159 results on '"Clinical Immunology"'
Search Results
2. Novel ADA2 Variants in a Romanian Case Series of DADA2.
- Author
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Cochino, Alexis Virgil, Ioan, Andreea, Farkas, Oana Maria, Liu, Meng, and Lee, Pui Y.
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ADENOSINE deaminase , *CLINICAL immunology , *ROMANIANS , *GENETIC mutation , *GENETIC variation - Abstract
This article, published in the Journal of Clinical Immunology, describes a case series of five Romanian patients with Deficiency of adenosine deaminase 2 (DADA2), an autoinflammatory disorder caused by mutations in the ADA2 gene. The patients presented with a range of symptoms including systemic inflammation, vasculitis, immune dysregulation, and hematologic dysfunction. The study identified novel variants in the ADA2 gene and confirmed reduced ADA2 activity in the patients. The authors emphasize the need for early detection of DADA2 to reduce morbidity and mortality, and highlight the heterogeneity of the disease and the importance of personalized treatment strategies. [Extracted from the article]
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- 2023
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3. Soluble Interleukin-2 Receptor/White Blood Cell Ratio Reflects Granulomatous Disease Progression in Common Variable Immune Deficiency.
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van Stigt, Astrid C., Dalm, Virgil A. S. H., Nagtzaam, Nicole M. A., van Hagen, P. Martin, Dik, Willem A., and IJspeert, Hanna
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COMMON variable immunodeficiency , *LEUCOCYTES , *INTERLEUKIN-2 , *DISEASE progression , *CLINICAL immunology - Abstract
This letter, published in the Journal of Clinical Immunology, discusses the challenges in detecting and monitoring granulomatous disease in patients with common variable immune deficiency (CVID). The authors conducted a retrospective cohort study to identify biomarkers that could indicate granuloma progression in CVID. They found that the ratio of soluble interleukin-2 receptor (sIL-2R) to white blood cell (WBC) count was a more accurate and discriminative monitoring tool for detecting and monitoring granulomatous disease and progression in CVID compared to sIL-2R alone or other markers. The authors suggest that this ratio could be easily implemented in routine diagnostics. [Extracted from the article]
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- 2023
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4. Split Immunological Reconstitution in a NEMO-Deficient Male with Incontinentia Pigmenti and Immunodeficiency.
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Sonoda, Motoshi, Ishimura, Masataka, Ogata, Reina, Oda, Hirotsugu, and Ohga, Shouichi
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ECTODERMAL dysplasia , *IMMUNODEFICIENCY , *B cells , *T cells , *CLINICAL immunology - Abstract
This document is a letter published in the Journal of Clinical Immunology. It discusses a case study of a male patient with Incontinentia Pigmenti and X-linked anhidrotic ectodermal dysplasia with immunodeficiency (IP-EDA-ID). The patient had a pathogenic variant in the NEMO/IKBKG gene, which caused split immunological reconstitution. The study examines the chronological changes in somatic mosaicism of T cells, B cells, and monocytes in the patient. The findings suggest that monitoring mosaicism can guide the appropriate treatment for NEMO deficiency. [Extracted from the article]
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- 2023
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5. Correction to: A Novel Case of IFNAR1 Deficiency Identified a Common Canonical Splice Site Variant in DOCK8 in Western Polynesia: The Importance of Validating Variants of Unknown Significance in Under-Represented Ancestries.
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Huynh, Aimee, E Gray, Paul, Sullivan, Anna, Mackie, Joseph, Guerin, Antoine, Rao, Geetha, Pathmanandavel, Karrnan, Della Mina, Erika, Hollway, Georgina, Hobbs, Matthew, Enthoven, Karen, O'Young, Patrick, McManus, Sam, H. Wainwright, Luke, Higgins, Megan, Noon, Fallon, Wong, Melanie, Bastard, Paul, Zhang, Qian, and Casanova, Jean-Laurent
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CLINICAL immunology , *GENEALOGY , *AUTHORS - Abstract
This document is a correction notice for an article titled "A Novel Case of IFNAR1 Deficiency Identified a Common Canonical Splice Site Variant in DOCK8 in Western Polynesia: The Importance of Validating Variants of Unknown Significance in Under-Represented Ancestries" published in the Journal of Clinical Immunology. The correction states that Alberto Pinzon-Charry, Cindy S Ma, and Stuart G. Tangye should have been denoted as equally contributing authors. The original version of the article has been corrected. The publisher, Springer Nature, remains neutral regarding jurisdictional claims and institutional affiliations. The correction notice is reported by various authors. [Extracted from the article]
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- 2024
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6. Knowledge, Attitudes, and Practices of Allergists/Immunologists Regarding Transition of Care for Primary Immunodeficiency Patients.
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Rai, Shipra, Treyster, Zoya, and Jongco III, Artemio M.
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PRIMARY immunodeficiency diseases , *IMMUNOLOGISTS , *ATTITUDE (Psychology) , *CLINICAL immunology , *NEEDS assessment - Abstract
Purpose: Only some allergists/immunologists provide care throughout the lifespan despite their training. Although transition of care (TOC) guidelines exist, research on provider perspectives on TOC for pediatric primary immunodeficiency (PID) patients is lacking. We aimed to characterize knowledge, attitudes, and practices and establish clinician needs using a needs assessment survey. Methods: The 15-min online survey was adapted from an existing rheumatology TOC survey and was emailed to the American Academy of Allergy Asthma and Immunology (AAAAI) and Clinical Immunology Society (CIS) members. Our primary hypothesis was that both AAAAI and CIS providers report being underprepared for TOC and would express interest in TOC resources and consensus. Results: Forty-nine of 1250 eligible AAAAI and 67 of 698 eligible CIS participants completed the survey (4.8% vs 11.3% participation rate). Many (53.1% vs 59.7%) respondents transition their own patients but also retain adult patients (59.2% vs 52.2%). Many accepted transition patients (85.7% vs 92.5%). In total, 24.1% of respondents did not have a TOC policy while 18.9% have an informal policy. Only 25.0% were satisfied with their current practices while 43.9% agreed that a consensus statement would be useful. Conclusion: Despite a small sample size and high rate of unanswered questions, our findings show that TOC remains overlooked in our specialty and that providers want and need additional training and resources. There is a clear need to develop and evaluate the effectiveness of evidence-based TOC guidelines, resources, and best practices for PID patients. [ABSTRACT FROM AUTHOR]
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- 2023
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7. Selected Abstracts from the 13th Annual Meeting of the Clinical Immunology Society: 2022 Annual Meeting: Immune Deficiency and Dysregulation North American Conference.
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CLINICAL immunology , *AUTOIMMUNE diseases , *IMMUNODEFICIENCY , *MEDICAL personnel , *SCIENCE education , *LIFE sciences - Abstract
Thus, we provide a proof of concept that FMT from CVID patients to GF-mice recapitulates the microbiome alteration seen in CVID patients. Figure 1: Plot to show FOXP3/CD4 values in controls (n=26, mean 8.5%, SD 2.1) compared with patients with IPEX (n=46, mean 18.0%, SD 10.6, p<0.0001), patients with overlapping IPEX-like phenotypes due to other monogenic autoimmunity (AI) (n=16, mean 9.6%, SD 4.3, p=0.9) and patients with polygenic multi-AI (n=13, mean 10.1%, SD 2.4, p=0.7). B (4) Gene Expression Profiling and Signaling Pathway Analysis of Tolerance in an IL-7R- SCID Patient Following Bilateral Orthotopic Lung Transplant and Bone Marrow Transplant from the Same Cadaveric Donor b U Paul Szabolcs* u SP 1 sp , Evelyn Garchar SP 1 sp , Dhivyaa Rajasundaram SP 1 sp , Xiaohua Chen SP 1 sp SP 1 sp Children's Hospital of Pittsburgh Rationale: Primary immunodeficiency (PID) patients may develop pulmonary complications, and most are ineligible for either Bilateral Orthotopic Lung Transplant (BOLT) or Bone Marrow Transplant (BMT) due to futility. CVID patients have two distinct phenotypes: patients who develop infections-only (INF), and patients who develop autoimmune and inflammatory complications (noninfectious complications (NIC)). [Extracted from the article]
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- 2022
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8. Selected Abstracts from the 12th Annual Meeting of the Clinical Immunology Society: 2021 Virtual Annual Meeting: Immune Deficiency and Dysregulation North American Conference.
