caa a22 4500 44582428X CHVBK 20180317145254.0 cr unu---uuuuu 170323e20111001xx s 000 0 eng 10.1007/s00246-011-0029-2 doi (NATIONALLICENCE)springer-10.1007/s00246-011-0029-2 Oral Budesonide Treatment for Protein-Losing Enteropathy in Fontan-Palliated Patients [Elektronische Daten] [Kurt Schumacher, Michael Cools, Bryan Goldstein, Viktoriya Ioffe-Dahan, Karen King, Diane Gaffney, Mark Russell] Protein-losing enteropathy (PLE) is a rare complication of Fontan palliation associated with significant morbidity and mortality. It is characterized by the loss of serum proteins into the intestinal lumen, and its pathophysiology likely involves enteral inflammation. Budesonide, an oral steroid, is an attractive treatment option because of its potent enteral activity and minimal systemic side effects. A single-center, retrospective review of Fontan-palliated PLE patients treated with oral budesonide for 6months or longer was performed. The patient characteristics reviewed were demographics, anatomic diagnosis, budesonide treatment (dose and duration), other medications and therapeutic interventions, hospitalizations, serum albumin levels, medical complications, and patient status at the time of follow-up assessment. The study enrolled 10 patients representing 228 patient-months of on-therapy follow-up evaluation. Serum albumin levels increased after initiation of budesonide for 90% of the patients, and clinical evidence of fluid overload improved for 60% of them. Symptomatic improvement was reported in 80% of the cases. During the treatment period, 50% of the patients met the primary end point of death or cardiac transplantation. In this series of PLE patients, oral budesonide therapy was associated with significant symptomatic improvement and sustained increases in serum albumin. However, budesonide therapy may not alter the long-term outcome for patients with advanced PLE. Springer Science+Business Media, LLC, 2011 Budesonide nationallicence Fontan nationallicence Protein losing enteropathy nationallicence Schumacher Kurt Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut Cools Michael Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut Goldstein Bryan Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut Ioffe-Dahan Viktoriya Soroka University Medical Center, Beer-Sheva, Israel aut King Karen Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut Gaffney Diane Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut Russell Mark Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut Pediatric Cardiology Springer-Verlag 32/7(2011-10-01), 966-971 0172-0643 32:7<966 2011 32 246 https://doi.org/10.1007/s00246-011-0029-2 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00246-011-0029-2 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Schumacher Kurt Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Cools Michael Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Goldstein Bryan Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Ioffe-Dahan Viktoriya Soroka University Medical Center, Beer-Sheva, Israel aut NATIONALLICENCE 700 1- King Karen Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Gaffney Diane Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 700 1- Russell Mark Division of Pediatric Cardiology, C.S. Mott Children's Hospital, University of Michigan, 1500 E. Medical Center Drive, L1242 Women's, SPC 5204, 48109, Ann Arbor, MI, USA aut NATIONALLICENCE 773 0- Pediatric Cardiology Springer-Verlag 32/7(2011-10-01), 966-971 0172-0643 32:7<966 2011 32 246 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer