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  • 1
    Article
    Article
    Language: Japanese
    In: Nihon rinsho. Japanese journal of clinical medicine, October 2014, Vol.72(10), pp.1861-9
    Description: GATA2 is a transcription factor that is involved in the lympho-hematopoiesis. Mutations of GATA2 cause MonoMAC syndrome (monocytopenia and mycobacterial infections)/DCML deficiency (dendritic cell, monocyte, B and natural killer (NK) lymphoid deficiency), Emberger syndrome (lymphoedema with MDS), and MDS/AML. In this review, we explain the new function of GATA2, and describe the clinical phenotypes, laboratory findings, pathology, genetic anomalies and etiology.
    Keywords: Gata2 Transcription Factor -- Deficiency
    ISSN: 0047-1852
    Source: MEDLINE/PubMed (U.S. National Library of Medicine)
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  • 2
    Language: English
    In: PLoS ONE, 01 January 2017, Vol.12(5), p.e0176957
    Description: We recently reported that the combination of the neutrophil-to-lymphocyte ratio (NLR) and the platelet-to-lymphocyte ratio (PLR) is a novel and useful predictor of intravenous immunoglobulin (IVIG)-resistance in Kawasaki disease (KD). In the present study, to evaluate the effectiveness of the new risk score, we compared its predictive validity to that of previously reported risk scores.The laboratory records of 437 patients with KD before IVIG therapy were retrospectively analyzed, and the IVIG-responsive (n = 344) and IVIG-resistant (n = 93) patients were compared. The validity of the new score (the combination of NLR≥3.83 and PLR≥150) for predicting IVIG resistance in KD was compared to that of the Kobayashi, Egami and Sano risk scores.The new score and the Kobayashi score displayed high sensitivity (0.72 and 0.70 respectively) and specificity (0.67 and 0.68 respectively), while the Egami and Sano scores showed high specificity (0.71 and 0.81 respectively) but relatively low sensitivity (0.56 and 0.45 respectively). The odds ratios (ORs) for the new score, the Kobayashi score, the Egami score and the Sano score were 5.34 (95% confidence interval [CI] 3.22-8.85), 4.87 (95% CI 2.96-8.01), 3.14 (95% CI 1.96-5.03) and 3.53 (95% CI 2.17-5.77) respectively.The predictive validity of the combination of NLR≥3.83 and PLR≥150, which is a simple and convenient indicator, was equal to or higher than that of the other risk scores. This suggests that the new score could be a widely available marker for predicting IVIG resistance in KD.
    Keywords: Sciences (General)
    E-ISSN: 1932-6203
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  • 3
    Language: English
    In: The Journal of Pediatrics, November 2016, Vol.178, pp.281-284.e1
    Description: The laboratory records of 405 patients with Kawasaki disease before and after intravenous immunoglobulin (IVIG) therapy were compared between the IVIG-responsive (n = 320) and IVIG-resistant (n = 85) groups. A high neutrophil-to-lymphocyte ratio and a high platelet-to-lymphocyte ratio before IVIG, especially when combined, were useful predictors for IVIG resistance in Kawasaki disease.
    Keywords: Coronary Artery Abnormality ; White Blood Cell ; Medicine
    ISSN: 0022-3476
    E-ISSN: 1097-6833
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  • 4
    In: Circulation, 2011, Vol.124(25), pp.2822-2828
    Description: BACKGROUND—: Markedly activated neutrophils or higher plasma levels of neutrophil elastase are involved in the poor response to intravenous immunoglobulin (IVIG) and the formation of coronary artery lesions (CAL) in patients with acute Kawasaki disease. We hypothesized that ulinastatin (UTI), by both direct and indirect suppression of neutrophils, would reduce the occurrence of CAL. METHODS AND RESULTS—: We retrospectively analyzed the clinical records of patients with Kawasaki disease between 1998 and 2009. Three hundred sixty-nine patients were treated with a combination of UTI, aspirin, and IVIG as an initial treatment (UTI group), and 1178 were treated with a conventional initial treatment, and IVIG with aspirin (control group). The baseline characteristics did not demonstrate notable differences between the two groups. The occurrence of CAL was significantly lower in the UTI group than in the control group (3% versus 7%; crude odds ratio [OR], 0.46; 95% confidence interval [CI], 0.25–0.86; P=0.01). The OR adjusted for sex, Gunma score (the predictive score for IVIG unresponsiveness), and dosage of initial IVIG (1 or 2 g/kg) was 0.32 (95% CI, 0.17–0.60; P〈0.001). In addition, most CAL occurred in patients requiring additional rescue treatment and the proportion of those patients was significantly lower in the UTI group than in the control group (13% versus 22%; crude OR, 0.52; 95% CI, 0.38–0.73; P〈0.001). The adjusted OR was 0.30 (95% CI, 0.20–0.44; P〈0.001). CONCLUSIONS—: UTI was associated with fewer patients requiring additional rescue treatment and reduction of CAL in this retrospective study.
