title: Nutritional rehabilitation in cystic fibrosis: a 5 year follow-up study.
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.
authors.affiliation: Cystic Fibrosis Clinic, Brisbane, Australia.
authors.forename: A M
authors.initials: AM
authors.lastname: Dalzell
authors.forename: R W
authors.initials: RW
authors.lastname: Shepherd
authors.forename: B
authors.initials: B
authors.lastname: Dean
authors.forename: G J
authors.initials: GJ
authors.lastname: Cleghorn
authors.forename: T L
authors.initials: TL
authors.lastname: Holt
authors.forename: P J
authors.initials: PJ
authors.lastname: Francis
mesh_terms: D000293:Adolescent; D000328:Adult; D001827:Body Height; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D004750:Enteral Nutrition; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009748:Nutrition Disorders / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D009752:Nutritional Status; D015430:Weight Gain