keyword
MENU ▼
Read by QxMD icon Read
search

Severe combined immunodeficiency

keyword
https://www.readbyqxmd.com/read/29325489/creating-effective-biocontainment-facilities-and-maintenance-protocols-for-raising-specific-pathogen-free-severe-combined-immunodeficient-scid-pigs
#1
Ellis J Powell, Sara Charley, Adeline N Boettcher, Lisa Varley, Justin Brown, Martine Schroyen, Malavika K Adur, Susan Dekkers, Dean Isaacson, Mary Sauer, Joan Cunnick, N Matthew Ellinwood, Jason W Ross, Jack Cm Dekkers, Christopher K Tuggle
Severe combined immunodeficiency (SCID) is defined by the lack of an adaptive immune system. Mutations causing SCID are found naturally in humans, mice, horses, dogs, and recently in pigs, with the serendipitous discovery of the Iowa State University SCID pigs. As research models, SCID animals are naturally tolerant of xenotransplantation and offer valuable insight into research areas such as regenerative medicine, cancer therapy, as well as immune cell signaling mechanisms. Large-animal biomedical models, particularly pigs, are increasingly essential to advance the efficacy and safety of novel regenerative therapies on human disease...
January 1, 2018: Laboratory Animals
https://www.readbyqxmd.com/read/29319822/an-animal-model-of-abacavir-induced-hla-mediated-liver-injury
#2
Binbin Song, Shigeki Aoki, Cong Liu, Takeshi Susukida, Kousei Ito
Genome-wide association studies indicate that several idiosyncratic adverse drug reactions are highly associated with specific human leukocyte antigen (HLA) alleles. For instance, abacavir, a human immunodeficiency virus reverse transcriptase inhibitor, induces multi-organ toxicity exclusively in patients carrying the HLA-B*57:01 allele. However, the underlying mechanism is unclear due to a lack of appropriate animal models. Previously, we developed HLA-B*57:01 transgenic mice and found that topical application of abacavir to the ears induced proliferation of CD8+ lymphocytes in local lymph nodes...
January 8, 2018: Toxicological Sciences: An Official Journal of the Society of Toxicology
https://www.readbyqxmd.com/read/29316956/next-generation-in-situ-hybridization-approaches-to-define-and-quantify-hiv-and-siv-reservoirs-in-tissue-microenvironments
#3
REVIEW
Claire Deleage, Chi N Chan, Kathleen Busman-Sahay, Jacob D Estes
The development of increasingly safe and effective antiretroviral treatments for human immunodeficiency virus (HIV) over the past several decades has led to vastly improved patient survival when treatment is available and affordable, an outcome that relies on uninterrupted adherence to combination antiretroviral therapy for life. Looking to the future, the discovery of an elusive 'cure' for HIV will necessitate highly sensitive methods for detecting, understanding, and eliminating viral reservoirs. Next-generation, in situ hybridization (ISH) approaches offer unique and complementary insights into viral reservoirs within their native tissue environments with a high degree of specificity and sensitivity...
January 9, 2018: Retrovirology
https://www.readbyqxmd.com/read/29310626/mgat1-is-a-novel-transcriptional-target-of-wnt-%C3%AE-catenin-signaling-pathway
#4
Izzet Akiva, Necla Birgül Iyison
BACKGROUND: The Wnt/β-catenin signaling pathway is an evolutionary conserved pathway, which has important functions in vertebrate early development, axis formation, cellular proliferation and morphogenesis. Additionally, Wnt/β-catenin signaling pathway is one of the most important intracellular pathways that controls cancer progression. To date most of the identified targets of this pathway are shown to harbor tumorigenic properties. We previously showed that Mannosyl glycoprotein acetylglucosaminyl-transferase (MGAT1) enzyme is among the Wnt/β-catenin signaling putative target genes in hepatocellular carcinoma cell lines (Huh7)...
January 8, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29304240/adipose-tissue-derived-stem-cells-in-a-fibrin-implant-enhance-neovascularization-in-a-peritoneal-grafting-site-a-potential-way-to-improve-ovarian-tissue-transplantation
#5
D D Manavella, L Cacciottola, C M Desmet, B F Jordan, J Donnez, C A Amorim, M M Dolmans
STUDY QUESTION: Do two different concentrations of human adipose tissue-derived stem cells (ASCs) embedded inside a fibrin scaffold have the potential to differentiate into vessels and aid vascularization in a peritoneal grafting site intended for ovarian tissue transplantation? SUMMARY ANSWER: Human ASCs in low and high concentrations differentiated into vessels when transplanted to mouse peritoneum inside a fibrin matrix, but only high ASC concentrations significantly increased human vessel area 14 days after transplantation...
