Long term nutritional rehabilitation by gastrostomy in Israeli patients with cystic fibrosis: clinical outcome in advanced pulmonary disease.
OBJECTIVE
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Efrati
Ori
O
Pediatric Pulmonary Unit, the Safra Children's Hospital, Chaim Sheba Medical Center, Tel-Hashomer 52621, Israel. oriefrati@sheba.health.gov.il
Mei-Zahav
Meir
M
Rivlin
Joseph
J
Kerem
Eitan
E
Blau
Hannah
H
Barak
Asher
A
Bujanover
Yoram
Y
Augarten
Arie
A
Cochavi
Brijit
B
Yahav
Yaacov
Y
Modan-Moses
Dalit
D
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000886:Anthropometry; D001827:Body Height / Q000502:physiology; D015992:Body Mass Index; D001835:Body Weight / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000628:therapy*; D004750:Enteral Nutrition; D005260:Female; D005541:Forced Expiratory Volume; D005774:Gastrostomy / Q000379:methods*; D006801:Humans; D007223:Infant; D007557:Israel; D008137:Longitudinal Studies; D008168:Lung / Q000503:physiopathology; D008297:Male; D009752:Nutritional Status; D012129:Respiratory Function Tests / Q000379:methods; D016896:Treatment Outcome; D015430:Weight Gain