Department Of Immunology Research Articles

Assessment of Anti-Ganglioside Antibodies in Patients Diagnosed With Guillain Barre Syndrome

Objective: To assess the frequency of patients positive for the presence of anti-ganglioside antibodies and factors related to it among the patients diagnosed with Guillian Barre Syndrome (GBS) at our hospital.
 Study Design: Prospective longitudinal study.
 Place and Duration of Study: Pak Emirates Military Hospital Rawalpindi, from Jan to Jun 2019.
 Methodology: Patients were included in the study once detailed assessment and investigations have been carried out by the consultant neurologist and he has declared the patient suffering from Guillian Barre Syndrome. Anti-ganglioside antibodies were performed on all the patients diagnosed with Guillian Barre Syndrome from the immunology department of the laboratory of our hospital. Factors like age, gender, preceding gastrointestinal infection; sensory involvement and need for mechanical ventilation were related with the presence of Guillian Barre Syndrome in our target population.
 Results: A total of 30 patients were enrolled in the study. Out of these 22 (73.3%) were positive for the presence of antiganglioside antibodies while 08 (26.4%) did not show the presence of detectable levels of these antibodies in their serum. On fisher exact test preexisting gastrointestinal infection and sensory involvement were statistically significantly related to the presence of anti-ganglioside antibodies among the patients suffering from Guillian Barre Syndrome.
 Conclusion: Relationship of presence of anti-ganglioside antibodies was strong with the Guillian Barre Syndrome among the patients enrolled in our study. It was even stronger in among the patients who presented with the sensory involvement or those who had a gastrointestinal infection prior to the onset of symptoms of...........

Association between Rheumatoid Arthritis Disease Activity and Risk of Ovarian Malignancy in Middle-Aged and Elderly Women.

Objective To investigate the risk of ovarian malignancy in middle-aged and elderly women with rheumatoid arthritis (RA) and its correlation with disease activity. Methods 219 middle-aged and elderly (age ≥ 40) female RA patients who were treated at the Department of Rheumatology and Immunology of the Second Affiliated Hospital of Guizhou University of Traditional Chinese Medicine from August 2019 to September 2020 were selected. Their general information such as age and medical history was collected. RA disease activity-related indicators include rheumatoid factor (RF), anticyclic citrullinated peptide antibody (ACPA), ESR, CRP, and ovarian malignancy risk-related indicators including alpha fetoprotein (AFP), carcinoembryonic antigen (CEA), CA125, CA199, and human epididymis protein 4 (HE4) were detected. According to Risk of Ovarian Malignancy Algorithm (ROMA), they were divided into a low-risk group (ROMA-low, premenopausal: ROMA ≤ 11.4%, postmenopausal: ROMA ≤ 29.9%) and a high-risk group (ROMA-high, premenopausal: ROMA > 11.4%, postmenopausal: ROMA > 29.9%) for ovarian malignancy. Meanwhile, according to the DAS28-ESR, they were divided into the general disease activity group (DAS28-ESR ≤ 5.1) and the high disease activity group (DAS28-ESR > 5.1). SPSS 25.0 software was used to compare the differences among groups and to analyze the correlation between ovarian malignancy risk and RA disease activity. Results Compared with the ROMA-low group, the levels of RF, ACCP, CDAI, SDAI, DAS28-ESR, and DAS28-CRP in the ROMA-high group were significantly increased (P < 0.05). HE4 and ROMA in the high disease activity group were significantly higher than general disease activity group (P < 0.05). Spearman correlation analysis showed that age (r = 0.472), RF (r = 0.221), ACPA (r = 0.156), CDAI (r = 0.226), SDAI (r = 0.221), DAS28-ESR (r = 0.254), DAS28-CRP (r = 0.208), medications (r = 0.189), and CA199 (r = 0.250) were correlated with ROMA (P < 0.05). Multivariate regression analysis showed that ESR (OR = 1.11), SDAI (OR = 1.02), DAS28-ESR (OR = 1.33), DAS28-CRP (OR = 1.26), and CA199 (OR = 1.03) were independent risk factors for high risk of ovarian malignancy (P < 0.05). Subgroup analysis showed that CA199 is an effect modification factor for DAS28-ESR (P < 0.05). Conclusion The risk of ovarian malignancy is significantly increased in middle-aged and elderly women with high disease activity with rheumatoid arthritis. In clinical, full attention should be paid to the risk of ovarian malignancy in this population. Screening in time, especially in patients with increased DAS28-ESR and CA199 at the same time, is needed.

