TY  - JOUR
A1  - Tanislav, Christian
A1  - Guenduez, Dursun
A1  - Liebetrau, Christoph
A1  - Giese, Anne-Kathrin
A1  - Eichler, Sabrina
A1  - Sieweke, Nicole
A1  - Speth, Maria
A1  - Bauer, Timm
A1  - Hamm, Christian
A1  - Rolfs, Arndt
T1  - Cardiac troponin I: a valuable biomarker indicating the cardiac involvement in fabry disease
T2  - PLoS one
N2  - Objectives: Assessment of the clinical severity of Fabry disease (FD), an X-linked, rare, progressive disorder based on a genetic defect in alpha-galactosidase is challenging, especially regarding cardiac involvement. The aim of the study was to evaluate the diagnostic value of cardiac troponin I (cTnI) in discriminating FD patients with cardiac involvement in a large FD patient cohort.
Methods: cTnI levels were measured with a contemporary sensitive assay in plasma samples taken routinely from FD patients. The assay was calibrated to measure cTnI levels ≥0.01 ng/ml. Elevated cTnI values (cut-off ≥0.04 ng/ml) were correlated with clinical data.
Results: cTnI was assessed in 62 FD patients (median age: 47 years, males: 36%). Elevated cTnI levels were detected in 23 (37%) patients. Patients with a cTnI elevation were older (median 55 years versus 36 years, p<0.001). Elevated cTnI levels were associated with the presence of a LVH (16/23 versus 1/39; OR 65.81, CI: 6.747–641.859; p<0.001). In almost all patients with a left ventricular hypertrophy (LVH) elevated cTnI levels were detected (16/17, 94%). Absolute cTnI levels in patients with LVH were higher than in those without (median 0.23 ng/ml versus 0.02 ng/ml; p<0.001). A cTnI level <0.04ng/ml had a high negative predictive value regarding the presence of a LVH (38/39, 97%). In a control group of non-FD patients (n = 17) with LVH (due to hypertension) none showed cTnI levels ≥0.01 ng/ml.
Conclusions: Elevated cTnI levels are common in FD patients, reflecting cardiac involvement. FD patients might benefit from a continuous cTnI monitoring.
Y1  - 2016
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/41507
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-415072
UR  - https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4913911/
SN  - 1932-6203
N1  - Copyright: © 2016 Tanislav et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
VL  - 11
IS  - (6): e0157640
SP  - 1
EP  - 11
PB  - PLoS
CY  - Lawrence, Kan.
ER  -