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SEVERE combined immunodeficiency , *AGAMMAGLOBULINEMIA , *CLINICAL immunology , *IMMUNODEFICIENCY , *MEDICAL sciences , *MONONUCLEAR leukocytes , *NUCLEAR factor of activated T-cells - Abstract
The most commonly reported endocrine-specific conditions among patients in the USIDNET Registry are thyroid specific, found in 356 patients (6.5% of registry patients); this is followed by pancreatic (N=105, 1.9%), parathyroid (N=82, 1.5%), ovarian (N=19, 0.3%, where 6 of these patients had undergone stem cell transplant), Addison's (N=16, 0.3%), polyglandular (N=4, 0.1%), and testicular (N=1, 0.02%). Indications included AD (6 patients), asthma (1 patient), AD and asthma (1 patient), and ABPA (2 patients). 25 patients with autoimmune thyroid disease with 80 available immunologic studies, 5 patients with idiopathic thrombocytopenia purpura (ITP) who had 24 immunologic evaluations and 5 patients with JIA with 13 immunologic evaluations were included in the study along with 668 patients with no autoimmune diagnosis who had 1207 immunologic evaluations. Fourteen patients (6.6%) were diagnosed with either SCID or T-cell lymphopenia: 4 patients (1.9%) with SCID, 2 patients (0.9%) with 22q11.2 deletion syndrome, and 8 patients (3.8%) with idiopathic T-cell lymphopenia. Patients were most likely to be sensitive to milk (6 patients), egg (4 patients), peanut (3 patients), and wheat (3 patients). [Extracted from the article]
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- 2021
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9. Enterobacter cloacae, a Rare Cause of Cervical Lymphadenitis in X-Linked Chronic Granulomatous Disease.
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Penner, Cooper, Datta, Rahul, Ebube, Jefferson, and Romberg, Neil
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CHRONIC granulomatous disease , *ENTEROBACTER cloacae , *LYMPHADENITIS , *ANTIBIOTIC prophylaxis , *CLINICAL immunology , *DRUG resistance in bacteria - Abstract
This letter published in the Journal of Clinical Immunology discusses a rare case of cervical lymphadenitis in a 6-year-old boy with X-linked chronic granulomatous disease (CGD). The patient had a history of severe infections and was admitted with a rapidly growing lymphadenitis despite antibiotic prophylaxis. The lymph node was surgically excised and cultured, revealing Enterobacter cloacae, a rare pathogen that is increasingly resistant to antibiotics. The authors suggest that E. cloacae should be considered in CGD patients with new infectious symptoms, and that changes in prophylaxis and treatment may be necessary to address its antibiotic resistance. [Extracted from the article]
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- 2023
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10. Inborn Errors of Immunity and Efforts to Diagnose Affected Children in the Absence of Training and Specialty Practice in Clinical Immunology in Ethiopia: a Brief Report.
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Alemayehu T, Asfaw YM, and Weldetsadik AY
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- Child, Humans, Ethiopia epidemiology, Genetic Diseases, Inborn epidemiology, Genetic Diseases, Inborn immunology, Immune System Diseases epidemiology, Immune System Diseases genetics
- Abstract
Four in five children with inborn errors of immunity globally remain undiagnosed. These figures are disproportionally high in low-income countries like Ethiopia. Apart from the inclusion of basic overviews of these disorders in to postgraduate pediatric curricula, little effort has been placed in to establishing clinical immunology training programs. This report summarizes the existing epidemiology of inborn errors of immunity in Ethiopia, unique presentations in Ethiopian children, challenges faced in diagnosing them, and efforts to improve their management., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
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- 2023
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11. Correction to: Antibody Deficiency in Patients with Biallelic KARS1 Mutations.
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Saettini, Francesco, Guerra, Fabiola, Fazio, Grazia, Bugarin, Cristina, McMillan, Hugh J., Ohtake, Akira, Ardissone, Anna, Itoh, Masayuki, Giglio, Sabrina, Cappuccio, Gerarda, Giardino, Giuliana, Romano, Roberta, Quadri, Manuel, Gasperini, Serena, Moratto, Daniele, Chiarini, Marco, Ishiguro, Akira, Fukuhara, Yasuyuki, Hayakawa, Itaru, and Okazaki, Yasushi
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ACQUISITION of manuscripts , *CLINICAL immunology , *IMMUNOGLOBULINS , *PERSONAL names , *PERIODICAL publishing - Abstract
This document is a correction notice for an article titled "Antibody Deficiency in Patients with Biallelic KARS1 Mutations" published in the Journal of Clinical Immunology. The correction states that during the submission of the manuscript, the first names and surnames of the authors were inverted. The list of authors has been corrected in the document. The original version of the article has been corrected. The publisher, Springer Nature, remains neutral regarding jurisdictional claims and institutional affiliations. The authors of the article are listed as Francesco Saettini, Fabiola Guerra, Grazia Fazio, Cristina Bugarin, Hugh J. McMillan, Akira Ohtake, Anna Ardissone, Masayuki Itoh, Sabrina Giglio, Gerarda Cappuccio, Giuliana Giardino, Roberta Romano, Manuel Quadri, Serena Gasperini, Daniele Moratto, Marco Chiarini, Akira Ishiguro, Yasuyuki Fukuhara, Itaru Hayakawa, Yasushi Okazaki, Mario Mauri, Rocco Piazza, Gianni Cazzaniga, and Andrea Biondi. [Extracted from the article]
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- 2023
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12. Correction to: Functional Relevance of CTLA4 Variants: an Upgraded Approach to Assess CTLA4‑Dependent Transendocytosis by Flow Cytometry.
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Rojas‑Restrepo, Jessica, Sindram, Elena, Zenke, Simon, Haberstroh, Hanna, Mitsuiki, Noriko, Gabrysch, Annemarie, Huebscher, Katrin, Posadas‑Cantera, Sara, Krausz, Máté, Kobbe, Robin, Rohr, Jan C., Grimbacher, Bodo, and Gamez‑Diaz, Laura
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FLOW cytometry , *CLINICAL immunology , *PERIODICAL publishing - Abstract
This document is a correction notice for an article titled "Functional Relevance of CTLA4 Variants: an Upgraded Approach to Assess CTLA4-Dependent Transendocytosis by Flow Cytometry" published in the Journal of Clinical Immunology. The correction addresses a typesetting mistake in Figure 2b, where there were overlapping values in the first histogram. The original version of the article has been corrected. The publisher, Springer Nature, maintains a neutral stance on jurisdictional claims and institutional affiliations. The authors of the article are Jessica Rojas-Restrepo, Elena Sindram, Simon Zenke, Hanna Haberstroh, Noriko Mitsuiki, Annemarie Gabrysch, Katrin Huebscher, Sara Posadas-Cantera, Máté Krausz, Robin Kobbe, Jan C. Rohr, Bodo Grimbacher, and Laura Gamez-Diaz. [Extracted from the article]
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- 2023
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13. Correction to: Immunologic and Genetic Contributors to CD46‑Dependent Immune Dysregulation.
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Meyer, Benedikt J, Kunz, Natalia, Seki, Sayuri, Higgins, Rebecca, Ghosh, Adhideb, Hupfer, Robin, Baldrich, Adrian, Hirsiger, Julia R, Jauch, Annaïse J, Burgener, Anne-Valerie, Lötscher, Jonas, Aschwanden, Markus, Dickenmann, Michael, Stegert, Mihaela, Berger, Christoph T, Daikeler, Thomas, Heijnen, Ingmar, Navarini, Alexander A, Rudin, Christoph, and Yamamoto, Hiroyuki
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CLINICAL immunology , *RESEARCH personnel , *PERIODICAL publishing , *PUBLISHING - Abstract
This document is a correction notice for an article titled "Immunologic and Genetic Contributors to CD46-Dependent Immune Dysregulation" published in the Journal of Clinical Immunology. The correction addresses a typesetting mistake in Figure 2B, where the labels above the panel had duplicate data. The original version of the article has been corrected. The publisher, Springer Nature, maintains a neutral stance on jurisdictional claims and institutional affiliations. The article was authored by a group of researchers including Benedikt J Meyer, Natalia Kunz, Sayuri Seki, and others. [Extracted from the article]
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- 2023
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14. Past, Present, and Future of The Journal of Clinical Immunology, the International Journal of Inborn Errors of Immunity.
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Bonagura, Vincent R. and Casanova, Jean-Laurent
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CLINICAL immunology , *HEALING , *IMMUNITY , *HEMATOPOIETIC stem cell transplantation , *LYMPHOPENIA , *MYELOFIBROSIS - Published
- 2020
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15. 2019 Clinical Immunology Society Compensation Survey.
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Williams, Kelli W., Derby, Morgan, and Lawrence, Monica G.
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CLINICAL immunology , *WAGES - Abstract
Therefore, to provide clinical immunologists with a guide for informed salary discussions, in 2019 the Clinical Immunology Society (CIS) surveyed its membership to capture compensation data from members working in a variety of practice settings. The median annual salary for an immunologist 3 years or less from fellowship was $153,700 whereas 4-7 years out made on average $160,000, and those 8+ years out of fellowship made a median salary of $185,000. While the value of a skilled clinical immunologist may be clear to patients, their families, and the other subspecialists with whom immunologists work closely to care for medically complex patients, it may be less clear to business managers closely tracking wRVU. [Extracted from the article]
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- 2020
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16. Prescribing Immunoglobulin Replacement Therapy for Patients with Non-classical and Secondary Antibody Deficiency: an Analysis of the Practice of Clinical Immunologists in the UK and Republic of Ireland.
