Academy Of Sciences Research Articles

Constructive Approaches to Analysis of Constructed Identity

The article deals with the main directions and outcomes of identity research along with the expansion of the research field as a whole and concept list. The author explores an interdisciplinary and multidisciplinary approach well established in identitarian research. By building a general outline and defining the comparative context of the analysis of identity in general and political identity in particular, the article explores collective monographs “Integration and disintegration potential of identity in history and modernity” and “Identity. Personality, society politics. New contours of the research field”. The interdisciplinary work of Tomsk scientists is structured into three parts, corresponding to the author’s methodology. This approach enables to characterize the key positions of the authors of each part, comparing them with the arsenal developed in the social and humanities. Scientific and political relevance characterize each of the dimensions chosen by the authors of the monograph, which also determines the high disputability of the proposed topics and assessments. The collective monograph edited by Corresponding Member of the Russian Academy of Sciences I.S. Semenenko has 44 sections written by a team of 32 authors. The article contains an overview of key sections and directions of research, and notes the role of the executive editor, who is one of the leaders of identitarian research in modern Russia. The collective monograph is aimed at promoting the issue of identity not only in scientific discussions, but also in the public space, since identity is considered as an important development resource. Despite all the differences in format, structure, objectives and approaches, these works are united by bringing identity into the mainstream of sociohumanitarian and political science, determining the potential of identity as a resource for social development, integration (or disintegration) of the society. Analysis of the research conducted gives grounds to attribute them to the successful search for an independent vector of domestic science.

On the History of Studying of Biological Motility at the Institute of Evolutionary Physiology and Biochemistry of the Russian Academy of Sciences

On the History of Studying of Biological Motility at the Institute of Evolutionary Physiology and Biochemistry of the Russian Academy of Sciences

Cardiorespiratory system of massage therapists when performing a dosed massage procedure

The article tells about the presentation of a number of studies on the effect of massage procedures on the physiological, biochemical and psychological status of a person, while work on evaluating the functional indicators of massage therapists during the procedure is rare. The study aims to determine the functional state of the cardiorespiratory system (CRS) of massage specialists in the dynamics of performing a 30-minute back massage session. The object of the study was 10 male massage therapists (age 36.0 (29.0;42.0); BMI 24.7(23.3;31.3). The study consisted in performing a classical back massage procedure for 30 minutes according to a generally accepted technique using six massage techniques. To determine the functional state during the work of specialists, an ergospirometric system and measurement of blood pressure (BP) by the Korotkiy method were used. During the massage procedure, specialists noted an increase in metabolic parameters, oxygen consumption (OC), respiratory coefficient (RC) and energy consumption, as well as blood pressure by the 30th minute of the procedure. When performing a 30-minute back massage, massage specialists significantly mobilize the links of the cardiorespiratory system. At the end of the procedure, there is an increase in blood pressure. However, the maximum increase in the parameters of the cardiorespiratory system occurs when performing a shock vibration reception. Limitations. Due to the small sample of the group, it is not possible to statistically reliably assess the significance of changes in some indicators. Ethics. The protocol of the experiment corresponded to the Helsinki Declaration. The study was developed and conducted in accordance with the methodological guidelines of the Local Scientific Research Committee on Bioethics of the Institute of Physiology of the Komi Scientific Center of the Ural Branch of the Russian Academy of Sciences.

Unveiling new fungal discoveries in Yunnan Province, China

Fungal taxa can be found almost anywhere and China is recognized as one of the most diverse regions globally. Within Asia, China leads major fungal hotspot studies, with Yunnan Province noted for its high plant and fungal diversity compared to other regions in the country. The Kunming Institute of Botany (KIB), Chinese Academy of Sciences (CAS), is a leading research institute with a botanical garden focused on the taxonomy and phylogeny of plants, fungi, lichen, and related projects. This study introduces new and known taxa from KIB collections based on comprehensive morphological and molecular analyses. Detailed morphological descriptions, illustrations and multigene phylogenetic analyses were used to identify four taxa within the class Dothideomycetes (Pleosporales). This includes a new genus (Paracrassiperidium), a new species (P. fusiforme), and two previously known species (Chaetopyrena penicillata and Pseudokeissleriella bambusicola). The new genus is characterized by dome-shaped, semi-immersed to immersed ascomata with a thick-walled peridium, a hamathecium composed of branched pseudoparaphyses, cylindrical to cylindric-clavate pedicellate asci with a well-developed ocular chamber, and fusiform, hyaline to light brown, septate ascospores.

