%0 Journal Article %J Central European Journal of Immunology %@ 1426-3912 %V 30 %N 3 %D 2005 %F Wolska-Kuśnierz2005 %T Twenty five years of investigations into primary immunodeficiency diseases in the Department of Immunology, The Children’s Memorial Health Institute, Warsaw %X Nine hundred and forty-six cases of primary immunodeficiency diseases were diagnosed in the Department of Immunology of the Children’s Memorial Health Institute in Warsaw during 1980-2006. The highest frequency was that of antibody immunodeficiencies: 547 (57.8%). Predominantly T cell deficiencies were recognised in 80 (8.5%) children. In 29 children severe combined immunodeficiencies with different genetic defects were diagnosed, and in 18 of them hematopoietic stem cell transplantation was done. In the group of phagocytic disorders (95; 10% of patients), neutropenia was the most common - 46 cases, followed by chronic granulomatous diseased recognised in 41 patients. In the group of other well-defined PIDs - immunodeficiencies associated with chromosomal instability were the most common (152; 16% of patients). Ataxia – teleangiectasia was diagnosed in 92 cases and, next, Nijmegen Breakage syndrome in 57 children. Hyper IgE syndrome was diagnosed in 22 patients, and Wiskott-Aldrich syndrome in 19 boys. %A Wolska-Kuśnierz, Beata %A Pac, Małgorzata %A Pietrucha, Barbara %A Heropolitańska-Pliszka, Edyta %A Klaudel-Dreszler, Maja %A Kurenko-Deptuch, Magdalena %A Gregorek, Hanna %A Michałkiewicz, Jacek %A Piątosa, Barbara %A Bernatowska, Ewa %P 3-4 %9 journal article %U https://www.termedia.pl/Twenty-five-years-of-investigations-into-primary-immunodeficiency-diseases-in-the-Department-of-Immunology-The-Children-8217-s-Memorial-Health-Institute-Warsaw,10,6799,1,1.html