POS0014 EFFICACY AND SAFETY OF JAK INHIBITORS IN REFRACTORY OR INITIAL TREATMENT OF ADULT ONSET STILL’S DISEASE

BackgroundInappropriate activation of pro-inflammatory cytokines such as interleukin (IL)-1, IL-6 or IL-18, is a pathogenic cornerstone in adult onset Still’s disease (AOSD). Beyond therapies targeting IL-1 and IL-6, Janus kinases (JAK) inhibitors have been suggested to be efficient in refractory AOSD patients [1].ObjectivesTo assess the efficacy and safety of JAK inhibitors in the treatment of AOSD patient refractory to, or with initial treatment.MethodsThis retrospective study was based on our single center of the department of rheumatology and immunology. The data was collected from the patients’ medical records using a standardized questionnaire and analyzed at different time points. The response to JAK inhibitors was categorized as complete remission (CR), partial remission (PR) or failure (F) [2].Results7 patients were recruited (Table 1), including 4 refractory patients and 3 initial patients. Mean age at JAK inhibitor treatment start was 27.5 years for refractory patients and 35 years for initial patients; and mean disease duration was 66.5 months and 1 month respectively. All patients had fever and polyarthritis, 5 patents had rash. In addition, pulmonary hypertension, abnormal liver function tests, abdominal pain, and heart failure were also observed in our patients. Response to corticosteroids, conventional synthetic or biological Disease Modifying Anti-Rheumatic Drugs (DMARDs) had been considered inadequate in 4 refractory patients. Tofacitinib was added in the initial treatment for 3 patients for high disease activity. In total, baricitinib was used in 2 patients and tofacitinib in 5 patients. Steroids were concurrently used in 6 patients, MTX in three, SASP and NSAIDs in one. At a mean follow up of 3.8 months, complete remission was observed in one patient (with tofacitinib), partial remission was in 5 patients (4 patients with tofacitinib and one with baricitinib), and failure in one (patients with baricitinib). At the last visit, steroids could be decreased but not stopped in those 6 patients. Tolerance of JAK inhibitors was excellent, none infectious disease or other severe side effect were observed.Table 1.Characteristics of the AOSD patientsNo.SexAge (year)Disease Duration (Month)Main symptomsTreatments before JAKionsetJAKiSteroids at onsetConconmitant treatmentSteroids at the end of F-UF-U(month)Outcome1M2629Fever, polyarthritisPred+MTXTofacitinib0MTX04PR2M259Fever, polyarthritis, rash, pulmonary hypertensionPred+MTXBaricitinib16MTX+Pred124F3M3812Fever, polyarthritis, rashPred+MTX+CsA+NSAIDsTofacitinib24Pred123PR4M21216Fever, polyarthritisPred+MTX+SASP+NSAIDs+TNFiBaricitinib4Pred+MTX+SASP+NSAIDs43PR5F331Fever, polyarthritis, rash, abnormal liver function testsPredTofacitinib40Pred25CR6F471Fever, polyarthritis, rash, abdominal painPredTofacitinib36Pred45PR7F251Fever, polyarthritis, rash, heart failurePredTofacitinib40Pred123PRPred: prednisone; MTX: Methotrexate; SASP: salicylazosulfapyridine; CsA: ciclosporin A; NSAIDs: Non-Steroidal Antiinflammatory Drugs; TNFi: Tumor necrosis factor inhibitor. CR: complete remission; PR: partial remission; F: failure.ConclusionJAK inhibitors treatment may be helpful for some patients with refractory AOSD, or patients with severe disease activity at initial treatment. Different treatment responses were observed in these short series of cases, which might be due to the phenotype of patients. However, the scale of patients in our study was too low to draw a conclusion. Further study and additional information are needed to evaluate more precisely the risk-benefit ratio of this treatment, and a possible difference in efficacy among the different groups of patients or JAK inhibitors.

BCG Moreau Polish Substrain Infections in Patients With Inborn Errors of Immunity: 40 Years of Experience in the Department of Immunology, Children's Memorial Health Institute, Warsaw.