Relationship between income inequality, social transfers, poverty, and employment in Ukraine

The impact of social transfers on income inequality and poverty remains a subject of debate, particularly regarding threshold effects, design, and integration with taxation and labor market dynamics. Using a linear regression model, the study analyzes the dependency of the Gini index on the percentage of social transfers in the average household’s monthly resources, the percentage of households with income below the median, and the percentage of the employed populace in Ukraine from 2010 to 2021. The results show that a 1% increase in social transfers in household income reduces income inequality by 0.13%, a 1% increase in employment decreases income inequality by 0.1%, whereas a 1% rise in poverty leads to a 0.34% increase in income inequality. In line with the results from EU and OECD countries, this study confirms that increasing the share of social transfers in household incomes contributes to the mitigation of income inequality in Ukraine. However, this remains valid only if the share of social transfers in households’ total income rises proportionally. The income and expenditure patterns of Ukrainian households, along with the Gini index, reflect poverty, which is partially mitigated by social transfers; however, their effectiveness is constrained by offsetting inflation. The rise in household incomes without a corresponding reduction in poverty suggests that employment is no longer the predominant factor in poverty alleviation in Ukraine. Acknowledgment This paper is funded as part of the project “Financial tools for reducing economic inequality in Ukraine” research project (No. 0124U002254), conducted at the State Organization “Institute for Economics and Forecasting of the National Academy of Sciences of Ukraine”.

COVID-19 and SMEs: An umbrella review of systematic literature (2020–2024) and future directions for entrepreneurship

AbstractThis special issue addresses the impact of COVID-19 on entrepreneurship. It builds on insights shared at the dedicated session – supported by the Hungarian Academy of Sciences' Post-COVID research grant (MTA PC-II-17/1/2022) – of the 2nd Danube Cup Conference in Belgrade in November 2023, featuring three papers presented there, along with four additional contributions. The goal of this issue is to advance scholarly understanding of post-COVID dynamics in entrepreneurship and share valuable findings with a broad academic audience. To provide a comprehensive overview of current research on this topic, we include an umbrella review examining the COVID-19 crisis' impact on small and medium-sized enterprises (SMEs) from 2020 to 2024. Drawing on 26 systematic literature reviews identified through Scopus, Google Scholar, and Elicit, this review captures key challenges, strategies, and emerging trends affecting SMEs. Major research themes identified include resilience, supply chain management, digitalization, crisis management, and the financial impact of COVID-19.

Global research progress of nanomedicine and colorectal cancer: a bibliometrics and visualization analysis

BackgroundSurgery and chemoradiotherapy are the main clinical treatment methods for colorectal cancer (CRC), but the prognosis is poor. The emergence of nanomedicine brings bright light to the treatment of CRC. However, there has not been a comprehensive and systematic analysis of CRC and nanomedicine by bibliometrics.MethodsWe searched the Web of Science Core Collection database (WOSCC) for relevant literature published from 2011 to 2024. We used VOSviewer and Citespace to analyze countries, institutions, authors, keywords, highly cited references, and co-cited references.Results3105 pieces of literatures were included in the research analysis, and PEOPLES R CHINA and the USA took the leading position in the number of papers published and had academic influence. The Chinese Academy of Sciences posted the most papers. The most prolific scholar was Abnous Khalil. The level of economic development is inversely proportional to the number of cases and deaths of colorectal cancer. Nanoparticles (NPs), the nanomedical drug delivery system (NDDS) is a hot topic in the field. Photodynamic therapy (PDT), immunogenic cell death (ICD), tumor microenvironment (TEM), folic acid, and pH are the cutting edge of the field.ConclusionThis paper introduces the research hotspot, emphasis, and frontier of CRC and nanomedicine, and points out the direction for this field.

Visualization and analysis of PM2.5 health effects, 2013 to 2023: Bibliometrics of PM2.5 and health effects.

To analyze the current status, hotspots, and cutting-edge trends of PM2.5 health effects of PM2.5, using CiteSpace. The Web of Science Core Collection Science Citation Index Expanded was searched for relevant articles from January 1, 2013, to December 31, 2023. Network maps identifying authors, institutions, countries, keywords, co-cited authors, journals, references, and research trends were then created using CiteSpace. A total of 2174 articles on the health effects of PM2.5 were identified between 2013 and 2023, with an increasing trend in annual publications. The United States had the highest number of articles on this topic, followed by China. The Chinese Academy of Sciences (CAS) is the leading institute in terms of paper production. Hoek was the most prolific author, focusing on the health consequences of air pollution. Among referenced journals, ENVIRON HEALTH PERSP ranked first, while Pope Ca was the most cited author. Current research focuses on reactive oxygen species (ROS), inflammation, oxidative stress, cardiovascular and respiratory diseases, PM2.5 sources, and chemical composition. The field is currently experiencing a phase of rapid expansion. The findings of this bibliometric analysis offer insight into the status and direction of research on PM2.5 and its health impacts, which aid scientists in coming up with new directions for their investigation.