    Keywords: Medicine ; Anatomy & Physiology;
    ISSN: 0009-7322
    ISSN: 15244539
    E-ISSN: 15244539
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  • 5
    In: Acta Pædiatrica, December 2010, Vol.99(12), pp.1759-1760
    Keywords: Diagnosis, Differential–Isolation & Purification ; Encephalitis, Viral–Complications ; Fatal Outcome–Diagnosis ; Female–Virology ; Herpesvirus 7, Human–Drug Therapy ; Humans–Drug Therapy ; Infant–Drug Therapy ; Roseolovirus Infections–Drug Therapy ; Shock, Hemorrhagic–Drug Therapy ; Status Epilepticus–Drug Therapy ; Syndrome–Drug Therapy;
    ISSN: 0803-5253
    E-ISSN: 1651-2227
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  • 6
    In: Acta Pædiatrica, December 2010, Vol.99(12), pp.1908-1909
    ISSN: 0803-5253
    ISSN: Acta Paediatrica
    E-ISSN: 1651-2227
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  • 7
    Language: English
    In: Biophysical Journal, 31 January 2012, Vol.102(3), pp.672a-672a
    Keywords: Biology
    ISSN: 0006-3495
    E-ISSN: 1542-0086
    Source: ScienceDirect Journals (Elsevier)
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  • 8
    Language: English
    In: The Journal of Allergy and Clinical Immunology, August 2014, Vol.134(2), pp.411-419.e1
    Description: The molecular mechanism of class-switch recombination (CSR) in human subjects has not been fully elucidated. The CSR-induced mutations occurring in the switch region of the IgM gene (Smu-SHMs) in CSR-activated and switched B cells have been analyzed in mice but not in human subjects. We sought to better characterize the molecular mechanism of CSR in human subjects. Smu-SHMs were analyzed and by using healthy control subjects and patients with molecularly defined CSR defects. We found that Smu-SHMs can be induced by means of CSR activation in human subjects. We also found large amounts of Smu-SHMs in class-switched memory B cells, smaller (although significant) amounts in unswitched memory B cells, and very low amounts in naive B cells. In class-switched memory B cells a high frequency of Smu-SHMs was found throughout the Smu. In unswitched memory B cells, the Smu-SHM frequency was significantly decreased in the 5′ part of the Smu. The difference between switched and unswitched B cells suggests that the extension of somatic hypermutation (SHM) to the 5′ upstream region of the Smu might be associated with the effective induction of CSR. The analysis of the pattern of mutations within and outside the WRCY/RGYW (W, A/T; R, A/G; and Y, C/T) motifs, as well as the Smu-SHMs, in CD27 B cells from CD40 ligand (CD40L)–, activation-induced cytidine deaminase (AID)–, and uracil-DNA glycosylase (UNG)–deficient patients revealed the dependence of Smu-SHM on CD40L, AID, UNG, and the mismatch repair system in human subjects. CD40L-, AID-, UNG-, and mismatch repair system–dependent Smu-SHMs and extension to the 5′ region of Smu are necessary to accomplish effective CSR in human subjects.
    Keywords: Activation-Induced Cytidine Deaminase ; Somatic Hypermutation ; Antibody Maturation ; Uracil-DNA Glycosylase ; Class-Switch Recombination ; Immunoglobulin Switch Region ; Medicine
    ISSN: 0091-6749
    E-ISSN: 1097-6825
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  • 9
    Language: English
    In: The Journal of Allergy and Clinical Immunology, October 2015, Vol.136(4), pp.1018-1024
    Description: The long-term outcome of X-linked hyper-IgM syndrome (XHIM) caused by mutations in is poor, and the only curative treatment is hematopoietic stem cell transplantation (HSCT). We sought to determine the clinical features and factors affecting outcomes in patients with XHIM. We enrolled and retrospectively analyzed data from 56 Japanese patients with XHIM, including 29 patients who received HSCT. The long-term survival rate was poor in those not undergoing HSCT (overall survival rate at 40 years of age, 28.2%). The overall survival rate of patients undergoing HSCT (n = 29) was significantly higher than that of those not undergoing HSCT (n = 27,  = .0231). Moreover, event-free and disease-free survival rates were significantly greater in patients 5 years old or younger at the time of transplantation (n = 14) than in older patients (n = 15). On the basis of these results, we concluded that HSCT improved the outcomes of patients with XHIM and that an age of 5 years or younger was optimal for the timing of HSCT because persistent infections and severe organ damage were frequently observed in patients older than 6 years.
    Keywords: Cd40 Ligand ; Hematopoietic Stem Cell Transplantation ; Pneumocystis Jirovecii ; Cryptosporidium Parvum ; Cryptococcus Neoformans ; Primary Immunodeficiency ; Hyper-Igm Syndrome ; Combined Immunodeficiency ; Class-Switch Recombination ; Medicine
    ISSN: 0091-6749
    E-ISSN: 1097-6825
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  • 10
    In: PLoS ONE, 2014, Vol.9(1)
    Description: Galectin-9 (Gal-9), a lectin having a β-galactoside-binding domain, can induce apoptosis of Th1 cells by binding to TIM-3. In addition, Gal-9 inhibits IgE/Ag-mediated degranulation of mast cell/basophilic cell lines by binding to IgE, thus blocking IgE/Ag complex formation. However, the role of Gal-9 in mast cell function in the absence of IgE is not fully understood. Here, we found that recombinant Gal-9 directly induced phosphorylation of Erk1/2 but not p38 MAPK in a human mast cell line, HMC-1, which does not express FcεRI. Gal-9 induced apoptosis and inhibited PMA/ionomycin-mediated degranulation of HMC-1 cells. On the other hand, Gal-9 induced cytokine and/or chemokine production by HMC-1 cells, dependent on activation of ERK1/2 but not p38 MAPK. In addition, the lectin activity of Gal-9 was required for Gal-9-mediated cytokine secretion by HMC-1 cells. These observations suggest that Gal-9 has dual properties as both a regulator and an activator of mast cells.
    Keywords: Research Article ; Biology ; Medicine
    E-ISSN: 1932-6203
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