Severe Combined Immunodeficiency (SCID)

3254  relevant articles (120 outcomes, 292 trials/studies) found for this Disease

SCID; Bare Lymphocyte Syndrome; Immunodeficiency Syndrome, Severe Combined; Immunologic Deficiency, Severe Combined; Omenn's Syndrome; Reticuloendotheliosis, Familial; Severe Combined Immunodeficiency Syndrome; Severe Combined Immunologic Deficiency; Bare Lymphocyte Syndromes; Combined Immunodeficiencies, Severe; Combined Immunodeficiency, Severe; Familial Reticuloendothelioses; Familial Reticuloendotheliosis; Immunodeficiencies, Severe Combined; Lymphocyte Syndrome, Bare; Lymphocyte Syndromes, Bare; Omenns Syndrome; Reticuloendothelioses, Familial; Severe Combined Immunodeficiencies; Syndrome, Bare Lymphocyte; Syndrome, Omenn; Syndrome, Omenn's; Syndromes, Bare Lymphocyte; Immunodeficiency, Severe Combined; Omenn Syndrome

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Severe Combined Immunodeficiency (SCID)
2.	Neoplasms (Cancer)
3.	Infection
4.	Leukemia
5.	Neoplasm Metastasis (Metastasis)

Experts

1.	Yamamoto, Naoki: 5 articles (04/2007 - 05/2003)
2.	Buckley, Rebecca H: 4 articles (04/2007 - 02/2002)
3.	Abinun, M: 4 articles (07/2003 - 05/2000)
4.	Aiuti, Alessandro: 3 articles (07/2007 - 09/2004)
5.	Gaspar, H Bobby: 3 articles (05/2007 - 10/2004)
6.	Peiris, J S M: 3 articles (04/2007 - 03/2005)
7.	Chen, Pei-Jer: 3 articles (04/2007 - 03/2005)
8.	Tashiro, Masato: 3 articles (04/2007 - 03/2005)
9.	Nomura, Tatsuji: 3 articles (04/2007 - 03/2005)
10.	Sakatani, Mitsunori: 3 articles (04/2007 - 03/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Severe Combined Immunodeficiency:

