные ловушки (НЭЛ) возникают в результате высвобождения гранулярного и ядерного содержимого нейтрофилов во внеклеточное пространство в ответ на различные классы микроорганизмов, растворимые факторы и собственные модифицированные антигены организма. Во многих исследованиях продемонстрировано, что образование НЭЛ является эффективным механизмом борьбы с внедряющимися микроорганизмами, поскольку недостаточность формирования НЭЛ или гидролиз основной нуклеотидной цепи НЭЛ бактериальными ДНКазами делает организм человека восприимчивым к инфекциям. Основная роль НЭЛ - предотвращение распространения микроорганизмов в организме. Принято считать, что образование нейтрофильных экстраклеточных ловушек должно строго регулироваться, чтобы избежать повреждения тканей и избыточной гемокоагуляции. Цель исследования - морфологическая характеристика основных типов структур нейтрофильных экстраклеточных ловушек в зависимости от вида воспалительного процесса. Методика. В исследование были включены 18 больных с различными видами воспаления (абсцесс брюшной полости, аппендицит, панкреонекроз, калькулезный холецистит) в острый период заболевания с высокими показателями лейкоцитоза (10-12 тыс/мкл). Для визуализации и подсчета нейтрофильных экстраклеточных ловушек использовали метод флуоресцентной микроскопии. Окрашивание НЭЛ проводили с помощью флюоресцентного красителя для двухцепочечной ДНК SYBR Green (Evrogen). Результаты. Показано, что при благоприятном течении острого инфекционного процесса нейтрофилы выбрасывают одну или несколько нитей c ДНК, которые затем ветвятся и формируют структуру сети. В дальнейшем происходит ретракция волокон сети с захватом микроорганизмов. При постнекротическом воспалении сеть не формируется, а вместо неё возникает вуалеобразная структура, состоящая из тонких нитей c ДНК. Асептическое воспаление характеризуется особой морфологической формой -- нитевидной. Нейтрофил, при этом виде воспаления, выбрасывает значительной длины одиночную нить c ДНК. Заключение. Предложенный нами метод визуализации нативных нейтрофильных экстраклеточных ловушек показал высокую диагностическую эффективность. Он позволяет выявлять различия в структурах нейтрофильных экстраклеточных ловушек при разных типах воспаления. Neutrophil extracellular traps (NETs) arise as a result of the release of granular and nuclear contents of neutrophils into the extracellular space in response to various classes of microorganisms, soluble factors and own modified antigens. Many studies have demonstrated that the formation of NETs is an effective mechanism for combating invading microorganisms, since insufficient release of NETs or hydrolysis of the main nucleotide chain of NET by bacterial DNases increases susceptibility to infections. The main role of NET is to prevent the spread of microorganisms. It is generally believed that the formation of NETs should be strictly regulated to avoid tissue damage and excessive hemocoagulation. The aim of the study was to identify the main morphological structures of NETs depending on the type of inflammatory process. Methods. The study included 18 patients with various types of inflammation (abdominal abscess, appendicitis, pancreatic necrosis, calculous cholecystitis) in the acute period of the disease with high rates of leukocytosis (10-12 103/µl). NETs were stained with a fluorescent dye for double-stranded DNA, SYBR Green (Evrogen), and visualized and counted using fluorescence microscopy. Results. The study showed that, with a benign course of an acute infectious process, neutrophils emit one or more DNA strands, which then branch and form the network structure. In the future, the fibers of the network retract, capturing microorganisms. With necrotic inflammation, the network is not formed, but instead a veil-like structure consisting of thin strands of DNA appears. Aseptic inflammation is characterized by a special morphological form, i.e., a threadlike form. Neutrophils, with this type of inflammation, emit a single strand with DNA of considerable length. Conclusion. Thus, our proposed method of visualization of native NETs has shown high efficiency. It allowed us to identify different structures of NETs in various types of inflammation (infectious, necrotic and aseptic), which seems relevant and opens up a new direction in pathophysiology of inflammation.