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Edgar, John David M., Richter, Alex G., Huissoon, Aarnoud P., Kumararatne, Dinakantha S., Baxendale, Helen E., Bethune, Claire A., Garcez, Tomaz, Misbah, Siraj A., Sorensen, Ricardo U., and United Kingdom Primary Immunodeficiency Network (UKPIN) Immunoglobulin Decision to Treat Study Group
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IMMUNOLOGICAL deficiency syndrome treatment , *THERAPEUTIC use of immunoglobulins , *CLINICAL immunology , *MEDICAL practice , *BRONCHIECTASIS - Abstract
Background: Immunologists are increasingly being asked to assess patients with non-classical and secondary antibody deficiency to determine their potential need for immunoglobulin replacement therapy (IGRT). Immunoglobulin is a limited, expensive resource and no clear guidance exists for this broad patient group. The purpose of this survey is to establish what factors influence the decision to commence IGRT in adult patients, when diagnostic criteria for primary antibody deficiency are not fulfilled.Methods: Under the auspices of the United Kingdom Primary Immunodeficiency Network (UKPIN), a study group was established which circulated an online questionnaire to the consultant body across the UK and Ireland. Results provided a snapshot of the current clinical practice of 71% of consultant immunologists, from 30 centers.Results: In order of importance, factors which influence the decision to commence IGRT include number of hospital admissions with infection, serum IgG level, bronchiectasis, radiologically proven pneumonia, number of positive sputum cultures, number of antibiotic courses, and results of immunization studies. The commonest test vaccine used was Pneumovax 23 with measurement of serotype-specific responses at 4 weeks, with a threshold of 0.35 μg/ml in 2/3 of serotypes measured. Eighty-six percent of patients are treated with a trial of prophylactic antibiotics prior to consideration of IGRT. Efficacy of IGRT trial is assessed at between 6 and 12 months.Conclusions: There was consistency in clinical practice using a combination of clinical history, evidence of infections, and vaccination testing for diagnosis. However, there was some variation in the implementation of this practice, particularly in vaccine choice and assessment of response to vaccination. [ABSTRACT FROM AUTHOR]
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- 2018
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17. Targeted Sequencing and Immunological Analysis Reveal the Involvement of Primary Immunodeficiency Genes in Pediatric IBD: a Japanese Multicenter Study.
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Suzuki, Tasuku, Sasahara, Yoji, Kikuchi, Atsuo, Kakuta, Humihiko, Kashiwabara, Toshihiko, Ishige, Takashi, Nakayama, Yoshiko, Tanaka, Masanori, Hoshino, Akihiro, Kanegane, Hirokazu, Abukawa, Daiki, and Kure, Shigeo
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INFLAMMATORY bowel diseases , *IMMUNODEFICIENCY , *CLINICAL immunology , *JUVENILE diseases , *JAPANESE people , *EXOMES , *GENETICS , *DISEASES - Abstract
Purpose: Pediatric inflammatory bowel disease (IBD) is a heterogeneous disorder caused by multiple factors. Although genetic and immunological analyses are required for a definitive diagnosis, no reports of a comprehensive genetic study of a Japanese population are available. Methods: In total, 35 Japanese patients <16 years of age suffering from IBD, including 27 patients aged <6 years with very early-onset IBD, were enrolled in this multicenter study. Exome and targeted gene panel sequencing was performed for all patients. Mutations in genes responsible for primary immunodeficiency diseases (PID) and clinical and immunological parameters were evaluated according to disease type. Results: We identified monogenic mutations in 5 of the 35 patients (14.3 %). We identified compound heterozygous and homozygous splice-site mutations in interleukin-10 receptor A ( IL- 10RA) in two patients, nonsense mutations in X-linked inhibitor of apoptosis protein ( XIAP) in two patients, and a missense mutation in cytochrome b beta chain in one patient. Using assays for protein expression levels, IL-10 signaling, and cytokine production, we confirmed that the mutations resulted in loss of function. For each patient, genotype was significantly associated with clinical findings. We successfully treated a patient with a XIAP mutation by allogeneic cord blood hematopoietic stem cell transplantation, and his symptoms were ameliorated completely. Conclusions: Targeted sequencing and immunological analysis are useful for screening monogenic disorders and selecting curative therapies in pediatric patients with IBD. The genes responsible for PID are frequently involved in pediatric IBD and play critical roles in normal immune homeostasis in the gastrointestinal tract. [ABSTRACT FROM AUTHOR]
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- 2017
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18. Correction to: De Novo Somatic Mosaicism of CYBB Caused by Intronic LINE‑1 Element Insertion Resulting in Chronic Granulomatous Disease.
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Yu, Lang, Li, Wenhui, Lv, Ge, Sun, Gan, Yang, Lu, Chen, Junjie, Zhou, Lina, Ding, Yuan, Zhang, Zhiyong, Tang, Xuemei, An, Yunfei, and Zhao, Xiaodong
- Subjects
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CHRONIC granulomatous disease , *MOSAICISM , *CLINICAL immunology - Published
- 2023
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19. Neurologic Complications of Common Variable Immunodeficiency.
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Nguyen, Jenna, Green, Ari, Wilson, Michael, DeRisi, Joseph, and Gundling, Katherine
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IMMUNODEFICIENCY , *AUTOIMMUNITY , *NEUROLOGICAL disorders , *CANCER relapse , *CLINICAL immunology , *PATIENTS - Abstract
Common variable immunodeficiency is a rare disorder of immunity associated with a myriad of clinical manifestations including recurrent infections, autoimmunity, and malignancy. Though rare, neurologic complications have been described in a small number of case reports and case series of CVID patients. In this article, we present a patient with CVID who suffered significant neurologic morbidity and categorize the reported range of neurologic complications associated with CVID. Our case highlights the complex nature of neurologic manifestations in CVID patients, and our review of the current database suggests that infection and inflammatory neurologic disorders are the cause of most neurologic presentations. [ABSTRACT FROM AUTHOR]
- Published
- 2016
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20. Life with a Primary Immune Deficiency: a Systematic Synthesis of the Literature and Proposed Research Agenda.
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Similuk, Morgan, Wang, Angela, Lenardo, Michael, and Erby, Lori
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IMMUNODEFICIENCY , *CLINICAL immunology , *PSYCHOSOCIAL development theory , *PSYCHOLOGY of the sick , *STAKEHOLDER analysis - Abstract
Purpose: The clinical immunology literature is punctuated with research on psychosocial dimensions of illness. Studies investigating the lived experiences and stated needs of patients with primary immune deficiencies and their families are essential to improving clinical management and determining the research questions that matter to patients and other stakeholders. Yet, to move the field forward, a systematic review of literature and proposed agenda is needed. Methods: A systematic review was conducted via PubMed and Scopus to include original research on psychological, social, or behavioral aspects of primary immune deficiencies published between 1999 and 2015. A Title/Abstract keyword search was conducted, 317 candidate article abstracts were manually reviewed, and forward/backward reference searches were completed. Results: Twenty-nine studies met inclusion criteria. These illuminate the complex psychological, social, and emotional experiences of primary immune deficiency. Themes included the potential for negative psychosocial impact from disease; adaptation over time; the multi-dimensional assessments of quality of life; familial impact; the important roles of hope, developing a sense of control, social support; and addressing anxiety/depression in our patients and their families. Methodological considerations and areas for improvement are discussed. Conclusion: We propose the research agenda focus on study creativity and rigor, with improved engagement with existing literature and critical study design (e.g., methodology with adequate statistical power, careful variable selection, etc.). This review highlights opportunities to advance psychosocial research and bring a brighter future to clinicians, researchers, and families affected by primary immune deficiency. [ABSTRACT FROM AUTHOR]
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- 2016
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21. Past, Present, and Future of The Journal of Clinical Immunology, the International Journal of Inborn Errors of Immunity
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Vincent R. Bonagura and Jean-Laurent Casanova
- Subjects
medicine.medical_specialty ,Clinical immunology ,business.industry ,Immunity ,Immunology ,medicine ,MEDLINE ,Immunology and Allergy ,Medical physics ,business - Published
- 2020
22. Correction to: Peeling Skin Syndrome Type 1: Dupilumab Reduces IgE, But Not Skin Anomalies.
- Author
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Barranca, Alexis, Jonca, Nathalie, Martin-Blondel, Audrey, Apoil, Pol-André, and Mazereeuw-Hautier, Juliette
- Subjects
- *
DUPILUMAB , *CLINICAL immunology , *PERSONAL names , *SYNDROMES - Abstract
Publisher's Note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Correction to: Peeling Skin Syndrome Type 1: Dupilumab Reduces IgE, But Not Skin Anomalies B Correction to b B : b B Journal of Clinical Immunology (2022) 42:873-875 b https://doi.org/10.1007/s10875-021-01194-1 This addendum is to clarify the names (first names and family names) of the authors as shown above. [Extracted from the article]
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- 2022
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23. Correction to: Malignancy in STAT3 Deficient Hyper IgE Syndrome.
