| Autor: |
Abraham SC; Department of Pathology and Laboratory Medicine, University of Pennsylvania Medical Center, Philadelphia, USA., DeNofrio D, Loh E, Minda JM, Tomaszewski JE, Pietra GG, Reynolds C |
| Jazyk: |
angličtina |
| Zdroj: |
Human pathology [Hum Pathol] 1998 Aug; Vol. 29 (8), pp. 876-82. |
| DOI: |
10.1016/s0046-8177(98)90460-9 |
| Abstrakt: |
Desmin myopathy is a rare idiopathic disorder characterized by abnormal aggregates of desmin-type intermediate filaments, which affects cardiac and skeletal muscle, and rarely the intestinal smooth muscle. We report a 42-year-old woman with atrial fibrillation and progressive restrictive cardiomyopathy. Left ventricular biopsy, cardiac explant, and subsequent autopsy study of skeletal muscle revealed cytoplasmic granulo-filamentous inclusions that were continuous with Z-lines and were immunoreactive for desmin filaments both at the light immunohistochemical and electron microscopic level. In addition, we report the presence of characteristic inclusions within the smooth muscle of intramural coronary blood vessels. This is the first description of desmin inclusions within vascular smooth muscle, and underscores the systemic nature of this rare myopathy. |
| Databáze: |
MEDLINE |
| Externí odkaz: |
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