经皮内镜下胃造口术在甲基丙二酸血症/丙酸血症合并喂养困难中的应用评估:7例病例分析

李伟艳, 曾永梅, 张纪泳, 陆喜燕, 汪无尽, 黄美, 丘晓颖, 黄秒, 王媛媛, 陈柚燕, 董国庆

中国当代儿科杂志 ›› 2026, Vol. 28 ›› Issue (3) : 330-335.

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中国当代儿科杂志 ›› 2026, Vol. 28 ›› Issue (3) : 330-335. DOI: 10.7499/j.issn.1008-8830.2507104
论著·临床研究

经皮内镜下胃造口术在甲基丙二酸血症/丙酸血症合并喂养困难中的应用评估:7例病例分析

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Application of percutaneous endoscopic gastrostomy in children with methylmalonic acidemia or propionic acidemia complicated by feeding difficulties: a case series of 7 patients

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摘要

目的 探讨经皮内镜下胃造口术(percutaneous endoscopic gastrostomy, PEG)对甲基丙二酸血症(methylmalonic acidemia, MMA)/丙酸血症(propionic acidemia, PA)合并喂养困难患儿的疗效。 方法 回顾性分析2021年6月—2025年4月在深圳市妇幼保健院行PEG的7例MMA/PA合并喂养困难患儿的临床资料,总结其术前评估、围手术期管理及术后随访情况。 结果 7例患儿中位PEG治疗年龄为20个月。PEG均一次性成功,平均手术时间(21.0±2.2)min,术中出血量1~2 mL,术后平均住院(5.7±1.1)d。与术前相比,术后6个月患儿体重、身长增加,酸中毒发作次数减少,每日喂养时间缩短,外出活动时间增加,血清前白蛋白升高(均P<0.05)。术后6~12个月最常见的并发症为造口周围肉芽增生(5例),1例术后7个月发生造口穿孔。 结论 PEG可有效改善MMA/PA合并喂养困难患儿的营养与代谢状态,是一种安全、有效的长期肠内营养支持方式。

Abstract

Objective To evaluate the efficacy and safety of percutaneous endoscopic gastrostomy (PEG) in children with methylmalonic acidemia (MMA) or propionic acidemia (PA) complicated by feeding difficulties. Methods Clinical data of seven children with MMA/PA and feeding difficulty who underwent PEG at Shenzhen Maternity and Child Healthcare Hospital from June 2021 to April 2025 were retrospectively analyzed. Preoperative assessment, perioperative management, and postoperative follow-up were summarized. Results The median age at PEG placement was 20 months. All PEG placements were successfully completed in a single attempt. The mean operative time was (21.0 ± 2.2) minutes, intraoperative blood loss was 1-2 mL, and the mean postoperative hospital stay was (5.7 ± 1.1) days. At 6 months postoperatively, compared with preoperative values, body weight and length increased, the frequency of acidosis episodes decreased, daily feeding time was shortened, time spent on outdoor activities increased, and serum prealbumin levels rose (all P<0.05). During 6-12 months of follow-up, the most common complication was peristomal hypergranulation (five cases). One case developed gastrostomy-site perforation 7 months postoperatively. Conclusions PEG effectively improves nutritional and metabolic status in children with MMA/PA complicated by feeding difficulties and is a safe, effective method for long-term enteral nutrition support.

关键词

甲基丙二酸血症 / 丙酸血症 / 喂养困难 / 经皮内镜下胃造口术 / 儿童

Key words

Methylmalonic acidemia / Propionic acidemia / Feeding difficulty / Percutaneous endoscopic gastrostomy / Child

引用本文

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李伟艳, 曾永梅, 张纪泳, . 经皮内镜下胃造口术在甲基丙二酸血症/丙酸血症合并喂养困难中的应用评估:7例病例分析[J]. 中国当代儿科杂志. 2026, 28(3): 330-335 https://doi.org/10.7499/j.issn.1008-8830.2507104
Wei-Yan LI, Yong-Mei ZENG, Ji-Yong ZHANG, et al. Application of percutaneous endoscopic gastrostomy in children with methylmalonic acidemia or propionic acidemia complicated by feeding difficulties: a case series of 7 patients[J]. Chinese Journal of Contemporary Pediatrics. 2026, 28(3): 330-335 https://doi.org/10.7499/j.issn.1008-8830.2507104

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脚注

所有作者均声明不存在利益冲突。

致谢

感谢广州医科大学附属广州市妇女儿童医疗中心龚四堂教授的技术指导。

基金

深圳市“医疗卫生三名工程”项目(SZSM202311021)

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