Update on Coronary Involvement in Fabry Disease
Fabry disease is a multisystemic disorder with consequent morbidity and mortality at an early age in patients of both genders. Although renal failure has been previously described as the cause of death with the highest prevalence, recent studies, based on the analysis of the population recorded in t...
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| Format: | Article |
| Language: | English |
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SciELO
2016-11-01
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| Series: | Journal of Inborn Errors of Metabolism and Screening |
| Online Access: | https://doi.org/10.1177/2326409816679427 |
| _version_ | 1811252260015964160 |
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| author | Gustavo Cabrera |
| author_facet | Gustavo Cabrera |
| author_sort | Gustavo Cabrera |
| collection | DOAJ |
| description | Fabry disease is a multisystemic disorder with consequent morbidity and mortality at an early age in patients of both genders. Although renal failure has been previously described as the cause of death with the highest prevalence, recent studies, based on the analysis of the population recorded in the Fabry Registry, reported that 40% of deaths had a cardiovascular origin. Data from the same registry emphasize the high risk for these patients suffering cardiovascular events—particularly acute myocardial infarction—and to the fact that this risk is higher for those patients who need dialysis. Microvascular dysfunction is a constant in patients with Fabry disease, which affects young patients, independently from other cardiac involvement manifestations—such as left ventricular hypertrophy—and from the patient’s gender. |
| first_indexed | 2024-04-12T16:32:08Z |
| format | Article |
| id | doaj.art-9a1fbbef7e1d4a7c81d46d9babe8d64e |
| institution | Directory Open Access Journal |
| issn | 2326-4594 |
| language | English |
| last_indexed | 2024-04-12T16:32:08Z |
| publishDate | 2016-11-01 |
| publisher | SciELO |
| record_format | Article |
| series | Journal of Inborn Errors of Metabolism and Screening |
| spelling | doaj.art-9a1fbbef7e1d4a7c81d46d9babe8d64e2022-12-22T03:25:07ZengSciELOJournal of Inborn Errors of Metabolism and Screening2326-45942016-11-01410.1177/232640981667942710.1177_2326409816679427Update on Coronary Involvement in Fabry DiseaseGustavo Cabrera0Centro Cardiovascular Bolivar, Pilar, Buenos Aires, ArgentinaFabry disease is a multisystemic disorder with consequent morbidity and mortality at an early age in patients of both genders. Although renal failure has been previously described as the cause of death with the highest prevalence, recent studies, based on the analysis of the population recorded in the Fabry Registry, reported that 40% of deaths had a cardiovascular origin. Data from the same registry emphasize the high risk for these patients suffering cardiovascular events—particularly acute myocardial infarction—and to the fact that this risk is higher for those patients who need dialysis. Microvascular dysfunction is a constant in patients with Fabry disease, which affects young patients, independently from other cardiac involvement manifestations—such as left ventricular hypertrophy—and from the patient’s gender.https://doi.org/10.1177/2326409816679427 |
| spellingShingle | Gustavo Cabrera Update on Coronary Involvement in Fabry Disease Journal of Inborn Errors of Metabolism and Screening |
| title | Update on Coronary Involvement in Fabry Disease |
| title_full | Update on Coronary Involvement in Fabry Disease |
| title_fullStr | Update on Coronary Involvement in Fabry Disease |
| title_full_unstemmed | Update on Coronary Involvement in Fabry Disease |
| title_short | Update on Coronary Involvement in Fabry Disease |
| title_sort | update on coronary involvement in fabry disease |
| url | https://doi.org/10.1177/2326409816679427 |
| work_keys_str_mv | AT gustavocabrera updateoncoronaryinvolvementinfabrydisease |