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Clinical Immunology: the Official Journal of the Clinical Immunology Society

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https://www.readbyqxmd.com/read/30326257/a-de-novo-monoallelic-ctla-4-deletion-causing-pediatric-onset-cvid-with-recurrent-autoimmune-cytopenias-and-severe-enteropathy
#1
LETTER
Vassilios Lougaris, Manuela Baronio, Luisa Gazzurelli, Tiziana Lorenzini, Maurizio Fuoti, Daniele Moratto, Anna Bozzola, Chiara Ricci, Maria Pia Bondioni, Alberto Ravelli, Vincenzo Villanacci, Alessandro Plebani
No abstract text is available yet for this article.
October 13, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30326256/the-role-of-surface-molecule-cd229-in-multiple-myeloma
#2
REVIEW
Michael Olson, Sabari Radhakrishnan, Tim Luetkens, Djordje Atanackovic
The outcome of Multiple Myeloma (MM) patients has dramatically improved, however, most patients will still succumb to their disease. Additional therapeutic options are urgently needed and novel immunotherapies are enormously promising in the therapeutic armamentarium against MM. The first step in the development of any immunotherapy needs to be the identification of an appropriate target structure. In this review we present the current knowledge on surface molecule CD229, a member of the Signaling Lymphocyte Activation (SLAM) family of immune receptors...
October 13, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30304699/serum-mirna-371b-5p-and-mirna-5100-act-as-biomarkers-for-systemic-lupus-erythematosus
#3
Li Zeng, Jia-Li Wu, Li-Min Liu, Ju-Qing Jiang, Hai-Jing Wu, Ming Zhao, Qian-Jin Lu
MicroRNAs (miRNAs) play important roles in the pathogenesis of systemic lupus erythematosus (SLE). Here, we investigated the serum miRNAs expression profiles in the serum of SLE and healthy controls, and identified the potential serum biomarkers for SLE. We screened and identified the differentially expressed miRNAs such as miR-371b-5p, miR-5100, miR-146a-5p among active SLE, inactive SLE and healthy controls based on the miRNAs expression array. Furthermore, the results of RT-qPCR confirmed that miR-371b-5p and miR-5100 expression was different among active SLE, inactive SLE and healthy controls...
October 7, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30296592/the-relationship-between-autophagy-increased-neutrophil-extracellular-traps-formation-and-endothelial-dysfunction-in-chronic-kidney-disease
#4
Jwa-Kyung Kim, Mi Jin Park, Hoi Woul Lee, Hyung Seok Lee, Sun Ryoung Choi, Young Rim Song, Hyung Jik Kim, Hyeong-Cheon Park, Sung Gyun Kim
In chronic kidney disease (CKD), the number of circulating neutrophils are increased, and this is usually accompanied by an increased basal activation state. However, the possible association between neutrophil extracellular traps (NETs) with vascular complications has not been evaluated. We assessed the relationship between NETs, autophagy and endothelial dysfunction in maintenance hemodialysis (MHD) patients. NET formation, neutrophil elastase (NE) activities, and serum nucleosome levels were measured in MHD (n = 60) and controls (n = 20)...
October 5, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30296591/podocyte-foot-process-width-is-a-prediction-marker-for-complete-renal-response-at-6-and-12-months-after-induction-therapy-in-lupus-nephritis
#5
Kunihiro Ichinose, Mineaki Kitamura, Shuntaro Sato, Keita Fujikawa, Yoshiro Horai, Naoki Matsuoka, Masahiko Tsuboi, Fumiaki Nonaka, Toshimasa Shimizu, Shoichi Fukui, Masataka Umeda, Tomohiro Koga, Shin-Ya Kawashiri, Naoki Iwamoto, Takashi Igawa, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Tomoya Nishino, Atsushi Kawakami
Morphological change that includes diffuse effacement of podocyte foot processes is correlated with proteinuria in patients with lupus nephritis (LN). We collected the data of clinico-pathological parameters and assessed foot process width (FPW) as an index of podocyte effacement in 73 patients with LN who had undergone renal biopsy. The multivariate analysis revealed that female gender (OR: 5.288; 95%CI: 1.197-37.29; p = .0267) and FPW (OR = 0.999, 95%CI = 0.997-0.999, p = .0150) were significantly predictive of a complete renal response (CR) at 6 months, while lymphocyte counts (OR = 1...
