Nutritional rehabilitation in cystic fibrosis: a 5 year follow-up study

J Pediatr Gastroenterol Nutr. 1992 Aug;15(2):141-5. doi: 10.1097/00005176-199208000-00007.

Abstract

Previously, we reported catch-up weight gain, growth, and improved lung function in a group of malnourished cystic fibrosis (CF) children receiving aggressive nutritional supplementation for 1 year compared with a forced expiratory volume in 1 s (FEV1)-, height-, and sex-matched comparison group receiving standard therapy. To evaluate long-term effects, the clinical progress of both groups has been studied over a 5 year period. The supplemented group (n = 10) received supplements for a median of 1.35 years to achieve nutritional rehabilitation. Compared with the nonsupplemented group (n = 14), the previously supplemented group had lower mortality (2 vs. 4, N.S.) and significantly greater weight and height z scores at 4 and 5 years. The progression of pulmonary function abnormalities as measured by FEV1 and forced vital capacity (FVC) slopes was greater at 3 years in the nonsupplemented group (FEV1, p less than 0.05) but no significant differences in rates of deterioration of pulmonary function were seen after 5 years in the two groups of survivors. We conclude that intensive nutritional support for 1 year has both short- and long-term effects on nutrition and growth, still evident some years after the cessation of this therapeutic modality. Supplementation for periods of longer than 1 year may produce greater gains and possibly prolong the improvement in pulmonary function observed in the earlier study.

MeSH terms

  • Adolescent
  • Adult
  • Body Height
  • Child
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / physiopathology
  • Cystic Fibrosis / therapy
  • Enteral Nutrition
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Nutrition Disorders / etiology
  • Nutrition Disorders / physiopathology
  • Nutrition Disorders / therapy*
  • Nutritional Status
  • Weight Gain