H Sonali Magdo1, Terri L Stillwell2, Matthew J Greenhawt3, Kathleen A Stringer4, Sunkyung Yu5, Carlen G Fifer6, Mark W Russell6, Kurt R Schumacher6. 1. University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI. Electronic address: hmagdo@med.umich.edu. 2. University of Michigan Pediatric Infectious Disease, Ann Arbor, MI. 3. University of Michigan Allergy and Immunology, Ann Arbor, MI. 4. University of Michigan College of Pharmacy, Ann Arbor, MI. 5. University of Michigan, Michigan Congenital Heart Outcomes Research and Discovery Ann Arbor, MI. 6. University of Michigan Congenital Heart Center, C.S. Mott Children's Hospital, Ann Arbor, MI.
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. STUDY DESIGN: 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.
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. STUDY DESIGN: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 depressedCD4 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 depressedCD8 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: Julia Moosmann; Okan Toka; Sören Lukassen; Arif B Ekici; Andreas Mackensen; Simon Völkl; Sven Dittrich Journal: Thorac Cardiovasc Surg Date: 2021-02-19 Impact factor: 1.827
Authors: Sven Dittrich; Anja Weise; Robert Cesnjevar; Oliver Rompel; André Rüffer; Martin Schöber; Julia Moosmann; Martin Glöckler Journal: Thorac Cardiovasc Surg Date: 2020-12-31 Impact factor: 1.827