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Urban, Amanda, Pittaluga, Stefania, and Freeman, Alexandra F.
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STAT proteins , *CLINICAL immunology , *SYNDROMES , *CONSORTIA , *PHYSICIANS - Published
- 2022
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24. Clinical Heterogeneity of Immunodysregulation, Polyendocrinopathy, Enteropathy, X-linked: Pulmonary Involvement as a Non-Classical Disease Manifestation.
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Baris, Safa, Schulze, Ilka, Ozen, Ahmet, Aydiner, Elif, Altuncu, Emel, Karasu, Gulsun, Ozturk, Nilufer, Lorenz, Myriam, Schwarz, Klaus, Vraetz, Thomas, Ehl, Stephan, and Barlan, Isil
- Subjects
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CLINICAL immunology , *HETEROGENEITY , *INTESTINAL diseases , *X-linked genetic disorders , *AUTOIMMUNITY , *EARLY death , *AUTOANTIBODIES - Abstract
Purpose: IPEX (Immunodysregulation, Polyendocrinopathy, Enteropathy, X-linked) is a rare X-linked recessive life-threatening disorder characterized by autoimmunity and early death. Pulmonary complication related with IPEX has not been elucidated exactly. Here, we report 4 IPEX patients, 3 of which died from severe pulmonary disease. Methods: Clinical data and laboratory findings including autoantibodies, immunoglobulin levels as well as number of T, B and NK cells were evaluated. FOXP3 expression and T reg activity were analyzed. The FOXP3 gene was sequenced and RNA analysis was performed. Results: Patient I (PI) presented with nephrotic syndrome at 3 years of age and then developed autoimmune hepatitis without eczema, enteropathy or high IgE and died at 9 years of age due to acute respiratory distress syndrome (ARDS). Two cousins of PI had the same hypomorphic splice site mutation leading to a deletion of 27 amino acids, but normal FOXP3 protein expression and normal suppressive capacity of T reg in a proliferation inhibition assay. However, they exhibited typical symptoms such as eczema, diabetes and enteropathy with eosinophilia at early age (PII, PIII) and were transplanted in infancy. One of them had severe respiratory distress right after birth (PIII). Patient IV from another family presented with chronic diarrhea without autoimmune manifestations and died due to ARDS. Conclusion: Lung disease related to IPEX syndrome has not been reported before and this entity could be a critical factor in disease outcome. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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25. Novel NFKB2 Mutation in Early-Onset CVID.
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Liu, Yiwen, Hanson, Steven, Gurugama, Padmalal, Jones, Alison, Clark, Barnaby, and Ibrahim, Mohammad
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IMMUNODEFICIENCY , *CLINICAL immunology , *NUCLEOTIDE sequence , *JUVENILE diseases , *DELETION mutation , *FRAMESHIFT mutation , *MUTANT proteins , *B cell differentiation , *DIAGNOSIS - Abstract
Common variable immunodeficiency (CVID) is heterogeneous, clinically, immunologically and genetically. The majority of genetic mechanisms leading to CVID remain elusive. We studied a Greek Cypriot family of non-consanguineous parents. Two children were diagnosed with CVID at an early age. Whole exome sequencing revealed 8bp deletion in the C-terminal part of NFKB2 gene associated with disease. The mutation leads to a frameshift (p.Asp865Valfs*17) altering 17 C-terminal amino acids from residue 865, and creating a premature stop-codon resulting in a truncated protein, 19 amino acids shorter than wild type (p100Δ19). We validated the results with Dye-termination sequencing and Western blot, and confirmed that the conserved residue at 866 is mutated from serine to arginine in p100Δ19, leaving the mutant protein unphosphorylated at this critical regulatory position. Consequently, NFKB2/p100 processing and nuclear translocation were abrogated. Using flow cytometry, we further demonstrated that there was a reduction in B cells (CD19+), switched memory B cells (CD27+IgD−) and T follicular helper (Tfh) cells (both CD4+CXCR5+ and CD4+CXCR5Hi) in a CVID patient with NFKB2/p100Δ19, compared to healthy controls. These data support the notion that the non-canonical NFκB pathway plays an important role in B cell differentiation and the development of Tfh cells, and may pave the way for better understanding of the pathology of CVID. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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26. Selected Abstracts from the 100th J Project Meeting, Antalya, Turkey, March 12-14, 2014.
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CLINICAL immunology , *IMMUNOGLOBULINS , *GENETIC recombination , *PUBLICATIONS , *PUBLISHING , *PUBLISHED articles - Published
- 2014
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27. Immunologist’s Perspectives on Assessment and Management of Lung Disease in CVID: a Survey of the Membership of the Clinical Immunology Society and the European Society for Immunodeficiencies
- Author
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Cheryl A. Lefaiver, Javeed Akhter, Christopher Scalchunes, Michael DiGirolamo, and Klaus Warnatz
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Lung Diseases ,medicine.medical_specialty ,Clinical immunology ,Immunology ,Treatment outcome ,03 medical and health sciences ,0302 clinical medicine ,X ray computed ,medicine ,Humans ,Immunology and Allergy ,030212 general & internal medicine ,Practice Patterns, Physicians' ,Disease management (health) ,Internet ,Practice patterns ,business.industry ,Disease Management ,Combined Modality Therapy ,Respiratory Function Tests ,Common Variable Immunodeficiency ,Treatment Outcome ,030228 respiratory system ,Lung disease ,Health Care Surveys ,Family medicine ,Tomography, X-Ray Computed ,business - Published
- 2018
28. 2019 Clinical Immunology Society Compensation Survey
- Author
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Monica G. Lawrence, Morgan Derby, and Kelli W. Williams
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medicine.medical_specialty ,Clinical immunology ,business.industry ,Compensation (psychology) ,Immunology ,MEDLINE ,Allergy and Immunology ,Surveys and Questionnaires ,Humans ,Workers' Compensation ,Immunology and Allergy ,Medicine ,Medical physics ,business - Published
- 2019
29. The Relationship Between Hypogammaglobulinemia, Monoclonal Gammopathy of Undetermined Significance and Humoral Immunodeficiency: a Case Series.
- Author
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Zemble, Robert, Takach, Patricia, and Levinson, Arnold
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AGAMMAGLOBULINEMIA , *MONOCLONAL gammopathies , *IMMUNOGLOBULIN producing cells , *IMMUNODEFICIENCY , *CLINICAL immunology , *BACTERIAL diseases , *MONOCLONAL antibodies - Abstract
Hypogammaglobulinemia of the non-monoclonal immunoglobulin heavy chain classes has been reported in monoclonal gammopathy of undetermined significance (MGUS) patients. Whether low polyclonal immunoglobulin levels are associated with impaired specific antibody production and whether they represent a risk factor for the development of recurrent bacterial infections have not been established in this population. We determined the frequency of MGUS in patients referred to a tertiary care clinical immunology ambulatory care practice for evaluation of hypogammaglobulinemia, who were assessed for deficits in specific antibody production and the presence of recurrent infections. Of the 133 patients evaluated for hypogammaglobulinemia, 68 were screened for monoclonal gammopathy and 5 were found to have MGUS. Three had MGUS associated hypogammaglobulinemia in the absence of a defining primary immunodeficiency, one possibly had common variable immunodeficiency, and one had an uncertain diagnosis. Thus, MGUS may be uncovered in patients presenting with hypogammaglobulinemia even in those who lack an elevated serum level of IgG, IgM, or IgA. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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30. The Antiasthma Effect of Neonatal BCG Vaccination Does Not Depend on the Th17/Th1 but IL-17/IFN-γ Balance in a BALB/c Mouse Asthma Model.
