Article ; Online: Hypertrophy of unaffected cardiomyocytes correlates with severity of cardiomyopathy in female patients with Fabry disease.
Orphanet journal of rare diseases
2021 Volume 16, Issue 1, Page(s) 169
Abstract: Aim: To investigate the contribution of unaffected cardiomyocytes in Fabry disease cardiomyopathy.: Findings: Left ventricular (LV) endomyocardial biopsies from twenty-four females (mean age 53 ± 11 ys) with Fabry disease cardiomyopathy were studied. ...
Abstract | Aim: To investigate the contribution of unaffected cardiomyocytes in Fabry disease cardiomyopathy. Findings: Left ventricular (LV) endomyocardial biopsies from twenty-four females (mean age 53 ± 11 ys) with Fabry disease cardiomyopathy were studied. Diagnosis of FD was based on the presence of pathogenic GLA mutation, Patients were divided in four groups according with LV maximal wall thickness (MWT): group 1 MWT ≤ 10.5 mm, group 2 MWT 10.5-15 mm, group 3 MWT 16-20 mm, group 4 MWT > 20 mm. At histology mosaic of affected and unaffected cardiomyocytes was documented. Unaffected myocytes' size ranged from normal to severe hypertrophy. Hypertrophy of unaffected cardiomyocytes correlated with severity of MWT (p < 0.0001, Sperman r 0,95). Hypertrophy of unaffected myocytes appear to concur to progression and severity of FDCM. It is likely a paracrine role from neighboring affected myocytes. |
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MeSH term(s) | Adult ; Cardiomyopathies ; Fabry Disease/genetics ; Female ; Heart Ventricles ; Humans ; Hypertrophy ; Hypertrophy, Left Ventricular ; Middle Aged ; Myocytes, Cardiac |
Language | English |
Publishing date | 2021-04-10 |
Publishing country | England |
Document type | Letter ; Research Support, Non-U.S. Gov't |
ISSN | 1750-1172 |
ISSN (online) | 1750-1172 |
DOI | 10.1186/s13023-021-01803-7 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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