Mentions of the disease nezhit in dietary texts of South Slavic medieval literature

The purpose of the present study is to trace how the disease Nezhit is represented in certain manuscripts, specifically dietary calendars preserved in the National Library of Serbia and the Archives of the Serbian Academy of Sciences and Arts. While the study is not exhaustive, it preliminarily outlines the framework for the depiction of this disease in the dietary calendars. For the first time, this publication highlights specific medical guidelines on how to treat individuals suffering from this condition.

A Study on Position and Lumen Geometry at the Origin of External Carotid Artery in Human Adult Cadavers

Background: External carotid artery is the predominant source of blood supply to the structures in the head and neck region. Morphological anomalies in the external and internal carotid arteries may lead to severe complications, when radiographic evaluations and surgical proceedings are done in the neck without any prior knowledge. Normal arterial diameters of external carotid arteries were important in-patient selection, preoperative planning, and design of new endovascular devices for arterial reconstruction. Material and Methods: A descriptive study was performed on 60 formalin fixed male and female Human adult cadavers, aged between 45 to 75 yrs in the department of Anatomy, Dr.Pinnamaneni Siddhartha Institute of Medical Sciences and Research foundation, chinoutapalli and Mamatha academy of medical sciences, Hyderabad , during the academic years, 2018- 2023 during the academic years . Observations from both right and left external carotid arteries (total- 120 sides) were noted. Results: In the present study, positions of external carotid artery observed were, antero-medial in 90%, anterolateral in 6.66% and lateral position in 3.33% of cases. The mean ± standard deviation calculated for the diameter of lumen at the origin of external carotid artery on the right side was 0.623±0.048 cm and on the left side was 0.608±0.050cm. For both right and left sides was 0.616±0.049cm. Conclusion: Positional variations of external carotid artery observed in the present study were anterolateral and lateral positions. In such cases Carotid endarterectomy can be safely done, after transposing the internal carotid artery to normal location. Data on normal arterial diameters were helpful during reconstructive surgeries and manufacture of endovascular devices, respective to different ethnic groups. KEYWORDS: Common Carotid Artery, Carotid Artery External, Positional Variations, Lumen Geometry, Carotid Endarterectomy.

Prilog proučavanju znanstvene i društvene aktivnosti Ferde Šišića na temelju korespondencije iz Arhiva HAZU-a u Zagrebu

On the basis of extensive archival materials related to the life, scientific and political activities of one of the most respected Croatian historians, Ferdo Šišić (1869–1940), which have been archived and kept in the Archives of the Croatian Academy of Sciences and Arts (HAZU) in Zagreb, the authors analyze his relations with his contemporaries. In this work, by processing material from Series A - Letters of various correspondents to Šišić, by analyzing the content of the letters, one can identify the circle of persons who addressed their letters to Šišić and especially the reasons for writing to him, one of the most respected members of the Croatian intellectual milieu at the end of the 19th and the first half of the 20th century. The mentioned archival fund contains letters from the period 1895 to 1940, and reveals that Šišić maintained lively scientific and everyday connections with persons from different social strata and different professions. However, by far the highest number of letters is devoted to historiography and related sciences (auxiliary historical sciences, philology, egdotics), which he discussed with distinguished scientists from Croatia, Central Europe, Russia, and Western Europe, as well. In addition to his scientific reputation, which his contemporaries emphasize in their letters in the Croatian, Slovenian, Serbian, Hungarian, German, French, English and Russian languages, many of them, especially the correspondents from Croatia, Slovenia and Bosnia and Herzegovina, reveal his character virtues, specifically that he was respected as a scientific authority, as well as a moral and empathic individual, who was always willing to help younger scientists, friends, and acquaintances.

Patient-Centric Mobile Medical Services Accessed Through Smartphones in the Top 100 Chinese Public Hospitals: Cross-Sectional Survey Study.

