PedAM
Pediatric Disease Annotations & Medicines
| Disease | severe combined immunodeficiency |
| Phenotype | |severe combined immunodeficiency |
| Sentences | 101 |
| PubMedID- 20594324 | 5-6 weeks old female severe combined immunodeficiency mice (scid) mice were obtained from harlan laboratory (harlan laboratories, inc, indianapolis, in) and maintained under pathogen free conditions in a temperature and humidity controlled animal care facility with a 12 hours light dark cycle. |
| PubMedID- 24069355 | Thus, for the first time we present t-b-nk+ severe combined immunodeficiency (scid) phenotype after spontaneously occurring modification of artemis gene in mice. |
| PubMedID- 20465788 | The spectrum of t cell defects is broad, from severe combined immunodeficiency to signaling defects to specific defects in lymphocyte apoptosis. |
| PubMedID- 23226942 | Such studies are complemented by a pediatric patient with t and b cell severe combined immunodeficiency (scid) that mounted a potent nk cell response to human cytomegalovirus (cmv) [19], indicating that nk cells may provide host protection against viral pathogens in the absence of adaptive immunity. |
| PubMedID- 23738757 | Four to five weeks old non-obese diabetic/severe combined immunodeficiency (nod/scid) male mice were used in all experiments. |
| PubMedID- 21067584 | By means of a non-obese diabetic/severe combined immunodeficiency disease (nod/scid) xenotransplant assay in combination with specific cell surface markers (cd44+cd24-/low), cscs were enriched from metastatic and primary breast tumors and were shown to have the ability to reestablish tumor heterogeneity after transplantation [1]. |
| PubMedID- 25387827 | severe combined immunodeficiency (scid) mice were subcutaneously injected with 6 × 106 m+g or m−g cells in 200 μl of dmem. |
| PubMedID- 25942583 | Sixteen severe combined immunodeficiency (cb17/scid) mice obtained from charles river (wilmington, ma) were housed in an animal care facility and held for 10 days to acclimatize. |
| PubMedID- PMC4407161 | Os characterized by symptoms of severe combined immunodeficiency (scid), in association with the cardinal triad of hepatosplenomegaly, lymphadenopathy and erythroderma. |
| PubMedID- 24723986 | The xenograft tumor volume of the severe combined immunodeficiency (scid) mice after 20 day treatment with the plga nanoparticles formulation combined with 3-methyladenine or chloroquine are found to be only about a half in comparison with the plga nanoparticles formulation only 58. thus, the incorporation of autophagy inhibitor in future theranostic platform, can improve the co-delivery of diagnostic and therapeutic agent. |
| PubMedID- 21233838 | The development of the scid (severe combined immunodeficiency)-hu model has been an important advance, as it was the first model to recapitulate a hubmm in mice.4, 5, 6 however, although the scid-hu system remains a highly relevant model for preclinical investigation, it does have important limitations: (i) restricted availability of human fetal bone chips; (ii) the allogeneic nature of the fetal bm milieu versus mm cells; and (iii) the significant heterogeneity of implanted human bone chips, collected from different individuals at different gestational age, that may produce experimental variability. |
| PubMedID- 26442241 | For example, prior to 2005, severe combined immunodeficiency (scid) could not be included in newborn screening as there was no method of testing for the condition using a dried bloodspot. |
| PubMedID- 24083030 | severe combined immunodeficiency (scid) or nude mice (charles river laboratories, wilmington, ma), approximately 4–6 weeks of age and weighing approximately 30 g received subcutaneous (sc) tumor implants performed using various ratios of gfp expressing sk-n-sh wild type cells (sk-n-sh gfp-wt) and sk-n-sh doxorubicin drug-resistant cells (sk-n-sh doxr) or sk-n-sh hmk (sk-n-sh hmk) cells with a total of 106 cells in 100 μl per implant. |
| PubMedID- 24454751 | Nonobese diabetic/severe combined immunodeficiency (nod/scid) mice (sankyo-lab service, tsukuba, japan) were bred and maintained in accordance with our institutional guidelines for the use of laboratory animals. |
| PubMedID- 19833883 | Mice deficient for nhej factors other than cernunnos/xlf (4) develop severe combined immunodeficiency due to their failure to join dna breaks generated during early lymphoid development in a dna rearrangement process termed v(d)j recombination (5). |
| PubMedID- 25738875 | Six-week-old non-obese diabetic–severe combined immunodeficiency (nod/scid) mice were supplied by the national laboratory animal center, taipei, taiwan, and housed in specific pathogen-free animal rooms. |
| PubMedID- 24198970 | Hyper-ige and wiskott-aldrich syndromes, cd40l deficiency, severe combined immunodeficiency, x-linked agammaglobulinemia, transient hypogammaglobulinemia of infancy, and chronic granulomatous disease were diagnosed in these children. |