The article presents the work of a multidisciplinary team of experts from various fields of medicine to optimize the «Questionnaire for assessing chronic pelvic pain and pelvic organ dysfunction (QCPPD) of the Ryzhikh National Medical Research Centre for Coloproctology» for use in clinical practice. The survey of respondents was conducted from June 28 to September 28, 2021. As a result of this survey, by repeatedly making edits and clarifications during communication with respondents, the final version was obtained, which allows assessing the patient's subjective sensations by the nature and localization of pelvic pain, sensitivity disorders and pelvic organ function. The main objective of this Questionnaire is to differentiate patients with neurogenic pain from a huge number of patients with chronic pelvic pain. This aspect will allow a more targeted approach to the diagnosis and pathogenetically justified treatment of patients, including after appropriate instrumental examinations. The work of a multidisciplinary team implies a higher degree of objectification and terminological accuracy of the Questionnaire under discussion. The presented version of the «Questionnaire for assessing chronic pelvic pain and pelvic organ dysfunction (QCPPD) of the Ryzhikh National Medical Research Centre for Coloproctology» will be primarily used in coloproctological patients with pelvic pain problems and anal incontinence and obstructive defecation. Further studies will be directed to the clinical evaluation of the results of the work carried out.

Актуальным вопросом для специалистов самых разных клинических дисциплин является совершенствование подходов к диагностике и лечению широкого круга заболеваний, в патогенезе которых большую роль играет рассогласованность циклических процессов, управляемых внутренними и внешними ритмогенными детерминантами. Цель лекции - представление накопленных к настоящему времени знаний о физиологии и патофизиологии биоритмов, причинах и последствиях десинхронозов. An urgent challenge for various clinical specialists is improving approaches to the diagnosis and treatment of multiple diseases, which pathogenesis includes a mismatch of cyclic processes controlled by internal and external rhythmogenic determinants. The purpose of this lecture was to present the current knowledge about physiology and pathophysiology of biorhythms as well as and about the causes and consequences of desynchronosis.

This paper presents interdisciplinary consensus on the use of protocols of high-intensity magnetic stimulation for the treatment of pelvic floor muscles dysfunction with anal incontinence in Russia. AIM: to highlight the discussion and the decision-making on the basis of an interdisciplinary consensus on the use of a new algorithm of peripheral and transcranial magnetic stimulation in the treatment of pelvic floor muscles dysfunction with the clinic of anal incontinence. RESULTS: the adoption of this consensus can serve as a basis for further research of this problem and optimize the results of treatment of patients with pelvic floor muscle dysfunction with anal incontinence. The data may be interesting for a wide range of medical specialists: general practitioners, gastroenterologists, coloproctologists, surgeons, neurosurgeons, gynecologists, urologists — anyone who encounter with manifestations of this disorder in a routine practice and chooses diagnostic and treatment options. CONCLUSION: protocols for the treatment of anal incontinence using high-intensity magnetic stimulation aimed at improving the quality of treatment of patients with anal incontinence are based on the Russian experience of using the methods discussed and the analysis of the results obtained are validated.

Postkovidnyj sindrom harakterizuetsya kognitivnymi i psihicheskimi narusheniyami, bolyami v grudi i sustavah, narusheniyami obonyaniya i vkusa, a takzhe zheludochno-kishechnymi i serdechnymi rasstrojstvami. Diagnostika postkovidnogo sindroma osnovyvaetsya preimushchestvenno na zhalobah bol'nyh. V nastoyashchee vremya optimal'nogo metoda diagnostiki ne predlozheno. Cel'yu issledovaniya bylo sravnit' informativnost' pokazatelej, poluchennyh pri tradicionnom obsledovanii bol'nyh s postkovidnym sindromom, s urovnem v krovi nejtrofil'nyh (NEL) i monocitarnyh (MEL) ekstrakletochnyh lovushek. Issledovali nejtrofily i monocity, poluchennye ot 21 bol'nogo v vozraste 18–59 let s diagnozom postkovidnyj sindrom. Dlya vizualizacii i podscheta ekstrakletochnyh lovushek ispol'zovali metod fluorescentnoj mikroskopii s primeneniem flyuorescentnogo krasitelya dlya dvuhcepochechnoj DNK SYBR Green (Evrogen). Kliniko-laboratornye pokazateli ne pozvolyayut vyyavit' specifichnye dlya postkovidnogo sindroma izmeneniya. Vmeste s tem, postkovidnyj sindrom harakterizuetsya vospalitel'nym processom v sosudistom endotelii. Laboratornym priznakom takogo asepticheskogo vospaleniya sluzhat najdennye nami v krovi NEL v nitevidnoj forme. Nitevidnye struktury NEL obnaruzheny tol'ko u tekh bol'nyh, kotorye perenesli SOVID-19 v legkoj i srednetyazheloj forme. A u bol'nyh, perenesshih etu infekciyu v tyazheloj forme, najdeny nitevidnye MEL. Rezul'taty issledovaniya demonstriruyut, chto naibolee informativnym diagnosticheskim priznakom postkovidnogo sindroma yavlyaetsya obnaruzhenie v krovi NEL i MEL v nitevidnoj forme.