Smartphone-based technology has been used to enhance the delivery of health care services to the public in numerous countries. This study aims to investigate the application of patient-centric mobile medical services accessed through smartphones in the top 100 Chinese public hospitals. Data on 124 tertiary public hospitals, ranked among the top 100 by the China Hospital Science and Technology Evaluation Metrics of the Chinese Academy of Medical Sciences (2019) and China's Hospital Rankings of the Hospital Management Institute of Fudan University (2019), were collected from the WeChat platform (Tencent Inc), mobile phone apps, and official websites until February 10, 2021. A total of 124 tertiary public hospitals, all of which were among the top 100 hospitals according to the 2 ranking lists, were selected for this study. Almost all (122/124, 98.39%) of the hospitals offered basic services such as appointment scheduling, registration, and health education. The majority also provided online access to test reports (95/124, 76.61%), consultations (72/124, 58.06%), and prescriptions (61/124, 49.19%). Among the hospitals offering online prescriptions, the majority (54/61, 88.52%) supported home delivery through third-party carriers. Slightly less than half (57/124, 45.97%) used artificial intelligence for medical guidance. Only a small fraction (8/124, 6.45%) managed chronic diseases through online monitoring and supervision by experienced doctors. Approximately half (60/124, 48.39%) of the included hospitals were officially licensed as internet hospitals approved to provide full online services. Hospitals with official internet hospital licenses provided more extensive digital health offerings. A significantly higher proportion of approved hospitals offered online consultations (29.69% vs 88.33%, r=43.741; P<.001), test reports (62.5% vs 91.67%, r=14.703; P<.001), and chronic disease management (1.56% vs 11.67%, r=5.238; P<.05). These officially approved hospitals tended to provide over 6 mobile medical services, mainly in the regions of Shanghai and Guangdong. This geographic distribution aligned with the overall layout of hospitals included in the study. Patient-centric mobile medical services offered by the top 100 Chinese public hospitals accessed through smartphones primarily focus on online appointment scheduling, registration, health education, and accessing test reports. The most popular features include online consultations, prescriptions, medication delivery, medical guidance, and early-stage chronic disease management. Approved internet hospitals offer a significantly greater variety of patient-centric mobile medical services compared with unapproved ones.

Csokonai és Debrecen

The paper asks how the literary legacy of Mihály Csokonai Vitéz (1773–1805) has been handed down: why did Csokonai’s legacy remain so intact that it is still accessible to modern research (a significant part of the material is currently housed in the Hungarian Academy of Sciences), and how did Csokonai become such a timeless marker of Debrecen’s urban space and local identity in the 19th century and later? The answer lies in the fact that there were people in Csokonai’s immediate Debrecen environment (his family and circle of acquaintances) who, immediately after his death, considered it important to preserve his intellectual legacy. The role played by Csokonai’s mother in this process should be highlighted. Later, after his mother’s death, there was a person (László Gaál) who not only preserved his own memories, but also passed on Csokonai’s legacy and his own memories to the persons and institutions of the slowly centralising scientific community in Pest, who were able to shape the outlook and canon. In other words, the survival of Csokonai’s legacy and its emphatic inclusion in the literary historical canon was the result of the cooperation of local and central factors. This did not mean, however, that Csokonai was discovered by the city of Debrecen itself: the discrepancy between the preservation of manuscripts and the preservation of the Csokonai-related material world is striking. While the former reaches the most important places of the institutionalised literary memory almost as fast as lightning, the musealisation of objects does not follow, and the Csokonai Circle (Csokonai Kör), which was founded in Debrecen only much later, in 1890, vainly tried to collect such relics, but could hardly find anything that could be authentically connected to Csokonai.

Bibliometric analysis of research trends in agricultural soil organic carbon components from 2000 to 2023

