腺苷脱氨酶缺乏的重症联合免疫缺陷中的双侧感音神经性耳聋。
Bilateral sensorineural deafness in adenosine deaminase-deficient severe combined immunodeficiency.
作者信息
Albuquerque Wendy, Gaspar Hubert B
机构信息
Department of Audiology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
出版信息
J Pediatr. 2004 Feb;144(2):278-80. doi: 10.1016/j.jpeds.2003.10.055.
PMID:14760277
Abstract
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.
摘要
腺苷脱氨酶缺乏症表现为严重联合免疫缺陷，可通过骨髓移植进行治疗。随着生存率的提高，这种疾病的非免疫表现正变得明显。我们报告了移植患者中双侧感音神经性耳聋的高发病率，这突出了该疾病的全身性。
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