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Primary immunodeficience

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https://www.readbyqxmd.com/read/29777376/comprehensive-molecular-diagnosis-of-epstein-barr-virus-associated-lymphoproliferative-diseases-using-next-generation-sequencing
#1
Shintaro Ono, Manabu Nakayama, Hirokazu Kanegane, Akihiro Hoshino, Saeko Shimodera, Hirofumi Shibata, Hisanori Fujino, Takahiro Fujino, Yuta Yunomae, Tsubasa Okano, Motoi Yamashita, Takahiro Yasumi, Kazushi Izawa, Masatoshi Takagi, Kohsuke Imai, Kejian Zhang, Rebecca Marsh, Capucine Picard, Sylvain Latour, Osamu Ohara, Tomohiro Morio
Epstein-Barr virus (EBV) is associated with several life-threatening diseases, such as lymphoproliferative disease (LPD), particularly in immunocompromised hosts. Some categories of primary immunodeficiency diseases (PIDs) including X-linked lymphoproliferative syndrome (XLP), are characterized by susceptibility and vulnerability to EBV infection. The number of genetically defined PIDs is rapidly increasing, and clinical genetic testing plays an important role in establishing a definitive diagnosis. Whole-exome sequencing is performed for diagnosing rare genetic diseases, but is both expensive and time-consuming...
May 18, 2018: International Journal of Hematology
https://www.readbyqxmd.com/read/29775036/consensus-on-updating-immunizations-in-patients-with-primary-immunodeficiencies
#2
(no author information available yet)
No abstract text is available yet for this article.
April 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29771377/alternative-splicing-analysis-in-human-monocytes-and-macrophages-reveals-mbnl1-as-major-regulator
#3
Hongfei Liu, Paolo A Lorenzini, Fan Zhang, Shaohai Xu, Mei Su M Wong, Jie Zheng, Xavier Roca
We report the detailed transcriptomic profiles of human innate myeloid cells using RNA sequencing. Monocytes migrate from blood into infected or wounded tissue to differentiate into macrophages, and control inflammation via phagocytosis or cytokine secretion. We differentiated culture primary monocytes with either GM- or M-CSF to obtain pro- or anti-inflammatory macrophages, and respectively activated them with either LPS/IFNγ or anti-inflammatory cytokines. We also treated the THP-1 monocytic cell line with PMA and similar cytokines to mimic differentiation and activation...
May 16, 2018: Nucleic Acids Research
https://www.readbyqxmd.com/read/29764952/samhd1-phosphorylation-coordinates-the-anti-hiv-1-response-by-diverse-interferons-and-tyrosine-kinase-inhibition
#4
Matthew A Szaniawski, Adam M Spivak, James E Cox, Jonathan L Catrow, Timothy Hanley, Elizabeth S C P Williams, Michel J Tremblay, Alberto Bosque, Vicente Planelles
Macrophages are susceptible to human immunodeficiency virus type 1 (HIV-1) infection despite abundant expression of antiviral proteins. Perhaps the most important antiviral protein is the restriction factor sterile alpha motif domain and histidine/aspartic acid domain-containing protein 1 (SAMHD1). We investigated the role of SAMHD1 and its phospho-dependent regulation in the context of HIV-1 infection in primary human monocyte-derived macrophages and the ability of various interferons (IFNs) and pharmacologic agents to modulate SAMHD1...
May 15, 2018: MBio
https://www.readbyqxmd.com/read/29761739/efficacy-safety-and-quality-of-life-in-patients-receiving-subcutaneous-igg-treatment-experience-in-bogot%C3%A3-colombia
#5
María Claudia Ortega-López, Javier Garay, Mónica León Pinilla
AIM: Investigate efficacy, safety and quality of life of gammanorm® 16.5% (subcutaneous immunoglobulin [SCIG]) in patients with primary immunodeficiencies (PIDs) and safety and to lesser extent efficacy in autoimmune diseases. PATIENTS & METHODS: Medical records were extracted from 31 pediatric and 12 adult patients who received SCIG as part of the Personalized Program at University Children's Hospital, Bogotá, Colombia. RESULTS: Mean SCIG dose was 28...
