TY - JOUR JO - Central European Journal of Immunology SN - 1426-3912 VL - 30 IS - 3 PY - 2005 ID - Wolska-Kuśnierz2005 TI - Twenty five years of investigations into primary immunodeficiency diseases in the Department of Immunology, The Children’s Memorial Health Institute, Warsaw AB - 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. AU - Wolska-Kuśnierz, Beata AU - Pac, Małgorzata AU - Pietrucha, Barbara AU - Heropolitańska-Pliszka, Edyta AU - Klaudel-Dreszler, Maja AU - Kurenko-Deptuch, Magdalena AU - Gregorek, Hanna AU - Michałkiewicz, Jacek AU - Piątosa, Barbara AU - Bernatowska, Ewa SP - 3 EP - 4 DA - 2005 UR - 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 ER -