巴西婴儿神经轴索营养不良（INAD）儿童和青少年面临的喂养挑战对营养素摄入和营养状况产生不利影响。
Feeding challenges faced by Brazilian children and adolescents with infantile neuroaxonal dystrophy (INAD) adversely impact nutrient intake and nutritional status.
文献信息
| PMID | 42820544 |
|---|---|
| 原文 | 在 PubMed 查看原文 ↗ |
| 发表日期 | 2026 |
| 作者 | Laura Fantazzini Grandisoli |
| 作者单位 | Universidade Paulista, Sorocaba, SP, Brazil. |
| 期刊 | Einstein (Sao Paulo, Brazil) |
| SCI 分区 | Q3 |
| IF | 1.2 |
| 研究类型 | 临床研究 · 临床 |
| 所属专科 | 咽喉科 |
中文摘要
目的： 评估巴西婴儿神经轴索营养不良儿童和青少年的营养状况、膳食摄入和喂养特征，并比较完全经口喂养与经胃造瘘喂养的参与者。
方法： 这项回顾性横断面研究纳入了2023年7月至2024年3月期间21例经基因确诊的婴儿神经轴索营养不良患者。数据收集包括24小时膳食回顾、人体测量以及使用功能性经口摄入量表评估吞咽困难。使用Mann-Whitney U检验比较营养状况和摄入量（p<0.05）。
结果： 12名参与者（57%）接受胃造瘘喂养。在经口喂养的参与者中（n=9），66.7%需要调整食物质地，88.9%表现出提示吞咽困难的症状。完全经口喂养组的年龄别体重Z评分倾向于低于胃造瘘喂养组（-2.10对-0.34，p=0.072）。完全经口喂养组的微量营养素摄入，包括维生素A、D、E、叶酸和B12，低于胃造瘘喂养组。超加工食品经常被消费，导致饮食单调。
结论： 喂养困难在婴儿神经轴索营养不良患者中很常见，并与营养素摄入受损和生长欠佳相关，尤其是在完全经口喂养的个体中。这些发现强调了采取个体化营养策略和多学科护理以应对吞咽困难并优化营养结局的重要性。
英文摘要
OBJECTIVE: To evaluate the nutritional profile, dietary intake, and feeding characteristics of Brazilian children and adolescents with infantile neuroaxonal dystrophy, comparing exclusively orally fed and gastrostomy-fed participants.
METHODS: This retrospective cross-sectional study included 21 genetically confirmed patients with infantile neuroaxonal dystrophy between July 2023 and March 2024. Data collection involved 24-hour dietary recalls, anthropometric measurements, and assessment of dysphagia using the Functional Oral Intake Scale. Nutritional status and intake were compared using the Mann-Whitney U test (p<0.05).
RESULTS: Twelve participants (57%) received gastrostomy feeding. Among orally fed participants (n=9), 66.7% required food texture modifications and 88.9% exhibited symptoms suggestive of dysphagia. Weight-for-age Z-scores tended to be lower in the exclusively orally fed group than in the gastrostomy-fed group (-2.10 versus -0.34, p=0.072). Micronutrient intake, including vitamins A, D, E, folic acid, and B12, was lower in the exclusively orally fed group than in the gastrostomy-fed group. Ultra-processed foods were frequently consumed, leading to a monotonous diet.
CONCLUSION: Feeding difficulties are common among patients with infantile neuroaxonal dystrophy and are associated with impaired nutritional intake and suboptimal growth, particularly in exclusively orally fed individuals. These findings underscore the importance of individualized nutritional strategies and multidisciplinary care to address dysphagia and optimize nutritional outcomes.