Plastic bronchitis in children with Fontan palliation: analogue to protein losing enteropathy?
We studied a 6-year-old boy and a 2-year-old 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.
Stiller
B
B
Department of Pediatric Cardiology, Deutsches Herzzentrum Berlin, Germany. stiller@dhzb.de
Riedel
F
F
Paul
K
K
van Landeghem
F K H
FK
D001991:Bronchitis / Q000188:drug therapy / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D004232:Diuretics / Q000627:therapeutic use; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D008297:Male; D011504:Protein-Losing Enteropathies / Q000188:drug therapy / Q000209:etiology*; D012709:Serum Albumin / Q000032:analysis
D004232:Diuretics; D012709:Serum Albumin; D006493:Heparin