Efrati Ori, Mei-Zahav Meir, Rivlin Joseph, Kerem Eitan, Blau Hannah, Barak Asher, Bujanover Yoram, Augarten Arie, Cochavi Brijit, Yahav Yaacov, Modan-Moses Dalit
Pediatric Pulmonary Unit, the Safra Children's Hospital, Chaim Sheba Medical Center, Tel-Hashomer 52621, Israel.
J Pediatr Gastroenterol Nutr. 2006 Feb;42(2):222-8. doi: 10.1097/01.mpg.0000189348.09925.02.
Several studies have shown a linear correlation between nutritional status and pulmonary function in patients with cystic fibrosis. Our study aims were: 1) To evaluate the effect of nutritional supplementation via gastrostomy on nutritional, clinical, and pulmonary parameters, and 2) To identify predicting factors for success of long-term nutritional rehabilitation.
Twenty-one Israeli patients, aged 8 months to 20 years, underwent gastrostomy insertion from 1992 to 2001. All patients were pancreatic insufficient, and all carried severe mutations (W1282X in 62% of the patients). Anthropometric and clinical data were obtained for each patient: 0-12 months before and 6-12 months and 18-24 months after gastrostomy placement. Standard deviation scores (SDS) for height, weight, and body mass index as well as percent of height-appropriate body weight were calculated.
The mean percent-of-predicted forced expiratory volume in 1 second (FEV1) decreased significantly during the first year of gastrostomy feeding (n = 16), from 44.2% +/- 13.9 to 41% +/- 13.3 (P = 0.05). However, during the second year of therapy (n = 10), a trend toward improvement was observed (from 39.4 +/- 12.1 to 41.4 +/- 16.1). Weight, and BMI z-scores as well as weight percent-of ideal body weight increased significantly. Height z-score for age decreased during the first year (from -1.9 +/- 1.3 to -2.1 +/- 1.4), However, a trend toward improvement was observed during the second year. A significant correlation was found between the change in weight z-score and height z-score during the first (r = 0.488, P = 0.016) and the second (r = 0.825, P < 0.001) years. There was no difference between compliers and noncompliers regarding height, weight, and BMI either before or after gastrostomy placement. A significant correlation between age at insertion of gastrostomy and improvement in height z-score (r = 0.52, P = 0.016) was observed. Cystic fibrosis related diabetes (n = 8) did not affect the response to supplemental feeding.
We observed a trend toward improvement of pulmonary disease during the second year, and a significant improvement in weight, height, and BMI z-scores. Compliance, diabetes, and young age prior to tube insertion did not predict success of nutritional rehabilitation.
多项研究表明，囊性纤维化患者的营养状况与肺功能之间存在线性相关性。我们的研究目的是：1）评估经胃造口术进行营养补充对营养、临床和肺部参数的影响，以及2）确定长期营养康复成功的预测因素。
1992年至2001年，21名年龄在8个月至20岁之间的以色列患者接受了胃造口术。所有患者均存在胰腺功能不全，且均携带严重突变（62%的患者为W1282X）。获取了每位患者的人体测量和临床数据：胃造口术放置前0 - 12个月、放置后6 - 12个月以及18 - 24个月的数据。计算了身高、体重和体重指数的标准差分数（SDS）以及身高适宜体重百分比。
在胃造口术喂养的第一年（n = 16），预计第1秒用力呼气量（FEV1）的平均百分比显著下降，从44.2%±13.9降至41%±13.3（P = 0.05）。然而，在治疗的第二年（n = 10），观察到有改善趋势（从39.4±12.1升至41.4±16.1）。体重、BMI z分数以及理想体重的体重百分比显著增加。年龄别身高z分数在第一年下降（从 - 1.9±1.3降至 - 2.1±1.4），不过在第二年观察到有改善趋势。在第一年（r = 0.488，P = 0.016）和第二年（r = 0.825，P < 0.001），体重z分数变化与身高z分数变化之间存在显著相关性。在胃造口术放置前后，依从者和非依从者在身高、体重和BMI方面均无差异。观察到胃造口术插入时的年龄与身高z分数改善之间存在显著相关性（r = 0.52，P = 0.016）。囊性纤维化相关糖尿病（n = 8）不影响补充喂养的反应。
我们观察到在第二年肺部疾病有改善趋势，体重、身高和BMI z分数有显著改善。依从性、糖尿病以及置管前年龄小并不能预测营养康复的成功。
