Marnix Lebeer1, Wim A Wuyts1, David Cassiman2, Wim Laleman2, Frederik Nevens2, Jacques Pirenne3, Diethard Monbaliu3, Tania Roskams4, Eric K Verbeken4, Arne P Neyrinck5, Dirk E Van Raemdonck6,7, Geert M Verleden1,7, Robin Vos1,7. 1. Department of Respiratory Diseases, University Hospitals Leuven. 2. Department of Gastroenterology and Hepatology, University Hospitals Leuven. 3. Department of Abdominal Transplant Surgery and Transplant Coordination, University Hospitals Leuven, Belgium. 4. Department of Histopathology, University Hospitals Leuven. 5. Department of Anesthesiology and Algology, University Hospitals Leuven. 6. Department of Thoracic Surgery, University Hospitals Leuven and Experimental Thoracic Surgery, KU Leuven. 7. Division of Respiratory Diseases, Department of Chronic Diseases, Metabolism and Ageing (CHROMETA), KU Leuven.
Abstract
BACKGROUND: Solid organ transplantation is a valid treatment option for selected patients with organ failure due to an underlying telomeropathy; however, the feasibility of multiple-organ transplantation if several organs are compromised is unclear. METHODS: We describe 2 patients with telomeropathy due to heterozygous telomerase RNA component or telomerase reverse transcriptase mutation, who successfully underwent serial or combined liver and lung transplantation for concurrent liver fibrosis/cirrhosis and pulmonary fibrosis. RESULTS: Despite a challenging posttransplant course, long-term outcomes were favorable, with both patients doing fine now, respectively, 12/20 and 24 months after multiple-organ transplantation. CONCLUSIONS: To our knowledge, this is the first report of multiple solid organ transplantation in documented telomeropathy. These cases highlight current difficulties of timely diagnosis, therapeutic approach, and postoperative complications in telomeropathy patients in whom several organs are affected.
BACKGROUND: Solid organ transplantation is a valid treatment option for selected patients with organ failure due to an underlying telomeropathy; however, the feasibility of multiple-organ transplantation if several organs are compromised is unclear. METHODS: We describe 2 patients with telomeropathy due to heterozygous telomerase RNA component or telomerase reverse transcriptase mutation, who successfully underwent serial or combined liver and lung transplantation for concurrent liver fibrosis/cirrhosis and pulmonary fibrosis. RESULTS: Despite a challenging posttransplant course, long-term outcomes were favorable, with both patients doing fine now, respectively, 12/20 and 24 months after multiple-organ transplantation. CONCLUSIONS: To our knowledge, this is the first report of multiple solid organ transplantation in documented telomeropathy. These cases highlight current difficulties of timely diagnosis, therapeutic approach, and postoperative complications in telomeropathypatients in whom several organs are affected.
Authors: Jennifer M Kolb; Kendra Conzen; Michael Wachs; Joseph Crossno; Brandon McMahon; Maheen Z Abidi; Elizabeth A Pomfret; Michael Kriss Journal: Hepatology Date: 2020-07 Impact factor: 17.425