title: The use of oral budesonide in adolescents and adults with protein-losing enteropathy after the Fontan operation.
abstract: BACKGROUND
  Approximately 5% to 15% of patients develop protein-losing enteropathy (PLE) after the Fontan operation. Oral controlled release (CR) budesonide has been used as a treatment strategy, but its use in the older Fontan population has not been described.
  
  
  METHODS
  Seven patients with refractory PLE after the Fontan operation were started on oral CR-budesonide at 9 mg. After 3 to 9 months, the dose was weaned to 3 mg. Response to treatment was assessed by clinical evaluation, serum albumin levels, and fecal α-1 antitrypsin clearance when available.
  
  
  RESULTS
  Median age at last evaluation was 20 years (range, 16 to 32 years). Six patients had increases in serum albumin levels but only 4 patients had symptomatic improvement. Systemic side effects included: cushingoid features (5), adrenal insufficiency (4), and new-onset type 2 diabetes mellitus (2). One patient had improvement in cushingoid features after weaning CR-budesonide to 3 mg. Older patients (ages 27 to 32 years) had the worst side effect profiles and were the most refractory to treatment. These patients had sonographic evidence of hepatic cirrhosis but normal serum liver function tests. Two deaths occurred: 1 from sepsis 1 month after CR-budesonide initiation and 1 from respiratory arrest 5 months after CR-budesonide discontinuation.
  
  
  CONCLUSIONS
  CR-budesonide can be used to treat PLE in certain patients, but careful assessment of hepatic function should be performed before initiation of therapy as systemic side effects can limit treatment. Normal serum liver function tests do not preclude hepatic dysfunction in the Fontan patient, and it is important to perform radiographic assessments as well.
authors.affiliation: Division of Pediatric Cardiology, Children's National Medical Center, George Washington University, Washington, DC, USA. anjohn@cnmc.org
authors.forename: Anitha S
authors.initials: AS
authors.lastname: John
authors.forename: David J
authors.initials: DJ
authors.lastname: Driscoll
authors.forename: Carole A
authors.initials: CA
authors.lastname: Warnes
authors.forename: Sabrina D
authors.initials: SD
authors.lastname: Phillips
authors.forename: Frank
authors.initials: F
authors.lastname: Cetta
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D019819:Budesonide / Q000008:administration & dosage*; D003692:Delayed-Action Preparations / Q000008:administration & dosage; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005243:Feces / Q000201:enzymology; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000601:surgery; D006801:Humans; D008297:Male; D011182:Postoperative Care / Q000379:methods*; D011504:Protein-Losing Enteropathies / Q000188:drug therapy* / Q000209:etiology / Q000378:metabolism; D012709:Serum Albumin / Q000378:metabolism; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult; D000515:alpha 1-Antitrypsin / Q000378:metabolism
chemical_list: D000893:Anti-Inflammatory Agents; D003692:Delayed-Action Preparations; D012709:Serum Albumin; D000515:alpha 1-Antitrypsin; D019819:Budesonide