1.	Adenosine DeaminaseIBA 01/01/1999 - "Gene therapy for severe combined immunodeficiency caused by adenosine deaminase deficiency: improved retroviral vectors for clinical trials." 02/01/2003 - "Children born with a life-threatening immune system disorder, severe combined immune deficiency (SCID), were cured after receiving gene therapy for replacement of their defective adenosine deaminase (ADA) gene. " 08/15/1999 - "In vivo kinetics of transduced cells in peripheral T cell-directed gene therapy: role of CD8+ cells in improved immunological function in an adenosine deaminase (ADA)-SCID patient." 12/01/1993 - "Severe combined immunodeficiency of reduced severity due to homozygosity for an adenosine deaminase missense mutation (Arg253Pro)." 06/01/2007 - "In humans, adenosine deaminase deficiency is linked to severe combined immunodeficiency and as such the enzyme has been approved for the first gene therapy trial. " Order ALL the reference details at left...
2.	AntibodiesIBA 06/01/2009 - "The protective efficacy of these antibodies was determined by injecting the absorbed serum into severe combined immunodeficiency mice, which were then challenged with highly pathogenic H5N1 virus (A/Vietnam/Jp1203/2004; Japanese isolate of A/Vietnam/1203/2004). " 01/01/2007 - "Also, as shown here, the scid-adapted MARV mouse models can be used to evaluate the efficacy of candidate antiviral therapeutic molecules, such as phosphorodiamidate morpholino oligomers or antibodies." 01/01/1997 - "To explore and prevent such resistance, we treated severe combined immunodeficiency mice (SCID) with anti-NK antibodies and analysed the improved engraftment of stem cells induced by this treatment. " 08/15/2004 - "In a pharmacokinetics study, the blood concentrations of the radiolabeled antibodies decreased with time similarly in SCID/NOD wild-type and SCID/NOD FcRgamma-/- mice. " 10/15/1992 - "The long term production of anti-mRBC antibodies and the modulation of the expressed xenoreactive repertoire observed in hu-PBL-SCID represents an opportunity to study the molecular genetics and cell biology of the human humoral immune response to a defined complex Ag." Order ALL the reference details at left...
3.	Immunotoxins (Immunotoxin)IBA 09/10/2004 - "Severe combined immunodeficiency (SCID) mice challenged with CD30-positive L540Cy cells were treated with the immunotoxin. " 02/01/2003 - "Treatment of SCID/human B cell precursor ALL with anti-CD19 and anti-CD22 immunotoxins." 09/01/1998 - "The use of an anti-CD3 immunotoxin to prevent the development of lymphoproliferative disease in SCID/PBL mice." 12/01/1994 - "To test the antitumor efficacy of these immunotoxins in vivo, we developed a disseminated human neuroblastoma model in severe combined immunodeficiency mice. " 05/01/1992 - "In vivo efficacy of B43 (anti-CD19)-pokeweed antiviral protein immunotoxin against human pre-B cell acute lymphoblastic leukemia in mice with severe combined immunodeficiency." Order ALL the reference details at left...
4.	Immunoglobulin G (IgG)IBA 02/01/1996 - "Results in SCID and MuMT mice demonstrate an important role for B cells or IgG in clearance of reovirus from the intestines. " 04/15/1997 - "Circulating human IgG was cleared 6-fold more rapidly in NOD/LtSz-scid/scid B2m(null) mice than in NOD/LtSz-scid/scid mice. " 12/01/2003 - "The purpose of this study was to evaluate the effect on the cardiac structure and function by the transfer of immunoglobulin G (IgG) and/or lymphocytes from rabbits immunized with a synthetic peptide corresponding to the sequence of the second extracellular loop of beta1-adrenoceptor (beta peptide) into severe combined immunodeficiency (SCID) mice. " 04/15/1997 - "This rapid IgG clearance suggested a failure of the engrafted human lymphoid cells to maintain high circulating levels of human IgG. The higher levels of human CD4+ T cells and the normalization of the CD4:CD8 ratio that are observed in human PBMC-engrafted NOD/LtSz-scid/scid B2m(null) mice suggest that this system may be an excellent model for studies of HIV pathogenesis." 06/24/1994 - "In this study, we report the development and characterization of an in vivo model for testing this parameter using SCID mice containing a physiological concentration of human IgG (hu-IgG-SCID). " Order ALL the reference details at left...
5.	purineIBA 12/01/2009 - " trials represented the first demonstration of long-term clinical efficacy of HSC gene therapy for ADA-SCID, underlining that gene therapy has a favorable safety profile and is effective in restoring normal purine metabolism and immune functions. " 01/01/2009 - "Recent studies have demonstrated that gene therapy for ADA-SCID has favorable safety profile and is effective in restoring normal purine metabolism and immune functions. " 04/15/2008 - "Overall, our findings provide a better understanding of the pathogenesis of the immune defects associated with an altered purine metabolism and confirm that ADA gene transfer is an efficacious treatment for ADA-SCID. " 12/01/1993 - "The development of these drugs emanated from an enhanced understanding of purine metabolism in lymphocytes and the mechanism of lymphocytotoxicity in severe combined immunodeficiency disease. " 01/01/1986 - "Purine enzyme activities as markers of lymphocytic differentiation: studies of lymphocytes from horses with severe combined immunodeficiency (SCID)." Order ALL the reference details at left...
