PDF(653 KB)
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.
PDF(653 KB)
PDF(653 KB)
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
Application of percutaneous endoscopic gastrostomy in children with methylmalonic acidemia or propionic acidemia complicated by feeding difficulties: a case series of 7 patients
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。
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.
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
Methylmalonic acidemia / Propionic acidemia / Feeding difficulty / Percutaneous endoscopic gastrostomy / Child
| [1] |
|
| [2] |
顾学范, 韩连书, 余永国. 中国新生儿遗传代谢病筛查现状及展望[J]. 罕见病研究, 2022, 1(1): 13-19. DOI: 10.12376/j.issn.2097-0501.2022.01.003 .
|
| [3] |
| [4] |
|
| [5] |
|
| [6] |
李慧雯, 龚四堂, 杨敏, 等. 经皮内镜下胃造瘘术在儿科应用的疗效分析[J]. 中华儿科杂志, 2016, 54(2): 145-149. DOI: 10.3760/cma.j.issn.0578-1310.2016.02.015 .
|
| [7] |
中国研究型医院学会肠外肠内营养学专业委员会. 经皮内镜下胃(空肠)造口术临床应用中国专家共识(2024版)[J]. 中华胃肠外科杂志, 2024, 27(3): 205-214. DOI: 10.3760/cma.j.cn441530-20231120-00183 .
|
| [8] |
余晓丹. 婴幼儿食物过敏的营养问题[J].中华实用儿科临床杂志, 2019, 34(21): 1614-1617. DOI: 10.3760/cma.j.issn.2095-428X.2019.21.004 .
|
| [9] |
|
| [10] |
|
| [11] |
冯围围, 张悦, 王惠珊, 等. 看护人焦虑水平与3岁以下儿童睡眠状况的关系[J]. 中国心理卫生杂志, 2023, 37(2): 122-127. DOI: 10.3969/j.issn.1000-6729.2023.02.005 .
|
| [12] |
|
| [13] |
|
| [14] |
赵红梅, 卢琪, 欧阳红娟, 等. 儿童经皮内镜下胃造口术临床应用5例报告[J]. 中国实用儿科杂志, 2019, 34(2): 158-160. DOI: 10.19538/j.ek2019020622 .
|
| [15] |
|
| [16] |
郑宏, 马丙祥, 陆相朋. 甲基丙二酸血症神经发育障碍分析与康复策略[J]. 中国实用儿科杂志, 2024, 39(2): 152-156. DOI: 10.19538/j.ek2024020615 .
|
| [17] |
|
所有作者均声明不存在利益冲突。
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。