- Author
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Deng, Yu, Chen, Weichao, Zang, Na, Li, Siming, Luo, Yan, Ni, Ke, Wang, Lijia, Xie, Xiaohong, Liu, Wei, Yang, Xiqiang, Fu, Zhou, and Liu, Enmei
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BCG vaccines , *ASTHMA , *ALLERGENS , *BRONCHOALVEOLAR lavage , *INFLAMMATION , *CLINICAL immunology - Abstract
Objective: This study aimed to determine whether the protective effects of the Mycobacterium bovis Bacillus Calmette-Guérin (BCG) vaccination on allergic asthma are associated with the T helper (Th) 17/Th1 balance in a murine asthma model. Methods: BALB/c neonates were vaccinated with BCG on the first day after birth, sensitized with ovalbumin, and then challenged with allergen. The resulting airway inflammation and responsiveness were measured. The levels of IL-17 and interferon (IFN)-γ in BALF and ratio of Th17/Th1 were investigated. Results: We found that although BCG neonatal vaccination inhibited airway hyperresponsiveness and inflammation following allergen challenge in a BALB/c mouse asthma model, reduced levels of Th2 cytokines were not observed. However, BCG neonatal vaccination reduced IL-17 production and increased IFN-γ production in both the bronchoalveolar lavage fluid and the lung lymphocytes in asthmatic mice. Conclusion: The antiasthma effects of neonatal BCG vaccination reversed the IL-17/IFN-γ imbalance in a murine asthma model but did not depend on modifying the Th17/Th1 balance. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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31. Role of Thymic Stromal Lymphopoietin (TSLP) in Palifermin-Mediated Immune Modulation and Protection from Acute Murine Graft-Versus-Host Disease.
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Ellison, Cynthia, Lissitsyn, Yuriy, Packiasamy, Juliet, Leonard, Warren, and Gartner, John
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PROTEINS , *CYTOKINES , *IMMUNOREGULATION , *SKIN diseases , *LABORATORY mice , *GROWTH factors , *CLINICAL immunology - Abstract
Using the C57BL/6→(C57BL/6 x DBA/2)F-hybrid model of acute graft-versus-host disease (GVHD), we previously showed that treating the donor mice with palifermin provides protection from morbidity and a shift from Th1 to Th2 cytokine production. To determine whether thymic stromal lymphopoietin (TSLP) is involved in palifermin-mediated immune modulation, we used donors from the following groups: (1) untreated wild-type donors, (2) palifermin-treated wild-type donors, (3) untreated TSLPR donors, and (4) palifermin-treated TSLPR donors. Survival in the recipients was 0%, 100%, 31%, and 0%, for groups 1-4, respectively, indicating that TSLP responsiveness is required for palifermin-mediated protection from GVHD. We also found that the increases in Th2 cytokine levels that are induced by palifermin treatment are obviated in TSLPR donors, and that protection from GVHD (group 2) is associated with a higher percentage of CD4CD25Foxp3 cells in the graft. Collectively, our findings show that when palifermin and TSLP act in concert, the predominant effect is protection in this model. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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32. Efficacy, Safety, and Pharmacokinetics of a 10% Liquid Immune Globulin Preparation (GAMMAGARD LIQUID, 10%) Administered Subcutaneously in Subjects with Primary Immunodeficiency Disease.
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Wasserman, Richard, Melamed, Isaac, Kobrynski, Lisa, Strausbaugh, Steven, Stein, Mark, Sharkhawy, Marlies, Engl, Werner, Leibl, Heinz, Sobolevsky, Luba, Gelmont, David, Schiff, Richard, and Grossman, William
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PHARMACOKINETICS , *IMMUNODEFICIENCY , *IMMUNOGLOBULIN G , *BACTERIAL diseases , *SUBCUTANEOUS surgery , *INTRAVENOUS therapy , *CLINICAL immunology - Abstract
multi-center, prospective, open-label study was conducted in primary immunodeficiency disease patients to determine the tolerability and pharmacokinetics of a 10% liquid IgG preparation administered subcutaneously. Forty-nine subjects (3-77 years old) were enrolled. Pharmacokinetic equivalence of subcutaneous treatment was achieved at a median dose of 137% of the intravenous dose, with a mean trough IgG level of 1,202 mg/dL at the end of the assessment period. The overall infection rate during subcutaneous treatment was 4.1 per subject-year. Three acute serious bacterial infections were reported, resulting in a rate of 0.067 per subject-year. A low overall rate of temporally associated adverse events (8%), and a very low rate of infusion site adverse events (2.8%), was seen at volumes up to 30 mL/site and rates ≤30 mL/h/site. Thus, subcutaneous replacement therapy with a 10% IgG preparation proved effective, safe and well-tolerated in our study population of subjects with primary immunodeficiency disease. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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33. CXCL10 and CXCL13 Expression were Highly Up-regulated in Peripheral Blood Mononuclear Cells in Acute Rejection and Poor Response to Anti-Rejection Therapy.
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Mao, Youying, Wang, Minmin, Zhou, Qin, Jin, Juan, Wang, Yucheng, Peng, Wenhan, Wu, Jianyong, Shou, Zhangfei, and Chen, Jianghua
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CHEMOKINES , *GENE expression , *GRAFT rejection , *HOMOGRAFTS , *CREATININE , *BLOOD cells , *CLINICAL immunology - Abstract
Background: Acute rejection is still one of the main complications which enhances the cost and the risk to renal graft failure. Chemokines, interacting with respective receptors, can recruit leukocytes into grafts and mediate allograft rejection. In this study, we aimed to analyze gene expression of chemokines including CCL5/RANTES, CXCL10/IP-10, CXCL13/BCA-1, and receptors of CCR5, CXCR3, CXCR5 in peripheral blood mononuclear cells (PBMCs) during acute renal allograft rejection Methods: Gene expression of all these chemokines and receptors in PBMCs were analyzed by real-time PCR from 14 stable recipients, 32 biopsy-proven acute rejection (AR), and 5 acute tubular necrosis (ATN). Results: Gene expression of CCL5, CXCL10, CXCL13, and CCR5 were up-regulated both in AR and ATN group compared to stable recipients (fold change > 2, P < 0.05). Serum creatinine recovered to baseline level after anti-rejection therapy was defined as AR-sensitive and creatinine maintained above 200 μmol/L as AR-resistant. Expression of CXCL10 and CXCL13 were 5.98-, 2.94-, and 20.5, 10.8-fold change in AR-resistant and AR-sensitive compared to stable recipients, respectively. The expression of CXCL10 and CXCL13 was a twofold change in AR-resistant compared to AR-sensitive recipients ( P < 0.05). Five out of ten AR-resistant recipients lost graft function in the follow-up. Conclusion: CXCL10 and CXCL13 expression were highly up-regulated in PBMCs in acute renal allograft rejection, especially in poor response to anti-rejection therapy and detrimental prognosis. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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34. Expression and Functional Role of HLA-G in Immune Cells from Patients with Systemic Lupus Erythematosus.
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Monsiváis-Urenda, Adriana, Baranda, Lourdes, Alvarez-Quiroga, Crisol, Abud-Mendoza, Carlos, and González-Amaro, Roberto
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HLA histocompatibility antigens , *AUTOIMMUNE diseases , *IMMUNE response , *DENDRITIC cells , *FLOW cytometry , *LYMPHOCYTES , *CLINICAL immunology - Abstract
Human leukocyte antigen (HLA)-G is a class I non-classical HLA molecule with an important regulatory role on the immune response. The possible role of this molecule in the pathogenesis of SLE has not been explored. In this work, we evaluated the expression and function of HLA-G in SLE patients. We studied 37 SLE patients as well as 25 healthy donors. Peripheral blood monocytes and in vitro-generated dendritic cells (DCs) were analyzed for HLA-G expression by flow cytometry. We found that monocytes from SLE patients as well as mature CD83+ DCs showed a diminished expression of HLA-G compared with healthy controls. In addition, monocytes from SLE patients showed a diminished induction of HLA-G expression in response to stimulation with IL-10. Furthermore, functional assays showed that these monocytes pre-treated with IFN-γ exhibited a diminished capability to inhibit the proliferation of autologous lymphocytes. Finally, lymphocytes from SLE patients tended to display a lower acquisition of HLA-G (by trogocytosis) from autologous monocytes compared to controls. Our results might have implications for the immune abnormalities observed in patients with SLE. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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35. Neural System Antigens Are Recognized by Autoantibodies from Patients Affected by a New Variant of Endemic Pemphigus Foliaceus in Colombia.
