Lindert Judith, Day Hannah, de Andres Crespo Marta, Amerstorfer Eva, Alexander Sabine, Backes Manouk, de Filippo Carlotta, Golebiewski Andrzej, Midrio Paola, Mohideen Mazeena, Modrzyk Anna, Lemli Anette, Rassouli-Kirchmeier Roxana, Pfaff-Jongman Marijke, Staszkiewicz Karolina, Telborn Lovisa, Stenström Pernilla, Holström Karolin, Kohl Martina, Curry Joe, Loukogeorgakis Stavros, Davidson Joseph R
Department of Paediatric Surgery, University Rostock, Ernst-Heydemann-Str. 8, 18057 Rostock, Germany.
Department of Specialist Neonatal and Paediatric Surgery Great, Ormond Street Hospital NHS Trust, London WC1N 3JH, UK.
Children (Basel). 2024 Sep 12;11(9):1118. doi: 10.3390/children11091118.
This study aimed to understand the influence of diet and nutrition items on gastrointestinal symptoms in patients with Hirschsprung Disease (HD). An online questionnaire was created to obtain patient-reported outcomes using the multinational Holistic Care in Hirschsprung Disease Network. This was distributed in Dutch, English, German, Italian, Polish, and Swedish via patient associations. Information on demographics, the extension of disease, current diet, and the influence of food ingredients on bowel function were obtained. In total, 563 questionnaires were answered by parents or patients themselves. The length of the aganglionic segment was short in 33%, long in 45%, total colonic aganglionosis (TCA) in 11%, and involved the small intestine in 10%. Overall, 90% reported following a mixed diet, and 31% reported taking probiotics, with twice as many patients taking probiotics in the TCA group compared to standard HD. Mealtimes and behaviours around eating were affected by 61%, while 77% had established food items that worsened symptoms, and of these, 80% stated that they had worked these items out themselves. A high-fibre diet was followed by 24% and 18% a low-fibre diet. Symptoms were reported, particularly from dairy in 30%, fruits in 39%, pulses in 54%, and sugar in 48%. This first multinational survey on diet and bowel function in HD reports an association between certain dietary items with gastrointestinal symptoms. This study can support an improved understanding of the interaction between food items and bowel function in children with HD. We suggest a multidisciplinary approach to balance dietary exclusions and support adequate growth, preventing nutrition deficiencies and enhancing quality of life.
本研究旨在了解饮食和营养项目对先天性巨结肠病（HD）患者胃肠道症状的影响。通过跨国先天性巨结肠病网络整体护理项目创建了一份在线问卷，以获取患者报告的结果。该问卷通过患者协会以荷兰语、英语、德语、意大利语、波兰语和瑞典语进行分发。收集了有关人口统计学、疾病范围、当前饮食以及食物成分对肠道功能影响的信息。共有563份问卷由家长或患者本人作答。无神经节段长度短的占33%，长的占45%，全结肠无神经节细胞症（TCA）占11%，累及小肠的占10%。总体而言，90%的人报告遵循混合饮食，31%的人报告服用益生菌，TCA组服用益生菌的患者数量是标准HD组的两倍。61%的人进餐时间和进食行为受到影响，77%的人确定了会使症状加重的食物，其中80%的人表示他们自己找出了这些食物。24%的人遵循高纤维饮食，18%的人遵循低纤维饮食。报告出现症状的食物，特别是乳制品占30%，水果占39%，豆类占54%，糖占48%。这项关于HD饮食和肠道功能的首次跨国调查报告了某些饮食项目与胃肠道症状之间的关联。本研究有助于更好地理解HD患儿食物与肠道功能之间的相互作用。我们建议采用多学科方法来平衡饮食限制并支持充足生长，预防营养缺乏并提高生活质量。
