title: Immune Abnormalities in Fontan Protein-Losing Enteropathy: A Case-Control Study.
abstract: OBJECTIVE
  To comprehensively characterize the immunologic characteristics of patients with protein-losing enteropathy (PLE) post-Fontan and compare them with patients without PLE post-Fontan.
  
  
  METHODS
  Patients with PLE post-Fontan and age-matched controls post-Fontan were prospectively studied with laboratory markers of immune function. Infectious history was obtained by interview and chart review. The groups' demographics, cardiac history, immune characteristics, and infection history were compared using appropriate 2-group statistics.
  
  
  RESULTS
  A total of 16 patients enrolled (8 patients with PLE and 8 controls). All patients with PLE had lymphopenia compared with 25% of controls (P = .01). All patients with PLE had markedly depressed CD4 T cell counts (median 58 cells/μL) compared with controls (median 450 cells/μL, P = .0002); CD4% was also low in the PLE group (12.3%) and normal in control (36.9%, P = .004). Both groups had mildly depressed CD8 T cells and normal to slightly elevated natural killer and B-cell subsets. A majority of patients with PLE (62.5%) had negative titers to measles, mumps, and rubella vaccination, compared with no control Fontan with a negative titer (P = .03). Despite profoundly low CD4 counts, the frequency of infection was not different between groups with no reported opportunistic infections.
  
  
  CONCLUSIONS
  Patients with Fontan-associated PLE have extensive quantitative immune abnormalities, particularly CD4 deficiency. These immune abnormalities are similar to those found in non-Fontan patients with PLE caused by intestinal lymphangiectasia.
authors.affiliation: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI. Electronic address: hmagdo@med.umich.edu.
authors.forename: H Sonali
authors.initials: HS
authors.lastname: Magdo
authors.affiliation: University of Michigan Pediatric Infectious Disease, Ann Arbor, MI.
authors.forename: Terri L
authors.initials: TL
authors.lastname: Stillwell
authors.affiliation: University of Michigan Allergy and Immunology, Ann Arbor, MI.
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: Greenhawt
authors.affiliation: University of Michigan College of Pharmacy, Ann Arbor, MI.
authors.forename: Kathleen A
authors.initials: KA
authors.lastname: Stringer
authors.affiliation: University of Michigan, Michigan Congenital Heart Outcomes Research and Discovery Ann Arbor, MI.
authors.forename: Sunkyung
authors.initials: S
authors.lastname: Yu
authors.affiliation: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
authors.forename: Carlen G
authors.initials: CG
authors.lastname: Fifer
authors.affiliation: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
authors.forename: Mark W
authors.initials: MW
authors.lastname: Russell
authors.affiliation: University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
authors.forename: Kurt R
authors.initials: KR
authors.lastname: Schumacher
mesh_terms: D018791:CD4 Lymphocyte Count; D015496:CD4-Positive T-Lymphocytes; D018414:CD8-Positive T-Lymphocytes; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000097:blood / Q000276:immunology / Q000601:surgery*; D006801:Humans; D007132:Immunoglobulin Isotypes / Q000097:blood; D007223:Infant; D008231:Lymphopenia / Q000453:epidemiology*; D008297:Male; D011446:Prospective Studies; D011504:Protein-Losing Enteropathies / Q000097:blood / Q000276:immunology*
chemical_list: D007132:Immunoglobulin Isotypes