October 5, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30296590/fra-2-is-a-novel-candidate-drug-target-expressed-in-the-podocytes-of-lupus-nephritis
#6
Changliang Xu, Yunjie Miao, Qingmeng Pi, Shoucao Zhu, Furong Li
Lupus nephritis (LN) is a common and devastating complication caused by systemic lupus erythematosus. In this study, we evaluated the expression and mechanism of Fos-related antigen 2 (Fra-2) in LN. The results showed that Fra-2 was significantly increased in kidney biopsies of LN patients compared with healthy controls and other kidney disease in glomerular podocytes. The MRL/lpr mouse strain is a murine model of lupus, and it was used to study the mechanisms of Fra-2 in LN. The results showed that Fra-2 was expressed in the glomerular podocytes...
October 5, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30290225/flow-cytometric-assessment-of-leukocyte-kinetics-for-the-monitoring-of-tissue-damage
#7
Wouter B L van den Bossche, Kyrill Rykov, Cristina Teodosio, Bas L E F Ten Have, Bas A S Knobben, Maurits S Sietsma, Karin Josiassen, Sandra de Bruin-Versteeg, Alberto Orfao, Jacques J M van Dongen, Jos J A M van Raay
Leukocyte populations quickly respond to tissue damage, but most leukocyte kinetic studies are not based on multiparameter flow cytometry. We systematically investigated several blood leukocyte populations after controlled tissue damage. 48 patients were assigned to either an anterior or posterolateral total hip arthroplasty. Peripheral blood was collected pre-operatively and at 2 h, 24 h, 48 h, 2 and 6 weeks postoperatively and assessed by flow cytometry for absolute counts of multiple leukocyte populations using standardized EuroFlow protocols...
October 2, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30287219/the-heterogeneity-of-autoimmune-polyendocrine-syndrome-type-1-clinical-features-new-mutations-and-cytokine-autoantibodies-in-a-brazilian-cohort-from-tertiary-care-centers
#8
Fernanda Guimarães Weiler, Pärt Peterson, Beatriz Tavares Costa-Carvalho, Mayra de Barros Dorna, Joya Emilie de Menezes Correia-Deur, Soraya Lopes Sader, Daniela Espíndola-Antunes, Gil Guerra-Junior, Magnus Régios Dias-da-Silva, Marise Lazaretti-Castro
Autoimmune polyendocrine syndrome type 1 (APS1) is characterized by multiorgan autoimmunity. We aim at characterizing a multi-center Brazilian cohort of APS1 patients by clinical evaluation, searching mutation in the AIRE gene, measuring serum autoantibodies, and investigating correlations between findings. We recruited patients based on the clinical criteria and tested them for AIRE mutations, antibodies against interferon type I and interleukins 17A, 17F and 22. We identified 12 unrelated families (13 patients) with typical signs of APS1 in the proband, and the screening of relatives recognized an asymptomatic child...
October 1, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30282051/exome-sequencing-confirms-diagnosis-of-kabuki-syndrome-in-an-adult-with-hodgkin-lymphoma-and-unusually-severe-multisystem-phenotype
#9
Charu Kaiwar, Teresa M Kruisselbrink, Yogish C Kudva, Eric W Klee, Pavel Pichurin
We report a 34-year-old male patient with a novel variant in KMT2D gene, which finally ended a quest for a diagnosis that was clinically suspected in the past, prior the molecular basis of Kabuki Syndrome (KS) was known. The patient showcases the multisystemic features, with involvement of all previously associated with KS body systems, presence of immune deficiency as well as autoimmune disorders, requiring three pancreatic transplants. We also report, for the first time to our knowledge, the presence of epidural lipomatosis and Hodgkin Lymphoma in a patient with KS...
September 30, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30266629/regulation-of-systemic-tissue-injury-by-coagulation-inhibitors-in-b6-mrl-lpr-autoimmune-mice
#10
C Moratz, R Robbins, J Eickhoff, J Edison, H Lui, S Peng
Impaired fibrinolysis and complement activation in Systemic Lupus Erythematosus contributes to disease amplification including increased risk of thrombosis and tissue Ischemia/Reperfusion (IR) injury. Previous work has demonstrated complement is a key regulator of tissue injury. In these studies inhibitors had varying efficacies in attenuating injury at primary versus systemic sites, such as lung. In this study the role of coagulation factors in tissue injury and complement function was evaluated. Tissue Factor Pathway Inhibitor (TFPI), an extrinsic pathway inhibitor, and Anti-Thrombin III, the downstream common pathway inhibitor, were utilized in this study...
