Views: 1,346 | Downloads:
21
| Responses: 0
| XML | Respond to this article | Alert & updates | Request permissions | Email to a friend |
Case ReportOpen Access
The Ultrastructural Study in a Case of Fabry Disease
Palungwachira P 
,
Yaguchi H
,
Yaguchi H An investigation of the ultrastructural study was conducted on specimens from a typical
patient with Fabry disease. Numerous characteristic cytoplasmic inclusions were observed in the
vascular endothelial cell, pericyte, smooth muscle cell, nerve and eccrine sweat glands, the lamellar
pattern of which were considerably variable in various types of gland cells. Large vacuolar inclusions
predominated in clear cells of the secretory coil; lesser vacuoles were also seen in the coiled duct,
and the basal cells of the straight duct toward the coiled duct displayed mulberry-like figures. There
were some clear cells showing cell damage and necrosis in the secretory coil. Lamellated bodies
were noted in the axons and schwann cells around the eccrine sweat glands. The small blood vessels
around the eccrine glands were narrowed by swollen endothelial cells with heavy inclusions. These
intracytoplasmic deposits may be responsible for the decreased sweating ability in Fabry disease. The
factors related to hypohidrosis are also discussed.
Key word : Fabry Disease, Ultrastructural Study
patient with Fabry disease. Numerous characteristic cytoplasmic inclusions were observed in the
vascular endothelial cell, pericyte, smooth muscle cell, nerve and eccrine sweat glands, the lamellar
pattern of which were considerably variable in various types of gland cells. Large vacuolar inclusions
predominated in clear cells of the secretory coil; lesser vacuoles were also seen in the coiled duct,
and the basal cells of the straight duct toward the coiled duct displayed mulberry-like figures. There
were some clear cells showing cell damage and necrosis in the secretory coil. Lamellated bodies
were noted in the axons and schwann cells around the eccrine sweat glands. The small blood vessels
around the eccrine glands were narrowed by swollen endothelial cells with heavy inclusions. These
intracytoplasmic deposits may be responsible for the decreased sweating ability in Fabry disease. The
factors related to hypohidrosis are also discussed.
Key word : Fabry Disease, Ultrastructural Study
Download:
PDF