eISSN: 1644-4124
ISSN: 1426-3912
Central European Journal of Immunology
Current issue Archive Manuscripts accepted About the journal Special Issues Editorial board Abstracting and indexing Subscription Contact Instructions for authors Publication charge Ethical standards and procedures
Editorial System
Submit your Manuscript
SCImago Journal & Country Rank
3-4/2005
vol. 30
 
Share:
Share:
abstract:

Twenty five years of investigations into primary immunodeficiency diseases in the Department of Immunology, The Children’s Memorial Health Institute, Warsaw

Beata Wolska-Kuśnierz
,
Małgorzata Pac
,
Barbara Pietrucha
,
Edyta Heropolitańska-Pliszka
,
Maja Klaudel-Dreszler
,
Magdalena Kurenko-Deptuch
,
Hanna Gregorek
,
Jacek Michałkiewicz
,
Barbara Piątosa
,
Ewa Bernatowska

(Centr Eur J Immunol 2005; 30 (3-4): 104-114)
Online publish date: 2006/10/10
View full text Get citation
 
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.
keywords:

primary immunodeficiencies, T cells defects, B cells defects, phagocytic defects, haematopoietic stem cell transplantation

Quick links
© 2024 Termedia Sp. z o.o.
Developed by Bentus.