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https://www.readbyqxmd.com/read/29790481/antiviral-combination-therapy-for-cytomegalovirus-infection-in-high-risk-infants
#1
Surabhi B Vora, Adam W Brothers, Alpana Waghmare, Janet A Englund
BACKGROUND: Cytomegalovirus (CMV) infection is a major risk factor for mortality in infants with severe combined immunodeficiency (SCID) and other profound immune defects. Specific antiviral therapy must be initiated early and aggressively because of the potential for antiviral resistance, rapid dissemination and poor transplant outcomes. Combination antiviral therapy is routinely administered for some viral infections, but the value of this approach for the treatment of CMV is unclear...
May 23, 2018: Antiviral Therapy
https://www.readbyqxmd.com/read/29787435/nhej-contributes-to-the-fast-repair-of-radiation-induced-dna-double-strand-breaks-at-late-prophase-i-telomeres
#2
Emad A Ahmed, Michael Rosemann, Harry Scherthan
Exposure of cells to ionizing radiation induces DNA double-strand breaks. To repair double-strand breaks correctly, cells must distinguish between the ends of chromosomes (telomeres) and DNA double-strand breaks within chromosomes. Double-strand breaks in telomeric DNA may lead to telomere shortening and mutagenesis. Eukaryotic cells repair double-strand breaks primarily by two mechanisms: error-free homologous recombination and error-prone nonhomologous end joining, of which homologous recombination is used in early meiotic prophase I to create recombined haploid gametes by two meiotic cell divisions lacking an intervening S-phase...
July 2018: Health Physics
https://www.readbyqxmd.com/read/29784015/induction-of-multiple-myeloma-cancer-stem-cell-apoptosis-using-conjugated-anti-abcg2-antibody-with-epirubicin-loaded-microbubbles
#3
Fangfang Shi, Miao Li, Jing Wang, Di Wu, Meng Pan, Mei Guo, Jun Dou
BACKGROUND: Multiple myeloma (MM) currently remains largely incurable. Cancer stem cells (CSCs) are believed to be responsible for drug resistance and eventual relapse. In this study, we exploited a novel agent to evaluate its inhibitory effect on MM CSCs. METHODS: Epirubicin (EPI)-loaded lipid microbubbles (MBs) conjugated with anti-ABCG2 monoclonal antibody (EPI-MBs + mAb) were developed and their effect on MM 138- CD34- CSCs isolated from human MM RPMI 8226 cell line plus ultrasound exposure in vitro and in vivo in a nonobese diabetic/severe combined immunodeficient mouse model were assessed...
May 21, 2018: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29783708/following-acute-encephalitis-semliki-forest-virus-is-undetectable-in-the-brain-by-infectivity-assays-but-functional-virus-rna-capable-of-generating-infectious-virus-persists-for-life
#4
Rennos Fragkoudis, Catherine M Dixon-Ballany, Adrian K Zagrajek, Lukasz Kedzierski, John K Fazakerley
Alphaviruses are mosquito-transmitted RNA viruses which generally cause acute disease including mild febrile illness, rash, arthralgia, myalgia and more severely, encephalitis. In the mouse, peripheral infection with Semliki Forest virus (SFV) results in encephalitis. With non-virulent strains, infectious virus is detectable in the brain, by standard infectivity assays, for around ten days. As we have shown previously, in severe combined immunodeficient (SCID) mice, infectious virus is detectable for months in the brain...
