Plastic bronchitis in children with fontan palliation: Analogue to protein losing enteropathy?

被引：26

作者：

Stiller, B

Riedel, F

Paul, K

van Landeghem, FKH

机构：

[1] Deutsch Herzzentrum Berlin, Dept Pediat Cardiol, D-13353 Berlin, Germany

[2] Altonaer Kinderkrankenhaus, Dept Pediat, Hamburg, Germany

[3] Charite, Dept Pulmonol, Berlin, Germany

[4] Charite, Inst Neuropathol, Berlin, Germany

来源：

PEDIATRIC CARDIOLOGY | 2002年 / 23卷 / 01期

关键词：

Fontan operation; plastic bronchitis; bronchial casts; high-molecular-weight heparin; protein-losing enteropathy;

D O I：

10.1007/s00246-001-0024-0

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

We studied a 6-year-old boy and a 2-yearold girl with bronchitis fibroplastica following Fontan operation. Large endobronchial casts of rubber-like consistency resulted in life-threatening pulmonary failure. In one patient symptoms improved after optimizing heart function with diuretics, and in the other a dramatic improvement with the resolution of the clinical symptoms and normalized serum albumin followed subcutaneous high-molecular-weight heparin treatment. The severe relapse after discontinuation of the heparin medication and the once more successful treatment with heparin suggest that in addition to optimizing heart function, high-molecular-weight heparin might be a therapeutic option for this poorly understood condition.

引用

页码：90 / 94

页数：5

共 15 条

[1] Heparin therapy and reversal of protein-losing enteropathy in a case with congenital heart disease [J].