Schlieper Georg, Grotemeyer Dirk, Aretz Anke, Schurgers Leon J, Krüger Thilo, Rehbein Hermann, Weirich Thomas E, Westenfeld Ralf, Brandenburg Vincent M, Eitner Frank, Mayer Joachim, Floege Jürgen, Sandmann Wilhelm, Ketteler Markus
Department of Nephrology and Clinical Immunology, RWTH University Hospital, and Central Facility for Electron Microscopy, RWTH University, Aachen, Germany.
Ann Vasc Surg. 2010 Apr;24(3):408-14. doi: 10.1016/j.avsg.2009.11.006. Epub 2010 Feb 7.
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.
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.
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.
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.
珊瑚礁样主动脉是一种罕见的血管疾病，其特征为内脏主动脉背侧腔内钙化。这种具有特定部位和形态学特征的疾病的发病机制尚不清楚。本研究的目的是调查珊瑚礁样主动脉患者钙化抑制剂及钙化的超微结构。
对10例珊瑚礁样主动脉患者进行检查。采用免疫组织化学技术研究钙化标本中钙化抑制剂基质γ-羧基谷氨酸蛋白（MGP）和胎球蛋白-A的表达。还通过包括电子能量损失谱、电子能谱和电子衍射在内的电子显微镜技术评估血管壁。分析患者血清中的胎球蛋白-A、未羧化MGP（ucMGP）和骨保护素。
通过免疫组织化学评估，大部分MGP在钙化区域附近被检测到。钙化抑制剂ucMGP、胎球蛋白-A和骨保护素的血清水平分别为370±107 nmol/L、0.57±0.03 g/L和5.64±0.79 pmol/L。钙化标本的超微结构分析显示具有多个钙化核的核壳结构。钙化表现出微晶特征，元素分析显示羟基磷灰石为化合物。
珊瑚礁样主动脉代表了一种具有多个核的血管钙化的极端外生性生长，类似于典型的中膜钙化。血管免疫染色阳性以及胎球蛋白-A和ucMGP的低血清水平提示这些钙化抑制剂在珊瑚礁样主动脉发生发展中具有病理生理作用。