September 25, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30248399/evaluation-of-circulating-invariant-t-cells-before-and-after-il-17-inhibitor-treatment-in-a-patient-with-psoriatic-arthritis
#11
LETTER
Tomohiro Koga, Megumi Matoba, Tomohito Sato, Yuta Koike, Yushiro Endo, Remi Sumiyoshi, Shin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Atsushi Kawakami
No abstract text is available yet for this article.
September 21, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30248398/tolerogenic-dendritic-cells-induced-the-enrichment-of-cd4-foxp3-regulatory-t-cells-via-tgf-%C3%AE-in-mesenteric-lymph-nodes-of-murine-lps-induced-tolerance-model
#12
Li Jia, Jia Lu, Ya Zhou, Yijing Tao, Hualin Xu, Wen Zheng, Juanjuan Zhao, Guiyou Liang, Lin Xu
Endotoxin tolerance is an important state for the prevention of lethal infection and inflammatory response, which is closely associated with the participation of innate immune cells. Moreover, mesenteric lymph nodes (MLNs)-resident immune cells, such as CD4+ Foxp3+ regulatory T (Treg) cells and dendritic cells, play important roles in the maintenance of peripheral immune tolerance. However, the potential roles of these cells in MLNs in the development of endotoxin tolerance remain largely unknown. Recent research work showed that CD4+ Foxp3+ Treg cells contributed to the development of endotoxin tolerance...
September 21, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30217791/safety-profile-after-prolonged-c3-inhibition
#13
Edimara S Reis, Nadja Berger, Xin Wang, Sophia Koutsogiannaki, Robert K Doot, Justin T Gumas, Periklis G Foukas, Ranillo R G Resuello, Joel V Tuplano, David Kukis, Alice F Tarantal, Anthony J Young, Tetsuhiro Kajikawa, Athena M Soulika, Dimitrios C Mastellos, Despina Yancopoulou, Ali-Reza Biglarnia, Markus Huber-Lang, George Hajishengallis, Bo Nilsson, John D Lambris
The central component of the complement cascade, C3, is involved in various biological functions, including opsonization of foreign bodies, clearance of waste material, activation of immune cells, and triggering of pathways controlling development. Given its broad role in immune responses, particularly in phagocytosis and the clearance of microbes, a deficiency in complement C3 in humans is often associated with multiple bacterial infections. Interestingly, an increased susceptibility to infections appears to occur mainly in the first two years of life and then wanes throughout adulthood...
September 20, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30244152/norovirus-specific-mucosal-antibodies-correlate-to-systemic-antibodies-and-block-norovirus-virus-like-particles-binding-to-histo-blood-group-antigens
#14
Kirsi Tamminen, Maria Malm, Timo Vesikari, Vesna Blazevic
The best acknowledged correlate of protection from norovirus (NoV) infection is the ability of serum antibodies to block binding of NoV virus-like particles (VLPs) to histo-blood group antigens (HBGAs). We investigated mucosal NoV-specific antibody levels in adult volunteers and used saliva from a single donor to determine whether purified saliva antibodies confer blocking. NoV-specific IgG and IgA levels in saliva and plasma samples were measured against four NoV genotype VLPs. NoV-specific IgG and IgA titers in saliva and plasma samples correlated significantly...
September 19, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30240602/cvid-enteropathy-is-characterized-by-exceeding-low-mucosal-iga-levels-and-interferon-driven-inflammation-possibly-related-to-the-presence-of-a-pathobiont
#15
Natalia Shulzhenko, Xiaoxi Dong, Dariia Vyshenska, Renee L Greer, Manoj Gurung, Stephany Vasquez-Perez, Ekaterina Peremyslova, Stanislav Sosnovtsev, Martha Quezado, Michael Yao, Kim Montgomery-Recht, Warren Strober, Ivan J Fuss, Andrey Morgun
Common variable immunodeficiency (CVID), the most common symptomatic primary antibody deficiency, is accompanied in some patients by a duodenal inflammation and malabsorption syndrome known as CVID enteropathy (E-CVID).The goal of this study was to investigate the immunological abnormalities in CVID patients that lead to enteropathy as well as the contribution of intestinal microbiota to this process.We found that, in contrast to noE-CVID patients (without enteropathy), E-CVID patients have exceedingly low levels of IgA in duodenal tissues...
