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severe combined immunodeficiency

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The text is the summary of recent articles on severe combined immunodeficiency at 75 thresold from National Library of Medicine (NLM). This information is subject to NCBI's Disclaimer and Copyright notice.

TEI from pan-EGFP enteroids was labeled for smooth Muscle alpha actin (SMA) to identify ISEMFs and SMCs and Green fluorescent protein (GFP) to identify Cells from pan-EGFP enteroids [1]. Fluorescence in situ hybridization (FISH) for the Y chromosome was applied to TEI from male LGR5-EGFP enteroids implanted into female nonobese diabetic/severe combined Immunodeficiency mice [2]. FISH of TEI from male LGR5-EGFP enteroids implanted into Female nonobese diabetic/severe combined Immunodeficiency mice revealed that only the epithelium was Y chromosome positive [3]. In vivo, RES-529 intraperitoneal injection significantly inhibited U2OS xenograft Tumor growth in severe combined immunodeficiency (SCID) mice [4]. A self-controlled case series design was applied on a sample of 231,693 Children who completed Newborn screening for severe combined Immunodeficiency in Ontario, Canada between August 2013 and December 2015 [5].

Also, preliminary studies of FMCA/FMCAP in chimeric mice and Female Non-obese diabetic/severe combined Immunodeficiency (NOD/SCID) mouse models having the LMV/ETV triple mutant have shown a high rate of reduction of HBV DNA levels compared to ETV [6]. The osteoblasts derived from CCD-iPSCs (CCD-OBs) expressed low levels of several osteoblast differentiation markers, and transplantation of these osteoblasts into calvarial Bone defects created in rats with severe combined immunodeficiency showed poor regeneration [7]. This article reviews latest developments in HSCT for conventional primary immunodeficiencies and presents data on outcome for emerging diseases, Areas covered: This article reviews recently published literature detailing advances, particularly in conditioning regimens and new methods of T-lymphocyte depletion, as well as new information regarding approach and out come of transplanting patients with conventional primary immunodeficiencies [8]. For in vivo studies, we first employed both ectopic and semi-orthotopic study models using severe combined Immunodeficiency mice [9]. Using autologous sDPSCs carried by hydrogel and transplanted into the mini-swine root canal space, we observed regeneration of vascularized pulp-like tissue with a Layer of newly deposited dentin-like (rD) tissue or osteodentin along the canal walls [10].

Mutations in the gene encoding adenylate kinase 2 (AK2) were identified to cause this phenotype [11]. Um die Fertilität präpubertärer Mädchen zu erhalten, besteht die einzige Option zurzeit aus der Kryokonservierung von Ovargewebe, gefolgt von einer Retransplantation des gefrorenen Eierstockgewebes nach Abschluss der onkologischen Behandlung, um die Fertilität wiederherzustellen [12]. 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 [13]. Three severe combined immunodeficiency mice, grafted with tissue fragments, were followed for 6 months and showed no macroscopic/microscopic signs for leukemia [14]. Like Severe Combined Immunodeficiency Syndrome, Pediatric Heart failure is a rare and life-threatening condition for which organ transplantation is often the only option for survival [15].

Nearly 15 years ago, the Berlin Heart EXCOR ventricular assist Device was developed, as the isolator was for David, to be a bridge to transplantation for Infants and Children with unstable Heart failure [16]. Four patients underwent successful retransplantation for graft loss or poor Immune reconstitution [17]. Both failed newborn screening for severe combined immunodeficiency (SCID) due to T-cell lymphopenia [18]. Primary Immunodeficiency diseases (PID) are a heterogeneous group of inborn errors of immunity [19]. Severe combined immunodeficiency (SCID) is one form of PID which is uniformly fatal without early, definitive therapy, and outcomes are significantly improved if Infants are diagnosed and treated within the first few months of life [20].

Here, we review the early origins of Newborn screening and the evolution of screening methodologies [21]. Cells from each group were injected into non-obese diabetic/severe combined Immunodeficiency mice and their tumorigenicity determined in vivo [22]. These data suggest that PGE2 improves lentiviral transduction and increases vector copy Number, therefore resulting in increased transgene expression [23]. Mutations of the recombination-activating genes RAG 1 and RAG 2 are associated with a range of clinical presentations including, severe combined immunodeficiency and autoimmunity [24]. Male severe combined immunodeficiency (SCID) mice were implanted subcutaneously with LAPC-4 Cells for in vivo studies [25].

In this study, lentiviral dynamics in a host without adaptive immunity were examined in order to determine kinetic parameters of infection and quantify the effect of neutralizing Antibodies in preventing infection, using mathematical modeling of data from equine infectious anemia virus (EIAV) infection of horses with severe combined immunodeficiency (SCID) [26]. The IL2RG gene is located on the X chromosome and is mutated in humans with X-linked severe combined immunodeficiency (XSCID) [27]. γc family cytokines collectively control broad aspects of lymphocyte development, growth, differentiation, and survival, and these cytokines are clinically important, related to allergic and autoimmune diseases and cancer as well as Immunodeficiency [28]. We generated a humanized X-linked severe combined immunodeficiency (SCID-X1) mouse model and evaluated the efficacy and safety of hematopoietic reconstitution from limited input of functional HSPCs, establishing thresholds for full correction upon different types of conditioning [29]. We have applied a liquid chromatography (LC-) and gas chromatography (GC-) Mass spectrometry (MS) metabolomics and lipidomics analysis of blood plasma and pancreas tissue to follow the progression of disease in three models related to autoimmune diabetes: the nonobese diabetic (NOD) mouse, susceptible to the development of autoimmune diabetes, and the NOD-E (transgenic NOD mice that express the I-E heterodimer of the major histocompatibility complex II) and NOD-severe combined Immunodeficiency (SCID) mouse strains, two models protected from the development of diabetes [30].

References: 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 ,

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Bell Raj Eapen
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