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Primary immunodeficience

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https://www.readbyqxmd.com/read/28214184/-systemic-lupus-erythematosus-and-lymphopenia-clinical-and-pathophysiological-features
#1
M Martin, A Guffroy, X Argemi, T Martin
Lymphopenia is frequent in systemic lupus erythematosus (SLE) and profound (<500/mm(3)) in 10% of cases. T lymphocytes, especially CD4+, are more affected than B cells. The pathophysiological mechanisms are complex, involving lymphocytotoxic antibodies, excess of apoptosis, increased susceptibility of T cells to complement mediated cytolysis, as well as lymphopoiesis impairment and lymphocyte sequestration. Lymphopenia in SLE is independent from other cytopenia and immunosuppressive drug regiments, and associated with disease activity, risk of flare and damage scores...
February 14, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28212436/antibody-deficiency-in-patients-with-frequent-exacerbations-of-chronic-obstructive-pulmonary-disease-copd
#2
Brian N McCullagh, Alejandro P Comellas, Zuhair K Ballas, John D Newell, M Bridget Zimmerman, Antoine E Azar
Chronic Obstructive Pulmonary Disease is the third leading cause of death in the US, and is associated with periodic exacerbations, which account for the largest proportion of health care utilization, and lead to significant morbidity, mortality, and worsening lung function. A subset of patients with COPD have frequent exacerbations, occurring 2 or more times per year. Despite many interventions to reduce COPD exacerbations, there is a significant lack of knowledge in regards to their mechanisms and predisposing factors...
2017: PloS One
https://www.readbyqxmd.com/read/28202953/fully-human-cd19-specific-chimeric-antigen-receptors-for-t-cell-therapy
#3
D Sommermeyer, T Hill, S M Shamah, A I Salter, Y Chen, K M Mohler, S R Riddell
Impressive results have been achieved by adoptively transferring T-cells expressing CD19-specific CARs with binding domains from murine mAbs to treat B-cell malignancies. T-cell mediated immune responses specific for peptides from the murine scFv antigen-binding domain of the CAR can develop in patients and result in premature elimination of CAR-T-cells increasing the risk of tumor relapse. As fully human scFv might reduce immunogenicity, we generated CD19-specific human scFvs with similar binding characteristics as the murine FMC63-derived scFv using human Ab/DNA-libraries...
February 16, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28201953/acute-hiv-1-infection-presenting-with-fulminant-encephalopathy
#4
Eun J Lee, Young H Kim, Jeong Y Lee, Jun-Sang Sunwoo, Se Y Park, Tae H Kim
Human immunodeficiency virus (HIV)-1 directly affects the nervous system, causes distinct neurological symptoms, and indirectly results in opportunistic infections, which include herpes virus simplex (HSV)-1, HSV-2, varicella zoster virus, and cytomegalovirus encephalitis caused by immunodeficiency. Early HIV-1 invasion of the central nervous system is also possible, and acute encephalopathy is a potentially lethal complication. We encountered a case of fulminant encephalopathy as a primary presentation of acute HIV-1 infection, in which highly active antiretroviral treatment resulted in a full clinical recovery...
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28199798/the-hotel-study-clinical-and-health-service-effectiveness-in-a-cohort-of-homeless-or-marginally-housed-persons
#5
William G Honer, Alejandro Cervantes-Larios, Andrea A Jones, Fidel Vila-Rodriguez, Julio S Montaner, Howard Tran, Jimmy Nham, William J Panenka, Donna J Lang, Allen E Thornton, Talia Vertinsky, Alasdair M Barr, Ric M Procyshyn, Geoffrey N Smith, Tari Buchanan, Mel Krajden, Michael Krausz, G William MacEwan, Kristina M Gicas, Olga Leonova, Verena Langheimer, Alexander Rauscher, Krista Schultz
OBJECTIVE: The Hotel Study was initiated in Vancouver's Downtown East Side (DTES) neighborhood to investigate multimorbidity in homeless or marginally housed people. We evaluated the clinical effectiveness of existing, illness-specific treatment strategies and assessed the effectiveness of health care delivery for multimorbid illnesses. METHOD: For context, we mapped the housing locations of patients presenting for 552,062 visits to the catchment hospital emergency department (2005-2013)...
