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P617 Primary immunodeficiencies (PID) of the child: about 16 cases

Introduction Primary immunodeficiency disorders (PIDs) in children are genetic diseases that affect adaptive or innate immunity. The importance and nature of the clinical signs depend on the deficit function predominantly within the immune system. Objective To study the epidemiological, clinical, paraclinical, therapeutic and evolutionary characteristics of PID in children. Patients and methods A retrospective study of 16 cases of primary immunodeficiencies (PID) of the child collected at the pediatric emergency and resuscitation departmt Hedi Chaker University Hospital Sfax over a period of 15 years (2004–2019). Results 14 boys and 2 girls, mean age 3 years (range 2 months to 10 years). A notion of consanguinity was present in 15 cases. The respiratory manifestations were revealing in 9 cases. Five patients had impaired expression of HLA class II molecules, 4 patients had agammaglobulinemia, 5 patients had chronic septic granulomatous disease, 2 patients had hyper IgM syndrome and one patient had ataxia telangiectasia. Therapeutically all our patients have benefited from symptomatic treatment based on veinoglobulins. The outcome was favorable in 6 patients, one patient had hematopoietic stem cell transplantation, 4 patients had frequent hospitalisations for infections, and 5 patients had died. Conclusion A better knowledge and characterization of the different immunodeficiencies allows an improvement of the care of these patients both preventive and curative, adapted to the deficit, as well as a more precise genetic advice.