May 18, 2018: Viruses
https://www.readbyqxmd.com/read/29779669/synthesis-and-profiling-of-benzylmorpholine-1-2-4-5-tetraoxane-analogue-n205-towards-tetraoxane-scaffolds-with-potential-for-single-dose-cure-of-malaria
#5
Paul M O' Neill, Paul A Stocks, Sunil Sabbani, Natalie L Roberts, Richard K Amewu, Emma R Shore, Ghaith Aljayyoussi, Iñigo Angulo-Barturén, María Belén, Jiménez-Díaz, Santiago Ferrer Bazaga, María Santos Martínez, Brice Campo, Raman Sharma, Susan A Charman, Eileen Ryan, Gong Chen, David M Shackleford, Jill Davies, Gemma L Nixon, Giancarlo A Biagini, Stephen A Ward
A series of aryl carboxamide and benzylamino dispiro 1,2,4,5-tetraoxane analogues have been designed and synthesized in a short synthetic sequence from readily available starting materials. From this series of endoperoxides, molecules with in vitro IC50s versus Plasmodium falciparum (3D7) as low as 0.84 nM were identified. Based on an assessment of blood stability and in vitro microsomal stability, N205 (10a) was selected for rodent pharmacokinetic and in vivo antimalarial efficacy studies in the mouse Plasmodium berghei and Plasmodium falciparum Pf3D70087/N9 severe combined immunodeficiency (SCID) mouse models...
May 17, 2018: Bioorganic & Medicinal Chemistry
https://www.readbyqxmd.com/read/29778502/hematopoietic-stem-cell-transplantation-for-progressive-combined-immunodeficiency-and-lymphoproliferation-in-activated-pi3k-delta-syndrome-type-1
#6
Tsubasa Okano, Kohsuke Imai, Yuki Tsujita, Noriko Mitsuiki, Kenichi Yoshida, Chikako Kamae, Kenichi Honma, Kanako Mitsui-Sekinaka, Yujin Sekinaka, Tamaki Kato, Katsuyuki Hanabusa, Eri Endo, Takehiro Takashima, Haruka Hiroki, Tzu-Wen Yeh, Keisuke Tanaka, Masakazu Nagahori, Ikuya Tsuge, Yuki Bando, Fuminori Iwasaki, Yoshiaki Shikama, Masami Inoue, Tomiko Kimoto, Naohiko Moriguchi, Yuki Yuza, Takashi Kaneko, Kyoko Suzuki, Tomoyo Matsubara, Yoshihiro Maruo, Tomoaki Kunitsu, Tomoko Waragai, Hideki Sano, Yuko Hashimoto, Kazuhiro Tasaki, Osamu Suzuki, Toshihiko Shirakawa, Motohiro Kato, Toru Uchiyama, Masataka Ishimura, Tetsuzo Tauchi, Hiroshi Yagasaki, Shiann-Tarng Jou, Hsin-Hui Yu, Hirokazu Kanegane, Sven Kracker, Anne Durandy, Daiei Kojima, Hideki Muramatsu, Taizo Wada, Yuzaburo Inoue, Hidetoshi Takada, Seiji Kojima, Seishi Ogawa, Osamu Ohara, Shigeaki Nonoyama, Tomohiro Morio
BACKGROUND: Activated phosphatidylinositol-3-OH kinase-delta (PI3Kδ) syndrome type 1 (APDS1) is a recently described primary immunodeficiency syndrome characterized by recurrent respiratory infections, lymphoid hyperplasia, and herpesviridae infections due to germline gain-of-function mutations of PIK3CD. Hematopoietic stem cell transplantation (HSCT) may be considered to ameliorate progressive immunodeficiency and associated malignancy, but appropriate indications, method, and outcomes of HSCT for APDS1 remain undefined...
May 17, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29761856/autoimmune-thyroiditis-following-hla-matched-sibling-hematopoietic-stem-cell-transplantation-for-wiskott-aldrich-syndrome
#7
Tristan Knight, Katherine Kotz, Süreyya Savaşan
WAS is a fatal X-linked combined immunodeficiency syndrome, the only cures for which are HSCT or gene therapy. AID occur in up to 72% of patients with WAS who do not receive HSCT, likely arising secondary to impaired multilineage immune autoregulatory function; AITD is not typically seen. In this article, we describe the case of a male patient who underwent HLA-matched HSCT for WAS at the age of 5 months, with his sister (a WAS carrier) acting as his donor and subsequently developed AITD 12 months post-transplant, with marked elevation of antithyroid peroxidase antibody titer...
