EVALUATION OF PRIMARY IMMUNE DEFICIENCY DISEASES IN CHILDREN

Erguven M¹, Hatipoglu S², Anıl M¹, Usta M¹, Ozcay S¹

¹ SSK Goztepe Training Hospital, Department of Pediatrics

² Dr Sadi Konuk Training Hospital, Department of Pediatrics

Istanbul, Turkey

Primary immune deficiency diseases (PIDs) are rare but important disorders, diagnosed predominantly in childhood. With the improvement in the immunology the diagnosis of immune deficiency syndromes increase. The diagnosis requires some special analyses. The most important point is the fact that in which patient group we will do these analyses. The aim of the study is to try to point out in which patients PIDs must be thought and which patient requires immunologic evaluation. In our study we evaluate 41 patients who are diagnosed PIDs in our clinic between the periods of January 97 and April 2001. In our study male/female is 4,8. Clinical manifestations are repeatedly fever %51,2, repeatedly wheezing %39,02, chronic diarrhea %29,2, chronic cough %39,02, recurrent otitis media %11,7, cutaneous lesions %9,7. As in other series antibody deficiencies predominated, accounting for %79,5. IgA deficiency is first seen as an antibody deficiency (%29,4). Sinopulmonary infections (%92,8) are the most common proceeding infections. We determined rotavirus (%17,6), giardia lamblia (%17,4), mycobacterium tuberculosis (%17,4) as the common pathogens in our series. Immundeficiency syndromes must be evaluated when children with repeatedly serious infections, infections that are resistant to adequate therapy, etiology of the chronic diseases , recurrent otitis media, diarrhea, cough, patients who have PID diagnosed family members.

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