Введение. В патогенезе расстройств дефекации у больных с ректоцеле могут участвовать как анатомические, так и функциональные нарушения. Однако до настоящего времени о возможности консервативной терапии, направленной на коррекцию функциональных расстройств дефекации, изучены недостаточно. Цель: изучение эффективности использования методов включающих БОС-терапию (терапия биологической обратной связью, biofeedback терапия) и тибиальной нейромодуляции в лечении больных с функциональными расстройствами дефекации на фоне пролапса тазовых органов. Методика. Материалом исследования служили результаты обследования пациенток в возрасте от 18 до 75 лет с наличием функционального расстройства дефекации (ФРД) в сочетании с ректоцеле без ранее проводившихся попыток хирургической коррекции. Проведены оценка общеклинических данных, опрос при помощи специализированного опросника выраженности расстройств эвакуаторной функции толстой кишки, рентгеновская дефектография, аноректальная манометрия высокого разрешения до и после комплекса консервативной реабилитации при помощи БОС-терапии и тибиальной нейромодуляции. Результаты. Конечному анализу были доступны данные 60 пациенток. Ректоцеле 1-й степени выявлено у 3 человек (5%), 2-й - у 37 (61,7%), 3-й степени - у 20 (33,3%) участниц исследования. Средний балл по симптомному опроснику составил 11,4±3,7. ФРД I типа выявлено у 41 (68,3%), II типа - у 6 (10%), III - у 10 (16,7%) и IV - у 3 (5%) участниц. После проведенной БОС-терапии признаки ФРД полностью устранены у 36,7% (22/60) женщин с ректоцеле. Неэффективной БОС-терапия оказалась у 11/41 (26,8 %) пациенток с I типом манометрического паттерна, 2/6 (33,3 %) со II типом и 4/10 (40,0 %) пациенток с III типом ФРД; (всего у 17/60 (28,3 %). У пациенток с IV типом паттерна неэффективные результаты лечения отсутствовали. Заключение. БОС-терапия и тибиальная нейромодуляция приводят к устранению симптомов в 36,7% случаев и положительной динамике у 35,0% больных. Методы могут быть рекомендованы к использованию в комплексной терапии эвакуаторных расстройств дефекации у больных с ректоцеле Aim. To assess the efficacy of conservative methods like biofeedback therapy (BFB) and tibial neuromodulation (TNM) for treatment of patients with functional FDD and rectocele. Methods. Information collected during examinations of female patients with FDD and rectocele and with no previous surgery served as source data. Before and after conservative treatment with BFB and TNM, symptoms were assessed, responses to a specialized questionnaire on the severity of rectal evacuatory function impairment were analyzed, and X-ray defecography and high-resolution anorectal manometry were performed. Data before and after treatment were compared with non-parametric statistics (Wilcoxon matched pairs test). Results. The data of 60 women (mean age 48.2±13.4 years) were analyzed. Rectocele grade 1 was detected in 3 (5%), grade 2 in 37 (61.7%), and grade 3 in 20 (33.3%) patients. Mean symptom score on the specialized questionnaire was 11.4±3.7. FDD type 1 manometric pattern was found in 41 (68.3%), type II in 6 (10%), type III in 10 (16.7%), and IV in 3 (5%) participants. Complete resolution of FDD after BFB and TNM therapy was found in 22/60 (36.7%) of women. BFB and TNM therapy was ineffective in 11/41 (26.8%) patients with FDD type I, in 2/6 (33.3%) with type II, and in 4/10 (40.0%) patients with type III FDD. This conservative treatment was effective in 100% patients with type IV pattern of FDD. Based on the results, we suggest further actions to improve the outcomes of conservative treatment. Conclusion. Conservative treatment with combined biofeedback therapy and tibial neuromodulation may help improve symptoms in 35% of patients and lead to complete resolution of functional component in 37% of patients with functional defecatory disorders and rectocele. This treatment was not effective in 28% of patients.

