Nutritional Status, nutrient intake and dietary practices of Brazilian children and adolescents with Infantile Neuroaxonal Dystrophy (INAD)

The objective fo the study was to delineate the nutritional profile, dietary intake, and feeding characteristics among orally
and tube-fed Brazilian patients with INAD. This is a retrospective cross-sectional study conducted between July 2023 and March 2024. Data were collected through a semi- structured electronic form and phone interviews. Food intake was assessed by three 24-hour
food recalls applied via phone and video calls. Reported body weight and height were used to determine BMI (body mass index). Nutritional status was assessed using weight-for-age (WAZ), height-for-age (HAZ), and BMI-for-age Z-scores (BMIAZ) from WHO child growth standards. The study enrolled 21 participants. Twelve of them (57%) had a gastrostomy for feeding. Considering the 9 participants exclusively orally fed, 66.7% needed modification in food texture, and 88.9% presented at least one sign of dysphagia, most commonly choking and coughing when eating. There was a trend toward a lower median weight-for-age Z-score in oral-fed participants compared to those fed by gastrostomy (-2.10 versus -0.34 respectively, p=0.072). Micronutrient intake, including vitamins A, D, E, folic acid, and B12, was lower in the oral-fed group.

本研究旨在阐述巴西经口和管饲的INAD患者群体的营养状况、膳食摄入及喂养特征。该研究为一项于2023年7月至2024年3月间进行的回顾性横断面研究。数据通过半结构化的电子问卷和电话访谈收集。通过电话和视频通话进行三次24小时食物回顾来评估食物摄入量。利用报告的体重和身高数据，根据世界卫生组织（WHO）儿童成长标准，计算了体重指数（BMI）。营养状况通过体重-年龄（WAZ）、身高-年龄（HAZ）和BMI-年龄Z分数（BMIAZ）进行评估。研究共纳入21名参与者。其中12名（占57%）因喂养需要行胃造口术。在仅经口喂养的9名参与者中，有66.7%需要调整食物质地，88.9%至少表现出一种吞咽困难症状，最常见为进食时的窒息和咳嗽。与胃造口术喂养的参与者相比，经口喂养的参与者的体重-年龄Z分数的中位数呈降低趋势（分别为-2.10与-0.34，p=0.072）。在经口喂养组中，包括维生素A、D、E、叶酸和B12在内的微量元素摄入量较低。