15例甲基丙二酸血症患儿营养状况评价及相关分析

Analysis of two methods for the nutritional evaluation on fifteen Chinese patients with methylmalonic acidemia

目的探讨甲基丙二酸血症(methylmalonic acidemia,MMA)患儿的入院营养评价方法及发现存在的营养问题。方法 2013年6月至2014年5月住院的15例3岁以下的MMA患者,男7例,女8例。年龄20天至29个月,平均6个月。采用膳食调查,生化检查,体格检查,人体测量的方法,按体重年龄比(W/A),分别采用2010年中国儿科肠内肠外营养支持指南中位数百分比标准和2013年中华人民共和国卫生行业标准WS 423-2013(Z评分),对患儿进行入院营养状况评价。结果 15例患儿在入院前均存在喂养困难。采用2010年中国儿科肠内肠外营养支持指南标准(W/A),15例中9例(60%)有营养不良存在。用Z评分,15例有5例(33.3%)属于营养不良。两个筛查方法发现营养不良率相比,差异有显著性(P<0.05),Z评分标准营养不良检出率低。血常规,15例有14例血色素低,12例红细胞数低。血生化,15例患者有5例白蛋白低,6例甘油三酯高,11例存在高半胱氨酸血症,3例空腹血糖低。结论 MMA婴幼儿患者营养不良发生率高,多数存在贫血、高脂血症、高半胱氨酸血症。用2010年中国儿科肠内肠外营养支持指南标准,营养不良检出率高,有利于及时进行营养干预。

Objective To compare two nutritional evaluation tools for Chinese pediatric patients with methylmalonic acidemia（MMA）. Method From June 2013 to May 2014, 15 MMA patients with age below 3 years old were selected. These pediatric patients included 7 males and 8 females, aged from 20 days to 29 months with the average age 6 months. Dietary recall, biochemical examination, physical examination and anthropometry were used to evaluate patients＇ nutritional status by two methods, which are according to weight-to-age（W/A） from Guidelines for pediatric clinical application of enteral and parenteral nutritional support in China（2010） and according to z-score from Health Industry Standard of the People＇s Republic of China WS 423-2013（2013）. Result All of the 15 patients had feeding difficulties before admission. According to the patients＇ W/As from Guidelines for pediatric clinical application of enteral and parenteral nutritional support in China（2010）, 9 cases（60%） of the 15 cases MMA patient were found to have malnutrition. According to the patients＇ Z scores from WS 423-2013, 5 cases（33.3%） of the 15 patients were found to have malnutrition. The difference between the two methods was statistically significant（P〈0.01）. 14 of the 15 patients were diagnosed with low hemoglobin. 12 of the patients was diagnosed with low red blood cells. 5 cases of the 15 patients were found to have low albumin. 6 cases were found to have hypertriglyceridemia. 11 cases were found to have homocysteinemia. 3 cases of them were found to have low fasting blood glucose（FBG）. Conclusion There is a higher conincidence of malnutrition in pediatric MMA patients. Most of the patients have anemia, hypertriglyceridemia and homocysteinemia. Guidelines for pediatric clinical application of enteral and parenteral nutritional support in China may have higher positive rate for malnutrition, which is helpful for nutritional treatment in time.