Wiskott-Aldrich综合征综合报告及文献复习

The synthetical report and reviews of Wiskott-Aldrich syndrome

综合报告26例Wiskott－Aldrich综合征．属X连锁隐性遗传，均为男性儿童．偶有女性报告。发病年龄＜3个月占69．2％，死亡年龄平均17个月。临床表现以湿疹（84．6％），出血（7．1％），反复而严重的感染（76．9％）为主要表现。死亡原因以出血和感染为主、骨髓移植是目前最佳治疗手段。探讨了其遗传学特点，发病机制和临床治疗进展。

The synthetical study about 26 Wiskott-Aldrich syndrome was repotred, they were X-linked recessive inheritance in genetics, males involved and females occasionally. Presenting age:< 3 months (69. 2% ), the average age of death was 17 months Clinical manifestation:eczema (84.6% ), bleeding (73.1% ), recurrent and severe infection (76. 9 % ). The reason of death was mainly infection and hemorrhage. Bone marrow transplantation was the best therapeutic method at present. It was discussed that genetic characteristics, mechanism and clinical therapeutic progress.