关注囊性纤维化儿童营养管理

Focus on nutritional management for children with cystic fibrosis

囊性纤维化(CF)是一种危害儿童健康的常染色体隐性遗传病,常累及呼吸、消化等多系统,出现多脏器受损。多种因素导致营养不良在CF儿童非常常见,伴胰腺功能障碍表型的患儿常在出生时或出生1年即可出现吸收不良和/或生长发育障碍。营养状态与CF儿童的肺功能及生存期密切相关,因此,营养管理对CF患儿非常重要。但在实际临床工作中,国内对CF儿童的营养管理远远不足,我国CF患儿的生存期亦远低于欧美国家。本研究从CF患儿营养管理的重要性、营养管理方法及营养评估和监测等方面进行介绍,以提高儿科临床医师对CF儿童营养状态的关注及营养管理的认识,做到早期识别及干预,改善预后,延长生存期。

Cystic fibrosis(CF)is a life-threatening autosomal recessive hereditary disease,involving multiple organs and systems such as respiratory and digestive systems.Malnutrition caused by various factors is common in CF children.CF children with pancreatic dysfunction suffer from malabsorption and/or growth disorder at birth or 1 year old.Nutritional status is associated with lung function and prognosis of CF patients,so nutritional management is extremely important to CF children.However,there is inadequate nutritional management for CF children in clinical practice in China,and Chinese CF children has shorter survival time than CF children in European and American countries.Thus,in this study,the importance of nutritional management,nutritional management methods,nutritional assessment and monitoring of CF patients were introduced.The aim is to enhance clinical attention to the nutritional status of CF children and clinical understanding of nutritional management.Early identification and intervention can improve prognosis and prolong the life of pediatric CF patients.