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儿童戈谢病的肺部受累:沙特阿拉伯的常见表现。

Pulmonary involvement of Gaucher's disease in children: a common presentation in Saudi Arabia.

作者信息

Banjar H

机构信息

Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

出版信息

Ann Trop Paediatr. 1998 Mar;18(1):55-9. doi: 10.1080/02724936.1998.11747927.

Abstract

All three types of Gaucher's disease may show pulmonary involvement but reports on children with pulmonary Gaucher's are rare. We present nine children with Gaucher's disease, seven of whom had lung involvement, who were followed regularly for between 1 and 7 years. Four of them received ceredase and two showed improvement of their lung pathology. Enzyme therapy with ceredase could be an effective alternative to bone marrow transplant in treating Gaucher's disease with lung involvement.

摘要

所有三种类型的戈谢病都可能出现肺部受累,但关于患有肺部戈谢病儿童的报道很少。我们报告了9例戈谢病患儿,其中7例有肺部受累,对他们进行了1至7年的定期随访。其中4例接受了思而赞治疗,2例肺部病理状况有所改善。在治疗伴有肺部受累的戈谢病时,使用思而赞进行酶替代治疗可能是骨髓移植的一种有效替代方法。

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