- Author
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Abreu-Velez, Ana, Howard, Michael, Yi, Hong, Gao, Weiqing, Hashimoto, Takashi, and Grossniklaus, Hans
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NERVOUS system , *ANTIGENS , *AUTOIMMUNE diseases , *SKIN diseases , *BIOMARKERS , *OPTIC nerve , *CLINICAL immunology - Abstract
Background: Endemic pemphigus foliaceus (EPF), is also known as 'fogo selvagem' or 'wild fire,' reflecting the intense burning sensation of the skin reported by patients with this disease. Based on this finding, we tested for neural autoreactivity in patients affected by a new variant of EPF (El Bagre-EPF). Methods: We tested 20 El Bagre-EPF patients, 20 normal controls from the endemic area, and 20 age- and sex-matched normal controls from outside the endemic area. We tested for autoreactivity to several immunoglobulins and complement. Both human skin and bovine tail were used as antigens. Results: We detected autoreactivity to neural structures, mechanoreceptors, nerves, perineural cell layers of the arachnoid envelope around the optic nerve, brain structures, and to neuromuscular spindles; these structures colocalized with several neural markers. The patient antibodies also colocalized with desmoplakins 1 and 2, with the armadillo repeat protein deleted in velo-cardio-facial syndrome and with p0071 antibodies. Autoreactivity was also found associated with neurovascular bundles innervating the skin, and immunoelectron microscopy using protein A gold against patient antibodies was positive against the nerve axons. Paucicellularity of the intraepidermal nerve endings and defragmentation of the neural plexus were seen in 70% of the cases and not in the controls from the endemic area ( p < 0.005). Neuropsychological and/or behavioral symptoms were detected in individuals from the endemic area, including sensorimotor axonal neuropathy. Conclusions: Our findings may explain for the first time the 'pose of pemphigus,' representing a dorsiflexural posture seen in EPF patients vis-a-vis the weakness of the extensor nerves, and furthermore, the autoreactivity to nerves in EPF could explain the 'burning sensation' encountered in EPF disease. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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36. H5N1 Influenza Vaccine Formulated with AS03 Induces Strong Cross-Reactive and Polyfunctional CD4 T-Cell Responses.
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Moris, Philippe, Most, Robbert, Leroux-Roels, Isabel, Clement, Frédéric, Dramé, Mamadou, Hanon, Emmanuel, Leroux-Roels, Geert, and Mechelen, Marcelle
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H5N1 Influenza , *VACCINES , *IMMUNE response , *HEMAGGLUTININ , *B cells , *PANDEMICS , *CLINICAL immunology - Abstract
Objective: Adjuvantation of an H5N1 split-virion influenza vaccine with AS03 substantially reduces the antigen dose required to produce a putatively protective humoral response and promotes cross-clade neutralizing responses. We determined the effect of adjuvantation on antibody persistence and B- and T-cell-mediated immune responses. Methods: Two vaccinations with a split-virion A/Vietnam/1194/2004 (H5N1, clade 1) vaccine containing 3.75-30 μg hemagglutinin and formulated with or without adjuvant were administered to groups of 50 volunteers aged 18-60 years. Results: Adjuvantation of the vaccine led to better persistence of neutralizing and hemagglutination-inhibiting antibodies and higher frequencies of antigen-specific memory B cells. Cross-reactive and polyfunctional H5N1-specific CD4 T cells were detected at baseline and were amplified by vaccination. Expansion of CD4 T cells was enhanced by adjuvantation. Conclusion: Formulation of the H5N1 vaccine with AS03 enhances antibody persistence and induces stronger T- and B-cell responses. The cross-clade T-cell immunity indicates that the adjuvanted vaccine primes individuals to respond to either infection and/or subsequent vaccination with strains drifted from the primary vaccine strain. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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37. Clinical and Host Genetic Characteristics of Mendelian Susceptibility to Mycobacterial Diseases in Japan.
- Author
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Hoshina, Takayuki, Takada, Hidetoshi, Sasaki-Mihara, Yuka, Kusuhara, Koichi, Ohshima, Koichi, Okada, Satoshi, Kobayashi, Masao, Ohara, Osamu, and Hara, Toshiro
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DISEASE susceptibility , *MYCOBACTERIAL diseases , *GENETIC mutation , *FLOW cytometry , *DISEASE progression , *CLINICAL immunology - Abstract
Purpose: The aim of this study is to investigate clinical characteristics and genetic backgrounds of Mendelian susceptibility to mycobacterial diseases (MSMD) in Japan. Methods: Forty-six patients diagnosed as having MSMD were enrolled in this study. All patients were analyzed for the IFNGR1, IFNGR2, IL12B, IL12RB1, STAT1, and NEMO gene mutations known to be associated with MSMD. Results: Six patients and one patient were diagnosed as having partial interferon-γ receptor 1 deficiency and nuclear factor-κB-essential modulator deficiency, respectively. Six of the seven patients had recurrent disseminated mycobacterial infections, while 93% of the patients without these mutations had only one episode of infection. Conclusions: The patients with a genetic mutation were more susceptible to developing recurrent disseminated mycobacterial infections. Recurrent disseminated mycobacterial infections occurred in a small number of patients even without these mutations, suggesting the presence of as yet undetermined genetic factors underlying the development and progression of this disease. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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38. Immunomodulatory Properties of Borage ( Echium amoenum) on BALB/c Mice Infected with Leishmania major.
- Author
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Hosseini, Nahid and Abolhassani, Mohsen
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IMMUNOREGULATION , *LABORATORY mice , *LEISHMANIA , *DISEASE susceptibility , *CLINICAL immunology , *PARASITES , *CYTOKINES - Abstract
Leishmaniasis is caused by parasitic protozoa transmitted by the bite of a female sand fly and is currently endemic in 88 countries. BALB/c mice are highly susceptible to the infection with the parasite Leishmania major, and this susceptibility has been attributed, in part, to the expansion of Th2 cells, production of their cytokines, and downregulation of Th1 cytokine, interferon gamma (IFN-γ). In this report, we used both aqueous and alcoholic extracts of Iranian borage ( Echium amoenum Fisch & C.A. Mey) for treatment of L. major infection in BALB/c mice. We found that both extracts had immunomodulatory properties and increased the level of IFN-γ and lowered the parasite burden in the proximal lymph nodes and prevented the necrosis of the footpad as compared with the untreated infected mice. These results may provide a basis for further studies directed toward the use of the Iranian borage against L. major infection. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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39. Leptin Activates Human B Cells to Secrete TNF-α, IL-6, and IL-10 via JAK2/STAT3 and p38MAPK/ERK1/2 Signaling Pathway.
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Agrawal, Sudhanshu, Gollapudi, Sastry, Su, Houfen, and Gupta, Sudhir
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LEPTIN , *B cells , *CYTOKINES , *MITOGEN-activated protein kinases , *BIOMARKERS , *IMMUNOREGULATION , *CLINICAL immunology - Abstract
Leptin, one of the adipokines, functions as a hormone and a cytokine. In this investigation, we show for the first time that leptin, in a concentration-dependent manner, activates human peripheral blood B cells to induce secretion of IL-6, IL-10, and TNF-α. Leptin increased B cells expressing CD25 and HLA-DR. Leptin induces phosphorylation of Janus activation kinase 2 (JAK2), signal transducer and activator of transcription 3 (STAT3), p38 mitogen-activated protein kinase (p38MAPK), and extracellular signal-regulated kinase (ERK1/2). Furthermore, leptin-induced cytokine secretion by B cells was blocked by inhibitors of JAK2, STAT3, p38MAPK, and ERK1/2. These data demonstrate that leptin activates human B cells to secrete cytokines via activation of JAK2/STAT3 and p38MAPK/ERK1/2 signaling pathways, which may contribute to its inflammatory and immunoregulatory properties. [ABSTRACT FROM AUTHOR]
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- 2011
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40. Circulating Tregs Correlate with Viral Load Reduction in Chronic HBV-Treated Patients with Tenofovir Disoproxil Fumarate.
- Author
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TrehanPati, Nirupma, Kotillil, Shyam, Hissar, Syed, Shrivastava, Shikha, Khanam, Arshi, Sukriti, Sukriti, Mishra, Siddartha, and Sarin, Shiv
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VIRAL load , *HEPATITIS B virus , *TENOFOVIR , *CHRONIC diseases , *IMMUNE response , *T cells , *CLINICAL immunology - Abstract
Limited response to current hepatitis B virus (HBV) drugs is possibly due to inadequate host cytotoxic cellular responses. Circulating Tregs have been shown to be associated with chronicity of HBV infection, but their profile during antiviral therapy has not been studied. We analyzed the frequency and effect of Tregs on cellular immune responses against HBV in 35 chronic hepatitis B eAg−ve and eAg+ve patients treated with tenofovir 300 mg/day. Frequency of Tregs and their modulatory role in cytokine-secreting cells were determined after stimulation with HBsAg or HBcAg in the absence or presence of Tregs and after blockage of PD-1/PDL-1 in peripheral blood mononuclear cells (PBMCs). Prior to therapy, eAg−ve patients had lower HBV DNA levels, reduced CD8 T cells, increased Tregs, and T cells expressing PD1. After 12 weeks of therapy, >2 log HBV viral reduction was observed in both groups, along with an increase frequencies of CD8 T cells in eAg−ve patients and increased expression of chemokine receptors/Toll-like receptors in both groups. PD-1 expression on CD8 cells in PBMCs was decreased in both groups during therapy but not on Tregs. In eAg-ve group, sustained increase of Tregs was observed till week 12, which declined at week 24. In both groups, after 24 weeks, depletion of CD4CD25 Tregs from PBMCs enhanced HBV-specific T cell responses, and blockage of PD-1/PDL1 pathway did enhance pro-inflammatory cytokine production in eAg+ve patients but not in eAg−ve. We conclude that Tregs induced by HBV replication in vivo are expanded in eAg−ve patients more. Reduction in HBV DNA by tenofovir partially restored adaptive immune responses and also reduced the Tregs. Blockage of PD-1/PDL1, enhanced cytokine production in eAg+ve patients but not in eAg−ve, suggests that distinctly different immunologic mechanisms are involved in eAg+ve and eAg−ve patients. [ABSTRACT FROM AUTHOR]
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- 2011
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41. Serodiagnostic Assays for Celiac Disease Based on the Open or Closed Conformation of the Autoantigen, Transglutaminase 2.
