小児 低ガンマグロブリン血症

1)UTDより ２）DyneMedではない ________________ UPDより 予後　 XLAでは多くの人が成人する。 成人した人の中でもその後死亡した人は少数だが、3人中２人が医原性であったことは特筆すべき。 人もPrognosis — The average age of diagnosis has dropped significantly since the initial description of XLA over 50 years ago [6]. In addition, immune globulin preparations have improved, allowing patients to maintain normal levels of IgG on replacement therapy. Most patients with XLA born in the last few decades and treated optimally can be expected to survive into adult life because of these improvements in initial diagnosis, immune globulin replacement therapy, and treatment of infections. In a 2006 report, over 40 percent of patients with XLA in a United States registry were adults. In the same series, only 3 of 80 patients followed prospectively over a four year period died, and, notably, two died of iatrogenic causes [6]. For patients who survive into adulthood, the quality of life is generally good [22,63]. Adults with XLA miss more days of school/work and they are hospitalized more frequently than males in the general population. However, most lead productive and fulfilling lives despite these limitations.