7-T (35)Cl and (23)Na MR Imaging for Detection of Mutation-dependent Alterations in Muscular Edema and Fat Fraction with Sodium and Chloride Concentrations in Muscular Periodic Paralyses.
7-T (35)Cl and (23)Na MR Imaging for Detection of Mutation-dependent Alterations in Muscular Edema and Fat Fraction with Sodium and Chloride Concentrations in Muscular Periodic Paralyses.
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7-T (35)Cl 和 (23)Na MR 成像用于检测肌肉周期性麻痹中钠和氯浓度引起的肌肉水肿和脂肪分数的突变依赖性变化
DOI：
10.1148/radiol.2016151617
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发表时间：
2016
期刊：
影响因子：
19.7
通讯作者：
Lehmann-Horn F
中科院分区：
文献类型：
--
作者：
Weber MA;Nagel AM;Marschar AM;Glemser P;Jurkat-Rott K;Wolf MB;Ladd ME;Schlemmer HP;Kauczor HU;Lehmann-Horn F
目的确定是否改变了钠（Na+）和氯 (Cl−）稳态可以在周期性 使用7-T钠23（23 Na）和氯35麻痹 材料和方法机构审查委员会批准和所有参与者的知情同意 23 Na（重复时间毫秒/回波时间毫秒， 160/0.35）和35 Cl（40/0.6）的双下肢MR成像。 使用7-T全身系统在遗传性 确诊的低钾型周期性麻痹（Cav1.1-R1239 H突变，n= 5; Cav1.1-R528 H突变，n= 8）和Andersen-Tawil综合征（n= 3）， 16名健康志愿者此外，每个参与者都接受了3-T质子 同一天使用T1加权短tau进行MR成像 反转恢复和Dixon型序列。评估肌肉水肿， 短tau反转恢复图像，评估脂肪变性， T1加权成像，并采用Dixon型定量肌脂肪分数 显像Na+和Cl−在 用含10-，20-， 30 mmol/L NaCl溶液和5%琼脂糖凝胶作为对照。参数数据 采用单因素方差分析对所有亚群进行了检验 Dunnett检验，相关性用斯皮尔曼等级进行评估 相关系数。结果中位肌23钠浓度较高， Cav1.1-R1239 H（34.7 mmol/L，P< .001），Cav1.1-R528 H (32.0 mmol/L，P<0.001）和Kir2.1（24.3 mmol/L，P= 0.035）突变 (19.9 mmol/L）的浓度。肌肉标准化35 Cl信号强度中位数 Cav1.1-R1239 H基因型患者的TNM分期明显高于正常对照组（27.6，P <0.01）。 Cav1.1-R528H（23.6，P < .001） 健康志愿者（12.6），但在Kir2.1突变患者中没有 (14.3，P= .517）。与志愿者相比， Cav1.1-R1239 H和Cav1.1-R528 H患者显示肌肉水肿增加 （P< .001和P = .003， 结论在7-T MR成像中，周期性麻痹患者的Na+和Cl−稳态变化最为明显 在严重表型Cav1.1-R1239 H中，每天使用麻痹剂 ©RSNA，2016这篇文章的早期错误版本出现在网上。这篇文章于2016年4月18日更正。
PurposeTo determine whether altered sodium (Na+) and chloride (Cl−) homeostasis can be visualized in periodic paralyses by using 7-T sodium 23 (23Na) and chlorine 35 (35Cl) magnetic resonance (MR) imaging.Materials and MethodsInstitutional review board approval and informed consent of all participants were obtained.23Na (repetition time msec/echo time msec, 160/0.35) and35Cl (40/0.6) MR imaging of both lower legs was performed with a 7-T whole-body system in patients with genetically confirmed hypokalemic periodic paralysis (Cav1.1-R1239H mutation,n= 5; Cav1.1-R528H mutation,n= 8) and Andersen-Tawil syndrome (n= 3) and in 16 healthy volunteers. Additionally, each participant underwent 3-T proton MR imaging on the same day by using T1-weighted, short-tau inversion-recovery, and Dixon-type sequences. Muscle edema was assessed on short-tau inversion-recovery images, fatty degeneration was assessed on T1-weighted images, and muscular fat fraction was quantified with Dixon-type imaging. Na+and Cl−were quantified in the soleus muscle by using three phantoms that contained 10-, 20-, and 30-mmol/L NaCl solution and 5% agarose gel as a reference. Parametric data for all subpopulations were tested by using one-way analysis of variance with the Dunnett test, and correlations were assessed with the Spearman rank correlation coefficient.ResultsMedian muscular23Na concentration was higher in patients with Cav1.1-R1239H (34.7 mmol/L,P< .001), Cav1.1-R528H (32.0 mmol/L,P< .001), and Kir2.1 (24.3 mmol/L,P= .035) mutations than in healthy volunteers (19.9 mmol/L). Median muscular normalized35Cl signal intensity was higher in patients with Cav1.1-R1239H (27.6,P< .001) and Cav1.1-R528H (23.6,P< .001) than in healthy volunteers (12.6), but not in patients with the Kir2.1 mutation (14.3,P= .517). When compared with volunteers, patients with Cav1.1-R1239H and Cav1.1-R528H showed increased muscular edema (P< .001 andP= .003, respectively) and muscle fat fraction (P< .001 andP= .017, respectively).ConclusionWith 7-T MR imaging, changes of Na+and Cl−homeostasis can be visualized in periodic paralyses and are most pronounced in the severe phenotype Cav1.1-R1239H, with up to daily paralytic episodes.©RSNA, 2016An earlier incorrect version of this article appeared online. This article was corrected on April 18, 2016.
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影响因子：
19.7
作者：
A. Nagel;F. Lehmann;M. Weber;K. Jurkat;M. Wolf;A. Radbruch;R. Umathum;W. Semmler
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影响因子：
19.7
作者：
Umathum, Reiner;Roesler, Manuela B.;Nagel, Armin M.
通讯作者：
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DOI：
10.1007/s10334-013-0398-z
发表时间：
2014-02-01
影响因子：
2.3
作者：
Baier, Sebastian;Kraemer, Philipp;Schad, Lothar R.
通讯作者：
Schad, Lothar R.