| PubMedID- 23318460 | Furthermore 24 severe combined immunodeficiency mice were divided into three groups and in each group, eight mice were injected with mock/293, b7-h4 wt/293 or b7-h4 h250q mt/293 cells, respectively. |
| PubMedID- 24487407 | Five- to six-week-old cb-17 severe combined immunodeficiency (scid) male mice (charles river laboratories international, wilmington, ma, usa) had xenograft tumors induced by injecting 1×106 pc3 cells suspended in 50 μl of pbs subcutaneously in the upper hind legs of the animals. |
| PubMedID- 26086199 | This mutation is also responsible for the severe combined immunodeficiency (scid) phenotype in mice and rats [59]. |
| PubMedID- 26097871 | Six to eight week-old female severe combined immunodeficiency (scid) and balb/c mice were purchased from charles river laboratories (calco, italy), and housed in our specific pathogen free (spf) animal facility. |
| PubMedID- 20226009 | Scid (severe combined immunodeficiency) mice were maintained in a specific pathogen-free environment in compliance with institutional policy and all animal procedures were previously approved by the iacuc (institutional animal care and use committee) at taipei medical university. |
| PubMedID- 26046360 | severe combined immunodeficiency (scid) female mice (harlan, indianapolis, in) received orthotopic pancreatic injections of s2-vp10 metastatic pancreatic adenocarcinoma cells expressing luciferase, resulting in pancreatic tumors within 7 days. |
| PubMedID- 22111066 | Three severe combined immunodeficiency (scid) mice (males, 6 weeks old) were injected with a suspension of 1×109 cells/ml in the back; however, no tumor formation was observed after 6 weeks. |
| PubMedID- 24078865 | They successfully corrected severe combined immunodeficiency (scid)-x1 in 11 children using a mouse leukemia virus-based vector (mlv) as the gene transfer vehicle. |
| PubMedID- 23226053 | This is true of patients with ataxia telangiectasia (at), ataxia telangiectasia-like disorder, severe combined immunodeficiency, ligase iv syndrome, rothmund–thompson syndrome, seckel syndrome, werner syndrome, nijmegen breakage syndrome, all due to defective repair of double-strand breaks (dsbs)5 or stalled replication forks.6 it is also true of patients with fanconi anemia caused by defective repair of dna interstrand crosslinks (icls) and patients with xeroderma pigmentosum due to a defect in nucleotide excision repair (ner) of helix-distorting dna adducts.7,8 since nsclc and hnscc are treated with cisplatin and radiation therapy, it is logical to predict that patients with reduced dsb repair, single-strand break (ssb) repair, icl repair, or ner due to polymorphisms affecting the expression or function of dna repair proteins might be most responsive to dna damaging agents. |
| PubMedID- 20547828 | Affected patients manifest with symptoms of severe combined immunodeficiency (scid), including an increased occurrence of life-threatening infections, failure to thrive, and, in particular, autoimmune-like clinical features including early-onset severe erythrodermia, alopecia, hepato-splenomegaly, and lymphadenopathy (omenn, 1965; ochs et al., 1974). |
| PubMedID- 23056487 | The severe combined immunodeficiency (scid) mice (6–8 weeks old, 18–20 g body weight), bred in-house, were maintained throughout in specific pathogen-free (spf) environment. |
| PubMedID- 23936793 | A mouse model of severe combined immunodeficiency disease (scid) [27] was developed in which conjoint implants of human fetal thymus and liver were placed under the kidney capsule with subsequent infection of hcmv [28]. |
| PubMedID- 23396406 | severe combined immunodeficiency (scid) is a fatal syndrome characterized by the absence of t cells and, in some molecular types, also of b or nk cells.1,2 without immune reconstitution by hematopoietic stem cell transplantation or gene therapy, infants with scid will die in the first two years of life. |
| PubMedID- 23122694 | Characterised traits range from severe combined immunodeficiency syndromes, with specific defects in cellular and humoral immunity, to defects in innate immunity such as impaired tlr signalling (box 1, figure 2). |
| PubMedID- 22174689 | The absence of cd45 leads to a severe combined immunodeficiency (scid) phenotype in humans [31]–[33] and mice [34]–[36]. |
| PubMedID- 23758766 | We used a severe combined immunodeficiency (scid) hiv encephalitis (hive) mouse model in which intracranial injection of clade b hiv-infected mdms leads to neuropathology and neurocognitive defects that parallel those found in had patients [20,21]. |
| PubMedID- 24116047 | Female non-obese diabetic/severe combined immunodeficiency (nod/scid) mice (5 week-old) were purchased (clea japan, inc., osaka, japan), and housed in laminar-flow cabinets under specific pathogen-free conditions. |