- Author
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Lindfors, Katri, Koskinen, Outi, Kurppa, Kalle, Laurila, Kaija, Collin, Pekka, Haimila, Katri, Partanen, Jukka, Saavalainen, Päivi, Mäki, Markku, and Kaukinen, Katri
- Subjects
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SERODIAGNOSIS , *FIRE assay , *CELIAC disease , *TRANSGLUTAMINASES , *GLUTEN , *AUTOANTIBODIES , *CLINICAL immunology - Abstract
The autoantigen of celiac disease, transglutaminase 2 (TG2), adopts an open conformation during enzymatic activation. We studied diagnostic accuracy of serodiagnostic assays using TG2 in its open and closed conformation as antigens in patients with diagnostic difficulties. The open TG2 antibody (TG2ab) test identified 93% of untreated celiac patients in contrast to 44%, 27%, and 68% detected by closed and conventional TG2ab and endomysial antibody (EmA) tests, respectively. The assay was able to detect 60% of non-responding celiac patients seronegative for conventional TG2ab and EmA. The titers of the openTG2abs were higher than those of the closed TG2abs. The serological test utilizing TG2 in an open conformation was more accurate than the other assays in finding active celiac disease even in patients having negative or borderline conventional celiac autoantibodies and in revealing poor dietary response non-invasively. It thus offers a promising tool in the diagnostics and follow-up of celiac disease. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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42. Disparity in FcεRI-Induced Degranulation of Primary Human Lung and Skin Mast Cells Exposed to Adenosine.
- Author
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Gomez, Gregorio, Zhao, Wei, and Schwartz, Lawrence
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LUNGS , *MAST cells , *ADENOSINES , *ASTHMATICS , *WHOOPING cough , *DERMATOLOGY , *CLINICAL immunology - Abstract
Inhaled and intravenously administered adenosine induces mast cell-mediated (histamine-dependent) bronchospasm in asthmatics without causing urticaria. A differential response to adenosine by human lung and skin mast cells is shown: low concentrations potentiate FcεRI-induced degranulation of human lung mast cells but not that of skin mast cells. Human lung mast cells were found to express ∼3-fold more A3AR messenger RNA (mRNA) than skin mast cells, suggesting the involvement of the G-linked A3AR. Indeed, the adenosine-induced potentiation was sensitive to inhibition by pertussis toxin and, furthermore, could be induced with an A3AR-specific agonist. This study reveals a previously unrecognized disparity in the response to adenosine by primary human mast cells from lung and skin that might explain why adenosine induces a pulmonary but not dermatologic allergy-like response in vivo. In addition, we identify the A3AR as a potentiating receptor of FcεRI-induced degranulation, thereby implicating it in the in vivo bronchoconstrictive response to adenosine in asthmatics. [ABSTRACT FROM AUTHOR]
- Published
- 2011
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43. Alteration of Interferon-α/β Receptors in Chronic Hepatitis B Patients.
- Author
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Meng, Fanli, Wang, Jiefei, Ge, Jian, Fan, Xiaopeng, Wang, Bing, Han, Liyan, Kisseleva, Tatiana, Paik, YongHan, Brenner, David, and Wang, Kai
- Subjects
- *
INTERFERONS , *HEPATITIS B , *FLOW cytometry , *IMMUNOFLUORESCENCE , *IMMUNOHISTOCHEMISTRY , *CHRONIC diseases , *CLINICAL immunology - Abstract
Background: The present study determined type I interferon (IFN) receptor (interferon-α/β receptor (IFNAR)) and its predicable role in interferon-α2b treatment in chronic hepatitis B (CHB) patients. Methods: Expression of IFN-α/βR-1 and IFN-α/βR-2 in peripheral blood mononuclear cells and in liver tissue was measured by flow cytometry, immunofluorescence, immunohistochemistry and RT-PCR. Results: IFN-α/βR-1 and IFN-α/βR-2 in monocytes and lymphocytes increased in CHB patients. Expression of IFNAR-1 and IFNAR-2 in liver had positive correlation with HBV-DNA in liver tissue. Expression of IFN-α/βR in lymphocytes and monocytes increased in the first month, but then decreased during the subsequent interferon-α2b treatment, patients who had higher levels of IFN-α/βR-2 in monocytes prior to therapy showed better viral response than those with lower levels. Conclusions: Expression of IFN-α/βR-2 in monocytes can be used as a predictable parameter to evaluate the effect of IFN-α treatment in CHB patients. [ABSTRACT FROM AUTHOR]
- Published
- 2011
- Full Text
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44. Inflammasomes and Anti-Viral Immunity.
- Author
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Rathinam, Vijay A. K. and Fitzgerald, Katherine A.
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INTERFERONS , *CYTOKINES , *VIRUS diseases , *IMMUNE system , *IMMUNOREGULATION , *CLINICAL immunology - Abstract
Type I Interferons are the hallmark cytokines deployed during infection to combat invading viruses. However, inflammatory cytokines such as Interleukin (IL)-1β and IL-18 also play important roles in anti-viral defenses. The activity of IL-1β and IL18 are regulated at the level of expression, processing, and secretion. Several classes of innate immune receptors control the expression of these cytokines, which must then be proteolytically processed via caspase-1. In this review, we provide an overview of how caspase-1-activating inflammasomes participate during viral infections and their role in regulating anti-viral immunity. [ABSTRACT FROM AUTHOR]
- Published
- 2010
- Full Text
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45. Inhibitor IκBα Promoter Functional Polymorphisms in Patients with Rheumatoid Arthritis.
- Author
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Ruei-Nian Li, Yu-Hung Hung, Chia-Hui Lin, Yen-Hsu Chen, and Jen-Hsien Yen
- Subjects
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RHEUMATOID arthritis , *CHRONIC diseases , *INFLAMMATION treatment , *GENETIC polymorphisms , *CLINICAL immunology - Abstract
Introduction: Rheumatoid arthritis (RA) is a chronic inflammation disease that may involve extra-articular organs in addition to joints. Many proinflammatory cytokines are involved in the inflammatory process of RA. IκBα conjugates with NF-κB and is a key player in regulation of the inflammatory process. We carried out experiments to define the effect of different promoter polymorphisms on the transcriptional activities of IκBα promoter and the development of RA. Methods: Different IκBα promoter reporters were constructed and were examined in human mononuclear cells, THP-1 cells. One hundred forty patients and 115 healthy controls were recruited from the Kaohsiung Medical University Hospital. Results: The activities of IκBα promoter constructs with -826C, -550A, -519T, and -826T, -550A, -519T genotypes were expressed at one half the activity level of other constructs. Promoter constructs containing the sites -550A/T and -519T had a reduced risk of rheumatoid arthritis. The odds ratio of -826C/T genotype was significantly associated with an increase of risk in causing rheumatoid arthritis, whereas -826T/T genotype was associated only with a slightly increased risk of RA, but without statistical significance (odds ratio = 1.2; 95% confidence interval, 0.4–3.8). Conclusion: The increase of T allele was associated with a significant increased risk and the tendency to the pathogenesis of RA. The association between IκBα promoter polymorphisms and disease severity of rheumatoid arthritis is partly due to different transcriptional activities of IκBα promoter and the activation of NF-κB. [ABSTRACT FROM AUTHOR]
- Published
- 2010
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46. Coculturing Dendritic Cells with Zoledronate Acid Efficiently Enhance the Anti-Tumor Effects of Cytokine-Induced Killer Cells.