May 15, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29757593/severe-combined-immunodeficiency-a-case-series-and-review-from-a-tertiary-pediatric-hospital
#8
Shahrzad Fallah, Mehrnaz Mesdaghi, Mahboubeh Mansouri, Delara Babaei, Abdollah Karimi, Seyed Alireza Fahimzad, Shahnaz Armin, Sedigheh Rafiei Tabatabaei, Roxana Azma, Ghamartaj Khanbabaee, Bahram Bashardoost, Mehrdad Amirmoeini, Saeed Sadr, Rozita Jalilianhasanpour, Roxana Ghanaei, Nima Rezaei, Zahra Chavoshzadeh
Severe combined immunodeficiency syndrome (SCID) is a life-threatening condition leading to early infant death as a result of severe infection, due to impaired cellular and humoral immune systems. Various forms of SCID are classified based on the presence or absence of T cells, B cells and natural killer cells. Patients usually present with recurrent infections and failure to thrive. Definitive treatment is hematopoietic stem cell transplantation. To achieve the best outcome, it should be performed prior to the development of severe infection...
April 2018: Iranian Journal of Allergy, Asthma, and Immunology
https://www.readbyqxmd.com/read/29753156/antiviral-t-cells-for-adenovirus-in-the-pre-transplant-period-a-bridge-therapy-for-severe-combined-immunodeficiency
#9
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/29750890/impact-of-age-on-disease-progression-and-microenvironment-in-oral-cancer
#10
V K Vincent-Chong, H DeJong, L J Rich, A Patti, M Merzianu, P A Hershberger, M Seshadri
Despite the recognized link between aging and cancer, most preclinical studies in experimental tumor models are conducted with 6- to 8-wk-old rodents. The goal of the present study was to examine the impact of age on tumor incidence, growth, and microenvironmental characteristics in mouse models of head and neck squamous cell carcinoma (HNSCC). Experimental studies were conducted with the 4-nitroquinoline-oxide (4NQO) oral carcinogenesis model and orthotopic FaDu HNSCC xenografts, established in young (7 to 12 wk of age) and old (65 to 70 wk of age) female C57BL/6 mice ( n = 44; 4NQO model) and severe combined immunodeficient mice ( n = 13; HNSCC xenografts)...
May 1, 2018: Journal of Dental Research
https://www.readbyqxmd.com/read/29750804/temporal-compartmental-changes-in-viral-rna-and-neuronal-injury-in-a-primate-model-of-neuroaids
#11
R Gilberto González, Robert Fell, Julian He, Jennifer Campbell, Tricia H Burdo, Patrick Autissier, Lakshmanan Annamalai, Faramarz Taheri, Termara Parker, Jeffrey D Lifson, Elkan F Halpern, Mark Vangel, Eliezer Masliah, Susan V Westmoreland, Kenneth C Williams, Eva-Maria Ratai
Despite the advent of highly active anti-retroviral therapy HIV-associated neurocognitive disorders (HAND) continue to be a significant problem. Furthermore, the precise pathogenesis of this neurodegeneration is still unclear. The objective of this study was to examine the relationship between infection by the simian immunodeficiency virus (SIV) and neuronal injury in the rhesus macaque using in vivo and postmortem sampling techniques. The effect of SIV infection in 23 adult rhesus macaques was investigated using an accelerated NeuroAIDS model...
2018: PloS One
https://www.readbyqxmd.com/read/29750285/updates-in-newborn-screening
#12
Farrah Rajabi
Newborn screening in the United States is an important public health measure to provide early detection for specified disorders when early treatment is both possible and beneficial. As technology improves, newborn screening can be offered for many more conditions. In the past 10 years, screening has expanded to include severe combined immunodeficiency, congenital heart disease, lysosomal storage disease, and X-linked adrenoleukodystrophy. This article reviews the current state of newborn screening with updates on recent developments...