Objective. To prove the role of interrelated autoimmune, hemostatic and infl ammatory mechanisms in the pathogenesis of angleclosure glaucoma on the basis of experimental morphological research. Material and methods. The work was performed on 3 denucleated eyes of patients with terminal “creeping” angle-closure glaucoma (ACG) and 2 eyes with terminal ACG during an intractable acute exacerbation. Sagittal sections through the area of Schlemm’s canal, as well as serial cross sections, were examined by the method of paraffi n sections stained with hematoxylin-eosin (HE). To assess the degree of the infl ammatory response in the eye tissues, the density of infl ammatory cells was calculated within the standard eyepiece micrometer grid at a magnifi cation of × 20.Results. The formation of peripheral anterior synechiae between the periphery of the iris and the trabecular meshwork in the iridocorneal angle is the main etiological factor in chronic angle closure. Several mechanisms contribute to the formation of anterior synechiae. First of all, in our opinion, it is autoimmune infl ammation. Edema and hyperemia of the ciliary processes pushes the iris anteriad, collagen fi bers of the trabecular meshwork are damaged; delayed endothelialization of the trabecular plate occurs, and the angle of the anterior chamber narrows and closes as a result. The resistance to the outfl ow of intraocular fl uid increases. Ischemia, due to increased intraocular pressure (IOP), causes the formation of new vessels in the iris, where aggregates of blood cells are observed. The walls of the newly formed vessels are defective, which contributes to hemorrhages. Thus, in addition to autoimmune infl ammation, we observe signs of endothelial dysfunction syndrome associated with infl ammatory processes with ACG.Conclusions. 1. The pathogenesis of chronic angleclosure glaucoma is based on autoimmune processes, as proved by lymphocytoplasmocytic infl ammatory infi ltration with an addition of pigment-containing macrophages and fi broblasts at the junction of the iris with the cornea. 2. The detection of intravascular aggregates is a proof of impaired hemostasis in angle-closure glaucoma. 3. Parietal thrombus formation in the newly formed vessels of the iris, fi brin in the tissues are evidence of chronic endothelial dysfunction in ACG. 4. The capillaries of the ciliary processes surrounded by a fi brin ring indicate an acute vasomotor disorder and the release of plasma containing fi brinogen into the surrounding tissue. This is indirect evidence of emotional and vasomotor instability in patients with this form of glaucoma. 5. Disturbances in the systems of immunity and hemostasis are interrelated processes. 6. Increased iris stiff ness is ACG biomarker and may serve as a further target for therapeutic intervention.

The purpose of this study was to identify the leading mechanisms of immunopathogenesis of inflammation diseases of different genesis. Using the indirect immune fluorescence method, the number of lymphocytes carrying surface receptors CD3, CD4, CD8, CD16, CD56, CD20, CD72, mIgM, mIgG, CD23, CD25, CD71, HLA-DR, CD54, CD95, CD30, CD38, CD178. Lymphocytes of patients with aseptic inflammation showed an increase in the number of CD25, CD71, and HLA-DR lymphocytes – markers of activation. But the complete absence of lymphocyte activation was found in severe cases of infectious inflammatory process in patients with osteomyelitis. In autoimmune inflammation, the lymphocytes activation process has a high intensity and is accompanied by a weakening of the expression of the activation receptor apoptosis CD95. Atopic diseases (atopic dermatitis and atopic bronchial asthma) are characterized by an intense lymphocytes activation process, which is accompanies with a significant decrease in the number of CD95 + lymphocytes, but different by another lymphocytes subpopulations.