Soil organic carbon is a vital component of the soil carbon pool. Investigation of its composition and dynamics is crucial for enhancing carbon sequestration in soils and for stabilizing the global carbon cycle. In recent years, considerable research has focused on the interactions between soil organic carbon components and their responses to varied land use and agricultural practices. However, the mechanism of soil organic carbon sequestration and response characteristics of soil organic carbon components to soil carbon pools are still subject to some debate. To the best of our knowledge, no researchers have used bibliometric analyses to explore the field of soil organic carbon components. This study thus involved the use of bibliometric techniques to identify research hotspots in the study of organic carbon components over the last 23 years and future trends in research development. Specifically, we performed a comprehensive literature review of 607 documents pertaining to organic carbon components using the Web of Science database, covering the period from 2000 to 2023. Employing CiteSpace, we visualized and analyzed the data across national, institutional, disciplinary, and keyword dimensions. In this study, we conducted a comprehensive, systematic, and quantitative analysis of publications pertaining to organic carbon component research. The results indicate that researchers in the United States and China have substantially influenced the study of soil organic carbon components. Since 2000, the United States has pioneered the study of soil organic carbon components, establishing a foundational role in this field of research. Meanwhile, China leads with the largest number of publications and the most diverse research directions in this field. Among the institutions involved in such research, the University of Chinese Academy of Sciences has the highest number of publications. The investigation of soil organic carbon components within agricultural systems is inherently multidisciplinary, with the most comprehensive research being performed within the soil sciences discipline. At present, the focal areas of research on soil organic carbon components predominantly revolve around the impacts of straw return to fields, varying land-use changes, restoration of vegetation, and the reciprocal effects of soil organic carbon components on the restoration of vegetation. The findings of this work highlight the research hotspots within the field of soil organic carbon components and the emerging trends within this field. This work offers novel insights into the dynamics of soil organic carbon components, potentially guiding future studies in this vital field.

Analysis of Spatial Clustering of Seismic Events in the Northwest Pacific Ocean

The spatial clustering of epicenters of seismic events in the Northwest Pacific Ocean is analyzed using the Discrete Perfect Sets (DPS) topological filtering algorithm. The results of the analysis are presented in this paper. Based on the data shallow earthquakes recorded from 1963 to 2022 by the seismic network of the Kamchatka Branch of the Geophysical Survey of the Russian Academy of Sciences, stable areas of epicenter clustering are identified. These areas are associated with the Northern and Southern segments of the Kuril-Kamchatka seismofocal zone and do not depend on the time of recording of the earthquakes. The characteristic clustering radius for the Kuril-Kamchatka seismofocal zone is determined and is found to be 42–44 km. The analysis has confirmed the unique pattern of seismicity in the territory of the Commander segment of the Aleutian arc, which differs from that observed in the Northern and Southern segments.

The September 21, 2020 (Mw = 5.6) Bystraya Earthquake at the South-Western Flank of the Baikal Rift Zone: a Milestone in Macroseismology of Eastern Siberia

The article presents analysis of macroseismic data on the September 21, 2020 (Mw = 5.6) Bystraya earthquake, which occurred in the eastern part of the Tunka basins system on the southwestern flank of the Baikal rift zone. Macroseismic data were collected mainly through an Internet questionnaire posted on the website of the Baikal Branch of the Geophysical Survey, Russian Academy Sciences. A total of 3013 eyewitness responses were collected, which is currently an unprecedented number in the entire history of macroseismic observations in the Baikal region. In total, we collected data for 263 Intensity Data Points. The maximal shaking intensity (VI–VII MSK-64) was observed in the Bystraya village and the Kultuk settlement. The shaking intensity V MSK-64 was noted at a distance of up to ∼180 km; intensity IV MSK-64 was recorded at a distance of up to ∼550 km. Analysis of data on the Bystraya earthquake revealed significantly lower attenuation compared to that expected from the regional macroseismic equation. Due to the large volume of macroseismic data collected, as well as the high efficiency of the data collection method used, the Bystraya earthquake can be considered an important milestone in macroseismic research in East Siberia.

Management of Velopharyngeal Impairment in Adults With Dysarthria: A Systematic Review.

Velopharyngeal impairment (VPI) is a common manifestation of dysarthria, yet there is uncertainty regarding the evidence for treatment options. The aim of this systematic review was to identify and evaluate published intervention studies of VPI and to update the original Practice Guidelines for VPI, as part of the Dysarthria Writing Group of the Evidence-Based Clinical Research Committee of the Academy of Neurologic Communication Disorders and Sciences. Four electronic databases were searched for articles published through September 2022 in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards. Multiple indices of methodological quality and risk of bias were used to evaluate studies. The search yielded 1,347 articles, of which 31 met inclusion criteria and were reviewed by two blinded co-investigators and a third consensus reviewer. Therapeutic approaches centered on prosthetic treatment (n = 13), prosthetic treatment combined with another form of therapy (n = 7), and behavioral exercise (n = 7), with four additional studies reporting unconventional approaches (e.g., use of medication). No single treatment approach garnered strong evidence. However, converging moderate evidence suggests positive treatment outcomes for prosthetic approaches for moderate-severe speakers with flaccid/spastic dysarthria and behavioral treatment approaches for speakers spanning a range of nonprogressive dysarthrias. There is a critical need for well-controlled treatment studies of VPI that incorporate comprehensive and systematic description of baseline speech function, treatment protocols, and operationally defined outcome measures, particularly related to social validity and participation goals. Suggestions for how clinicians can apply extant research findings to practice are provided. https://doi.org/10.23641/asha.27655566.

