The aim of this study was to estimate the disease burden of PIDs in a department of general pediatrics in Algiers and to appreciate the trends.
A retrospective single center study conducted in the department of pediatrics at Mustapha Bacha teaching hospital in Algiers. All cases of PIDs seen in our department between January 1st 2003 and June 30th 2019 were enrolled. PIDs were classified according to the International Union of Immunological Societies expert committee for Primary Immunodeficiency.
70 PIDs patients were identified with 40 boys. Mean age at diagnosis was 40 months. Parental consanguinity was found in 38% of cases. Main clinical manifestations were recurrent respiratory infections (55%), growth failure (45%), prolonged fever (40%), chronic diarrhea (32%) and eczema (25%). The represented categories were combined T and B cell immunodeficiency (34%), well define syndromes with immunodeficincy (23%), predominantly antibody deficiencies (21%), congenital defects of phagocyte (8.5%), diseases of immune dysregulation (7%) and autoinflammatory diseases (6%). Among combined ID category, SCID was the most common condition (24%) followed by CMH II deficiency (8.5%). The global mortality of our series was 27 % at a mean age of 31 months.
Children with recurrent infections, growth failure and chronic diarrhea should raise high index of suspicion on possible PID. In the absence of routine screening, physician awareness of the relative frequency of these disorders is critical to early diagnosis and treatment.