ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis
ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis
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DOI：
10.1016/j.clnu.2016.03.004
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发表时间：
2016-06-01
影响因子：
6.3
通讯作者：
Wilschanski, Michael
中科院分区：
文献类型：
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作者：
Turck, Dominique;Braegger, Christian P.;Wilschanski, Michael
背景资料：营养不良是囊性纤维化（CF）的常见特征和合并症，营养状况与肺功能和生存率密切相关。因此，营养管理是CF患者的标准治疗。ESPEN，ESPGHAN和ECFS建议的指南，以涵盖CF患者的营养管理。方法：该指南是由一个国际多学科工作组按照官方认可的标准。GRADE系统用于确定证据等级和推荐强度。声明进行了讨论，提交给德尔菲轮，ESPGHAN和ECFS审查，并接受在网上调查中ESPEN members.Results：工作组建议，启动营养管理应开始后尽早诊断，随后定期随访和患者/家庭教育。建议完全母乳喂养，但如果不可能，则使用常规配方奶粉。应调整能量摄入，以达到正常的体重和身高年龄。如有指征，应尽早引入胰酶和脂溶性维生素治疗并定期监测。胰腺功能充足的患者应进行年度评估，包括粪便胰腺弹性蛋白酶测量。建议补充钠，并应测量尿钠：肌酐比值，对应于钠排泄分数。如果怀疑缺铁，应该解决潜在的炎症。葡萄糖耐量试验应在10岁时进行。骨密度检查应在8-10岁进行。当生长或营养状况受损时，推荐口服营养补充剂，然后进行聚合物肠内管喂养。可根据临床情况考虑补锌。必需脂肪酸，抗肿瘤药物，生长激素，食欲刺激剂和益生菌可以recommended.Conclusion：营养护理和支持应该是CF管理的一个组成部分之前，需要进一步的研究。获得儿童的正常生长模式和维持成人的充足营养状态是多学科囊性纤维化中心的主要目标。(C)2016爱思唯尔有限公司和欧洲临床营养与代谢学会。All rights reserved.
Background: Malnutrition is both a frequent feature and a comorbidity of cystic fibrosis (CF), with nutritional status strongly associated with pulmonary function and survival. Nutritional management is therefore standard of care in CF patients. ESPEN, ESPGHAN and ECFS recommended guidelines to cover nutritional management of patients with CF.Methods: The guidelines were developed by an international multidisciplinary working group in accordance with officially accepted standards. The GRADE system was used for determining grades of evidence and strength of recommendation. Statements were discussed, submitted to Delphi rounds, reviewed by ESPGHAN and ECFS and accepted in an online survey among ESPEN members.Results: The Working Group recommends that initiation of nutritional management should begin as early as possible after diagnosis, with subsequent regular follow up and patient/family education. Exclusive breast feeding is recommended but if not possible a regular formula is to be used. Energy intake should be adapted to achieve normal weight and height for age. When indicated, pancreatic enzyme and fat soluble vitamin treatment should be introduced early and monitored regularly. Pancreatic sufficient patients should have an annual assessment including fecal pancreatic elastase measurement. Sodium supplementation is recommended and a urinary sodium:creatinine ratio should be measured, corresponding to the fractional excretion of sodium. If iron deficiency is suspected, the underlying inflammation should be addressed. Glucose tolerance testing should be introduced at 10 years of age. Bone mineral density examination should be performed from age 8-10 years. Oral nutritional supplements followed by polymeric enteral tube feeding are recommended when growth or nutritional status is impaired. Zinc supplementation may be considered according to the clinical situation. Further studies are required before essential fatty acids, anti-osteoporotic agents, growth hormone, appetite stimulants and probiotics can be recommended.Conclusion: Nutritional care and support should be an integral part of management of CF. Obtaining a normal growth pattern in children and maintaining an adequate nutritional status in adults are major goals of multidisciplinary cystic fibrosis centers. (C) 2016 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.