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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.

DOI:10.1080/02724936.1998.11747927
PMID:9692003
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。

相似文献

1
Pulmonary involvement of Gaucher's disease in children: a common presentation in Saudi Arabia.儿童戈谢病的肺部受累:沙特阿拉伯的常见表现。
Ann Trop Paediatr. 1998 Mar;18(1):55-9. doi: 10.1080/02724936.1998.11747927.
2
Pulmonary pathology in Gaucher's disease.戈谢病的肺部病理学
Hum Pathol. 1999 Jun;30(6):666-70. doi: 10.1016/s0046-8177(99)90092-8.
3
[Course of a case of Gaucher's disease type 1 treated over a year with glucocerebrosidase (Cérédase)].[1例1型戈谢病患者接受葡萄糖脑苷脂酶(思而赞)治疗1年的病程]
Rev Med Interne. 1993;14(10):996. doi: 10.1016/s0248-8663(05)80114-6.
4
[Enzyme therapy in children with severe forms of Gaucher's disease].[酶疗法治疗重型戈谢病患儿]
Cas Lek Cesk. 1997 Feb 5;136(3):95-7.
5
[Persistence of pulmonary lesions in a 6-year-old boy with type I Gaucher's disease treated by alglucerase since the age of 20 months].[一名自20个月起接受阿糖苷酶治疗的1型戈谢病6岁男孩肺部病变的持续情况]
Arch Pediatr. 1998 Dec;5(12):1341-3. doi: 10.1016/s0929-693x(99)80053-0.
6
Marked clinical and histologic improvement in a patient with type-1 Gaucher's disease following long-term glucocerebroside substitution. A case report and review of current diagnosis and management.长期葡萄糖脑苷脂替代治疗后1型戈谢病患者临床及组织学显著改善。病例报告及当前诊断与治疗综述
Pathol Res Pract. 2003;199(3):159-63. doi: 10.1078/0344-0338-00369.
7
Lung involvement and enzyme replacement therapy in Gaucher's disease.戈谢病中的肺部受累与酶替代疗法
QJM. 2001 Aug;94(8):407-15. doi: 10.1093/qjmed/94.8.407.
8
Ceredase dosing concerns for Gaucher's disease.
Am J Med. 1994 Oct;97(4):402-3. doi: 10.1016/0002-9343(94)90314-x.
9
Successful treatment of bone marrow failure in Gaucher's disease with low-dose modified glucocerebrosidase.低剂量改良葡萄糖脑苷脂酶成功治疗戈谢病的骨髓衰竭
Q J Med. 1992 Jul;83(303):541-6.
10
The health-related quality of life of adults with Gaucher's disease receiving enzyme replacement therapy: results from a retrospective study.接受酶替代疗法的戈谢病成年患者的健康相关生活质量:一项回顾性研究的结果
Qual Life Res. 1998 Jul;7(5):373-86. doi: 10.1023/a:1008814105603.

引用本文的文献

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Allogeneic hematopoietic stem cell transplantation for treating severe lung involvement in Gaucher disease.异基因造血干细胞移植治疗戈谢病严重肺部受累
Mol Genet Metab Rep. 2020 Oct 20;25:100652. doi: 10.1016/j.ymgmr.2020.100652. eCollection 2020 Dec.
2
Pulmonary hemorrhage in type 3 Gaucher disease: a case report.3 型戈谢病中的肺出血:病例报告。
J Inherit Metab Dis. 2010 Dec;33 Suppl 3:S329-31. doi: 10.1007/s10545-010-9176-5. Epub 2010 Aug 6.