January 1, 2017: Canadian Journal of Psychiatry. Revue Canadienne de Psychiatrie
https://www.readbyqxmd.com/read/28197791/clinical-manifestations-and-genetic-analysis-of-17-patients-with-autosomal-dominant-hyper-ige-syndrome-in-mainland-china-new-reports-and-a-literature-review
#6
Jing Wu, Ji Chen, Zhi-Qing Tian, Hao Zhang, Ruo-Lan Gong, Tong-Xin Chen, Li Hong
PURPOSE: Autosomal dominant hyper-IgE syndrome (AD-HIES) is a rare complicated primary immunodeficiency disease (PID). Signal transducer and activator of transcription 3 (STAT3) gene mutation is found to cause AD-HIES. The distribution of AD-HIES patients with STAT3 deficiency in the Chinese population is not clear. Herein, we retrospectively report 17 AD-HIES patients with STAT3 deficiency and demonstrate their clinical, immunological, and genetic features. METHODS: Patients' clinical data were collected from their medical records...
February 14, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28197149/treatment-of-infantile-inflammatory-bowel-disease-and-autoimmunity-by-allogeneic-stem-cell-transplantation-in-lps-responsive-beige-like-anchor-deficiency
#7
Shahrzad Bakhtiar, Laura Gámez-Díaz, Andrea Jarisch, Jan Soerensen, Bodo Grimbacher, Bernd Belohradsky, Klaus-Michael Keller, Christoph Rietschel, Thomas Klingebiel, Sibylle Koletzko, Michael H Albert, Peter Bader
Inflammatory bowel disease (IBD) in young children can be a clinical manifestation of various primary immunodeficiency syndromes. Poor clinical outcome is associated with poor quality of life and high morbidity from the complications of prolonged immunosuppressive treatment and malabsorption. In 2012, mutations in the lipopolysaccharide-responsive beige-like anchor (LRBA) gene were identified as the cause of an autoimmunity and immunodeficiency syndrome. Since then, several LRBA-deficient patients have been reported with a broad spectrum of clinical manifestations without reliable predictive prognostic markers...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28192253/hematopoietic-stem-cell-transplantation-using-preimplantation-genetic-diagnosis-and-hla-typing-for-hla-matched-sibling-donor-a-turkish-multicenter-study
#8
Emin Kurekci, Alphan Küpesiz, Sema Anak, Gülyüz Öztürk, Orhan Gürsel, Serap Aksoylar, Talia Ileri, Barış Kuşkonmaz, Ibrahim Eker, Mualla Cetin, Gülsün Tezcan Karasu, Zühre Kaya, Tunç Fışgın, Mehmet Ertem, Savaş Kansoy, Mehmet Akif Yeşilipek
Preimplantation genetic diagnosis involves the diagnosis of a genetic disorder in embryos obtained through in vitro fertilization, selection of healthy embryos, and transferring them to the mother's uterus. Preimplantation genetic diagnosis has been used not only to avoid the risk of having an affected child, but also by using HLA matching together, it offers preselection of potential HLA-genoidentical healthy donor progeny for an affected sibling, who requires bone marrow transplantation. Here, we share the hematopoietic stem cell transplantation results of 52 patients with different benign and malign hematological or metabolic diseases or immunodeficiencies, whose donors were their siblings borned with this technique in Turkey since 2008...
February 10, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28192236/diagnosis-of-primary-antibody-and-complement-immunodeficiencies-in-young-adults-after-a-first-invasive-bacterial-infection
#9
Sébastien Sanges, Frédéric Wallet, Nicolas Blondiaux, Didier Theis, Isabelle Verin, Anne Vachée, Rodrigue Dessein, Karine Faure, Nathalie Viget, Eric Senneville, Olivier Leroy, Fleur Maury, Nicolas Just, Julien Poissy, Daniel Mathieu, Anne Prévotat, Cécile Chenivesse, Arnaud Scherpereel, Grégoire Smith, Benjamin Lopez, Jérémie Rosain, Véronique Fremeaux-Bacchi, Eric Hachulla, Pierre-Yves Hatron, Mathilde Bahuaud, Frédéric Batteux, David Launay, Myriam Labalette, Guillaume Lefèvre
OBJECTIVES: Screening for primary immunodeficiencies (PIDs) in adults is recommended after two severe bacterial infections. We aimed to evaluate if screening should be performed after the first invasive infection in young adults. METHODS: Eligible patients were retrospectively identified using hospital discharge and bacteriology databases in 3 centers during a 3-year period. Eighteen to forty year-old patients were included if they had experienced an invasive infection with encapsulated bacteria commonly encountered in PIDs (Streptococcus pneumoniae (SP), Neisseria meningitidis (NM), Neisseria gonorrhoeae (NG), Haemophilus influenzae (HI) or group A Streptococcus (GAS))...
