Nutritional Considerations in Cystic Fibrosis

Hubbard, Van S.

doi:10.1055/s-2007-1011509

Cited by 12 publications

(2 citation statements)

References 25 publications

(35 reference statements)

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“…Children who participated in the clinical trial experienced an average decline of 0.05 in their BMI z -score over 27 months, whereas the Comparison Sample declined four times as much during the same period. While there is no corresponding CFF Registry data on caloric intake, numerous studies have reported that the typical intake of children with CF is about 100% of the recommended energy for children 18–21 rather than the 120–150% recommended for CF 22,23 and achieved by children who received the intensive intervention provided in the clinical trial. 12 Thus, our data suggests that standard of care, as it was being delivered during the time of the study was not achieving optimal growth outcomes in comparison to what could be achieved with more intensive and structured interventions.…”

Section: Discussionmentioning

confidence: 99%

The effects of an intensive behavior and nutrition intervention compared to standard of care on weight outcomes in CF

Stark

Opipari-Arrigan

Quittner

et al. 2010

Pediatric Pulmonology

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Summary Inadequate intake and suboptimal growth are common problems for patients with CF and a critical target for intervention. The purpose of this study was to compare the growth outcomes of children with CF who participated in a randomized clinical trial to improve energy intake and weight to children with CF receiving standard of care during the same time period. Our primary outcome was change in body mass index z-score (BMI z-score) over 2 years. An exploratory outcome was forced expiratory volume at 1-sec (FEV1) over 2 years. Participants were children ages 4–12 with CF, who participated in a randomized clinical trial of behavior plus nutrition intervention versus nutrition education alone, and a matched Comparison Sample receiving standard of care drawn from the Cystic Fibrosis Foundation (CFF) Registry. Children in the Clinical Trial Group (N = 67) participated in a 9-week, nutrition intervention and were followed at regular intervals (3, 6, 12, 18, and 24 months) for 2 years post-treatment to obtain anthropometric and pulmonary function data. For each child in the Comparison Sample (N = 346), these measures were obtained from the CFF Registry at matching intervals for the 27-month period corresponding to the clinical trial. Over 27 months, children in the Clinical Trial Group (the combined sample of the behavior plus nutrition and the nutrition alone) demonstrated significantly less decline in BMI z-score, −0.05 (SD = 0.68, CI = −0.23 to 0.13), as compared to children in the Comparison Sample, −0.21 (SD = 0.67, CI = −0.31 to −0.11). No statistically significant differences were found for decline in FEV1 between children in the Clinical Trial Group and the Comparison Sample. The key implication of these findings is that intensive behavioral and nutritional intervention is effective and needs to be adapted so that it can be broadly disseminated into clinical practice.

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Section: Discussionmentioning

confidence: 99%

The effects of an intensive behavior and nutrition intervention compared to standard of care on weight outcomes in CF

Stark

Opipari-Arrigan

Quittner

et al. 2010

Pediatric Pulmonology

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“…Recent work has suggested that malabsorption alone cannot explain the altered fatty acid status in CF patients and that total calorie intake relative to caloric need may be the most important determinant of measured deficiences. 23 Rapid correction of deficiencies with intravenous fat infusions may permit a normalization, a new baseline from which normal levels can then be maintained via oral sources. Further work is required in this area to document the specific advantages of normal linoleic acid levels for CF patients.…”

Section: Complicationsmentioning

confidence: 99%

Supplemental Parenteral Nutrition in Cystic Fibrosis

Lester

Rothberg

Dawson

et al. 1986

J Parenter Enteral Nutr

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The efficacy and safety of short-term supplemental peripheral hyperalimentation (PH) was evaluated in 15 hospitalized cystic fibrosis (CF) patients who exhibited varying degrees of pulmonary disease severity and nutritional impairment. An average of 1000 supplemental calories/day were administered intravenously for a 2- to 3-week period to patients being treated with parenteral antibiotics for exacerbation of their pulmonary disease. Eleven of 15 patients responded with a weight gain of greater than 2.0 kg and showed continued weight gain and stabilized pulmonary status for the 6- to 12-month follow-up period; two patients showed dramatic reversal of poor weight gain and growth following PH. Total calorie intake (oral + PH) equaled 141 +/- 40% of the recommended dietary allowances (RDA) in responders, with 45 +/- 12% RDA contributed by PH, in contrast to 68 +/- 20% of the RDA for total calories with 31 +/- 13% supplied using PH achieved in the nonresponders. Linoleic acid deficiency was documented in these patients (linoleic acid level as a percent of total fatty acid = 21.9% +/- 1.41 SEM vs 31.8% +/- 1.16 SEM in normal controls), and all seven patients achieved normalization of linoleic acid level after PH. Prior assessment of nutritional status (anthropometric measurements) or of severity of pulmonary disease (NIH clinical score) did not allow prediction of response to PH. No complications resulted from administration of PH to these hospitalized CF patients.

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