Progression of transthyretin (TTR) amyloidosis in donors and recipients after domino liver transplantation-a prospective single-center cohort study
| Autor: | Frank Birklein, Christian Geber, Maria Hoppe-Lotichius, Veronica Weyer, Tim Zimmermann, Mimoun Azizi, Ana Paula Barreiros, Gerd Otto, Julia C Schmid, Hauke Lang, Jens Mittler, Johanna Vollmar, Peter R. Galle, Arno Schad, Tilman Emrich, Michael Heise |
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| Rok vydání: | 2018 |
| Předmět: |
Transplantation
medicine.medical_specialty biology medicine.diagnostic_test business.industry medicine.medical_treatment Amyloidosis 030204 cardiovascular system & hematology Liver transplantation medicine.disease Single Center Gastroenterology 03 medical and health sciences Transthyretin Tilt table test 0302 clinical medicine Internal medicine Cohort Biopsy biology.protein Medicine business 030217 neurology & neurosurgery Cohort study |
| Zdroj: | Transplant International. 31:1207-1215 |
| ISSN: | 0934-0874 |
| Popis: | Liver transplantation (LT) is the first-line therapy in patients with transthyretin (TTR) amyloidosis and progressive familial amyloid polyneuropathy (FAP). Explanted organs from these patients can be used for domino liver transplantation (DLT). After DLT, de novo amyloidosis may develop in domino recipients (DR). Data were collected prospectively in a transplant database. Electroneurography by nerve conduction velocity (NCV), quantitative sensory testing, heart rate variability (HRV), sympathetic skin response, orthostatic reaction (tilt table test), transthoracic echocardiography, cardiac MRI and organ biopsy results were evaluated. The cohort included 24 FAP- (11 Val30Met, 13 nonVal30Met) and 23 DR-patients. DR symptoms referred to post-DLT only, while those of FAP patients were both pre- and post-transplantation. Symptoms of TTR-amyloidosis in Val30Met and Non-Val30Met patients pre- and post-LT were similarly distributed. Biopsy-proven de novo amyloidosis occurred in 4/23 DR after a mean observation of 10 years. Analysis for manifestations of amyloidosis only included patients with available 5-year follow-up data (n = 13 FAP, n = 12 DR). Compared to Val30Met FAP patients pre-LT, Val30Met DR patients had better NCV (P = 0.04) and HRV (P = 0.015). In the Non-Val30Met group no differences were found between DR and FAP patients pre-LT. TTR-amyloidosis symptoms showed no differences in FAP patients pre- and 5 years post-LT, irrespective of Val30Met status. In DR patients, de novo amyloidosis occurred earlier than expected. Therefore, recipients for DLT need to be carefully selected and followed. |
| Databáze: | OpenAIRE |
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