| PubMedID- 20427943 | severe combined immunodeficiency (scid) is a primary immunodeficiency disorder with heterogeneous genetic etiologies, characterized by a profound defect in both t and b lymphocytes.12 affected individuals usually present in early infancy with severe and persistent infections.3 without hematopoietic stem cell transplantation (hsct) or gene therapy, most patients die in early childhood.45 transplacentally derived maternal t lymphocytes are frequently detected in healthy newborns; however, they are rapidly eliminated by immune competent t lymphocytes.67 on the contrary, scid infants do not usually reject maternally engrafted cells; therefore maternal t cells were detected in 24% to 40% of patients undergoing hematopoietic stem cell transplantation.89 since these t cells are usually non-functional, they do not alter the course of the disease and patients present typically in early infancy with severe infection.9 we present a typical case of scid masked by a clinically functional maternal t-cell engraftment leading to late presentation of the disease at the age of 9 years with pneumocystis jiroveci pneumonia (pjp) and cytomegalovirus (cmv) infections, probably following exhaustion of maternally engrafted t lymphocytes. |
| PubMedID- 23574525 | On the other hand, genetic deficiency of jak3 does not lead to lethality but causes severe combined immunodeficiency (scid) in humans [45,46] and a corresponding phenotype with severe lymphocyte developmental defects in mice [47,48]. |
| PubMedID- 24076575 | severe combined immunodeficiency due to adenosine-deaminase defect (ada-scid) is usually deadly in childhood because of severe recurrent infections. |
| PubMedID- 20158571 | Eight-week-old severe combined immunodeficiency (scid) mice were treated with streptozotocin (stz, 200 mg/kg, sigma) freshly dissolved in 0.025 m sodium citrate (ph 4.0). |
| PubMedID- 21994645 | Approval of adagen® (pegademase) for the treatment of severe combined immunodeficiency disease (scid) by the u.s. fda in the early 1990s illustrated the potential for pegylation to significantly impact modern therapeutics. |
| PubMedID- 24402744 | Six-week-old female severe combined immunodeficiency (scid)/cb17 mice were purchased from charles river breeding laboratories (calco, italy), housed under specific pathogen-free conditions in the bl2 containment laboratory in our animal facility, and allowed to acclimate to local conditions for 1 week. |
| PubMedID- 23298290 | Hairless (severe combined immunodeficiency) scid or athymic nu/nu (or nude) female mice, 5 to 6 wk old (charles river laboratories, inc., wilmington, ma) were maintained under pathogen-free conditions in hepa-filtered cages under controlled light (12 h light and dark cycle), temperature (22-24°c), and humidity (25%). |
| PubMedID- 22567029 | Children with severe combined immunodeficiency can develop bcgosis after bcg immunization as a result of their deficient immune system, which clearly illustrates that vaccinations should only be performed in immunocompetent individuals [20]. |
| PubMedID- 23226020 | About 2 weeks before the experiment, female beige severe combined immunodeficiency mice were injected subcutaneously into the left flank with 2.5 × 106 u-87 mg cells and tumors were left to grow until they reached a size of about 1 cm in diameter. |
| PubMedID- 23135762 | Because chimeric mice have the characteristic of severe combined immunodeficiency, the viral kinetics in chimeric mice sera during ifn treatment could be contributed by the innate immune response of hcv-infected human hepatocytes. |
| PubMedID- 26495209 | severe combined immunodeficiency beige mice (charles river laboratories, lyon, france) were used for in vivo fat grafting experiments. |
| PubMedID- 23300447 | severe combined immunodeficiency (scid) mice engrafted with human pbmc depleted of nk cells are more susceptible to fatal lymphoproliferation of infused ebv-infected b cells than non-depleted controls, indicating a role for nk cells in preventing outgrowth of infected b cells [35]. |
| PubMedID- 23776540 | Male nonobese diabetic (nod)/severe combined immunodeficiency (scid) mice, 6–8 weeks of age (slac laboratory animal company, shanghai, china), were fed in laminar flow cabinets under specific pathogen-free conditions. |
| PubMedID- 23879992 | Three-week old female non-obese/severe combined immunodeficiency (nod/scid) mice were obtained from jackson labs (bar harbor, me, usa) and maintained in a pathogen-free animal facility. |
| PubMedID- 26151747 | Immunodeficient nonobese diabetous/shi-severe combined immunodeficiency/interleukin-2rgammanull (nsg) mice under 2.5% isoflurane anesthesia (belamont, piramal healthcare, northumberland, uk) were injected subcutaneously into the right flank with 105 ags reporter cells, gc10 or gc06 gastric tumor cells resuspended in 100 μl of 7 mg/ml matrigel (bd biosciences, le pont de claix, france) in ice cold pbs. |
| PubMedID- 24799913 | Hypomorphic variants of rag genes with null mutations cause severe combined immunodeficiency (scid) as was found in an increasing number of patients with combined immunodeficiency [20]. |