Publications (14) View all
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Article: Elevated serum BAFF levels in patients with autoimmunity and lymphoproliferation.
Scandinavian Journal of Immunology 07/2011; 74(5):518-9. · 2.23 Impact Factor -
Article: Characterization of lymphocyte subsets in patients with common variable immunodeficiency reveals subsets of naive human B cells marked by CD24 expression.
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ABSTRACT: Increased proportions of naive B cell subset and B cells defined as CD27(neg)CD21(neg)CD38(neg) are frequently found in patients with common variable immunodeficiency (CVID) syndrome. Current methods of polychromatic flow cytometry and PCR-based detection of κ deletion excision circles allow for fine definitions and replication history mapping of infrequent B cell subsets. We have analyzed B cells from 48 patients with CVID and 49 healthy controls to examine phenotype, frequency, and proliferation history of naive B cell subsets. Consistent with previous studies, we have described two groups of patients with normal (CVID-21norm) or increased (CVID-21lo) proportions of CD27(neg)CD21(neg)CD38(neg) B cells. Upon further analyses, we found two discrete subpopulations of this subset based on the expression of CD24. The B cell subsets showed a markedly increased proliferation in CVID-21lo patients as compared with healthy controls, suggesting developmental arrest rather than increased bone marrow output. Furthermore, when we analyzed CD21(pos) naive B cells, we found two different subpopulations based on IgM and CD24 expression. They correspond to follicular (FO) I and FO II cells previously described in mice. FO I subset is significantly underrepresented in CVID-21lo patients. A comparison of the replication history of naive B cell subsets in CVID patients and healthy controls implies refined naive B cell developmental scheme, in which human transitional B cells develop into FO II and FO I. We propose that the CD27(neg)CD21(neg)CD38(neg) B cells increased in some of the CVID patients originate from the two FO subsets after loss of CD21 expression.The Journal of Immunology 11/2010; 185(11):6431-8. · 5.79 Impact Factor -
Article: Multicenter survey on the outcome of transplantation of hematopoietic cells in patients with the complete form of DiGeorge anomaly.
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ABSTRACT: Seventeen patients transplanted with hematopoietic cells to correct severe T lymphocyte immunodeficiency resulting from complete DiGeorge anomaly were identified worldwide, and retrospective data were obtained using a questionnaire-based survey. Patients were treated at a median age of 5 months (range, 2-53 months) between 1995 and 2006. Bone marrow was used in 11 procedures in 9 cases: 6 from matched unrelated donors, 4 from human leukocyte antigen (HLA)-identical siblings, and one haploidentical parent with T-cell depletion. Unmobilized peripheral blood was used in 8 cases: 5 from HLA-identical siblings, one from a matched unrelated donor, one from an HLA-identical parent, and one unrelated matched cord blood. Conditioning was used in 5 patients and graft-versus-host disease prophylaxis in 11 patients. Significant graft-versus-host disease occurred in 9 patients, becoming chronic in 3. Median length of follow-up was 13 months, with transplantation from HLA-matched sibling showing the best results. Median survival among deceased patients (10 patients) was 7 months after transplantation (range, 2-18 months). The overall survival rate was 41%, with a median follow-up of 5.8 years (range, 4-11.5 years). Among survivors, median CD3 and CD4 counts were 806 (range, 644-1224) and 348 (range, 225-782) cells/mm(3), respectively, CD4(+)/CD45RA(+) cells remained very low, whereas mitogen responses were normalized.Blood 09/2010; 116(13):2229-36. · 9.90 Impact Factor -
Article: Prolonged impairment of polymorphonuclear cells functions in one infant with transient zinc deficiency: a case report.
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ABSTRACT: Zinc is an essential trace element for the immune system. The zinc deficiency diminishes antibody- and cell-mediated responses in man. Lymphopenia and thymic atrophy are usually the early hallmarks of zinc deficiency. Surprisingly, only scarce data are available about polymorphonuclear cells (PMNs) functions in infants with zinc deficiency. We present the results of immunological analyses in one infant with transient zinc deficiency due to decreased zinc concentration in mother milk resulting in severe lactogenic acrodermatitis enteropathica. Nine repeated examination of oxidative burst of PMNs and immunoglobulin levels using nitroblue tetrazolium dye test, chemiluminescence, flow cytometry and nephelometry were performed in the infant with severe zinc deficiency during 28 months period. The unusual prolonged but transient impairment of PMNs respiratory burst accompanied with hypogammaglobulinaemia developed since the age of 2.5 months. Dramatic improvement of the skin was observed within days with total resolution of skin lesions on the 9th day of zinc therapy, but decreased PMNs respiratory burst persisted until the age of 23 months. We conclude that zinc deficiency may lead to prolonged impairment of polymorphonuclear cells functions and hypogammaglobulinaemia.Prague medical report 02/2008; 109(2-3):184-93. -
Conference Proceeding: Evaluation of health care related web resources based on web citation analysis and other quality criteria
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ABSTRACT: Automatic system for ranking of educational health care related Web resources based on quality criteria was developed. Automatic retrieval of new Web resources from well-known directories followed by their automatic evaluation is a keystone of the system. The system provides this information: a) location of the resource and its author, b) number of back links to the resource, c) checks presence of electronic labels of quality. Currently the system encompasses more than 500 links to educational Web resources divided into 47 medical specialities. The resources in all categories are ranked according to their link popularity, the electronic labels of quality are presented. History of link popularity is recorded and might be displayed at every resource. There are 2 language editions. The system operates automatically, the editors check and correct the retrieved values. The described system adds to Website indexing criteria for objective evaluation of quality of Webpages. It is useful for the selection of optimum education resources in health careEngineering in Medicine and Biology Society, 2005. IEEE-EMBS 2005. 27th Annual International Conference of the; 02/2006