ObjectiveWe aimed to assess BCG (Bacillus Calmette-Guérin) complications in patients with Inborn Errors of Immunity (IEI), according to the inherited disorders and associated immunological defects, as well as the different BCG substrains.MaterialWe studied adverse reactions to the locally-produced BCG Moreau vaccine, analyzed in patients with IEI diagnosed between 1980 and 2020 in the Department of Immunology, Children's Memorial Health Institute (CMHI), Warsaw. These results were compared with previously published studies.ResultsSignificantly fewer disseminated BCG infections (BCGosis) were found in 11 of 72 (15%) SCID (Severe Combined Immunodeficiency) NK (Natural Killer)-phenotype patients, when compared with the 119 out of 349 (34%) (p = 0.0012) patients with SCID with BCG in other countries. Significantly fewer deaths caused by BCGosis were observed (p = 0.0402). A significantly higher number of hematopoietic stem cell transplantations (HSCTs) were performed in the CMHI study (p = 0.00001). BCGosis was found in six patients with Mendelian susceptibility to mycobacterial diseases (MSMD). Other patients with IEI prone to BCG complications, such as CGD (Chronic Granulomatous Disease), showed no case of BCGosis.ConclusionThe BCG Moreau substrain vaccine, produced in Poland since 1955, showed genetic differences with its parental Brazilian substrain together with a superior clinical safety profile in comparison with the other BCG substrains, with no BCGosis in patients with IEI other than SCID and MSMD. Our data also confirmed significantly fewer cases of BCGosis and deaths caused by BCG infection in patients with SCID with this vaccine substrain. Finally, they confirmed the protecting role of NK cells, probably via their production of IFN-γ.

Novel microRNA biomarkers of systemic lupus erythematosus in plasma: miR-124-3p and miR-377-3p

BackgroundTo explore the clinical value of miR-124-3p and miR-377-3p in plasma, and to examine whether these microRNAs can serve as biomarkers for systemic lupus erythematosus. MethodsSamples from 50 patients with SLE and 47 healthy individuals were collected from the Rheumatology and Immunology Department at the Second Affiliated Hospital of Chongqing Medical University. The expression of miR-124-3p and miR-377-3p in peripheral blood mononuclear cells (PBMCs) and serum were determined by quantitative reverse-transcription PCR (RT-PCR). We then further analyzed the associations between these microRNAs and clinically relevant indicators. We employed receiver operating characteristic (ROC) curves and the area under the ROC curves (AUCs) to validate the diagnostic value of miR-124-3p and miR-377-3p. ResultsThe expression of miR-124-3p and miR-377-3p was significantly upregulated in PBMCs and serum from SLE patients compared with the normal control group (P < 0.05). ROC curve analysis indicated that plasma miR-124-3p and miR-377-3p were thus candidate diagnostic biomarkers for SLE, with AUCs of 0.714 (95% CI, 0.610 to 0.820, P < 0.05) and 0.705 (95% CI, 0.600 to 0.809, P < 0.05), respectively. Furthermore, the combined diagnostic efficiency of miR-124-3p and miR-377-3p was higher than that for miR-124-3p or miR-377-3p alone, and the AUCs for miR-124-3p and miR-377-3p in plasma were higher than in PBMCs. Plasma miR-124-3p expression was also associated with various clinicopathological parameters such as antiC1q and C3. Binary logistic regression analysis revealed that the expression of miR-377-3p was an independent predictor for SLE, and the plasma miR-124-3p was independently associated with the remission rate of SLE. ConclusionCirculating miR-124-3p and miR-377-3p may constitute promising biomarkers for the diagnosis of SLE.

