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        In Vivo Assessment of Neurodegeneration in Type C Niemann-Pick Disease by IDEAL-IQ

        Ruo-Mi Guo,Qing-Ling Li,Zhong-Xing Luo,Wen Tang,Ju Jiao,Jin Wang,Zhuang Kang,Shao-Qiong Chen,Yong Zhang 대한영상의학회 2018 Korean Journal of Radiology Vol.19 No.1

        Objective: To noninvasively assess the neurodegenerative changes in the brain of patients with Niemann-Pick type C (NPC) disease by measuring the lesion tissue with the iterative decomposition of water and fat with echo asymmetry and least square estimation-iron quantification (IDEAL-IQ). Materials and Methods: Routine brain MRI, IDEAL-IQ and 1H-proton magnetic resonance spectroscopy (1H-MRS, served as control) were performed on 12 patients with type C Niemann-Pick disease (4 males and 8 females; age range, 15−61 years; mean age, 36 years) and 20 healthy subjects (10 males and 10 females; age range, 20−65 years; mean age, 38 years). The regions with lesion and the normal appearing regions (NARs) of patients were measured and analyzed based on the fat/water signal intensity on IDEAL-IQ and the lipid peak on 1H-MRS. Results: Niemann-Pick type C patients showed a higher fat/water signal intensity ratio with IDEAL-IQ on T2 hyperintensity lesions and NARs (3.7−4.9%, p < 0.05 and 1.8−3.0%, p < 0.05, respectively), as compared to healthy controls (HCs) (1.2−2.3%). After treatment, the fat/water signal intensity ratio decreased (2.2−3.4%), but remained higher than in the HCs (p < 0.05). The results of the 1H-MRS measurements showed increased lipid peaks in the same lesion regions, and the micro-lipid storage disorder of NARs in NPC patients was detectable by IDEAL-IQ instead of 1H-MRS. Conclusion: The findings of this study suggested that IDEAL-IQ may be useful as a noninvasive and objective method in the evaluation of patients with NPC; additionally, IDEAL-IQ can be used to quantitatively measure the brain parenchymal adipose content and monitor patient follow-up after treatment of NPC.

      • SCIE

        Single-index composite quantile regression

        Jiang, Rong,Zhou, Zhan-Gong,Qian, Wei-Min,Shao, Wen-Qiong 한국통계학회 2012 Journal of the Korean Statistical Society Vol.41 No.3

        In this paper, we extend the composite quantile regression (CQR) method to a single-index model. The unknown link function is estimated by local composite quantile regression and the parametric index is estimated through the linear composite quantile. It is shown that the proposed estimators are consistent and asymptotically normal. The simulation studies and real data applications are conducted to illustrate the finite sample performance of the proposed methods.

      • KCI등재

        Single-index composite quantile regression

        Rong Jiang,Zhan-Gong Zhou,Wei-Min Qian,Wen-Qiong Shao 한국통계학회 2012 Journal of the Korean Statistical Society Vol.41 No.3

        In this paper, we extend the composite quantile regression (CQR) method to a single-index model. The unknown link function is estimated by local composite quantile regression and the parametric index is estimated through the linear composite quantile. It is shown that the proposed estimators are consistent and asymptotically normal. The simulation studies and real data applications are conducted to illustrate the finite sample performance of the proposed methods.

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