Bilateral sensorineural deafness in adenosine deaminase-deficient severe combined immunodeficiency
Bilateral sensorineural deafness in adenosine deaminase-deficient severe combined immunodeficiency
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DOI：
10.1016/j.jpeds.2003.10.055
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发表时间：
2004-02-01
影响因子：
5.1
通讯作者：
Gaspar, HB
中科院分区：
文献类型：
--
作者：
Albuquerque, W;Gaspar, HB
腺苷脱氨酶缺乏症表现为严重的联合免疫缺陷，可通过骨髓移植治疗。随着生存率的提高，这种疾病的非免疫学表现变得越来越明显。我们报告了双侧感音神经性耳聋在移植患者中的高发病率，这突出了该疾病的系统性。
Adenosine deaminase deficiency presents with severe combined immunodeficiency and is treatable by bone marrow transplantation. With improved survival, the nonimmunologic manifestations of this condition are becoming apparent. We report a high incidence of bilateral sensorineural deafness in transplanted patients, which highlights the systemic nature of the disease.