Castano Ekaterina, Palmer Matthew B, Vigneault Christine, Luciano Randy, Wong Serena, Moeckel Gilbert
Department of Pathology, Yale University School of Medicine, New Haven, CT, USA.
Department of Medicine, Yale University School of Medicine, New Haven, CT, USA.
BMC Nephrol. 2015 Apr 29;16:64. doi: 10.1186/s12882-015-0046-0.
Amyloidosis leads to deposition of abnormal protein with beta-pleated sheet structure in specific compartments of the affected organs. The histological localization of these amyloid deposits determines the overall survival of the patient.
In this study we have assessed the histological localization and severity of amyloid deposition in 35 patients with biopsy-proven renal amyloidosis and have compared those to clinical parameters, histo-pathological injury criteria and respective patient outcome. Comparisons were statistically analyzed using thus comparison between the different study groups, which was done using Student t-test and analysis of variance.
We find that the glomerulus is by far the most commonly and most severely affected renal compartment and patients with severe glomerular amyloidosis advance faster towards end stage renal disease (ESRD) and death, compared to those patients without glomerular amyloid deposits. Patients with severe glomerular amyloidosis showed higher serum creatinine and urine protein levels, while patients with severe vascular amyloidosis showed higher levels of interstitial inflammatory infiltrate.
In kidneys affected by amyloidosis, the amyloid proteins are predominantly deposited along vessels, especially the small vessels including glomerular capillary loops. The severity of glomerular amyloid deposition enhances the risk of developing ESRD and increases the risk for premature death.
淀粉样变性导致具有β-折叠片层结构的异常蛋白质在受影响器官的特定区域沉积。这些淀粉样沉积物的组织学定位决定了患者的总体生存期。
在本研究中，我们评估了35例经活检证实为肾淀粉样变性患者淀粉样沉积的组织学定位和严重程度，并将其与临床参数、组织病理学损伤标准及相应的患者预后进行比较。使用Student t检验和方差分析对不同研究组之间的比较进行统计学分析。
我们发现，肾小球是迄今为止受影响最常见且最严重的肾部分，与无肾小球淀粉样沉积物的患者相比，严重肾小球淀粉样变性患者向终末期肾病（ESRD）和死亡进展得更快。严重肾小球淀粉样变性患者的血清肌酐和尿蛋白水平较高，而严重血管淀粉样变性患者的间质炎症浸润水平较高。
在受淀粉样变性影响的肾脏中，淀粉样蛋白主要沿血管沉积，尤其是包括肾小球毛细血管袢在内的小血管。肾小球淀粉样沉积的严重程度增加了发生ESRD的风险，并增加了过早死亡的风险。
