A Retrospective Study Of Clinical Data Of 34 Inpatients With Primary Immunodeficiency Disease

Objective:Primary immunodeficiency disease(PID)is a heterogeneous disease characterized by functional defects in the immune system.This study retrospectively analyzed the clinical data of 34 hospitalized children with PID.By analyzing the clinical onset and main clinical manifestations of various types of PID,the clinical characteristics of the disease are revealed and help to improve the understanding of the disease.This study provides an effective basis for clinical identification and management.Methods:34 hospitalized patients with primary immunodeficiency disease at the Children's Hospital,Zhejiang University School of Medicine from January 2015 to January 2019 were retrospectively analyzed.Analysis of the clinical data of these children including the age of onset and diagnosis,gender,history of family and pregnancy,vaccination history,growth and development history,clinical manifestations,pathogen test detection,chest CT,treatment outcomes and prognosis.Result:Of the 34 patients with PID,28 were male and 6 were female,7 had a positive family history.Predominantly antibody deficiencies represented the largest category(18 patients,52.9%of all PID patients)followed by combined immunodeficiency(5 patients,14.7%),diseases of immune dysregulation(5 patients,14.7%),combined immunodeficiencies with associated or syndromic features(3 patients,8.8%),congenital defects of phagocyte number or function(2 patients,5.9%),autoinflammatory disorders(1 patient,2.9%).All cases had infection performance,and the respiratory system(30/34 cases)being the most common site of infection.Respiratory pathogen detection results and chest CT changes are various in different PID disease types.In addition,nine patients had autoinflammatory diseases,including 4 cases of arthritis and 2 cases of lymphoma.One patient died during hospitalization due to serious infection,and the rest were better discharged.Conclusions:There are many kinds of PID,and the clinical manifestations and onset time are different.All children in this group had recurrent infections.Children with recurrent infections need to be highly suspected of PID in clinical work.Family history investigation and immune function testing can help clinical diagnosis and if necessary,take genetic testing.Infections occur most frequently in the respiratory tract,and the susceptible pathogens of these PID types are different.In addition to the infection,PID patients also have the risk of combining autoimmune diseases and tumors.In long-term clinical management,it is necessary to be alert to the possibility of concurrent diseases.