No differences in native T1 of the renal cortex between Fabry disease patients and healthy subjects in cardiac dedicated native T1 maps.

IF 4.2 1区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS
Anna Damlin, Felix Kjellberg, Raquel Themudo, Kelvin Chow, Henrik Engblom, Mikael Oscarson, Jannike Nickander
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引用次数: 0

Abstract

Background: Fabry disease (FD) is an X-linked inherited lysosomal storage disease that is caused by deficient activity of the enzyme alpha-galactosidase A. Cardiovascular magnetic resonance (CMR) imaging can detect cardiac sphingolipid accumulation using native T1 mapping. The kidneys are often visible in cardiac CMR native T1 maps, however it is currently unknown if the maps can be used to detect sphingolipid accumulation in the kidneys of FD patients. Therefore, the aim of this study was to evaluate if cardiac dedicated native T1 maps can be used to detect sphingolipid accumulation in the kidneys.

Methods: FD patients (n=18, 41 ± 10 years, 44% male) and healthy subjects (n=38, 41 ± 16 years, 47% male) were retrospectively enrolled. Native T1 maps were acquired at 1.5T (MAGNETOM Aera) using MOLLI research sequences. Native T1 values were measured by manually delineating regions of interest (ROI) in the renal cortex, renal medulla, heart, spleen, blood, and liver. Endo- and epicardial borders were delineated in the myocardium and averaged across all slices. Blood ROIs were placed in the left-ventricular blood pool in the midventricular slice.

Results: There were no differences in native T1 between the FD patients and the healthy subjects in the renal cortex (1034±88 ms vs 1056±59 ms, p=0.29), blood (1614±111 ms vs 1576 ± 100 ms, p=0.22), spleen (1143±45 ms vs 1132±70 ms, p=0.54) or liver (568±49 ms vs 557±47 ms, p=0.41). Native T1 was lower in the hearts of the FD patients compared to healthy subjects (951±79 vs 1006±38, p<0.01), and higher in the renal medulla (1635±144 vs 1514±81, p<0.01). The results were similar when stratified for sex.

Conclusion: Compared to healthy subjects, patients with FD and cardiac involvement had no differences in native T1 of the renal cortex. FD patients had higher native T1 in the renal medulla, which is not totally explained by differences in blood native T1 but may reflect a hyperfiltration state in the development of renal failure. The findings suggest that sphingolipid accumulation in the renal cortex in FD patients could not be detected with cardiac dedicated research native T1 maps.

在心脏专用原生 T1 图中,法布里病患者与健康人的肾皮质原生 T1 无差异。
背景:法布里病(FD)是一种X连锁遗传性溶酶体贮积病,由α-半乳糖苷酶A活性不足引起。心血管磁共振(CMR)成像可利用原位T1图谱检测心脏鞘脂堆积。肾脏在心脏 CMR 原位 T1 图谱中经常可见,但目前尚不清楚该图谱是否可用于检测 FD 患者肾脏中的鞘脂堆积。因此,本研究旨在评估心脏专用原位 T1 图是否可用于检测肾脏中的鞘脂堆积:方法:回顾性招募 FD 患者(18 人,41 ± 10 岁,44% 为男性)和健康受试者(38 人,41 ± 16 岁,47% 为男性)。使用MOLLI研究序列在1.5T(MAGNETOM Aera)采集原生T1图。通过手动划定肾皮质、肾髓质、心脏、脾脏、血液和肝脏的感兴趣区(ROI)来测量原生 T1 值。在心肌中划定心内膜和心外膜边界,并在所有切片中取平均值。血液 ROI 放置在左心室中室切片的左心室血池中:肾皮质(1034±88 ms vs 1056±59 ms,P=0.29)、血液(1614±111 ms vs 1576±100 ms,P=0.22)、脾脏(1143±45 ms vs 1132±70 ms,P=0.54)或肝脏(568±49 ms vs 557±47 ms,P=0.41)的原生 T1 在 FD 患者和健康受试者之间没有差异。与健康受试者相比,FD 患者心脏的原生 T1 更低(951±79 vs 1006±38,p 结论:与健康人相比,FD和心脏受累患者的肾皮质原生T1没有差异。FD患者肾髓质的原生T1较高,这不能完全用血液原生T1的差异来解释,但可能反映了肾衰竭发展过程中的高滤过状态。研究结果表明,心脏专用研究原生T1图无法检测到FD患者肾皮质中的鞘脂堆积。
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来源期刊
CiteScore
10.90
自引率
12.50%
发文量
61
审稿时长
6-12 weeks
期刊介绍: Journal of Cardiovascular Magnetic Resonance (JCMR) publishes high-quality articles on all aspects of basic, translational and clinical research on the design, development, manufacture, and evaluation of cardiovascular magnetic resonance (CMR) methods applied to the cardiovascular system. Topical areas include, but are not limited to: New applications of magnetic resonance to improve the diagnostic strategies, risk stratification, characterization and management of diseases affecting the cardiovascular system. New methods to enhance or accelerate image acquisition and data analysis. Results of multicenter, or larger single-center studies that provide insight into the utility of CMR. Basic biological perceptions derived by CMR methods.
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