September 19, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30236770/nlrp3-inflammasome-regulates-th17-differentiation-in-rheumatoid-arthritis
#16
Chunmei Zhao, Yibin Gu, Xiaoyun Zeng, Jing Wang
Rheumatoid arthritis (RA) is one of the most common autoimmune diseases. Th17 has been shown to play am important role in the pathogenesis of RA. Accumulating data suggest the involvement of NLRP3 inflammasome in Th17 differentiation in autoimmune diseases. In the current study, we found that NLRP3 inflammasome is activated in CD4 T cells from RA patients. The activation of NLRP3 inflammasome was correlated with disease activities and IL-17A concentration in RA sera. Knockdown of NLRP3 suppressed Th17 differentiation...
September 17, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30219667/increase-of-follicular-helper-t-cells-skewed-toward-a-th1-profile-in-cvid-patients-with-non-infectious-clinical-complications
#17
Delphine Turpin, Adeline Furudoi, Marie Parrens, Patrick Blanco, Jean-François Viallard, Dorothée Duluc
Common variable immunodeficiency (CVID) is characterized by low levels of circulating immunoglobulins and defects in B cell maturation leading to an increased susceptibility to infections. Some patients develop complications such as autoimmune diseases, enteropathy, and lymphoproliferation, resulting in higher morbidity and mortality. Follicular helper T (Tfh) cells are specialized in helping B cell differentiation into Ig-producing cells. Three subsets have been described, namely non B-cell helper Tfh1 and the two B-helper cell subsets Tfh2 and Tfh17...
September 13, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30218707/th17-and-treg-lymphocytes-in-obesity-and-type-2-diabetic-patients
#18
REVIEW
Mei Wang, Fuqiong Chen, Jingli Wang, Zhixuan Zeng, Qin Yang, Shiying Shao
Assumption that the pathogenesis of obesity-associated type 2 diabetes (T2DM) encompasses inflammation and autoimmune aspects is increasingly recognized. In the state of obesity and T2DM, the imbalance of T helper 17 (Th17) cells and regulatory T (Treg) cells are observed. These alterations reflect a loss of T cell homeostasis, which may contribute to tissue and systemic inflammation and immunity in T2DM. In this review we will discuss the accumulating data supporting the concept that Th17/Treg mediated immune responses are present in obesity-related T2DM pathogenesis, and provide evidences that restoration of Th17/Treg imbalance may be a possible therapeutic avenue for the prevention and treatment of T2DM and its complications...
September 13, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30205177/immunogenicity-to-cerliponase-alfa-intracerebroventricular-enzyme-replacement-therapy-for-cln2-disease-results-from-a-phase-1-2-study
#19
Anu Cherukuri, Heather Cahan, Greg de Hart, Andrea Van Tuyl, Peter Slasor, Laurie Bray, Joshua Henshaw, Temitayo Ajayi, Dave Jacoby, Charles A O'Neill, Becky Schweighardt
Treatment with intracerebroventricular (ICV)-delivered cerliponase alfa enzyme replacement therapy (ERT) in a Phase 1/2 study of 24 subjects with CLN2 disease resulted in a meaningful preservation of motor and language (ML) function and was well tolerated. Treatment was associated with anti-drug antibody (ADA) production in the cerebrospinal fluid (CSF) of 6/24 (25%) and in the serum of 19/24 (79%) of clinical trial subjects, respectively, over a mean exposure of 96.4 weeks (range 0.1-129 weeks). Neutralizing antibodies (NAb) were not detected in the CSF of any of the subjects...
September 8, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30196070/pathophysiology-and-inhibition-of-il-23-signaling-in-psoriatic-arthritis-a-molecular-insight
#20
Cuong Thach Nguyen, Yehudi Bloch, Katarzyna Składanowska, Savvas N Savvides, Iannis E Adamopoulos
Psoriatic arthritis (PsA) is a chronic inflammatory arthritis of unknown etiology, and currently the cellular and molecular interactions that dictate its pathogenesis remain elusive. A role of the interleukin-23 (IL-23)/IL-23R (IL-23 receptor) interaction in the development of psoriasis and PsA is well established. As IL-23 regulates the differentiation and activation of innate and adaptive immunity, it pertains to a very complex pathophysiology involving a plethora of effectors and transducers. In this review, we will discuss recent advances on the cellular and molecular pathophysiological mechanisms that regulate the initiation and progression of PsA as well as new therapeutic approaches for IL-23/IL-23R targeted therapeutics...
September 6, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
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