6.	rituximab (Mabthera)FDA Link 12/01/2003 - "Recent studies performed in severe combined immunodeficiency (SCID) mouse models suggest that in vivo rituximab-associated ADCC is mediated via the FcgammaRIII receptor on effector cells. " 07/01/2006 - "In severe combined immunodeficient (SCID)-Ramos mice the combination of rituximab + BU12-SAPORIN was significantly better therapeutically than either single agent. " 01/01/2008 - "In combination with rituximab, we have previously demonstrated that IMiDs have synergistic in vivo anti-tumour activity in preclinical studies in a human lymphoma severe combined immunodeficiency mouse model. " 11/01/2007 - "An in vivo model of human CD20+ B-lymphoma was established in severe combined immunodeficiency mice to test the ability of human neutralizing miniantibodies to CD55 and CD59 (MB55 and MB59) to enhance the therapeutic effect of rituximab. " 08/01/2007 - "Herein, we describe the case of a 23-month-old male child with Omenn's syndrome, who had undergone BMT and was successfully treated with rituximab (375 mg/m(2) intravenously, weekly for three times) for refractory post-BMT hemolytic anemia. " Order ALL the reference details at left...
7.	Interleukin-10 (Interleukin 10)IBA 08/25/1997 - "This study confirms that IL-10 is dependent on NK activity, since no therapeutic effect is seen in C.B-17/IcrCrl-SCID/Beige mice which lack T, B, and NK cell function. " 08/01/2001 - "Human myeloid dendritic cells transduced with an adenoviral interleukin-10 gene construct inhibit human skin graft rejection in humanized NOD-scid chimeric mice." 12/15/2000 - "Moreover, injection of IL-10-deficient NOD splenocytes into transgenic IL-10-NOD.scid/scid mice elicited accelerated disease, demonstrating that pancreatic IL-10 but not endogenous IL-10 is sufficient for the acceleration of diabetes. " 08/01/1999 - "IL-10 transgenic mice present a defect in T cell development reminiscent of SCID patients." 04/01/1997 - "A role for T cells in the early mortality of IL-10 -/- mice was suggested by experiments in which SCID IL-10 -/- mice infected with T. " Order ALL the reference details at left...
8.	Interleukin-3 (Interleukin 3)IBA 09/15/1999 - "Clinically, IL-3 may therefore be useful treatment for X-linked and Jak3-deficient SCID patients who lack bone marrow donors." 12/01/2004 - "The protocol based on the T/F/S + IL-3 combination was included in a gene therapy clinical trial for ADA-SCID, resulting in long-term engraftment of stem/progenitor cells. " 01/01/2001 - "Interleukin 3 improves the ex vivo expansion of primitive human cord blood progenitor cells and maintains the engraftment potential of scid repopulating cells." 12/01/2004 - "Moreover, the use of IL-3 or IL-7 significantly improved the maintenance of in vitro B cell progenitors from ADA-SCID BM cells and allowed the efficient transduction of B and NK cell progenitors. " 12/01/2004 - "IL-3 or IL-7 increases ex vivo gene transfer efficiency in ADA-SCID BM CD34+ cells while maintaining in vivo lymphoid potential." Order ALL the reference details at left...
9.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/1992 - "In mice given cyclophosphamide followed by human TILs i.p., the TILs were demonstrated at 7 days in the SCID peritoneum (leu 4 = 4%) and at 2 days in the SCID spleen (leu 4 = 2%). " 07/01/2001 - "In attempts to further assess the origins and evolution of these LDVs we have determined their competitiveness by monitoring their fate in mixed infections of wild type, SCID, nude, and cyclophosphamide-treated mice by reverse transcription/polymerase chain reaction assays that distinguish between them. " 02/01/1991 - "BP-3+ reticular cells were observed in the collapsed periarterial lymphatic sheaths of adult mice depleted of T and B cells by cyclophosphamide treatment and in mice with severe combined immunodeficiency (scid), indicating that development of this reticular network is lymphocyte independent. " 04/01/2003 - "Equitoxic doses of busulfan (ALL-SCID 4, 7, 19) or cyclophosphamide (ALL-SCID 19) were less effective with regard to the numbers of complete regressions and the number of cured animals. " 06/01/1999 - "In both groups of patients, the use of busulfan (8 mg/kg total dose) and cyclophosphamide (200 mg/kg total dose) as a conditioning regimen provided the best cure rate (74% for patients with B+ SCID and 43% for patients with B- SCID, respectively), although results were not statistically significantly different from other regimens. " Order ALL the reference details at left...
10.	matrigelIBA 10/01/2007 - "To enhance safety, these cells were implanted subcutaneously in a Matrigel scaffold, and the efficacy of this strategy was compared with i.v. delivery of engineered BOECs in nonhemophilic nonobese diabetic/severe combined immunodeficiency mice. " 04/01/2004 - "Finally, studies in nonobese diabetic/severe combined immunodeficiency (NOD/SCID) mice demonstrated that human circulating EPCs are able to colonize a Matrigel plug. " 02/01/2009 - "Moreover, the peptide 4.10 inhibited the in vitro endothelial cell motility and organization into capillary-like structures, and the in vivo angiogenesis of human umbilical cord-derived endothelial cells implanted in Matrigel in severe combined immunodeficiency mice. " 12/01/2005 - "Moreover, these cells colonized a Matrigel plug in immunodeficient mice (NOD/SCID). " 11/01/1998 - "To test the in vivo activity of the expanded CIK cells, CML was engrafted into severe combined immunodeficiency disease (SCID) mice using matrigel. " Order ALL the reference details at left...