February 10, 2017: Clinical Microbiology and Infection
https://www.readbyqxmd.com/read/28192146/autoimmune-and-inflammatory-manifestations-occur-frequently-in-primary-immunodeficiencies
#10
Alain Fischer, Johan Provot, Jean-Philippe Jais, Alexandre Alcais, Nizar Mahlaoui
BACKGROUND: Primary immunodeficiencies (PIDs) are inherited diseases associated with a considerably increase in susceptibility to infections. It is known that PIDs can also predispose to cancer and immune diseases including allergy, autoimmunity and inflammation. OBJECTIVE: We aimed at determining the incidence of autoimmunity and inflammation in PID patients. METHODS: We have retrospectively screen 2183 consecutive cases of PID in the French CEREDIH registry for the occurrence of autoimmunity and inflammation...
February 9, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28191765/generation-of-osteosarcomas-from-a-combination-of-rb-silencing-and-c-myc-overexpression-in-human-mesenchymal-stem-cells
#11
Jir-You Wang, Po-Kuei Wu, Paul Chih-Hsueh Chen, Chia-Wen Lee, Wei-Ming Chen, Shih-Chieh Hung
Osteosarcoma (OS) was a malignant tumor occurring with unknown etiology that made prevention and early diagnosis difficult. Mesenchymal stem cells (MSCs), which were found in bone marrow, were claimed to be a possible origin of OS but with little direct evidence. We aimed to characterize OS cells transformed from human MSCs (hMSCs) and identify their association with human primary OS cells and patient survival. Genetic modification with p53 or retinoblastoma (Rb) knockdown and c-Myc or Ras overexpression was applied for hMSC transformation...
February 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28188718/-hematopoietic-stem-cells-transplant-in-patients-with-common-variable-immunodeficiency-is-a-therapeutic-option
#12
Julio César Cambray-Gutiérrez, Diana Andrea Herrera-Sánchez, Patricia López-Pérez, Aurora Chávez-García, Marco Antonio Yamazaki-Nakashimada
BACKGROUND: Patients with common variable immunodeficiency show higher incidence of sinopulmonary and gastrointestinal infections, as well as lymphoproliferative and autoimmune diseases. The treatment of choice is replacement therapy with human gamma-globulin. Hematopoietic stem cell transplantation is a non-conventional therapeutic modality. CASE REPORT: Twenty-six-year old woman with no family or hereditary history of primary immune deficiencies or consanguinity, with repeated episodes of otitis, sinusitis, gastroenteritis and bronchitis since childhood...
January 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28188716/-immunological-alterations-in-common-variable-immunodeficiency
#13
Laura Berrón-Ruiz
Common variable immunodeficiency (CVID) is the largest group of symptomatic primary immune deficiencies; it is characterized by hypogammaglobulinemia, poor response to vaccines and increased susceptibility to infections. Cellular phenotypes and abnormalities have been described both in adaptive and innate immune response. Several classifications of common variable immunodeficiency are based on defects found on T and B cells, which have been correlated with clinical manifestations. In recent years, significant progress has been made in elucidating the genetic mechanisms that result in a IDCV phenotype...
January 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28188711/-clinical-symptoms-in-iga-deficiency
#14
Flavio Augusto De Oliveira-Serra, Tainá Mosca, Maria da Conceição Santos de Menezes, Wilma Carvalho-Neves Forte
BACKGROUND: IgA deficiency is the most common primary immunodeficiency. Early diagnosis and clinical follow-up may improve the quality of life of patients with IgA deficiency. To this end, IgA deficiency should be further studied and better understood on its clinical manifestations. OBJECTIVE: To determine IgA deficiency clinical manifestations. METHODS: Cross-sectional, retrospective, exploratory study, where the medical records of 39 patients with IgA deficiency were analyzed...
January 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28187791/primary-mediastinal-large-b-cell-lymphoma-in-a-woman-who-is-human-immunodeficiency-virus-positive-presenting-with-superior-vena-cava-syndrome-a-case-report
#15
Pedro Pallangyo, Paulina Nicholaus, Frederick Lyimo, Elikaanany Urio, Peter Kisenge, Mohamed Janabi
BACKGROUND: The risk of non-Hodgkin lymphoma is increased 200-fold in individuals seropositive for human immunodeficiency virus compared to those free from human immunodeficiency virus. Human immunodeficiency virus-associated non-Hodgkin lymphoma is known for its atypical presentation, aggressive ability, widespread involvement, poor response to chemotherapy, and high relapse potential which makes both the diagnosis and management a difficult undertaking especially in resource-poor settings...
February 11, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28187060/risk-factors-in-children-older-than-5-years-with-pneumococcal-meningitis-data-from-a-national-network
#16
Fanny Hénaff, Corinne Levy, Robert Cohen, Capucine Picard, Emmanuelle Varon, Christèle Gras Le Guen, Elise Launay
INTRODUCTION: The occurrence of meningitis in children > 5 years old may be associated with specific predisposing factors that can be anatomic, such as cerebrospinal fluid (CSF) fistula or breach, or related to genetic susceptibility or N inborn or acquired immunologic defect. This study aimed to assess the anatomical and immunologic risk factors in children > 5 years old with pneumococcal meningitis and prospectively enrolled in the French national meningitis network. METHODS: We analyzed all data for children 5 to 15 years old with a diagnosis of pneumococcal meningitis between 2001 and 2013...
