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

被引：41

作者：

Brodszki, Nicholas ^{[1
]}

Lansberg, John-Kalle ^{[1
]}

Dictor, Michael ^{[2
]}

Gyllstedt, Erik ^{[3
]}

Ewers, Sven-Borje ^{[4
]}

Larsson, Marcus K. ^{[1
]}

Eklund, Erik A. ^{[1
]}

机构：

[1] Lund Univ, Dept Clin Sci, Paediat Sect, BUT Team, Lund 22184, Sweden

[2] Lund Univ, Dept Clin Sci, Sect Pathol, Lund 22184, Sweden

[3] Lund Univ, Dept Clin Sci, Thorac Surg Sect, Lund 22184, Sweden

[4] Lund Univ, Dept Clin Sci, Sect Oncol, Lund 22184, Sweden

来源：

ACTA PAEDIATRICA | 2011年 / 100卷 / 11期

关键词：

Acute respiratory failure; Alpha-interferon therapy; Heparin; PROTEIN-LOSING ENTEROPATHY; ENDOTHELIAL GROWTH-FACTOR; HEPARIN-BINDING PROTEIN; MASSIVE OSTEOLYSIS; DIFFUSE LYMPHANGIOMATOSIS; THERAPY; BONE; INTERFERON-ALPHA-2B; MECHANISMS; SULFATE;

D O I：

10.1111/j.1651-2227.2011.02361.x

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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-alpha-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.

引用

页码：1448 / 1453

页数：6

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