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波兰雷特综合征女孩的饮食和营养状况-病例对照研究。

Diet and Nutritional Status of Polish Girls with Rett Syndrome-A Case-Control Study.

机构信息

Department of Clinical Dietetics, Faculty of Health Sciences, Medical University of Warsaw, 01-445 Warsaw, Poland.

Polish Rett Syndrome Association, 62-500 Konin, Poland.

出版信息

Nutrients. 2023 Jul 27;15(15):3334. doi: 10.3390/nu15153334.

Abstract

(1) Background: Rett syndrome may be considered a disease strongly associated with nutritional disorders that are likely to require special management strategies, extending beyond what is usually required for children with other developmental disorders. The aim of the study was to assess the nutritional status and diet of Polish girls with Rett syndrome. (2) Methods: Each patient (study group = 49, control group = 22) underwent anthropometric measurements, including body weight and height, waist, hip and arm circumference, and skinfold measurement. The assessment of the diet was based on the analysis of 7-day menus and the Food Frequency Questionnaire (FFQ-6). Data were analyzed using Statistica 13.3. (3) Results: The majority of the girls with Rett syndrome were deficient in weight and height, and consumed fewer calories, less protein, dietary fiber, calcium, and iron than the control group. They also drank less fluid. Soft products that were easy to chew and considered to be high in energy value were significantly more common in the menus. (4) Conclusions: Girls with Rett syndrome are characterized by weight deficiencies, poor growth that deteriorates with age, and are at risk of food shortages. Various nutritional intervention strategies should be explored to reduce and, if possible, prevent malnutrition and cachexia in such patients.

摘要

(1) 背景:Rett 综合征可能被认为是一种与营养障碍密切相关的疾病,可能需要特殊的管理策略,超出了其他发育障碍儿童通常所需的策略。本研究旨在评估波兰 Rett 综合征女孩的营养状况和饮食。

(2) 方法:每位患者(研究组=49 例,对照组=22 例)均进行了人体测量学测量,包括体重和身高、腰围、臀围和臂围以及皮褶厚度测量。饮食评估基于 7 天菜单和食物频率问卷(FFQ-6)的分析。数据使用 Statistica 13.3 进行分析。

(3) 结果:大多数 Rett 综合征女孩体重和身高不足,摄入的热量、蛋白质、膳食纤维、钙和铁比对照组少。她们也喝较少的液体。菜单中更常见的是那些柔软、易于咀嚼且被认为热量高的软产品。

(4) 结论:Rett 综合征女孩的特点是体重不足、随年龄增长而恶化的生长不良,并且存在食物短缺的风险。应探索各种营养干预策略,以减少并在可能的情况下预防此类患者的营养不良和恶病质。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba3b/10420679/d33b2a13ec54/nutrients-15-03334-g001.jpg

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