Correlations Between Serum Cholesterol and Vascular Lesions in Fabry Disease Patients
| Autor: | Hiroki Katsuta, Hiroshi Yamamoto, Kazuya Tsuboi, Hiromi Goto |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
Adult
Male 0301 basic medicine medicine.medical_specialty Adolescent Arteriosclerosis Adhesion (medicine) Disease 030204 cardiovascular system & hematology Gastroenterology 03 medical and health sciences chemistry.chemical_compound Sex Factors 0302 clinical medicine Internal medicine medicine Humans Enzyme Replacement Therapy Clinical significance Endothelial dysfunction Child Sphingolipids business.industry Cholesterol HDL General Medicine Enzyme replacement therapy Middle Aged medicine.disease Fabry disease Vascular endothelial growth factor 030104 developmental biology chemistry Fabry Disease Female lipids (amino acids peptides and proteins) Endothelium Vascular Glycolipids Cardiology and Cardiovascular Medicine business Body mass index |
| Zdroj: | Circulation Journal. 82:3058-3063 |
| ISSN: | 1347-4820 1346-9843 |
| Popis: | Background Fabry disease is an X-linked lysosomal storage disorder and shows globotriosylceramide (Gb3) accumulation in multiple organs, resulting from a deficiency of α-galactosidase. In patients with Fabry disease, cardiovascular disease occurs at an early age. Previous studies have shown that serum levels of high-density lipoprotein-cholesterol (HDL-C) increase in this disease, yet its clinical significance for cardiovascular disease remains unclear. Methods and Results: In order to determine why the serum HDL-cholesterol is high in various cardiovascular diseases of Fabry disease patients, we evaluated the serum lipid profiles, ocular vascular lesions, and levels of serum vascular endothelial growth factor (VEGF) and intercellular adhesion molecule-1 in 69 patients with Fabry disease diagnosed by genetic examination. The serum HDL-C/total cholesterol (T-Chol) ratio was significantly high, especially in male patients (41.5±1.7%) regardless of body mass index. Ocular vascular lesions were more likely to occur in female patients with a high HDL-C/T-Chol ratio compared with most male patients. Female patients with a high HDL-C/T-Chol ratio also presented a high serum VEGF level, suggesting that vascular endothelium dysfunction and arteriosclerotic changes progress more severely than in patients with a normal HDL-C/T-Chol ratio. In most patients, enzyme replacement therapy improved serum Gb3 and lyso-Gb3 levels, but these Gb3 and lyso-Gb3 still remained higher than in healthy controls, which appears to result in continuous vascular arteriosclerotic changes. Conclusions We concluded that increased low-density lipoprotein-cholesterol uptake to the vascular wall caused by endothelial dysfunction is likely to contribute to the high HDL-C/T-Chol ratio observed in Fabry disease patients. |
| Databáze: | OpenAIRE |
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