Abstract:

Primary immunodeficiencies which is diagnosed especially in childhood period are important group of the disorders. Clinically they appear as recurrent infections. In our study, 103 patients with primary immunodeficiency syndromes followed in our clinic between January 1997-August 2004 are analyzed retrospectively in terms of age at diagnosis, gender, symptoms at admission, family history, type of immunodeficiency, origin of infections, type of infections, accompanying diseases and complications. The aim was to evaluate in which group of patients primary immunodeficiency syndromes should be suspected and more detailed investigations must be carried out. In this study 37 of 103 patients with primary immunodeficiency were girls, while 66 of them were boys. The average age of patients at diagnosis was 30.4 months. While % 81.2 of all grup is humoral immune deficienc, most frequent diagnosed type of primary immüne deficiencies was selective IgA deficiency (% 42.7). The main observed complaints were: recurrent cough episodes (% 59.2), fever (% 45.6), recurrent wheezing attacks (% 36.9), frequent otitis media (% 34.9), prolonged diarrhea (% 28.2), and recurrent skin infections (8.8 %). Most frequently encountered infections were pneumonia (% 48.5), upper respiratory tract infections (% 46.6), bronchiolitis (% 33.0), otitis media (% 35.0), gastroenteritis (% 28.2) and sinüsitis (% 27.2). In conclusion in a case with recurrent and or complicated sinopulmonary infections; urinary tract infections without an identified cause; chronic diarrhea or recurrent gastroenteritis; infections with unusual or opportunistic organisms; infections in unusual locations; recurrent deep skin or organ absess; extremely serious vaccine reactions; if there were atopy, early infant death or autoimmune disease present in family history of child we should investigate for immune deficiencies.

Keywords: