The health status and quality of life of adults with X-linked agammaglobulinemia

作者: Vanessa Howard , Jeffrey M. Greene , Savita Pahwa , Jerry A. Winkelstein , John M. Boyle

DOI: 10.1016/J.CLIM.2005.11.002

关键词: MedicineQuality of lifeHepatitisFamily historyX-linked agammaglobulinemiaDiarrheaImmunologySinusitisImmunopathologySkin infectionPediatrics

摘要: Forty-one adults (mean age 33) with a definitive diagnosis of X-linked agammaglobulinemia (XLA) completed questionnaire concerning current and past medical problems quality life. Thirty-six the 41 were working full time or students; 18 had not missed any work school due to infection in previous year. Their life was equivalent that general US male population. Thirteen reported they chronic lung disease, 33 indicated one more episodes sinusitis preceding Arthritis, diarrhea skin infections common but debilitating. The study subjects likely have prior family history XLA, milder mutations Btk, gene responsible for XLA. These results indicate most XLA are moderately healthy lead productive lives.

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