May 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29749587/newborn-screening-for-severe-combined-immunodeficiency
#13
REVIEW
John Routes, James Verbsky
PURPOSE OF REVIEW: This review provides a brief history of newborn screening (NBS) for severe combined immunodeficiency (SCID), discusses the theoretical basis for the T cell receptor excision circle (TREC) assay, highlights the results of recent studies using the TREC, and provides practical advice for the evaluation of infants with an abnormal TREC assay. RECENT FINDINGS: Currently, all but three states perform NBS for SCID in the USA. NBS using the TREC assay is highly sensitive in identifying infants with SCID and may also identify infants with T cell lymphopenia due to other causes such as congenital syndromes, multiple congenital anamolies, and some combined immunodeficiencies...
May 10, 2018: Current Allergy and Asthma Reports
https://www.readbyqxmd.com/read/29749435/spag6-silencing-induces-apoptosis-in-the-myelodysplastic-syndrome-cell-line-skm%C3%A2-1-via-the-pten-pi3k-akt-signaling-pathway-in-vitro-and-in-vivo
#14
Jiaxiu Yin, Xinxin Li, Zaili Zhang, Xiaohua Luo, Li Wang, Lin Liu
Apoptosis is a multi-step mechanism of cell self‑destruction for maintaining cellular homeostatic balance. Accumulating evidence indicates that abnormal apoptosis promotes the evolution and progression of myelodysplastic syndromes (MDS). As a novel cancer-testis antigen, sperm‑associated antigen 6 (SPAG6) has been reported to regulate apoptosis through the tumor necrosis factor-related apoptosis-inducing ligand signaling pathway in the MDS cell line SKM‑1. However, the mechanism of the intrinsic cell death pathway for apoptosis induction by SPAG6 silencing is unclear...
May 2, 2018: International Journal of Oncology
https://www.readbyqxmd.com/read/29742062/global-perspective-of-novel-therapeutic-strategies-for-the-management-of-neuroaids
#15
Swatantra Kumar, Vimal K Maurya, Himanshu R Dandu, Madan Lb Bhatt, Shailendra K Saxena
Among Human immunodeficiency virus (HIV) infected individuals, around two-thirds of patients present with neuroAIDS, where HIV-associated neurocognitive disorders (HAND), and HIV-associated dementia (HAD) are the most prevailing neurological complications. The neuropathology of neuroAIDS can be characterized by the presence of HIV infected macrophages and microglia in the brain, with the formation of multinucleated giant cells. Global predominant subtypes of HIV-1 clade B and C infections influence the differential effect of immune and neuronal dysfunctions, leading to clade-specific clinical variation in neuroAIDS patient cohorts...
May 8, 2018: Biomolecular Concepts
https://www.readbyqxmd.com/read/29739091/different-biological-characteristics-of-macrophages-from-balb-c-mice-and-severe-combined-immunodeficient-mice
#16
Chong Luo, Yu Zhang, Xuemei Tang, Lan Yao, Yating Wang, Xiqiang Yang
BACKGROUND: Our previous research has found that absence of lymphocytes during development may affect innate activity of immune cells. To better understand the effect of lymphocyte defects on macrophages during development, we compared the biological characteristics of peritoneal macrophages (PMa) from BALB/c and severe combined immunodeficient (SCID) mice. METHODS: Cytokines such as TNF-α, IL-6, and IL-10 produced by PMa and mRNA of those cytokines of PMa, phagocytosis of chicken red cells by PMa, and cell surface markers of PMa from BALB/c and SCID mice were tested...
