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primary immundeficiency

Justyna Rak, Katie Foster, Katarzyna Potrzebowska, Mehrnaz Safaee Talkhoncheh, Natsumi Miharada, Karolina Komorowska, Therese Torngren, Anders Kvist, Åke Borg, Lena Svensson, Dominique Bonnet, Jonas Larsson
Adhesion is a key component of hematopoietic stem cell regulation mediating homing and retention to the niche in the bone marrow. Here, using an RNA interference screen, we identify cytohesin 1 (CYTH1) as a critical mediator of adhesive properties in primary human cord blood-derived hematopoietic stem and progenitor cells (HSPCs). Knockdown of CYTH1 disrupted adhesion of HSPCs to primary human mesenchymal stroma cells. Attachment to fibronectin and ICAM1, 2 integrin ligands, was severely impaired, and CYTH1-deficient cells showed a reduced integrin β1 activation response, suggesting that CYTH1 mediates integrin-dependent functions...
February 23, 2017: Blood
Eda Kepenekli, Ahmet Soysal, Canan Kuzdan, Nezih Onur Ermerak, Mustafa Yüksel, Mustafa Bakır
Invasive aspergillosis is an important cause of morbidity and mortality in immunocompromised patients. Among primary immunodefiencies, chronic granulomatous disease (CGD) has the highest prevalence of invasive fungal diseases. Voriconazole is recommended for the primary treatment of invasive aspergillosis in most patients. In patients whose aspergillosis is refractory to voriconazole, therapeutic options include changing class of antifungal, for example using an amphotericin B formulation, an echinocandin, combination therapy, or further use of azoles...
2014: Italian Journal of Pediatrics
Alessandro Aiuti, Maria Grazia Roncarolo
Gene therapy with hematopoietic stem cells (HSC) is an attractive therapeutic strategy for several forms of primary immunodeficiencies. Current approaches are based on ex vivo gene transfer of the therapeutic gene into autologous HSC by vector-mediated gene transfer. In the past decade, substantial progress has been achieved in the treatment of severe combined immundeficiencies (SCID)-X1, adenosine deaminase (ADA)-deficient SCID, and chronic granulomatous disease (CGD). Results of the SCID gene therapy trials have shown long-term restoration of immune competence and clinical benefit in over 30 patients...
2009: Hematology—the Education Program of the American Society of Hematology
M Schlaak
Lymphocytes can transform into blasts. This basic biological property is the prerequisite for many reactions of the cellular immune system. Therefore, lymphocyte transformation tests appear to be a valuable diagnostic approach to define primary and secondary immundeficiency associated with different diseases. The validity of the test system is based on a standardization wich can be achieved sufficiently with regard to culture conditions and measurement of DNA-synthesis. But for phytomitogen-induced lymphocyte transformation a cooperative system of lymphocytes and macrophages is necessary...
July 1975: Immunität und Infektion
O Sauer, G Spelger
The main features of Dubowiz syndrome are bird headed dwarfism and a typical facial configuration. Including two own patients 13 cases among 8 siblings are known in the literature. Two own cases of Dubowitz syndrome in two sisters are described, one of them with hypogammaglobulinemia and neuroblastoma, the other one with complete Ig A deficiency and malignant lymphoma. A propable relationship between immundeficiency and malignancies is discussed. Until 1973 151 malignant tumors with primary immundeficiency had been registrated...
October 1977: Monatsschrift Für Kinderheilkunde
R J Haas, D Niethammer, M Dietrich, E Kleihauer
Immunological responses are divided into those mediated by humoral antibody (B-Cells) and those by cells (T-Cells). Both depend upon the activity of small lymphocytes which become thymus or bursa analogue dependent during embryonic development. Primary immundeficiencies are therefor defects of lymphocytes attributed in most cases to gene defects. Early diagnosis is the necessary prerequisit for possible treatment. Tests for assessing the cellular immune status are: skin testes and in vitro tests. Clinical syndroms are: lymphocytopenia, hypoplasia, deficiency of lymphocytes in lymphatic tissues normal cortical germinal centres...
November 1975: Klinische Pädiatrie
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