V obzore predstavlena kontseptsiya antivirusnoy sistemy vrozhdennogo immuniteta i opisany glavnyye strukturnyye komponenty etoy sistemy v organizme cheloveka, deystvuyushchey protiv RNK-soderzhashchikh virusov. Antivirusnaya sistema vrozhdennogo immuniteta vklyuchayet v sebya dva glavnykh komponenta: mitokhondrial'nyy antivirusnyy sensor (MAVS) — belok naruzhnoy membrany mitokhondriy i neytrofily perifericheskoy krovi, sposobnyye formirovat' neytrofil'nyye ekstrakletochnyye lovushki. V zavisimosti ot puti aktivatsii MAVS pri infitsirovanii kletki RNK-soderzhashchim virusom razvivayutsya dva vozmozhnykh varianta yeye gibeli — apoptoz ili degeneratsiya kletok s nekroticheskimi izmeneniyami. Razvitiye virus-indutsirovannogo apoptoza infitsirovannykh kletok vyzyvayet formirovaniye neytrofil'nykh ekstrakletochnykh lovushek, sekretsiyu vospalitel'nykh tsitokinov, generatsiyu AFK, tkanevoye povrezhdeniye, gemokoagulyatsiyu i vozniknoveniye ostrogo vospalitel'nogo protsessa s razvitiyem COVID-19-pnevmonii. Narusheniye prionopodobnoy reaktsii MAVS v otvet na virusnoye infitsirovaniye kletki zapuskayet al'ternativnyy put' aktivatsii autofagii. Kletki v usloviyakh prodolzhitel'noy aktivatsii autofagii ispytyvayut degenerativnyye izmeneniya i eliminiruyutsya iz organizma monotsitami/makrofagami, kotoryye sekretiruyut protivovospalitel'nyye tsitokiny. Takoy tip reaktsii antivirusnoy sistemy vrozhdennogo immuniteta sootvetstvuyet bessimptomnomu techeniyu zabolevaniya. Iz privedennykh naiboleye sushchestvennykh storon patogeneza koronavirusnoy infektsii COVID-19 vytekayut rekomendatsii po profilakticheskomu lecheniyu etogo opasnogo zabolevaniya. Predlagayemoye lecheniye pozvolit znachitel'no oslabit' tyazhest' zabolevaniya Covid-19 i snizit' letal'nost'.

An important role in airway inflammation in cases of bronchial asthma (BA) and chronic obstructive pulmonary disease (COPD) is played by immune responses. Methods. In order to identify the signs of impaired functioning of the immune system in patients with COPD (n = 39) and BA (n = 37), the number and activity of lymphocytes expressing CD20, CD23, CD25, CD71, CD72, HLA-DR antigens were studied in blood serum; their role in inflammation during the period of exacerbation and a stable course of diseases was evaluated. Results. Examined patients, being in both stages of the disease, revealed a significant increase in the number of B-lymphocytes of all stages of differentiation. Insufficient apoptosis was also noted, which indicates the activation of the B-cell component of the immune system. Conclusion . According to the study results, it was found that the increased activity of B-cells, on the one hand, protects the body from colonization by microorganisms and infection (in COPD), by blocking the formation of immunoglobulin E (in BA), on the other hand, the autoantibodies, which they produce, can damage their own tissue, and insufficient apoptosis process may lead to maintenance of the inflammation process.