EDITORIAL FOREWORD

New Trends in High-Energy Physics is a series of conferences initiated by the Bogolyubov Institute for Theoretical Physics, National Academy of Sciences of Ukraine in 1992. It is focused on topical problems in experiment, phenomenology and theory of particle, nuclear and astroparticle physics. The conferences were held yearly or biannually at various sites in Ukraine and abroad, namely in Kyiv, Alushta, Yalta, Novy Svit, Odesa (Ukraine), Sukhumi (Georgia), Budva/Beˇci´ci (Montenegro), and Natal (Brazil).

Virtual collection of butterflies of Ivan Verhratskyi

The article examines the history of the formation of Ivan Verkhratskyi's collection of butterflies - from the first collections to the transfer to the funds of the State Natural History Museum of the National Academy of Sciences of Ukraine and digitization. In total, as of now, the collection includes 5,278 units of butterflies collected at the end of the 19th - beginning of the 20th centuries. The work uses generally accepted methods of digitizing naturalistic collections. Digitized samples have been included in the database of the web resource of the State Museum of Natural History of the National Academy of Sciences of Ukraine - Data Center «Biodiversity of Ukraine». As part of the project, 2,060 specimens belonging to 247 species from 18 families of Lepidoptera were digitized. As a result, the availability of digital data of the author's collection for various target audiences is ensured. The priorities of the "Cultural Heritage" competition program were implemented in two directions, namely: digitization of museum objects and digitization of intangible cultural heritage. The Lepidoptera collection of Ivan Verkhratskyі can be used for long-term monitoring of the state of the diversity of butterflies in the region, chronological analysis of changes in their species composition and distribution, as well as the study of individual imago variability and the accumulation of data on rare protected species. In addition, the digitized collection of butterflies has an important aesthetic and cognitive value.

Two cases of deficiency in ELF4 gene X-linked and literature review

Objective: To summarize the clinical phenotype and genetic characteristics of deficiency in ELF4 gene X-linked (DEX). Methods: A case series study was conducted to retrospectively analyze the clinical data and genetic testing results of 2 cases of DEX treated at Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and the First Hospital of Jilin University from January 2023 to April 2024. And literature up to April 2024 was searched from the PubMed database, as well as CNKI and Wanfang databases, using keywords such as "ELF4 deficiency" "deficiency in ELF4, X-linked""ELF4 gene". The main clinical manifestations and genotypes of DEX were summarized. Results: Both patients were male, with onset ages of 3 months and 3 years, respectively. Both patients presented with recurrent oral ulcers and abdominal pain. And the laboratory examination showed a significant increase in inflammatory markers. Intestinal examinations showed multiple intestinal ulcers, and both patients developed intestinal fistulas. Whole exome sequencing found ELF4 c.799C>T, p Arg267Trp and ELF4 c. 248-7G>A, both maternal variants. Based on clinical and genetic results, DEX were diagnosed. In terms of treatment, both patients underwent surgical treatment during the acute phase of the disease and received anti-tumor necrosis factor α therapy, but recurrent gastrointestinal symptoms were still observed in Patient 1, while the clinical effect in Patient 2 was still acceptable. However, the inflammatory markers in both patients were not normal even after treatment. Literature review found 18 patients including 2 patients in this study, reported in 5 English articles and no Chinese reports. Thirteen patients had disease onset age before 5. The main clinical manifestations were fever (12/17), oral ulcers (14/18), abdominal pain (8/18), diarrhea (6/18), perianal ulcers (5/17), ileum ulcers (6/16), colon ulcers (7/16), skin involvement (7/17) and recurrent infections (7/18); laboratory examinations found increased erythrocyte sedimentation rate (13/15) as well as C-reactive protein (9/9), and anemia (13/15); in terms of immunological function, there is a decrease in natural killer cells (9/15) as well as a decrease in class switching memory B cells (8/9). The main types of gene variantions were missense variantions (6/18), nonsense variantions (4/18) or frameshift variantions (3/18). Conclusions: DEX should be considered when an early-onset male patient manifested with recurrent fever, oral ulcers or mucosal ulcers, with elevated inflammatory markers, with or without recurrent infection. It is recommended to perform lymphocyte subsets analysis, gastrointestinal endoscopy and genetic testing to support the diagnosis.