April 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29737242/bms-493-modulates-retinoic-acid-induced-differentiation-during-expansion-of-human-hematopoietic-progenitor-cells-for-islet-regeneration
#17
Ruth Marise Elgamal, Gillian I Bell, Sarah Krause, David A Hess
Cellular therapies are emerging as a novel treatment strategy for diabetes. Thus, the induction of endogenous islet regeneration in situ represents a feasible goal for diabetes therapy. Umbilical cord blood (UCB)-derived hematopoietic progenitor cells (HPC) isolated by high aldehyde dehydrogenase activity (ALDH<sup>hi</sup>) have previously been shown to reduce hyperglycemia after intrapancreatic (iPan) transplantation into streptozotocin (STZ)-treated non-obese diabetic (NOD) severe combined immunodeficient (SCID) mice...
May 8, 2018: Stem Cells and Development
https://www.readbyqxmd.com/read/29737200/human-peripheral-blood-mononuclear-cells-incubated-in-vasculogenic-conditioning-medium-dramatically-improve-ischemia-reperfusion-acute-kidney-injury-in-mice
#18
Takayasu Ohtake, Shuzo Kobayashi, Shimon Slavin, Yasuhiro Mochida, Kunihiro Ishioka, Hidekazu Moriya, Sumi Hidaka, Ryo Matsuura, Maki Sumida, Daisuke Katagiri, Eisei Noiri, Kayoko Okada, Hiroshi Mizuno, Rica Tanaka
Acute kidney injury (AKI) is a major clinical problem that still has no established treatment. We investigated the efficacy of cultured human peripheral blood mononuclear cells (PBMNCs) for AKI. Ischemia/reperfusion injury (IRI) was used to induce AKI in male nonobese diabetic (NOD/severe combined immunodeficiency) mice aged 7 to 8 wk. PBMNCs were isolated from healthy volunteers and were subjected to quality and quantity controlled (QQc) culture for 7 d in medium containing stem cell factor, thrombopoietin, Flt-3 ligand, vascular endothelial growth factor, and interleukin 6...
January 1, 2018: Cell Transplantation
https://www.readbyqxmd.com/read/29731008/molecular-classification-of-primary-immunodeficiencies-of-t-lymphocytes
#19
William A Comrie, Michael J Lenardo
Proper regulation of the immune system is required for protection against pathogens and preventing autoimmune disorders. Inborn errors of the immune system due to inherited or de novo germline mutations can lead to the loss of protective immunity, aberrant immune homeostasis, and the development of autoimmune disease, or combinations of these. Forward genetic screens involving clinical material from patients with primary immunodeficiencies (PIDs) can vary in severity from life-threatening disease affecting multiple cell types and organs to relatively mild disease with susceptibility to a limited range of pathogens or mild autoimmune conditions...
2018: Advances in Immunology
https://www.readbyqxmd.com/read/29728406/b-cell-differentiation-and-il-21-response-in-il2rg-jak3-scid-patients-after-hematopoietic-stem-cell-transplantation
#20
Alexandra M Miggelbrink, Brent R Logan, Rebecca H Buckley, Roberta E Parrott, Christopher C Dvorak, Neena Kapoor, Hisham Abdel-Azim, Susan E Prockop, David Shyr, Hélène Decaluwe, Imelda C Hanson, Alfred Gillio, Blachy J Dávila Saldaña, Hermann Eibel, Gregory Hopkins, Jolan E Walter, Jennifer S Whangbo, Donald B Kohn, Jennifer M Puck, Morton J Cowan, Linda M Griffith, Elie Haddad, Richard J O'Reilly, Luigi D Notarangelo, Sung-Yun Pai
Allogeneic hematopoietic stem cell transplant (HSCT) typically results in donor T cell engraftment and function in patients with severe combined immunodeficiency (SCID), but humoral immunity, particularly when using donors other than matched siblings, is variable. B cell function after HSCT for SCID depends on the genetic cause, the use of pre-HSCT conditioning and whether donor B cell chimerism is achieved. Patients with defects in IL2RG or JAK3 undergoing HSCT without conditioning often have poor B cell function post-HSCT, perhaps due to impairment of IL-21 signaling in host-derived B cells...
May 4, 2018: Blood
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