December 13, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28183848/long-term-observation-reveals-high-frequency-engraftment-of-human-acute-myeloid-leukemia-in-immunodeficient-mice
#17
Anna M Paczulla, Stephan Dirnhofer, Martina Konantz, Michael Medinger, Helmut R Salih, Kathrin Rothfelder, Dimitrios A Tsakiris, Jakob R Passweg, Pontus Lundberg, Claudia Lengerke
Repopulation of immunodeficient mice remains the primary method to func-tionally assess human acute myeloid leukemia. Published data report en-graftment of ~40-66% of cases, mostly belonging to intermediate or poor risk subtypes. Here we report that extending follow-up beyond the standard analysis end-points of 10 to 16 weeks post-transplantation permitted leukemic engraftment from nearly every xenotransplanted acute myeloid leukemia case (18/19, ~95%). Xenogeneic leukemic cells showed conserved immune phenotypes and genetic signatures when compared to corresponding pre-transplant cells, and were furthermore able to induce leukemia in re-transplantation assays...
February 9, 2017: Haematologica
https://www.readbyqxmd.com/read/28178646/menin-mediates-tat-induced-neuronal-apoptosis-in-brain-frontal-cortex-of-siv-infected-macaques-and-in-tat-treated-cells
#18
Jun Wang, Yu Zhang, Qiping Xu, Jinhua Qiu, Honghua Zheng, Xiang Ye, Yuhua Xue, Yongmei Yin, Zhou Zhang, Ying Liu, Yanling Hao, Qiang Wei, Wei Wang, Kazuyasu Mori, Shuji Izumo, Ryuji Kubota, Yiming Shao, Hui Qin Xing
The molecular mechanisms involved in human immunodeficiency virus (HIV)-associated neurocognitive disorder (HAND) remain poorly understood. It has been recently reported that HIV-1 Tat transactivation requires menin, suggesting that menin may be involved in HAND pathogenesis. But the role of menin is not clear. Here, we found that protein level of menin was increased in simian-human immunodeficiency chimeric virus (SHIV)-SF162.P4 and simian immunodeficiency virus (SIV) sm543-3-infected rhesus macaques compared with the controls by immunohistochemistry (IHC) and western blot...
February 2, 2017: Oncotarget
https://www.readbyqxmd.com/read/28178146/pairwise-diversity-and-tmrca-as-potential-markers-for-hiv-infection-recency
#19
Sikhulile Moyo, Eduan Wilkinson, Alain Vandormael, Rui Wang, Jia Weng, Kenanao P Kotokwe, Simani Gaseitsiwe, Rosemary Musonda, Joseph Makhema, Max Essex, Susan Engelbrecht, Tulio de Oliveira, Vladimir Novitsky
Intrahost human immunodeficiency virus (HIV)-1 diversity increases linearly over time. We assessed the extent to which mean pairwise distances and the time to the most recent common ancestor (tMRCA) inferred from intrahost HIV-1C env sequences were associated with the estimated time of HIV infection. Data from a primary HIV-1C infection study in Botswana were used for this analysis (N = 42). A total of 2540 HIV-1C env gp120 variable loop region 1 to conserved region 5 (V1C5) of the HIV-1 envelope gp120 viral sequences were generated by single genome amplification and sequencing, with an average of 61 viral sequences per participant and 11 sequences per time point per participant...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28174252/trbp-maintains-mammalian-embryonic-neural-stem-cell-properties-by-enhancing-the-notch-signaling-pathway-as-a-novel-transcriptional-coactivator
#20
Sung-Hyun Byun, Juwan Kim, Dasol Han, Mookwang Kwon, Jae Youl Cho, Hui Xuan Ng, Samuel J Pleasure, Keejung Yoon
Transactivation response element RNA-binding protein (TRBP) is known to play important roles in human immunodeficiency virus (HIV) replication and microRNA biogenesis. However, recent studies implicate TRBP in a variety of biological processes as a mediator for cross-talk between signal transduction pathways. Here, we provide the first evidence that TRBP is required for efficient neurosphere formation, and expression of neural stem cell markers and Notch target genes in primary neural progenitor cells in vitro Consistent with this, introduction of TRBP into the mouse embryonic brain in utero increased the fraction of cells expressing Sox2 in the ventricular zone (VZ)...
February 7, 2017: Development
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