Intravascular ultrasound assessment of coronary artery involvement in Fabry disease
Jazyk angličtina Země Spojené státy americké Médium print-electronic
Typ dokumentu časopisecké články, práce podpořená grantem
- MeSH
- cévní endotel patologie MeSH
- Fabryho nemoc komplikace diagnóza diagnostické zobrazování MeSH
- fibroblasty metabolismus MeSH
- koronární angiografie metody MeSH
- koronární cévy diagnostické zobrazování patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nemoci koronárních tepen komplikace diagnóza diagnostické zobrazování MeSH
- senioři MeSH
- studie případů a kontrol MeSH
- trihexosylceramidy metabolismus MeSH
- ultrasonografie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Názvy látek
- globotriaosylceramide MeSH Prohlížeč
- trihexosylceramidy MeSH
AIM: We used intravascular ultrasound (IVUS) to characterize coronary artery involvement in patients with Fabry disease (FD). METHODS: Nine FD patients (5 women) were matched to 10 control patients (5 women) chosen from our IVUS database. Standard volumetric IVUS analyses were performed along with assessment of plaque echodensity. RESULTS: Plaques in FD patients were diffuse and hypoechogenic compared with more focal and more echogenic lesions in control patients. Echogenicity of plaques was significantly lower in FD patients (median 30.7 +/- 12.9 vs 55.9 +/- 15.7, p = 0.0052, mean 37.2 +/- 15.6 vs 66.2 +/- 13.3, p = 0.0014). Diffusiveness was assessed as differences between mean and median plaque burden versus the plaque burden in each of the analysed cross-sections. These differences were lower in FD vs controls (5.8 +/- 4.8 vs 8.7 +/- 6.6, p < 0.001 for mean, and 5.8 +/- 4.9 vs 8.8 +/- 7.3, p < 0.001 for median) indicating a more diffuse involvement. The occurrence of lipid cores was significantly higher in FD patients than in controls (2.4 +/- 1.5 vs 1.0 +/- 0.94, p = 0.02). CONCLUSION: IVUS showed diffuse hypoechogenic plaques in patients with FD. The explanation may be higher lipid content in plaques and accumulation of glycosphingolipid in smooth-muscle and endothelial cells.
2nd Department of Internal Medicine 1st Faculty of Medicine Charles University Prague Czech Republic
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