May 15, 2018: Immunotherapy
https://www.readbyqxmd.com/read/29758076/high-throughput-generation-and-characterization-of-replication-competent-clade-c-transmitter-founder-simian-human-immunodeficiency-viruses
#6
Debashis Dutta, Samuel Johnson, Alisha Dalal, Martin J Deymier, Eric Hunter, Siddappa N Byrareddy
Traditional restriction endonuclease-based cloning has been routinely used to generate replication-competent simian-human immunodeficiency viruses (SHIV) and simian tropic HIV (stHIV). This approach requires the existence of suitable restriction sites or the introduction of nucleotide changes to create them. Here, using an In-Fusion cloning technique that involves homologous recombination, we generated SHIVs and stHIVs based on epidemiologically linked clade C transmitted/founder HIV molecular clones from Zambia...
2018: PloS One
https://www.readbyqxmd.com/read/29757775/development-and-calibration-of-a-mathematical-model-of-anal-carcinogenesis-for-high-risk-hiv-infected-men
#7
Emily A Burger, Michael A Dyer, Stephen Sy, Joel M Palefsky, Alexandra De Pokomandy, François Coutlee, Michael J Silverberg, Jane J Kim
OBJECTIVES: Men who have sex with men (MSM) who are living with human immunodeficiency virus (HIV) are at highest risk for anal cancer. Our objective was to use empirical data to develop a comprehensive disease simulation model that reflects the most current understanding of anal carcinogenesis, which is uniquely positioned to evaluate future anal cancer screening strategies as well as provide insight on the unobservable course of the disease. SETTING: North America METHODS...
May 9, 2018: Journal of Acquired Immune Deficiency Syndromes: JAIDS
https://www.readbyqxmd.com/read/29757592/the-profile-of-toll-like-receptor-2-tlr2-tlr4-and-their-cytosolic-downstream-signaling-pathway-in-common-variable-immunodeficiency-cvid-patients
#8
Laleh Sharifi, Asghar Aghamohammadi, Nima Rezaei, Reza Yazdani, Mahdi Mahmoudi, Mohammad Mehdi Amiri, Farimah Masoumi, Saied Bokaie, Parsova Tavasolian, Rouzbeh Sanaei, Mona Moshiri, Naeimeh Tavakolinia, Tina Alinia, Gholamreza Azizi, Abbas Mirshafiey
Common variable immunodeficiency (CVID) is the most common clinical primary antibody deficiency, characterized by increased susceptibility to recurrent bacterial infections. Since Toll-like receptors (TLRs) play an important role in the maturation and differentiation of B-cells, TLRs' defect can be involved in the pathogenesis of CVID. Therefore, we evaluated the expression of TLR2 and TLR4 and their signaling pathway; also their association with autoimmunity, B-cell subtypes and response to pneumovax-23 were assessed in CVID patients...
April 2018: Iranian Journal of Allergy, Asthma, and Immunology
https://www.readbyqxmd.com/read/29755533/new-onset-colitis-in-an-adult-patient-with-chronic-granulomatous-disease-treated-with-hematopoietic-stem-cell-transplantation-a-diagnostic-dilemma
#9
Kara Robertson, Stephen Couban, Desmond Leddin, Imran Ahmad, Lori Connors
Background: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency characterized by recurrent life-threatening bacterial and fungal infections, granuloma formation and intestinal disease. This disease is caused by defects in NADPH oxidase, which result in the inability of phagocytes (neutrophils, monocytes and macrophages) to destroy certain microbes. The only established curative therapy for CGD is hematopoietic stem cell transplantation. Case presentation: A 23-year-old Caucasian male with X-linked chronic granulomatous disease underwent a reduced-intensity conditioning, matched unrelated donor peripheral blood stem cell transplant, after which he was started on tacrolimus and mycophenolate for graft-versus-host disease prophylaxis...