Medical Mycology dissertation topics require prioritisation among Postgraduate Microbiology trainees of Makerere University, Uganda

BackgroundAs elsewhere worldwide, there is an increasing burden of fungal diseases in Uganda. However, expertise in medical mycology (the study of fungal diseases of medical importance) among clinicians and laboratory personnel remains low. ObjectiveThis study sought to determine the proportion of dissertations on medical mycology among postgraduate medical microbiology trainees at the College of Health Sciences, Makerere University, Uganda. MethodsWe retrospectively reviewed the topics of dissertations submitted to the Departments of Medical Microbiology and Immunology & Molecular Biology from 2011 through 2018. The proportion of dissertation topics on medical mycology was analysed using descriptive statistics. ResultsA total of 152 dissertations were retrieved. Of these, only 5 (3.3%) were on medical mycology compared to bacteriology (50.7%, n = 77), virology (27.6%, n = 42), parasitology (14.5%, n = 22) and immunology (4.0%, n = 6). Of the 5 dissertations on fungal diseases, the distribution was as follows: cryptococcal meningitis (40%, n = 2), Candidiasis (20%, n = 1), superficial mycoses (20%, n = 1) and other invasive fungal diseases (20%, n = 1). The most common method that was used for studying the fungal diseases was culture 60%, n = 3. ConclusionThere is limited research on medical mycology among the postgraduate medical microbiology trainees of Makerere University, Uganda.

Association of Panel Reactive Antibodies (PRA) with Complement Dependent Cytotoxicity (CDC) Cross-Match in Pre-Renal Transplant Recipients

Objective: To determine the association of panel reactive antibodies (PRA) with complement-dependent cytotoxicity (CDC) cross-match in Pakistani pre-renal transplant recipients.&#x0D; Study Design: Cross-sectional study.&#x0D; Place and Duration of Study: Immunology Department, Armed Forces Institute of Pathology, Rawalpindi from Oct 2017 to Oct 2018.&#x0D; Methodology: A total of 162 patients referred to the Department of Immunology for pre-transplant workup for renal transplantation were included. Informed consent was taken, and detailed history was recorded. Frequency and percentages were calculated for cross-match positivity and most frequent anti-HLA antibodies.&#x0D; Results: Panel reactive antibodies (PRA) were present in 48 patients (30%), while complement-dependent cytotoxicity (CDC) cross-match was positive in 16 patients (10%). Out of 141 male patients, 35 (25%) were positive for PRA, while 10 (7%) had positive CDC cross-match. Out of 21 female recipients, 13 (62%) were positive for PRA, and 6 (28%) had positive CDC crossmatch. One male patient positive for CDC cross-match was negative for PRA. Patients positive for both CDC cross-match and PRA have an average mean fluorescent intensity (MFI) of more than 4000. CDC cross-match and PRA were strongly associated, whereas no significant association was found between CDC cross-match and anti-MIC antibodies.&#x0D; Conclusion: Complement dependent cytotoxicity (CDC) cross-match and panel reactive antibodies (PRA) should be routine in patients undergoing renal transplants as alone CDC cross-match can give false negative or false-positive results. At the same time, CDC cross-match lacks detection of anti MIC antibodies involved in graft rejection.

Inborn Errors of Immunity in Algerian Children and Adults: A Single-Center Experience Over a Period of 13 Years (2008-2021).

BackgroundInborn errors of immunity (IEI) predispose patients to various infectious and non-infectious complications. Thanks to the development and expanding use of flow cytometry and increased awareness, the diagnostic rate of IEI has markedly increased in Algeria the last decade.AimThis study aimed to describe a large cohort of Algerian patients with probable IEI and to determine their clinical characteristics and outcomes.MethodsWe collected and analyzed retrospectively the demographic data, clinical manifestations, immunologic, genetic data, and outcome of Algerian IEI patients - diagnosed in the department of medical immunology of Beni Messous university hospital center, Algiers, from 2008 to 2021.ResultsEight hundred and seven patients with IEI (482 males and 325 females) were enrolled, 9.7% of whom were adults. Consanguinity was reported in 50.3% of the cases and a positive family history in 32.34%. The medium age at disease onset was 8 months and at diagnosis was 36 months. The median delay in diagnosis was 16 months. Combined immunodeficiencies were the most frequent (33.8%), followed by antibody deficiencies (24.5%) and well-defined syndromes with immunodeficiency (24%). Among 287 patients tested for genetic disorders, 129 patients carried pathogenic mutations; 102 having biallelic variants mostly in a homozygous state (autosomal recessive disorders). The highest mortality rate was observed in patients with combined immunodeficiency (70.1%), especially in patients with severe combined immunodeficiency (SCID), Omenn syndrome, or Major Histocompatibility Complex (MHC) class II deficiency.ConclusionThe spectrum of IEI in Algeria is similar to that seen in most countries of the Middle East and North Africa (MENA) region, notably regarding the frequency of autosomal recessive and/or combined immunodeficiencies.

