title: Oral budesonide as a therapy for protein-losing enteropathy in patients having undergone Fontan palliation.
abstract: BACKGROUND
  Protein-losing enteropathy is associated with Fontan palliation for single-ventricle physiology and has been difficult to treat. Limited data suggest the successful use of oral budesonide (Entocort, AstraZeneca) as a palliative measure.
  
  
  METHODS
  We examine our single-institution retrospective experience in eight patients who were treated with this therapy.
  
  
  RESULTS
  Median pretherapy albumin level was 1.7 g/dL (range 1.0-2.7 g/dL), 3 months after therapy was 3.1 g/dL (range 2-4.8 g/dL), and by the end of the first year was 3.4 g/dL (range 2.1-5.3 g/dL). All patients had at least a transient improvement, and at latest follow-up (median 29 months, range 3-51 months) five patients remain on therapy. Five of eight patients had required pretherapy albumin transfusions; one patient required albumin infusions after therapy. Four patients had side effects associated with the medication.
  
  
  CONCLUSIONS
  Oral budesonide is an additional therapy that has the potential to improve symptoms and delay need for heart transplantation in this patient population.
authors: Heart Center, Children's Hospital of Pittsburgh of UPMC, 4401 Penn Ave., Pittsburgh, PA 15224, USA.
authors: Zachary
authors: Z
authors: Turner
authors: Lizabeth
authors: L
authors: Lanford
authors: Steven
authors: S
authors: Webber
mesh_terms: D000284:Administration, Oral; D000418:Albumins / Q000008:administration & dosage; D015415:Biomarkers / Q000097:blood; D019819:Budesonide / Q000008:administration & dosage* / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D005938:Glucocorticoids / Q000008:administration & dosage* / Q000009:adverse effects; D006330:Heart Defects, Congenital / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities / Q000601:surgery*; D006801:Humans; D007223:Infant; D007263:Infusions, Parenteral; D008297:Male; D010166:Palliative Care; D010414:Pennsylvania; D011504:Protein-Losing Enteropathies / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D012189:Retrospective Studies; D012709:Serum Albumin / Q000378:metabolism; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000418:Albumins; D015415:Biomarkers; D005938:Glucocorticoids; D012709:Serum Albumin; D019819:Budesonide