2018: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/29754192/gastrointestinal-disorders-associated-with-primary-immunodeficiency-diseases
#10
REVIEW
Stella Hartono, Michelina Rosa Ippoliti, Maria Mastroianni, Ricardo Torres, Nicholas L Rider
There are now 354 inborn errors of immunity (primary immunodeficiency diseases (PIDDs)) with 344 distinct molecular etiologies reported according to the International Union of Immunological Sciences (IUIS) (Clin Gastroenterol Hepatol 11: p. 1050-63, 2013, Semin Gastrointest Dis 8: p. 22-32, 1997, J Clin Immunol 38: p. 96-128, 2018). Using the IUIS document as a reference and cross-checking PubMed ( www.ncbi.nlm.nih.pubmed.gov ), we found that approximately one third of the 354 diseases of impaired immunity have a gastrointestinal component [J Clin Immunol 38: p...
May 13, 2018: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/29753658/feasibility-and-biological-rationale-of-repurposing-sunitinib-and-erlotinib-for-dengue-treatment
#11
Szuyuan Pu, Fei Xiao, Stanford Schor, Elena Bekerman, Fabio Zanini, Rina Barouch-Bentov, Claude M Nagamine, Shirit Einav
There is an urgent need for strategies to combat dengue virus (DENV) infection; a major global threat. We reported that the cellular kinases AAK1 and GAK regulate intracellular trafficking of multiple viruses and that sunitinib and erlotinib, approved anticancer drugs with potent activity against these kinases, protect DENV-infected mice from mortality. Nevertheless, further characterization of the therapeutic potential and underlying mechanism of this approach is required prior to clinical evaluation. Here, we demonstrate that sunitinib/erlotinib combination achieves sustained suppression of systemic infection at approved dose in DENV-infected IFN-α/β and IFN-γ receptor-deficient mice...
May 10, 2018: Antiviral Research
https://www.readbyqxmd.com/read/29753569/first-presentation-with-psychotic-symptoms-to-the-emergency-department
#12
Yousef Etlouba, Abdullah Laher, Feroza Motara, Muhammed Moolla, Nazeema Ariefdien
BACKGROUND: Mental health conditions account for 52.8 million (4.9-6.3%) emergency department (ED) visits in the United States. Psychotic conditions are responsible for approximately 10% of all mental health presentations. OBJECTIVE: We aimed to determine the underlying etiology and characteristics of patients presenting to the ED with a first episode of psychotic symptoms. METHODS: Medical records of 159 African, Asian, white, and mixed-race patients were prospectively reviewed...
May 10, 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29753156/antiviral-t-cells-for-adenovirus-in-the-pre-transplant-period-a-bridge-therapy-for-severe-combined-immunodeficiency
#13
Holly K Miller, Patrick J Hanley, Haili Lang, Christopher A Lazarski, Elizabeth A Chorvinsky, Sarah McCormack, Lauren Roesch, Shuroug Albihani, Marcus Dean, Fahmida Hoq, Roberta H Adams, Catherine Bollard, Michael D Keller
Viral infections can be life-threatening in patients with severe combined immunodeficiency (SCID) and other forms of profound primary immunodeficiency disorders both before and after hematopoietic stem cell transplant (HSCT). Adoptive immunotherapy with virus-specific T-cells (VST) has been utilized in many patients in the setting of HSCT, but has very rarely been attempted for treatment of viral infections before HSCT. Here we describe the use of VSTs in an infant with RAG1 SCID who had developed disseminated adenovirus which failed to improve on cidofovir...
May 9, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29750773/missed-opportunities-for-human-immunodeficiency-virus-and-syphilis-testing-among-men-who-have-sex-with-men-in-china-a-cross-sectional-study
#14
Jason J Ong, Hongyun Fu, Stephen Pan, M Kumi Smith, Dan Wu, Chongyi Wei, Bolin Cao, Wei Ma, Ligang Yang, Weiming Tang, Joseph D Tucker
BACKGROUND: Men who have sex with men (MSM) are at high risk of human immunodeficiency virus (HIV)/syphilis. To inform strategies for dual testing, we describe patterns of HIV/syphilis testing, and examine factors associated with never testing for HIV/syphilis in China. METHODS: An online survey (2016) was completed by MSM from 8 cities: men born biologically male, 16 years or older, and had sex with another man at least once during their lifetime. Demographic, sexual behavioral, and HIV/syphilis testing data were collected...