B lymphocytes and plasma cells as drivers of rheumatic diseases

Verschiedene Arbeitsgruppen am Deutschen Rheuma-Forschungszentrum Berlin haben in enger Zusammenarbeit mit der Medizinischen Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie an der Charité wichtige Beiträge zur Bedeutung der B‑Zellen und Plasmazellen bei rheumatischen Erkrankungen geleistet, die nicht nur für die Rheumatologie, sondern für alle klinischen Fachgebiete, in denen antikörpervermittelte Erkrankungen eine Rolle spielen, relevant sind. Insbesondere wird auf die gestörte B‑Zell-Homöostase, die Bedeutung des Immunglobulin M(IgM)-Fc-Rezeptors für die Regulation der Autoimmunität, die Rolle der langlebigen Gedächtnis-Plasmazelle bei der Aufrechterhaltung der Autoimmunität sowie die Sicherung ihres Überlebens in speziellen, von Stromazellen organisierten Nischen im Knochenmark und in entzündeten Geweben eingegangen. Die Forschungsergebnisse haben zu einem besseren Verständnis der immunologischen und molekularen Mechanismen bei rheumatischen Erkrankungen und ihrer Therapie beigetragen. Die Identifizierung der langlebigen Gedächtnis-Plasmazelle hat zu vielversprechenden therapeutischen Ansätzen mit kurativem Potenzial bei Autoimmunerkrankungen geführt.

Progressive Thrombocytopenia and Clinical Deterioration with Initial Diagnosis of Esophageal Cancer-Secondary Hemophagocytic Lymphohistiocytosis as a Complicating Cause

Gastroenterology Medicine & Research Progressive Thrombocytopenia and Clinical Deterioration with Initial Diagnosis of Esophageal Cancer-Secondary Hemophagocytic Lymphohistiocytosis as a Complicating Cause Mani Nassir1*, Adrian Schreiber2, Georg Hilfenhaus1, Sebastian Stintzing1 and Uwe Pelzer1 1Department of Hematology, Oncology and Tumor Immunology (CCM), Charity-University medicine Berlin, Berlin, Germany 2Department of Nephrology and Medical Intensive Care, Charity-University medicine Berlin, Berlin, Germany *Corresponding author: Mani Nassir, Department of Hematology, Oncology and Tumor Immunology (CCM), Charity- University medicine Berlin, Berlin, Germany Submission:March 18, 2022;Published: March 29, 2022 DOI: 10.31031/GMR.2022.06.000648 ISSN 2637-7632Volume6 Issue5

Immune Dysregulation in Pediatric Common Variable Immunodeficiency: Implications for the Diagnostic Approach

Infections and infectious complications are hallmarks of common variable immunodeficiency (CVID) and the leading cause of morbidity and mortality in affected patients at any age. However, the pediatric CVID is no longer perceived as a primary immunodeficiency associated solely with infectious manifestations; autoimmune, allergic, lymphoproliferative, and malignant disorders and organ-specific immunopathology also characterize the spectrum of non-infectious complications. In this study, we sought to determine the role of immune dysregulation and frequency of non-infectious sequelae in children affected with CVID. We also aimed at providing an insight into the pathogenesis of non-infectious complications and at delineating the diagnostic approach to pediatric CVID with immune dysregulation. An in-depth retrospective analysis of clinical manifestations and their correlations with selected immune parameters was performed in a group of 39 CVID children, followed by our pediatric immunology department. Whereas recurrent sinopulmonary infections were present in all (100%) of the children studied, an unexpectedly high rate of non-infectious disorders and immune dysregulation phenotypes were observed in as many as 32 (82.05%) patients, compared with infection-only phenotypes limited to 7 (17.95%) male patients. The most common inflammatory comorbidity was asthma, diagnosed in 21 (53.85%) patients. The second most frequent immune dysregulation group was autoimmune disorders, present in 18 (46.15%) of the children studied with a high rate of autoimmune thyroiditis in as many as 10 (25.64%) of the CVID-affected children. Lymphoproliferation was seen in 14 children (35.90%), and, among them, lymphadenopathy occurred in nine (23.08%) cases and granulomatous lymphocytic interstitial lung disease in seven (17.95%) cases. Finally, malignancies occurred in two female patients (5.13%), papillary thyroid cancer in the first one and T-cell lymphoblastic leukemia in the other one. The most prominent abnormalities in the B- and T-cell compartment contributing to complex immune deficiency and immune dysregulation phenotypes were seen in the autoimmunity group, showing significant reductions in the switched memory B cell, naive T helper cell, and regulatory T-cell subsets. Herein, we document the previously unreported high rate of immune dysregulation in pediatric CVID as a clinical and diagnostic challenge with the variability of defects in the humoral and cellular immune responses.

