Frequency of primary immune deficiency disorders in patients with Down syndrome

Abstract

Down syndrome is the most common genetic disorder. These patients have impaired immune system that predisposes them to recurrent infections, which itself is a main cause of hospitalization, morbidity and mortality among them. The aim of this study was to determine the relation between immunologic disorders and recurrent infections in a group of patients with Down syndrome. Methods: Clinical and immunological data were collected from medical records of 30 patients with Down syndrome, who were referred to the Children Hospital of Tabriz during 2018-2019. For comparison of immunological parameters with age-matched reference values, patients were divided into same age groups as used in reference study. Results: Recurrent pneumonia (86.7%) followed by otitis media (53.3%) were the most common infections in these patients; respectively. All patients had more than one history of hospitalization due to infection. CD19+ was significantly lower than the normal in all age groups, while CD3+, CD4+ and CD4/CD8 ratio were significantly lower than the normal in patients less than one-year-old and also in patients between 6-12 years old. On the other hands, Serum IgG levels were higher than the normal in 7-12 months' age group while serum IgA level was significantly lower than the normal in patients between 4-6 years old and also 10-11 years old groups but was higher in patients between 1-3 years old group. Also in the study of isohemaglotination a total of 74% of patients had B lymphocyte dysfunction.