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Published on: August 28, 2018
Analysis of calcifications in patients with coral reef aorta
Georg Schlieper1, Dirk Grotemeyer, Anke Aretz
1Department of Nephrology and Clinical Immunology, RWTH University Hospital, and Central Facility for Electron Microscopy, RWTH University, Aachen, Germany. gschlieper@ukaachen.de
Insights
Coral reef aorta involves extensive vascular calcification. Low levels of calcification inhibitors, including matrix Gla protein (MGP) and fetuin-A, suggest their role in this rare disease.
Area of Science:
- Vascular Biology
- Mineral Metabolism
- Pathology
Background:
- Coral reef aorta is a rare vascular condition characterized by dorsal visceral aorta calcifications.
- The underlying pathogenesis and morphology remain largely unknown.
Purpose of the Study:
- To investigate calcification inhibitors and the ultrastructure of calcifications in patients with coral reef aorta.
Main Methods:
- Examined ten patients with coral reef aorta.
- Utilized immunohistochemistry for matrix Gla protein (MGP) and fetuin-A expression.
- Conducted electron microscopy and spectroscopy on calcified specimens.
- Analyzed serum levels of fetuin-A, uncarboxylated MGP (ucMGP), and osteoprotegerin.
Main Results:
- MGP was predominantly found near calcified areas.
- Serum levels of ucMGP, fetuin-A, and osteoprotegerin were measured.
- Ultrastructural analysis revealed a core-shell structure with multiple nuclei, composed of hydroxyl apatite.
Conclusions:
- Coral reef aorta exhibits extensive calcification with multiple nuclei, similar to medial calcification.
- Positive vascular MGP staining and low serum fetuin-A and ucMGP suggest their involvement in the disease's pathophysiology.
Background:
Coral reef aorta is a rare vascular disease with intraluminal calcifications of the dorsal part of the visceral aorta. The pathogenesis of this disease with its topographic and morphologic characteristics is unknown. The aim of our study was to investigate calcification inhibitors and the ultrastructure of calcifications in patients with coral reef aorta.
Methods:
Ten patients with coral reef aorta were examined. Calcified specimens were investigated by immunohistochemical techniques for the expression of the calcification inhibitors matrix gla protein (MGP) and fetuin-A. Vessel walls were also assessed by electron microscopic techniques including electron energy-lost spectroscopy, electron dispersive spectroscopy, and electron diffraction. Sera of patients were analyzed for fetuin-A, uncarboxylated MGP (ucMGP), and osteoprotegerin.
Results:
As assessed by immunohistochemistry, most MGP was detected in the vicinity of calcified regions. Serum levels of the calcification inhibitors ucMGP, fetuin-A, and osteoprotegerin were 370+/-107 nmol/L, 0.57+/-0.03 g/L, and 5.64+/-0.79 pmol/L, respectively. Ultrastructural analysis of calcified specimens showed a core-shell structure with multiple calcification nuclei. Calcifications displayed a fine-crystalline character, and elemental analysis revealed hydroxyl apatite as the chemical compound.
Conclusion:
The coral reef aorta represents an extreme exophytic growth of vascular calcification with multiple nuclei which resemble typical media calcification. Positive vascular immunostaining and low serum levels of both fetuin-A and ucMGP suggest a pathophysiologic role of these calcification inhibitors in the development of coral reef aorta.
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