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Abstract #2257

The Targeted 1H MRS of Methylmalonic Acid and Lactate In Vivo

Mengyuan Zhuo1, Yan Yun2, Jiaxiang Xin3, Yufan Chen4, Yang Zhao4, Tao Gong4, Xuewen Xing4, Fei Liu4, Yuxin Li4, Yao Zou5, and Guangbin Wang1,4
1Shandong University, Jinan, China, 2Shandong University QILU Hospital, Jinan, China, 3MR Research Collaboration, Siemens Healthineers Ltd, Shang Hai, China, 4Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China, 5Binzhou Medical University, Jinan, China

Synopsis

Keywords: Neuro, Metabolism, Methylmalonic Acid(MMA),MRS

Motivation: Methylmalonic acidemia is an extremely harmful organic acidemia causing multi-system damage mainly in the central nervous system.

Goal(s): To selectively probe the 1H MR signals of methylmalonic acid and lactate (Lac) in vivo in the brain of methylmalonic acidemia (MMA) patients.

Approach: The optimal J-editing 1H MRS approach was used to probe the MMA and Lac signals in vivo.

Results: The methylmalonic acid and lactate 1H MR signals were selectively probed in a phantom and 8 MMA patients, confirming their correlation with urine and blood concentration levels.

Impact: We successfully detected methylmalonic acid and Lac signals in MMA patients, providing crucial support for exploring MMA metabolic pathways in vivo.

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Keywords