Koletzko S, Reinhardt D
Kinderklinik und Kinderpoliklinik im Dr. v. Haunerschen Kinderspital, Ludwig-Maximilians-Universität, Pettenkoferstr. 8a, D-80336, München, Germany.
Early Hum Dev. 2001 Nov;65 Suppl:S53-61. doi: 10.1016/s0378-3782(01)00206-7.
Nutrition plays an essential role in the management of cystic fibrosis (CF), particularly in infants with their high energy requirement due to rapid growth. Most infants are already malnourished at the time of clinical diagnosis due to an energy imbalance with increased losses and needs not compensated by energy intake. Even in very young asymptomatic CF patients diagnosed by neonatal screening programs, nutritional deficits can be detected in a high proportion of infants, since about half of all CF patients have evidence of pancreatic insufficiency at the time of birth. This review provides recommendations for assessment of the nutritional status at diagnosis and nutritional management including supplementation with pancreatic enzymes during the first year of life.
营养在囊性纤维化（CF）的管理中起着至关重要的作用，对于因快速生长而能量需求高的婴儿尤其如此。大多数婴儿在临床诊断时就已经营养不良，原因是能量失衡，损失增加且需求未通过能量摄入得到补偿。即使是通过新生儿筛查项目诊断出的非常年幼的无症状CF患者，也有很大比例的婴儿存在营养缺乏，因为所有CF患者中约有一半在出生时就有胰腺功能不全的证据。本综述提供了诊断时营养状况评估和营养管理的建议，包括在生命的第一年补充胰酶。