Achromatopsia (ACHM) is a rare autosomal recessive disease. Its mutation spectrum is well described in other populations, but the data on ACHM prevalence and features in Russia are insufficient. Purpose . To describe clinically and genetically the Russian cohort of AHCM for the potential use of targeted treatment approaches, including gene therapy. Material and methods . Out of 18 patients with clinical manifestations of ACHM, 10 patients were chosen (6 with no kinship relatedness and 4 with kinship relatedness) aged 12.3 ± 5.8 years. These patients underwent standard ophthalmologic examination: visometry, perimetry, biomicroscopy, ophthalmoscopy, as well as optical coherence tomography, electroretinography, and color test on distinguishing color shades, in order to determine the clinical characteristics of ACHM. Molecular genetic confirmation of the clinical diagnosis was performed by high-performance parallel DNA sequencing. An in silico analysis of pathogenetic pathways of the clinical picture in 10 patients with confirmed ACHM was performed. Results . In the examined Russian patients, previously determined mutations in the CNGA3 and CNGB3 genes were confirmed. The most common mutation was a single nucleotide deletion with a reading frame shift in the 10th exon of the CNGB3 gene; a missense mutation in the 8th exon of the CNGA3 gene was second frequent. One patient had mutations in the CNGA3 and CNGB3 genes. Segregation analysis confirms the autosomal recessive nature of disease inheritance. Mutations in the CNGB3 gene have been observed to lead to more serious clinical manifestations than mutations in CNGA3. Conclusions . The analysis of the Russian ACHM cohort shows that mutations in the CNGA3 and CNGB3 genes are the main cause of the development of the disease. A complete molecular genetic confirmation of the clinical diagnosis has been obtained, which is necessary for prescribing targeted treatment to patients, including gene therapy.

Achromatopsia (ACHM) is a rare autosomal recessive disease. Its mutation spectrum is well described in other populations, but the data on ACHM prevalence and features in Russia are insufficient. Purpose. To describe clinically and genetically the Russian cohort of AHCM for the potential use of targeted treatment approaches, including gene therapy. Material and methods. Out of 18 patients with clinical manifestations of ACHM, 10 patients were chosen (6 with no kinship relatedness and 4 with kinship relatedness) aged 12.3 ± 5.8 years. These patients underwent standard ophthalmologic examination: visometry, perimetry, biomicroscopy, ophthalmoscopy, as well as optical coherence tomography, electroretinography, and color test on distinguishing color shades, in order to determine the clinical characteristics of ACHM. Molecular genetic confirmation of the clinical diagnosis was performed by high-performance parallel DNA sequencing. An in silico analysis of pathogenetic pathways of the clinical picture in 10 patients with confirmed ACHM was performed. Results. In the examined Russian patients, previously determined mutations in the CNGA3 and CNGB3 genes were confirmed. The most common mutation was a single nucleotide deletion with a reading frame shift in the 10th exon of the CNGB3 gene; a missense mutation in the 8th exon of the CNGA3 gene was second frequent. One patient had mutations in the CNGA3 and CNGB3 genes. Segregation analysis confirms the autosomal recessive nature of disease inheritance. Mutations in the CNGB3 gene have been observed to lead to more serious clinical manifestations than mutations in CNGA3. Conclusions. The analysis of the Russian ACHM cohort shows that mutations in the CNGA3 and CNGB3 genes are the main cause of the development of the disease. A complete molecular genetic confirmation of the clinical diagnosis has been obtained, which is necessary for prescribing targeted treatment to patients, including gene therapy.

The complete form of X-linked congenital stationary night blindness (CSNB) is a rare genetic disease caused by a mutation in the NYX gene. CSNB is associated with the mutations taking place in 17 genes, whilst its CSNB1A form is caused by the mutations in the NYX gene, which were characterized earlier, although nothing had been reported so far about the Russian founder principle. The paper analyzes the pathogenetic mechanisms in a family with diagnosed CSNB1A and a new genetically confirmed mutation in the NYX gene in four members of one Russian family. Two brothers of the four siblings (two boys, two girls) with congenital stationary night blindness, diagnosed in early childhood, and high myopia underwent a standard ophthalmic examination, supplemented with OCT, electroretinography and color blind test with tables by Rabkin and Farnsworth test, whereupon they were sent to molecular genetics confirmation of the diagnosis by whole exome sequencing with subsequent Sanger sequencing confirmation of the detected mutation in the proband and proband’s relatives. In members of the family with clinical features of CSNB1A the reading frame shift mutation was genetically confirmed in the NYX gene (c.283delC, p.His95fs, NM_022567.2). This mutation is inherited in X-linked form. This is the first report of a case with a novel and probable founder mutation from Russia associated with CSNB1A. Since the mRNA of a NYX gene consists of only 2696 base pairs, a gene replacement therapy, or CRISPR-based gene editing, or a similar approach may be envisaged for the correction of frameshift in His95fs position.