Rakhim M. Khaitov (06.01.1944 – 11.03.2022)

Russian science has suffered a heavy loss. On March 11, 2022, Rakhim Musaevich Khaitov died - Academician of the Russian Academy of Sciences, Doctor of Medical Sciences, Professor, Honored Scientist of the Russian Federation, Scientific Director of the Federal State Budgetary Institution "SSC Institute of Immunology" of the Federal Medical and Biological Agency of Russia, Chief Allergist-Immunologist of the Ministry of Health of Russia, member of the Bureau of the Department of Physiology and Fundamental Medicine RAS, Head of the Department of Clinical Allergology and Immunology, Moscow State Medical University named after A.I. Evdokimov of the Ministry of Health of Russia.

Mechanism of Xinfeng Capsule in the Treatment of Hypercoagulable State of Ankylosing Spondylitis Based on Data Mining and Network Pharmacology.

Background Ankylosing spondylitis (AS) is a rheumatism that mainly affects the axial bones and joints. Xinfeng capsule (XFC) is a preparation with a remarkable clinical effect that is used in our hospital. And it has definite curative effect and less side effects in the treatment of AS. Objective Data mining and network pharmacology were used to analyze the efficacy of Chinese medicine Xinfeng capsule on treating the hypercoagulable state of ankylosing spondylitis and the underlying mechanism behind it. Methods Clinical data were collected and compiled from the Department of Rheumatology and Immunology of the First Affiliated Hospital of Anhui University of Chinese Medicine. Cluster analysis was used to investigate herbs that frequently used to treat AS, Apriori module was used to analyze the association rules between herbs and laboratory indexes, and the random walk model was used to reveal the therapeutic efficacy of XFC against AS. The TCMSP database was used to acquire the active components and targets of XFC, and the GeneCards and OMIM database were used to obtain the targets of AS. Afterward, an active ingredient-target network was established and core targets were screened for; overlapping targets were screened for the protein-protein interaction (PPI) network analysis, the Gene Ontology (GO) enrichment analysis, and the Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analysis. Molecular docking was adopted to investigate the interactions between main active components and core targets. Results Frequently used herbs could be divided into three groups, and according to the analysis of Apriori module, there is a strong correlation between XFC and the improvement of ESR and hs-CRP, and the results of the random walk model demonstrated that the effect of XFC on improving PLT, ESR, and hs-CRP was superior to the use of traditional Chinese medicine alone. In total, 103 active compounds of XFC and 59 overlapping targets were obtained. The PPI relationships were obtained through the STRING database, and 13 core targets were identified. 1786 GO enrichment results and 205 KEGG enrichment results were obtained, including NF-kappa B signaling pathway, TNF signaling pathway, and IL17 signaling pathway. The outcomes of molecular docking revealed a close relationship between the active compounds of XFC and core targets. Conclusion This study demonstrated that XFC can effectively improve the hypercoagulable state and the inflammatory indices of AS patients through data mining, and it has a strong correlation with the clinical improvement of inflammation. The active compounds of formononetin, triptolide, quercetin, and kaempferol may be the key active components of XFC in regulating AS, possibly through inhibiting the activation of NF-kappa B signaling pathway to improve hypercoagulable state.

First person – Harmen Koning

ABSTRACT First Person is a series of interviews with the first authors of a selection of papers published in Biology Open, helping early-career researchers promote themselves alongside their papers. Harmen Koning is first author on ‘ A deep-dive into fictive locomotion – a strategy to probe cellular activity during speed transitions in fictively swimming zebrafish larvae’, published in BiO. Harmen is a PhD student in the lab of Henrik Boije at the Department of Immunology, Genetics and Pathology, Uppsala University, Sweden, investigating the neuronal networks of locomotion in the zebrafish spinal cord to get a better understanding how hardwired circuits produce the flexible output needed for certain behaviours.