- Author
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Xiaosan Su, Lei Zhang, Liangkun Jin, Junsong Ye, Zheng Guan, and Rui Chen
- Subjects
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DENDRITIC cells , *KILLER cells , *CELLULAR mechanics , *IMMUNE system , *CLINICAL immunology ,TUMOR prevention - Abstract
Introduction: Dendritic cells (DCs) have greater stimulating activity on innate and adaptive immunity following short-term sensitization with zoledronate acid (DCs). We identified the phenotype, cytotoxicity, and mechanisms of killing of cytokine-induced killer (CIK) cells which were cocultured with DCs. Methods: Adherent and nonadherent cells of peripheral blood mononuclear cell from myeloma patients were incubated for DCs and CIK cells. Then, the CIK cells were cocultured with DCs (DCs-CIK). Expression of markers for DCs-CIK cells was measured using flow cytometry. Cytotoxicity was evaluated by against human myeloma cell lines and mechanisms of killing were tested by selectively blocking NKG2D receptor. The anti-tumor activity of these effector cells was further evaluated using a nude mice tumor model. Results: γδ TCR expression of CIK cells significantly increased after coculture with immature or mature DCs (iDCs/mDCs-CIK) and these cells aggressively lysed myeloma cells compared with mDCs-CIK and zoledronate acid pulsed CIK cells (CIK; 50.8 ± 7.9% and 48.2 ± 4.7% versus 31.9 ± 5.1% and 20.5 ± 3.6%, effector versus target ratio was 60:1). Both αβ T and γδ T cells in the iDCs-CIK cells performed the majority of lysis. The iDCs/mDCs-CIK cells greatly increased NKG2D expression compared with mDCs-CIK and CIK during culture (71.5 ± 11.3% and 67.7 ± 9.3% versus 51.3 ± 6.2% and 47.1 ± 5.7%). iDCs-CIK cell-mediated lysis dropped 69.21% when the NKG2D receptor was blocked and the cytotoxicity correlated with NKG2D ligand–MICA expression on the target cells. In a human myeloma bearing nude mice model, iDCs-CIK and mDCs-CIK cells treatment groups obtained 75% and 62.5% long-term survival (>120 days) respectively, as compared with none of the control animals or 37.5% treated with mDCs-CIK cells. Conclusion: Large numbers of CIK cells with greater anti-tumor activities are rapidly generated by Zol-treated iDCs/mDCs. This strategy is worthy of further investigation to improve adoptive cell therapy against tumors. [ABSTRACT FROM AUTHOR]
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- 2010
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47. Diversity in CD8 T Cell Function and Epitope Breadth Among Persons with Genital Herpes.
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Laing, Kerry J., Magaret, Amalia S., Mueller, Dawn E., Lin Zhao, Johnston, Christine, De Rosa, Stephen C., Koelle, David M., Wald, Anna, and Corey, Lawrence
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T cells , *HERPES simplex virus , *ANTIGENS , *VIRAL disease treatment , *CLINICAL immunology - Abstract
CD8 T cells are known to be important in clearing herpes simplex virus (HSV) infections. However, investigating the specific antiviral mechanisms employed by HSV-2-specific T cell populations is limited by a lack of reagents such as CD8 T cell epitopes and specific tetramers. Using a combination of intracellular cytokine staining flow cytometry and ELISpot methods, we functionally characterized peripheral HSV-2-specific CD8 T cells from peripheral blood mononuclear cell (PBMC) that recognize 14 selected HSV-2 open-reading frames (ORFs) from 55 HSV-2 seropositive persons; within these ORFs, we subsequently identified more than 20 unique CD8 T cell epitopes. CD8 T cells to HSV-2 exhibited significant heterogeneity in their functional characteristics, proliferation, production of inflammatory cytokines, and potential to degranulate ex vivo. The diversity in T cell response in these ex vivo assessments offers the potential of defining immune correlates of HSV-2 reactivation in humans. [ABSTRACT FROM AUTHOR]
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- 2010
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48. IL-2 Immunotherapy to Recently HIV-1 Infected Adults Maintains the Numbers of IL-17 Expressing CD4+ T (T17) Cells in the Periphery.
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Ndhlovu, Lishomwa C., Sinclair, Elizabeth, Epling, Lorrie, Qi Xuan Tan, Ho, Terence, Jha, Aashish R., Eccles-James, Ijeoma, Tincati, Camilla, Levy, Jay A., Nixon, Douglas F., Hecht, Frederick M., and Barbour, Jason D.
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T cells , *HIV , *INTERLEUKIN-2 , *CYTOKINES , *ANTIRETROVIRAL agents , *CLINICAL immunology - Abstract
Little is known about the manipulation of IL-17 producing CD4+ T cells (T17) on a per-cell basis in humans in vivo. Previous studies on the effects of IL-2 on IL-17 secretion in non-HIV models have shown divergent results. We hypothesized that IL-2 would mediate changes in IL-17 levels among recently HIV-1-infected adults receiving anti-retroviral therapy. We measured cytokine T cell responses to CD3/CD28, HIV-1 Gag, and CMV pp65 stimulation, and changes in multiple CD4+ T cell subsets. Those who received IL-2 showed a robust expansion of naive and total CD4+ T cell counts and T-reg counts. However, after IL-2 treatment, the frequency of T17 cells declined, while counts of T17 cells did not change due to an expansion of the CD4+ naïve T cell population (CD27+CD45RA+). Counts of HIV-1 Gag-specific T cells declined modestly, but CMV pp65 and CD3/CD28 stimulated populations did not change. Hence, in contrast with recent studies, our results suggest IL-2 is not a potent in vivo regulator of T17 cell populations in HIV-1 disease. However, IL-2-mediated T-reg expansions may selectively reduce responses to certain antigen-specific populations, such as HIV-1 Gag. [ABSTRACT FROM AUTHOR]
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- 2010
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49. B-Cell and T-Cell Phenotypes in CVID Patients Correlate with the Clinical Phenotype of the Disease.
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Mouillot, Gaël, Carmagnat, Maryvonnick, Gérard, Laurence, Garnier, Jean-Luc, Fieschi, Claire, Vince, Nicolas, Karlin, Lionel, Viallard, Jean-François, Jaussaud, Roland, Boileau, Julien, Donadieu, Jean, Gardembas, Martine, Schleinitz, Nicolas, Suarez, Felipe, Hachulla, Eric, Delavigne, Karen, Morisset, Martine, Jacquot, Serge, Just, Nicolas, and Galicier, Lionel
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IMMUNOLOGICAL deficiency syndromes , *IMMUNOGLOBULINS , *ANTIGEN presenting cells , *CLINICAL immunology ,INFECTION treatment - Abstract
Background: Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by recurrent infections and defective immunoglobulin production. Methods: The DEFI French national prospective study investigated peripheral T-cell and B-cell compartments in 313 CVID patients grouped according to their clinical phenotype, using flow cytometry. Results: In patients developing infection only (IO), the main B-cell or T-cell abnormalities were a defect in switched memory B cells and a decrease in naive CD4 T cells associated with an increase in CD4CD95 cells. These abnormalities were more pronounced in patients developing lymphoproliferation (LP), autoimmune cytopenia (AC), or chronic enteropathy (CE). Moreover, LP and AC patients presented an increase in CD21 B cells and CD4HLA-DR T cells and a decrease in regulatory T cells. Conclusion: In these large series of CVID patients, the major abnormalities of the B-cell and T-cell compartments, although a hallmark of CVID, were only observed in half of the IO patients and were more frequent and severe in patients with additional lymphoproliferative, autoimmune, and digestive complications. [ABSTRACT FROM AUTHOR]
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- 2010
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50. The NBS1 Genetic Polymorphisms and the Risk of the Systemic Lupus Erythematosus in Taiwanese Patients.
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Ying-Ju Lin, Yu-Ching Lan, Lei Wan, Chung-Ming Huang, Cheng-Wen Lin, Kai-Chung Hsueh, Da-Yuan Chen, Ting-Hsu Lin, and Fuu-Jen Tsai
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SYSTEMIC lupus erythematosus , *GENETIC polymorphisms , *AUTOIMMUNE disease treatment , *MEDICAL genetics , *CLINICAL immunology - Abstract
Introduction: Systemic lupus erythematosus (SLE), a multisystemic autoimmune disease, is characterized by the production of a range of autoantibodies against nuclear constituents and other self-antigens. The studies in DNA repair deficiencies in SLE patients have been recently investigated. Aims: Few studies have been conducted on DNA repair gene polymorphisms and their role in autoimmune diseases. Our study purpose was to examine and compare NBS1 genotype distributions in a group of Taiwanese SLE patients and controls in Taiwan. Patients and Methods: Participants were Taiwanese SLE patients and healthy controls. We studied associations among NBS1 polymorphisms—rs1061302, rs709816, and rs1805794—considering clinical features for the entire group and stratified subgroups. No statistically significant differences between the patients and controls were noted. However, we observed significant decreases in Ht1-GGG, Ht2-AAC, and Ht3-AGC in the SLE patients (Ht1-GGG, OR = 0.26, 95% CI: 0.16–0.41; Ht2-AAC, OR = 0.30, 95% CI: 0.17–0.53; Ht3-AGC, OR = 0.35, 95% CI: 0.19–0.71) and significant increases in Ht4-AAG, Ht5-AGG, and Ht8-GGC among the SLE patients. Combined, these results suggest an association between NBS1 genetic polymorphisms and Taiwanese SLE patients. [ABSTRACT FROM AUTHOR]
- Published
- 2010
- Full Text
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