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1

Uremic autonomic neuropathy - pathogenesis and diagnosis

100%
Rozentryt P., Stryjewski D., Zembala M.
Postępy Higieny i Medycyny Doświadczalnej
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1995
|
vol. 49
|
issue 5
671-689
EN
Autonomic impairment is associated with poor prognosis in many diseases.The pathogenesis of the uremic autonomic neuropathy has not been convincingly established, so the article reviews available information on factors involved in its development, futhermore the autors put forward their own hypothetical scheme of the pathogenesis.The last section outlines the methods most commonly used for its diagnosis and finally the autors discuss symptoms suggestive of this neuropathy in clinical setting.
2

Prevention and treatment of uremic autonomic neuropathy

100%
Rozenryt P., Stryjewski D., Zembala M.
Postępy Higieny i Medycyny Doświadczalnej
|
1995
|
vol. 49
|
issue 6
767-782
EN
The autonomic impairment, which is the frequent complication of end-stage renal failure, may eventually trigger numerous cripping consequences.It is doubtful if standard dialysis techiques may effectively treat once developed autonomic lesions.Renal transplantation seems to be much more efficient.The article points out the discrepancies in symptoms attributable to autonomic impairment and results of autonomic testing, discussed also differences between somatic and autonomic nervous system involvement in uremic patients.The one of the most important part of the article describes the practical approach to the patient with severely symptomatic uremic autonomic neuropathy.Finally the autors articulate their proposals for further research on uremic autonomic neuropathy.
3

Biological activity of dendritic cells generated from cord blood CD34+ hematopoietic progenitors in IL-7- and IL-13-conditioned cultures

100%
Mytar B., Stec M., Weglarczyk K., Zembala M.
Archivum Immunologiae et Therapiae Experimentalis
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2009
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vol. 59
|
issue 1
67-74
EN
Introduction: Dendritic cells (DCs) are required for initiation of the immune response and may therefore be used for the production of cancer vaccines. As mature DCs (mDCs) are the most potent antigen-presenting cells, there is increasing interest in generating them ex vivo. The present study was designed to obtain mDCs from CD34+ hematopoietic progenitors by culturing them in different media. Materials and Methods: Cord blood CD34+ hematopoietic progenitors were expanded for 7 days in FST medium containing fms-related tyrosine kinase 3 ligand (Flt3-L), stem cell factor (SCF), and thrombopoietin (TPO). Then the cells were divided into three parts and cultured for 21 days in different media: FST medium or FST enriched in interleukin (IL)-3 (FST3 medium) or supplemented with IL-7 and IL-13 (FST713 medium). At the end of culture part of the cells was harvested, counted, and analyzed while the other part was matured with proinflammatory cytokines for 2 days. The cells' phenotypes, ability to induce proliferation of allogeneic lymphocytes in the mixed lymphocyte reaction (allo-MLR), chemotaxis, phagocytosis, and O2? production were determined. Results: The average fold increase of DCs at the end of culture in FST medium was 127, in FST3 1043, and in FST713 71. In comparison with the other media, FST713 medium supported the generation of mDCs that were characterized by higher expressions of CD83, costimulatory molecules, and HLA-DR, enhanced ability to induce allo-MLR and migration to macrophage inflammatory protein (MIP) 3?, poor phagocytosis, and O2? production. Conclusions: This study indicates that FST713 medium allows the generation of limited numbers of more mature DCs, while FST3 medium leads to the production of immature DCs in high numbers.
4

Familial occurrence of warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome

64%
Siedlar M., Rudzki Z., Strach M., Trzyna E., Pituch-Noworolska A., Blaut-Szlosarczyk A., Bukowska-Strakova K., Lenart M., Grodzicki T., Zembala M.
Archivum Immunologiae et Therapiae Experimentalis
|
2008
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vol. 56
|
issue 6
419-425
EN
Introduction: Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a rare immunodeficiency disorder with an autosomal-dominant pattern of inheritance and low fatality rate but significant lifelong morbidity. Materials and Methods: A 27-year-old mother of two children has been suffering from severe neutropenia and recurrent infections with the diagnosis of sporadic WHIM established by sequencing the CXCR4 gene and the finding of a heterozygous 1000 CT nonsense mutation in the second CXCR4 exon. The first child was an apparently healthy boy delivered at full term. Umbilical cord blood cells were obtained for genetic analysis. Peripheral blood cells were also analyzed at 8 months of life. Both analyses revealed the same mutation as that of his mother. The child was in a good condition, manifesting neutropenia without infections until 11 months of life. He subsequently developed pneumonia requiring a more aggressive treatment. After that, the regular substitution of immunoglobulins (IVIGs) and G-CSF has been preventing serious infections. Six months ago the second boy was delivered who also demonstrated neutropenia without severe infections. Genetic studies using cord blood and also peripheral blood cells in the fourth month showed an identical mutation of the CXCR4 gene as in his mother. Moreover, the mother and her first son demonstrated monocytopenia. Results: The results indicate that genetic defects connected with WHIM syndrome may influence not only the granulocyte, but also the monocytic lineage. Moreover, a perinatal diagnosis of WHIM syndrome made by sequencing the CXCR4 gene should be performed in cases where either parent is known to be affected with this disease. Conclusions: This would facilitate an earlier detection of the deficiency in children, thereby allowing a more comprehensive follow-up and administration of appropriate therapy.
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