January 3, 2018: Human Reproduction
https://www.readbyqxmd.com/read/29301799/posterior-reversible-encephalopathy-syndrome-in-an-hiv-infected-patient-on-antiretroviral-treatment-what-is-the-risk-factor
#6
Barbara Birner, Cédric Hirzel, Franca Wagner, Gabriel Waldegg
Posterior reversible encephalopathy syndrome (PRES) is a rare but well-described syndrome associated with a high morbidity and a substantial mortality. We present an illustrative case of an HIV-infected but virologically suppressed patient who complained of visual impairment accompanied by severe headache and epileptic seizures. The cerebral CT scan and the follow-up cranial MRI confirmed the diagnosis of PRES. Unlike the cases of HIV-infected patients with PRES published so far, our patient suffered neither from advanced immunodeficiency nor from opportunistic infection or from any other evident predisposing factor...
January 4, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29298406/a-miniature-swine-model-for-stem-cell-based-de-novo-regeneration-of-dental-pulp-and-dentin-like-tissue
#7
Xiaofei Zhu, Jie Liu, Zongdong Yu, Chao-An Chen, Hacer Aksel, Adham A Azim, George T-J Huang
The goal of this study was to establish mini-swine as a large animal model for stem cell-based pulp regeneration studies. Swine dental pulp stem cells (sDPSCs) were isolated from mini-swine and characterized in vitro. For in vivo studies, we first employed both ectopic and semi-orthotopic study models using severe combined immunodeficiency mice. One is hydroxyapatite-tricalcium phosphate (HA/TCP) model for pulp-dentin complex formation, and the other is tooth fragment model for complete pulp regeneration with new dentin depositing along the canal walls...
January 3, 2018: Tissue Engineering. Part C, Methods
https://www.readbyqxmd.com/read/29281718/the-burden-of-hepatitis-b-virus-hbv-infection-genotypes-and-drug-resistance-mutations-in-human-immunodeficiency-virus-positive-patients-in-northwest-ethiopia
#8
Tekalign Deressa, Debasu Damtie, Kevin Fonseca, Shan Gao, Ebba Abate, Shitaye Alemu, Yetemwork Aleka, Mark G Swain, Guido van Marle, Carla S Coffin
BACKGROUND: In sub-Saharan Africa, the hepatitis B virus (HBV) and human immunodeficiency virus (HIV) infections are endemic. Although there has been great progress in HIV care, universal HBV vaccination and care is lacking. In this study, we aimed to determine the prevalence of HBV, HBV genotypes, and drug resistance mutations in dual infected cases in a cohort of HIV patients in Northwest Ethiopia. METHODS: A total of 308 HIV-1 positive patients were enrolled into the study and tested for HBsAg in plasma...
2017: PloS One
https://www.readbyqxmd.com/read/29277761/antitumor-effects-of-lidocaine-on-human-breast-cancer-cells-an-in-vitro-and-in-vivo-experimental-trial
#9
Thiên-Nga Chamaraux-Tran, Carole Mathelin, Marc Aprahamian, Girish P Joshi, Catherine Tomasetto, Pierre Diemunsch, Cherif Akladios
AIM: Retrospective studies have suggested a protective effect of regional anesthesia against recurrence after cancer surgery. But confirmation of the in vivo antitumor effects is lacking. We examined the in vitro antitumor effects of lidocaine on various breast cancer cell lines and then assessed these properties in vivo at clinically relevant concentrations. MATERIALS AND METHODS: In vitro experiments: normal breast epithelial cells (NBEC) MCF-10A and three tumor breast epithelial cells (TBEC) lines (MCF-7 luminal A, MDA-MB-231 triple-negative and SKBr3 HER2 positive) were exposed to increasing concentrations of lidocaine...
January 2018: Anticancer Research
https://www.readbyqxmd.com/read/29273275/modulatory-effect-of-voriconazole-on-the-production-of-proinflammatory-cytokines-in-experimental-cryptococcosis-in-mice-with-severe-combined-immunodeficiency
#10
E Gonçalves Silva, S Marilia de Souza Silva, C Rodrigues Paula, L da Silva Ruiz, A Latercia Tranches Dias
Cryptococcosis is a subacute or chronic disease. For many years, amphotericin B has been used in severe fungal infections. Voriconazole is a triazole with high bioavailability, a large distribution volume, and excellent penetration of the central nervous system (CNS). The objective of this study was to evaluate the production of pro-inflammatory cytokines in the lungs during an experimental infection caused by C. neoformans in murine model (SCID) that was treated with amphotericin B and voriconazole. After intravenous inoculation with 3...
