Primary immunodeficiency disease: A retrospective study of 112 Chinese children in a single tertiary care center
BMC Pediatrics Nov 17, 2019
Wu J, et al. - Given that an inherited flaw in the immune system causes primary immunodeficiency disease (PID), a disorder that enhances the susceptibility to infections, researchers here sought to describe the clinical features of PID children. From a single tertiary care center, they assessed 112 children with PID diagnosed and classified based on the 2017 criteria presented by the International Union of Immunological Societies (IUIC). This population had a male/female ratio of 6:1. Severe combined immunodeficiency (SCID) (28.6%) and hyper-IgM (HIGM) syndrome (24.1%) were the most frequent diagnosed PID, followed by predominantly antibody deficiencies (17.8%). The 2-year overall survival rate for SCID, HIGM syndrome, and the remaining of PID patients who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) was 14.3, 83.3, and 100%, respectively. The emergence of PID was typically observed at an early age. The most common clinical manifestations were a recurrent infection and serious infection. For PID patients, Allo-HSCT seems to be a relatively effective therapeutic strategy.
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