Health-related Quality Of Life Of Children With Primary Immunodeficiency Disease

ObjectiveThe aim of this study was to analyze the reliability and validity by thePedsQL^TM4.0Generic Core Scales proxy-report in the children with primaryimmunodeficiency disease, and to compare the quality of life among normalchildren and the children with primary immunodeficiency disease, amongpatients receiving routine medication treatment and those who underwenthematopoietic stem cell transplantation.Materials and methods1.A total of56patients with various primary immunodeficiencydiseases and their families were surveyed by using the questionnaire throughthe telephone interview or outpatient follow-up. And80healthy childrenaged2-18were enrolled as controls, of which all had been excluded withchronic diseases and immunocompromise conditions.2.To analyze the reliability and validity by the PedsQL^TM4.0GenericCore Scales proxy-report in the children with primary immunodeficiencydisease.3.Compared the quality of life among normal children and the childrenwith primary immunodeficiency disease.4.Compared the quality of life among medication treatment and hematopoietic stem cell transplant.Results1. PedsQL^TM4.0Generic Core Scales proxy-report showed acceptablereliability and validity and could be applied to the children with primaryimmunodeficiency disease.2. As compared with normal children, each dimension score in thegroup of the children with primary immunodeficiency disease was lowerwith statistically significant differences （P<0.05）.3. As compared with those of the patients upon medication treatment,the total score and the score in the physical functioning, school functioningand psychosocial scale of patients who underwent a successfulhematopoietic stem cell transplantion is significantly higher （P<0.05）.Whilethe score in the emotional functioning and social functioning showed nosignificant difference between the patients receiving different therapies.Conclusion1.PedsQL^TM4.0Generic Core Scales proxy-report can be applied toevaluate the quality of life in the children with primary immunodeficiencydisease.2.The quality of life in the children with primary immunodeficiencydisease is impaired and stem cell transplantation is currently the best mean torevert this situation to normal mode.