December 19, 2017: Journal de Mycologie Médicale
https://www.readbyqxmd.com/read/29259832/gain-of-function-mutations-in-stat1-a-recently-defined-cause-for-chronic-mucocutaneous-candidiasis-disease-mimicking-combined-immunodeficiencies
#11
Sanem Eren Akarcan, Ezgi Ulusoy Severcan, Neslihan Edeer Karaca, Esra Isik, Guzide Aksu, Mélanie Migaud, Ferda Evin Gurkan, Elif Azarsiz, Anne Puel, Jean-Laurent Casanova, Necil Kutukculer
Chronic Mucocutaneous Candidiasis (CMC) is the chronic, recurrent, noninvasive Candida infections of the skin, mucous membranes, and nails. A 26-month-old girl was admitted with the complaints of recurrent oral Candidiasis, diarrhea, and respiratory infections. Candida albicans grew in oral mucosa swab. CMV and EBV DNA titers were elevated. She had hypergammaglobulinemia; IgE level, percentages of lymphocyte subgroups, and in vitro T-cell proliferation responses were normal. She had parenchymal nodules within the lungs and a calcific nodule in the liver...
2017: Case Reports in Immunology
https://www.readbyqxmd.com/read/29241731/efficacy-of-lentiviral-mediated-gene-therapy-in-an-omenn-syndrome-rag2-mouse-model-is-not-hindered-by-inflammation-and-immune-dysregulation
#12
Valentina Capo, Maria Carmina Castiello, Elena Fontana, Sara Penna, Marita Bosticardo, Elena Draghici, Luigi P Poliani, Lucia Sergi Sergi, Rosita Rigoni, Barbara Cassani, Monica Zanussi, Paola Carrera, Paolo Uva, Kerry Dobbs, Nicolò Sacchetti, Luigi D Notarangelo, Niek P van Til, Gerard Wagemaker, Anna Villa
BACKGROUND: Omenn syndrome (OS) is a rare severe combined immunodeficiency associated with autoimmunity, caused by defects of the lymphoid-specific V(D)J recombination. Most patients carry hypomorphic mutations in recombination activating genes (RAG) 1 or 2. Hematopoietic stem cell (HSC) transplantation is the standard treatment, however gene therapy (GT) may represent a valid alternative, especially for patients lacking a matched donor. OBJECTIVE: To determine the efficacy of lentiviral vector (LV) mediated GT in the murine model of OS (Rag2R229Q/R229Q) in correcting immunodeficiency and autoimmunity...
December 11, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29241728/an-actin-cytoskeletal-barrier-inhibits-lytic-granule-release-from-natural-killer-cells-in-chediak-higashi-syndrome
#13
Aleksandra Gil-Krzewska, Mezida B Saeed, Anna Oszmiana, Elizabeth R Fischer, Kathryn Lagrue, William A Gahl, Wendy J Introne, John E Coligan, Daniel M Davis, Konrad Krzewski
BACKGROUND: Chediak-Higashi syndrome (CHS) is a rare disorder caused by biallelic mutations in the LYST gene, resulting in formation of giant lysosomes or lysosome-related organelles in several cell types. The disease is characterized by immunodeficiency and a fatal hemophagocytic lymphohistiocytosis due to impaired function of cytotoxic lymphocytes, mainly Natural Killer (NK) cells. OBJECTIVE: We sought to determine the underlying biochemical cause of the impaired cytotoxicity of NK cells in CHS...
December 11, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29238427/hiv-associated-nephropathy-in-africa-pathology-clinical-presentation-and-strategy-for-prevention
#14
REVIEW
Nazik Elmalaika Husain, Mohamed H Ahmed, Ahmed O Almobarak, Sufian K Noor, Wadie M Elmadhoun, Heitham Awadalla, Clare L Woodward, Dushyant Mital
The human immunodeficiency virus (HIV) infection can lead to progressive decline in renal function known as HIV-associated nephropathy (HIVAN). Importantly, individuals of African ancestry are more at risk of developing HIVAN than their European descent counterparts. An in-depth search on Google Scholar, Medline and PubMed was conducted using the terms "HIVAN" and "pathology and clinical presentation", in addition to "prevalence and risk factors for HIVAN", with special emphasis on African countries for any articles published between 1990 and 2017...
January 2018: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/29233117/elizabethkingia-miricola-as-an-opportunistic-oral-pathogen-associated-with-superinfectious-complications-in-humoral-immunodeficiency-a-case-report
#15
Przemysław Zdziarski, Mariola Paściak, Klaudia Rogala, Agnieszka Korzeniowska-Kowal, Andrzej Gamian
BACKGROUND: Elizabethkingia miricola is a rare Gram-negative bacterium found in water and clinical specimens. Typical culturing methods often misidentify Elizabethkingia spp. as Flavobacterium or Chryseobacterium. Although diagnosis is based on culturing samples taken from sterile sites, such as blood, a proper identification of this bacterium requires an expertise that goes beyond the capabilities of a typical clinical laboratory. CASE PRESENTATION: A 35-year-old woman diagnosed with common variable immunodeficiency was admitted to our center...
