Clinical and Immunological Profile of Suspected Primary Immunodeficiency Disorders (PIDs) in Children: A Tertiary care Experience from Bangladesh

Background: Primary immunodeficiency disorders (PIDs) are a heterogeneous group of inborn errors of immunity presenting with recurrent, severe, or unusual infections. Early diagnosis is challenging in resource-limited settings. Objective: To describe the clinical profile and immunological patterns of children with suspected primary immunodeficiency disorders in a tertiary care center in Bangladesh. Methods: This observational study was conducted at the Department of Pediatrics, Ad-din Women’s Medical College Hospital, Dhaka, from January 2017 to July 2019. A total of 129 children aged ≤18 years with recurrent infections were evaluated. Clinical features, serum immunoglobulins, lymphocyte subset analysis by flow cytometry, and relevant investigations were analyzed. Final classification was based on clinical and immunological findings. Genetic testing, functional antibody response and functional flow cytometry assays were not available. Results: Out of 129 children, 100 were classified as primary immunodeficiency disorders. The most common presentations were recurrent wheeze (51%) and recurrent pneumonia (39%). Severe wasting and stunting were seen in 31% and 29%, respectively. Immunological evaluation showed predominant antibody deficiency in 49%, B/NK-cell deficiency in 24%, combined T/B/NK-cell deficiency in 20%, and T-cell abnormalities in 7%. Selective IgA deficiency was the most frequent diagnosis (26%), followed by antibody deficiency with normal B-cell counts (23%), severe combined immunodeficiency (8%), combined immunodeficiency (8%), agammaglobulinaemia (4%), hyper-IgE syndrome (1%), and DiGeorge syndrome (1%). Conclusion: Primary immunodeficiency should be suspected in children with recurrent or severe infections, particularly with poor growth. Clinical evaluation with basic immunological testing enables early recognition in resource-limited settings. Improved access to advanced diagnostic facilities is needed for accurate classification. J Com Med Col Teachers’ Asso July 2026; 30(2): 119-126