Therapies and Procedures

1.	Transplantation (Transplant Recipients) 05/07/2009 - "Although this in part reflects that postnatal transplantations have overall given good results, there are no direct comparisons between IUHCT and postnatal transplantations of X-SCID. " 02/15/2003 - "FINDINGS: In SCID, 3-year survival with sustained engraftment was significantly better after HLA-identical than after mismatched transplantation (77% vs 54%; p=0.002) and survival improved over time. " 01/01/2009 - "Moreover, the B6 mArt(-/-) mouse provides a more accurate model for the human disease and a more appropriate system for studying human Artemis deficiency and for developing improved transplantation and gene therapy regimens for the treatment of children with SCID." 01/01/2003 - "The only effective treatment for SCID since 1968 is a hematopoetic stem cells (HSC) transplantation. " 11/01/2009 - "METHODS: In the present study we reviewed the medical records of these 23 consecutive, mostly conditioned patients with SCID who underwent transplantation between 1998 and 2007. " Order ALL the reference details at left...
2.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 01/01/1992 - "Significant improvement in the therapy of SCIDs has been made in the last 10 years leading to cure of at least 3/4 patients with SCID by either HLA identical or non identical bone marrow transplantation. " 07/01/2008 - "Long-term outcomes of bone marrow transplantation for severe combined immunodeficiencies confirmed the efficacy of this therapy." 01/01/2007 - "Uniformly fatal before 1968, SCID was first cured by allogeneic bone marrow transplantation (BMT). " 01/01/2000 - "The aim was to determine the efficacy of T cell depleted bone marrow transplantation (TCD BMT) in the treatment of children with SCID. " 02/18/1999 - "BACKGROUND: Since 1968 it has been known that bone marrow transplantation can ameliorate severe combined immunodeficiency, but data on the long-term efficacy of this treatment are limited. " Order ALL the reference details at left...
3.	Transplants (Transplant) 01/01/2008 - "Survival for SCID following HSCT has significantly improved over the past several decades, and ranges from 70% to 95% depending on the clinical condition of the child at the time of transplant, the availability of an HLA-matched sibling donor, and the SCID genotype/phenotype. " 07/01/2008 - "In order to study the ability of costimulation blockade to induce tolerance to human embryonic stem cells (HESC), severe combined immunodeficient (SCID), and immunocompetent C57BL/6 mice treated with costimulation blockade received intratesticular and intramyocardial HESC transplants. " 06/01/2006 - "Multiple human mesenteric arterial grafts from the same donor to study human chronic vascular rejection in humanized SCID/beige mice." 07/01/2005 - "BACKGROUND: We developed an original experimental model to study chronic vascular rejection (CVR) consisting of a graft of human mesenteric artery followed by human immune reconstitution into CB.17 SCID/Beige mice. " 09/01/2004 - "In this study, we describe the development of an immunodeficient SCID/bg murine model combining a transplanted human synthetic microvascular bed with adoptive transfer of human T lymphocytes allogeneic to the cells of the graft that more fully recapitulates T cell responses in natural tissues. " Order ALL the reference details at left...
4.	Heterologous Transplantation (Xenotransplantation) 10/01/2008 - "Severely immunocompromised NOD/Shi-scid IL2Rg (null) (NOG) mice, which show higher engraftment efficiency, are useful as recipients in xenotransplantation studies. " 10/01/2007 - "A controlled four-group parallel study design comprising 24-40 mice per tumor cell line was used in a severe combined immunodeficiency (SCID)-mouse xenotransplantation model. " 02/01/2006 - "This study was aimed to evaluate the in vivo antitumor effect of genetically modified myeloma cell vaccine on human myeloma xenografts implanted into nonobese diabetic/severe combined immunodeficiency (NOD/SCID) mice. " 03/01/2004 - "In vivo studies were performed in a severe combined immunodeficient (SCID)/Beige mouse xenograft model. " 01/01/2004 - "Previous FV vector marking studies in the NOD/SCID xenotransplantation model used umbilical cord blood (UCB)-derived SCID repopulating cells (SRCs) that were assayed 5-10 weeks posttransplantation. " Order ALL the reference details at left...
5.	Hematopoietic Stem Cell Transplantation 12/01/2007 - "RECENT FINDINGS: Severe combined immunodeficiency is treatable by hematopoietic stem cell transplantation, with best outcome if recognized and treated early in life. " 02/01/2002 - "Hematopoietic stem cell transplantation for severe combined immunodeficiency in the neonatal period leads to superior thymic output and improved survival." 09/01/2006 - "The effective treatment for SCID is a hematopoietic stem cell transplantation (HSCT). " 11/01/2009 - "CONCLUSIONS: Hematopoietic stem cell transplantation in patients with SCID results in engraftment, long-term survival, and a good quality of life for the majority of patients with or without pretransplantation conditioning." 11/01/2009 - "Outcomes of patients with severe combined immunodeficiency treated with hematopoietic stem cell transplantation with and without preconditioning." Order ALL the reference details at left...