Impact of Biological and Lifestyle Factors on Cognitive Aging and Work Ability in the Dortmund Vital Study: Protocol of an Interdisciplinary, Cross-sectional, and Longitudinal Study.

BackgroundPrevious research revealed several biological and environmental factors modulating cognitive functioning over a human’s lifespan. However, the relationships and interactions between biological factors (eg, genetic polymorphisms, immunological parameters, metabolic products, or infectious diseases) and environmental factors (eg, lifestyle, physical activity, nutrition, and work type or stress at work) as well as their impact on cognitive functions across the lifespan are still poorly understood with respect to their complexity.ObjectiveThe goal of the Dortmund Vital Study is to validate previous hypotheses as well as generate and validate new hypotheses about the relationships among aging, working conditions, genetic makeup, stress, metabolic functions, the cardiovascular system, the immune system, and mental performance over the human lifespan with a focus on healthy working adults. The Dortmund Vital Study is a multidisciplinary study involving the Departments of Ergonomics, Immunology, Psychology and Neurosciences, and Toxicology at the Leibniz Research Centre for Working Environment and Human Factors at the Technical University of Dortmund (IfADo) in Germany, as well as several national and international partners.MethodsThe Dortmund Vital Study is designed as a combined cross-sectional and longitudinal study. Approximately 600 healthy subjects aged between 20 and 70 years will participate. A wide range of demographic, psychological, behavioral, sensory, cardiovascular, immunological, and biochemical data, a comprehensive electroencephalography (EEG)-based cognitive test battery as well as structural and functional magnetic resonance imaging (MRI) have been included in the study.ResultsThe study was approved by the Ethics Committee of IfADo in October 2015. The baseline testing was conducted between 2016 and 2021 and will be repeated every 5 years (3 follow-up measures until 2035). As of March 2020 (until the outbreak of the COVID-19 pandemic), 593 participants have been enrolled. Some results from the cross-sectional part of the study were already published, further results will be published soon. Longitudinal data will be analyzed and published by 2025.ConclusionsWe anticipate that the study will shed light on sources of interindividual differences in the alterations of cognitive functioning with increasing age and reveal biological and lifestyle markers contributing to work ability, longevity, and healthy aging on the one hand, and to risk factors for cognitive decline, mild cognitive impairment, or even dementia on the other hand.Trial RegistrationClinicalTrials.gov NCT05155397; https://clinicaltrials.gov/ct2/show/NCT05155397International Registered Report Identifier (IRRID)DERR1-10.2196/32352

Pathogenesis of anti-melanoma differentiation-associated gene-5 (MDA5) dermatomyositis.

Department of Rheumatology and Immunology, The Second Affiliated Hospital of Soochow University, Suzhou, Jiangsu 215000, China. Correspondence to: Zhichun Liu, Department of Rheumatology and Immunology, The Second Affiliated Hospital of Soochow University, Sanxiang Road No.1055, Suzhou, Jiangsu 215000, China E-Mail: [email protected] How to cite this article: Weng C, Liu Z. Pathogenesis of anti-melanoma differentiation-associated gene-5 (MDA5) dermatomyositis. Chin Med J 2022;00:00–00. doi: 10.1097/CM9.0000000000002049 Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (www.cmj.org). Received July 1, 2021 This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0

Treatment of 11 cases of juvenile idiopathic arthritis by intra-articular injection of adalimumab

