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目的:调查初发急性淋巴细胞白血病(ALL)患儿使用左旋门冬酰胺酶(L-Asp)化疗时营养状况的改变，评估添加高蛋白、高中链甘油三酯(MCT)特殊医学用途配方食品(FSMP)作为口服营养补充对改善其营养状况及临床结局的作用。方法:前瞻性纳入2014年7月至2015年4月在上海儿童医学中心确诊ALL并使用含L-Asp的化疗方案进行化疗的患儿共121例，其中63例为对照组，58例为干预组。两组患儿年龄、性别等比较差异无统计学意义(n P>0.05)。对照组在使用L-Asp化疗期间使用常规低脂饮食，干预组在对照组基础上添加高蛋白、高MCT的FSMP，根据中国居民膳食指南建议的热卡摄入，其中1/4热卡由FSMP提供。记录两组1周饮食并进行膳食分析，计算平均热卡及营养素摄入情况，并计算其占指南建议值的百分比。记录入院、出院时患儿体重和白蛋白变化并进行人体成分测定，分析患儿营养状况、住院时间、住院费用等情况。n 结果:ALL患儿使用L-Asp化疗期间摄入均低于指南建议值，其中对照组热卡摄入和营养素摄入均显著低于干预组；化疗期结束时，体脂肪较入院时显著增加，肌肉量显著减少，去脂体重显著减少。上臂围和三头肌皮褶厚度显著减少。出院体重-入院体重：在使用L-ASP化疗期间的体重变化与热卡摄入比较(spearman值0.419，n P＝0.009；与蛋白质摄入比较spearman值0.395，n P＝0.014；与碳水化合物摄入比较spearman值0.473，n P＝0.003，均呈显著正相关。两组患儿体重、白蛋白和前白蛋白在出院时均有下降，其中体重和白蛋白对照组下降更明显，两组比较差异有统计学意义(n P<0.05)。n 结论:ALL患儿化疗期间均有不同程度体重丢失和蛋白水平下降，其状态与营养摄入情况密切相关。口服营养补充是营养治疗的重要途径之一，适当添加高蛋白、高MCT的FSMP有助于缓解ALL患儿的体重丢失及白蛋白下降。“,”Objective:This study aims to survey the changes of the nutritional status of the children with primary acute lymphoblastic leukemia (ALL) using L-asparaginase (L-Asp) and evaluate the effect of Oral nutritional supplements (ONS) added high protein and medium-chain triglyceride (MCT)(FSMP) improving their nutritional status and clinical outcome.Methods:121 ALL patients receiving the L-Asp chemotherapy were randomized administered conventional low-fat formula (control group, 63 cases) and high protein and MCT FSMP (intervention group, 58 cases). We recorded the one-week diet and performed dietary analysis. The changes of body weight and albumin were recorded on admission and discharge, and the body composition was determined, the nutritional status, number of hospitalization days, and medical costs were assessed.Results:ALL patients had lower intake than the guideline′s recommended value during chemotherapy. The control group was significantly lower in calorie intake and nutrients intake than the intervention group. Weight changes during chemotherapy were positivelycorrelated with calorie intake (n P=0.009), protein intake(n P=0.014), and carbohydrate intake(n P=0.003).n Conclusions:ALL children have different degrees of weight loss and protein level decline during chemotherapy, and their nutritional status was closely related to nutritional intake.ONS is an important nutritional therapy. The appropriate addition of high protein and MCT FSMP can alleviate the loss of thin body weight and the decline of albumin in ALL children.