December 12, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/29229829/psma-targeted-polyinosine-polycytosine-vector-induces-prostate-tumor-regression-and-invokes-an-antitumor-immune-response-in-mice
#16
Yael Langut, Alaa Talhami, Samarasimhareddy Mamidi, Alexei Shir, Maya Zigler, Salim Joubran, Anna Sagalov, Efrat Flashner-Abramson, Nufar Edinger, Shoshana Klein, Alexander Levitzki
There is an urgent need for an effective treatment for metastatic prostate cancer (PC). Prostate tumors invariably overexpress prostate surface membrane antigen (PSMA). We designed a nonviral vector, PEI-PEG-DUPA (PPD), comprising polyethylenimine-polyethyleneglycol (PEI-PEG) tethered to the PSMA ligand, 2-[3-(1, 3-dicarboxy propyl)ureido] pentanedioic acid (DUPA), to treat PC. The purpose of PEI is to bind polyinosinic/polycytosinic acid (polyIC) and allow endosomal release, while DUPA targets PC cells. PolyIC activates multiple pathways that lead to tumor cell death and to the activation of bystander effects that harness the immune system against the tumor, attacking nontargeted neighboring tumor cells and reducing the probability of acquired resistance and disease recurrence...
December 26, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29228703/dual-inhibition-of-brd4-and-pi3k-akt-by-sf2523-suppresses-human-renal-cell-carcinoma-cell-growth
#17
Hua Zhu, Jia-Hui Mao, Yin Wang, Dong-Hua Gu, Xiao-Dong Pan, Yuxi Shan, Bing Zheng
Bromodomain-containing protein 4 (BRD4) and PI3K-AKT are both important for renal cell carcinoma (RCC) development and progression. SF2523 is a BRD4 and PI3K-AKT dual inhibitor. The present study demonstrated that SF2523 was cytotoxic and anti-proliferative to established RCC cell lines (786-O and A498) and primary human RCC cells. SF2523 induced activation of caspase and apoptosis in RCC cells. Further, SF2523 disrupted RCC cell cycle progression and inhibited cell migration in vitro. At the signaling level, SF2523 in-activated PI3K-AKT-mTOR, and downregulated BRD4-dependent proteins, Bcl-2 and Myc, in RCC cells...
November 17, 2017: Oncotarget
https://www.readbyqxmd.com/read/29225858/fatal-ctla-4-heterozygosity-with-autoimmunity-and-recurrent-infections-a-de-novo-mutation
#18
Maria Francisca Moraes-Fontes, Amy P Hsu, Iris Caramalho, Catarina Martins, Ana Carolina Araújo, Filipa Lourenço, Anna V Taulaigo, Ana Lladó, Steven M Holland, Gulbu Uzel
Primary immunodeficiency disorders are rarely diagnosed in adults but must be considered in the differential diagnosis of combined recurrent infections and autoimmune disease. We describe a patient with CTLA-4 haploinsufficiency and an abnormal regulatory T-cell phenotype. Unusually, infections were more severe than autoimmunity, illustrating therapeutic challenges in disease course.
December 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/29219174/igfbp3-deposited-in-the-human-umbilical-cord-mesenchymal-stem-cell-secreted-extracellular-matrix-promotes-bone-formation
#19
Moyuan Deng, Keyu Luo, Tianyong Hou, Fei Luo, Zhao Xie, Zehua Zhang, Aijun Yang, Bo Yu, Shaoxuan Yi, Tan Jiulin, Shiwu Dong, Jianzhong Xu
The extracellular matrix (ECM) contains rich biological cues for cell recruitment, proliferation and even differentiation. The osteoinductive potential of scaffolds could be enhanced through human bone marrow mesenchymal stem cell (hBMSC) directly depositing extracellular matrix (ECM) on surface of scaffolds. However, the role and mechanism of human umbilical cord MSC-secreted ECM in bone formation remain unknown. We tested the osteoinductive properties of a human umbilical cord MSC-secreted ECM construct (hUCMSC-ECM) in a large femur defect of a severe combined immunodeficiency mouse model...
December 8, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29204384/approach-to-a-child-with-primary-immunodeficiency-made-simple
#20
REVIEW
Dhrubajyoti Sharma, Ankur K Jindal, Amit Rawat, Surjit Singh
Primary immunodeficiency disorders (PIDs) are a group of disorders affecting the capability to fight against infection. These include defects in T cells and B cells affecting cell-mediated and humoral immunity, respectively, combined humoral and cell-mediated immunodeficiency, defects in phagocytosis, complement defects, and defects in cytokine or cytokine signalling pathways which are detrimental for immune function. Depending upon the type and severity, age at onset of symptoms can vary from neonatal period to late childhood...
November 2017: Indian Dermatology Online Journal
keyword
keyword
78039
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"