Objective: To evaluate the efficacy and safety of intra-articular injection of adalimumab (ADA) in the treatment of refractory oligoarticular juvenile idiopathic arthritis (JIA). Methods: This was a retrospective study. Clinical data on age, gender, and symptoms of joint swelling and pain were collected from 11 children with refractory oligoarticular JIA involving only knee joints admitted to Department of Rheumatism and Immunology of Children's Hospital, Capital Institute of Pediatrics from November 2019 to October 2020. The physician and parent-child evaluation of disease activity, the number of active joints, and the level of erythrocyte sedimentation rate (ESR) at different treatment time points were analyzed at every 4-week observation point after drug administration, and the non-parametric Kruskal-Wallis test was used to compare the differences in clinical evaluation indicators and changes in laboratory tests at different treatment times. The follow-up period was 6 months. Results: Among the 11 children, 5 were boys and 6 were girls. The age was 3.0 (2.8) years. All 11 children had symptoms of joint swelling and pain as well as limitation of movement. After 3 intra-articular injections of ADA, the joint symptoms of 11 children were better than before treatment; the joint symptoms of 7 children disappeared completely, and no recurrence occurred during the 6-month follow-up period. At different treatment times, physician and parent-child evaluation of disease activity, a gradual decrease in the number of active joints in the children, ESR, and juvenile arthritis disease activity score with 27 joints were all statistically significant (χ2=53.99, 59.37, 32.87, 40.07, 54.00, all P<0.001).No significant adverse drug reactions were observed in any of the 11 children during treatment and follow-up. Conclusion: Intra-articular injection of ADA in the treatment of refractory oligoarticular JIA has a significant effect in controlling joint symptoms and is relatively safe.

Interleukin-29 profiles in COVID-19 patients: Survival is associated with IL-29 levels.

Interleukin-29 profiles in COVID-19 patients: Survival is associated with IL-29 levels.

Prevalence of Primary Immunodeficiency Diseases and Related Risk Factors Among Children Attending a Hospital in Ahvaz, Iran

Background and Objectives Primary immunodeficiency (PI) diseases are those occur because of a primary disorder in the immune system. Subjects and Methods This retrospective study was conducted on 70 children with PI diseases referred to the allergy and immunology department of Abuzar Children Hospital in Ahvaz, Iran from 2015 to 2018. Results The most common PI diseases in the majority of male children (56.92%) were severe combined immunodeficiency disease (21.42%) and chronic granulomatous disease (8.57%). Parents in most of patients (83%) were blood relatives. About 66.67% of all death cases (9.23%) was due to severe combined immunodeficiency disease; hypogammaglobulinemia and Griscelli syndrome accounted for about 16.67% of deaths. The most common manifestations were pneumonia, fever, diarrhea, and sepsis. Based on the family history, 33 children had allergies, 9 had malignancy, one had autoimmune disease, and 39 had mothers with a history of abortion. Most of children were under treatment with intravenous immunoglobulin and prophylactic antibiotics, (99.88% and 88.99%, respectively), while 2.81% were under interferon-gamma therapy and transplantation, and 1.40% were receiving corticosteroids and granulocyte colony-stimulating factor therapy. Conclusion The severe combined immunodeficiency disease, chronic granulomatous disease, and Bruton disease are the most common PI diseases in children living in Ahvaz.

Comparison of procedure times and collection efficiencies using integrated and multistep nonintegrated procedures for extracorporeal photopheresis.

BackgroundExtracorporeal photopheresis (ECP) is a blood‐based therapeutic procedure increasingly used for modulation of immune dysregulation in various underlying disease settings. The aim of this study was to compare the procedure times and blood collection efficiencies between the two approaches currently utilized in European centers: the integrated versus the multistep nonintegrated procedures.MethodsA retrospective data analysis was conducted, comparing treatment data from patients who received ECP therapy at the Central Institute for Blood Transfusion & Department of Immunology (ZIB) of the Tirol Kliniken GmbH, where the integrated and multistep nonintegrated procedures are routinely used in an approximated setup.ResultsDuring the observation period, a total of 15 patients who were treated with alternating systems on 2 consecutive days were identified. This allowed treatment pair comparisons with minimal interpatient variabilities, similar to a cross‐over design even though analyzed retrospectively. Total average procedure times with the integrated system were 99.3 vs 122.0 minutes with the multistep nonintegrated procedures, respectively. Significant differences were observed for all steps of the ECP procedure: (a) time for buffy coat collection, 66.5 vs 74.7; (b) handling/transfer, 2.8 vs 18.7; (c) irradiation, 20.3 vs 11.7; and (d) reinfusion/handling time, 9.6 vs 16.3 minutes. The calculated collection throughput was 7.79 mL/min for the integrated and 7.84 mL/min for the multistep nonintegrated procedures, and with a white blood cell (WBC) collection efficiency of 34.2% and 21.0%, respectively.ConclusionThe data presented in this study show a significant shorter overall procedure time and higher WBC collection efficiency for the integrated ECP system.