title: Fontan-associated protein-losing enteropathy and plastic bronchitis.
abstract: OBJECTIVE
  To characterize the medical history, disease progression, and treatment of current-era patients with the rare diseases Fontan-associated protein-losing enteropathy (PLE) and plastic bronchitis.
  
  
  METHODS
  A novel survey that queried demographics, medical details, and treatment information was piloted and placed online via a Facebook portal, allowing social media to power the study. Participation regardless of PLE or plastic bronchitis diagnosis was allowed. Case control analyses compared patients with PLE and plastic bronchitis with uncomplicated control patients receiving the Fontan procedure.
  
  
  RESULTS
  The survey was completed by 671 subjects, including 76 with PLE, 46 with plastic bronchitis, and 7 with both. Median PLE diagnosis was 2.5 years post-Fontan. Hospitalization for PLE occurred in 71% with 41% hospitalized ≥ 3 times. Therapy varied significantly. Patients with PLE more commonly had hypoplastic left ventricle (62% vs 44% control; OR 2.81, 95% CI 1.43-5.53), chylothorax (66% vs 41%; OR 2.96, CI 1.65-5.31), and cardiothoracic surgery in addition to staged palliation (17% vs 5%; OR 4.27, CI 1.63-11.20). Median plastic bronchitis diagnosis was 2 years post-Fontan. Hospitalization for plastic bronchitis occurred in 91% with 61% hospitalized ≥ 3 times. Therapy was very diverse. Patients with plastic bronchitis more commonly had chylothorax at any surgery (72% vs 51%; OR 2.47, CI 1.20-5.08) and seasonal allergies (52% vs 36%; OR 1.98, CI 1.01-3.89).
  
  
  CONCLUSIONS
  Patient-specific factors are associated with diagnoses of PLE or plastic bronchitis. Treatment strategies are diverse without clear patterns. These results provide a foundation upon which to design future therapeutic studies and identify a clear need for forming consensus approaches to treatment.
authors: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI. Electronic address: kurts@med.umich.edu.
authors: Kurt R
authors: KR
authors: Schumacher
authors: University of Michigan College of Pharmacy, Ann Arbor, MI.
authors: Kathleen A
authors: KA
authors: Stringer
authors: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Janet E
authors: JE
authors: Donohue
authors: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Sunkyung
authors: S
authors: Yu
authors: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Ashley
authors: A
authors: Shaver
authors: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Regine L
authors: RL
authors: Caruthers
authors: University of Michigan School of Public Health, Ann Arbor, MI.
authors: Brian J
authors: BJ
authors: Zikmund-Fisher
authors: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Carlen
authors: C
authors: Fifer
authors: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Caren
authors: C
authors: Goldberg
authors: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
authors: Mark W
authors: MW
authors: Russell
mesh_terms: D000293:Adolescent; D001991:Bronchitis / Q000453:epidemiology / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D015994:Incidence; D008297:Male; D008824:Michigan / Q000453:epidemiology; D010865:Pilot Projects; D011183:Postoperative Complications; D011504:Protein-Losing Enteropathies / Q000453:epidemiology / Q000209:etiology*; D012307:Risk Factors