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一种治疗儿童戈勒姆-斯图特综合征伴乳糜胸的新方法。

A novel treatment approach for paediatric Gorham-Stout syndrome with chylothorax.

机构信息

Department of Clinical Sciences, Section for Paediatrics, The BUT team, Lund University, Sweden.

出版信息

Acta Paediatr. 2011 Nov;100(11):1448-53. doi: 10.1111/j.1651-2227.2011.02361.x. Epub 2011 Jun 15.

Abstract

AIM

To expand the treatment options in paediatric Gorham-Stout syndrome (GSS) when conventional therapy is ineffective.

METHOD

Two children with biopsy confirmed GSS, a rare disorder with progressive lymphangiomatosis, were treated with a combination of interferon-α-2b, low anticoagulant, low molecular weight heparin, radiotherapy and surgery.

RESULTS

The combined therapy resolved the symptoms in the acute phase, and both patients have since been free of symptoms for >2 years.

CONCLUSION

The successful addition of a low anticoagulant, low molecular weight heparin (tafoxiparin) to the treatment protocol in two paediatric cases of the GSS may justify the use of this approach in similar cases.

摘要

目的

当常规治疗无效时,为儿科 Gorham-Stout 综合征(GSS)患者提供更多治疗选择。

方法

对 2 例经活检证实为 GSS 的儿童患者(一种罕见的进行性淋巴管瘤病)采用干扰素-α-2b、低抗凝剂、低分子肝素、放疗和手术联合治疗。

结果

联合治疗缓解了急性期症状,且两名患者此后均无任何症状超过 2 年。

结论

在 2 例儿科 GSS 患者的治疗方案中成功添加低抗凝剂、低分子肝素(tafoxiparin)可能证明该方法在类似病例中的应用是合理的。

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