June 2018: Sexually Transmitted Diseases
https://www.readbyqxmd.com/read/29746681/prevention-of-infectious-complications-in-patients-with-chronic-granulomatous-disease
#15
Maria A Slack, Isaac P Thomsen
Chronic granulomatous disease (CGD) is a primary immunodeficiency that confers a markedly increased risk of bacterial and fungal infections caused by certain opportunistic pathogens. Current evidence supports the use of prophylactic antibacterial, antifungal, and immunomodulatory therapies designed to prevent serious or life-threatening infections in patients with CGD. In this review, we discuss current strategies for the prevention of infections in children and adults with CGD and the evidence that supports those strategies...
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746679/noninfectious-manifestations-and-complications-of-chronic-granulomatous-disease
#16
Sarah E Henrickson, Artemio M Jongco, Kelly F Thomsen, Elizabeth K Garabedian, Isaac P Thomsen
Chronic granulomatous disease (CGD), a primary immunodeficiency characterized by a deficient neutrophil oxidative burst and the inadequate killing of microbes, is well known to cause a significantly increased risk of invasive infection. However, infectious complications are not the sole manifestations of CGD; substantial additional morbidity is driven by noninfectious complications also. These complications can include, for example, a wide range of inflammatory diseases that affect the gastrointestinal tract, lung, skin, and genitourinary tract and overt autoimmune disease...
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746675/chronic-granulomatous-disease-epidemiology-pathophysiology-and-genetic-basis-of-disease
#17
N L Rider, M B Jameson, C B Creech
Chronic Granulomatous Disease is one of the classic primary immunodeficiencies of childhood. While the incidence and severity of bacterial and fungal infections have been greatly reduced in this patient population, much remains to be learned about the pathophysiology of the disease, particularly for autoinflammatory manifestations. In this review, we examine the epidemiology, pathophysiology, and genetic basis for CGD.
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746674/considerations-in-the-diagnosis-of-chronic-granulomatous-disease
#18
Joyce E Yu, Antoine E Azar, Hey J Chong, Artemio M Jongco, Benjamin T Prince
Chronic granulomatous disease (CGD) is a rare primary immunodeficiency that is caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. The disease presents in most patients initially with infection, especially of the lymph nodes, lung, liver, bone, and skin. Patients with CGD are susceptible to a narrow spectrum of pathogens, and Staphylococcus aureus, Burkholderia cepacia complex, Serratia marcescens, Nocardia species, and Aspergillus species are the most common organisms implicated in North America...
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29745101/simulations-of-centriole-of-polarized-centrosome-as-a-monopole-antenna-in-immune-and-viral-synapses
#19
Josef Dvorak, Bohuslav Melichar, Alzbeta Filipova, Jana Grimova, Nela Grimova, Aneta Rozsypalova, David Buka, Rene Voboril, Radek Zapletal, Tomas Buchler, Igor Richter, David Buka
The immune synapse (IS) is a temporary interface between an antigen-presenting cell and an effector lymphocyte. Viral synapse is a molecularly organized cellular junction that is structurally similar to the IS. Primary cilium is considered as a functional homologue of the IS due to the morphological and functional similarities in architecture between both micotubule structures. It has been hypothesized that endogenous electromagnetic field in the cell is generated by a unique cooperating system between mitochondria and microtubules...
March 2018: Journal of B.U.ON.: Official Journal of the Balkan Union of Oncology
https://www.readbyqxmd.com/read/29744952/individualized-immunoglobulin-treatment-in-pediatric-patients-with-primary-humoral-immunodeficiency-disease
#20
REVIEW
Niraj C Patel
Primary immunodeficiency diseases (PIDD) are a group of genetic conditions that are generally considered to be underdiagnosed, and gaps may exist in the knowledge of treatment options. This review focuses on the diagnosis of pediatric patients with primary antibody deficiency and considerations for treatment with immunoglobulin (IgG) to optimize multiple dosing variables and minimize adverse events. The possibility of individualizing IgG therapy in clinical practice represents, in this field, the next pivotal step with the goal of improving the quality of life of pediatric patients with PIDD...
May 9, 2018: Pediatric Allergy and Immunology
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