Published online February 1, 2006
PEDIATRICS Vol. 117 No. 2 February 2006, pp. 391-400 (doi:10.1542/peds.2004-2832)
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via ISI Web of Science (6)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Shoff, S. M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Shoff, S. M.
Related Collections
Right arrow Respiratory Tract

Temporal Associations Among Energy Intake, Plasma Linoleic Acid, and Growth Improvement in Response to Treatment Initiation After Diagnosis of Cystic Fibrosis

Suzanne M. Shoff, PhDa, Hong-Yup Ahn, PhDf, Lisa Davis, MSc, HuiChuan Lai, PhDa,b the Wisconsin CF Neonatal Screening Groupb,c,d,e

a Nutritional Sciences
b Biostatistics and Medical Informatics
c General Clinical Research Center
d Department of Pediatrics and the State Laboratory of Hygiene, University of Wisconsin, Madison, Wisconsin
e Department of Pediatrics at the Medical College of Wisconsin, Milwaukee, Wisconsin
f Department of Statistics, Dongguk University, Seoul, Korea

OBJECTIVE. It is unclear why some patients with cystic fibrosis (CF) succeed ("responders") in recovering from malnutrition and growth faltering after treatment initiation whereas others fail to do so ("nonresponders"). We conducted a study to test the hypothesis that sustained high energy intake ({uparrow}EN) and normal plasma essential fatty acid status are critical determinants of treatment responsiveness within 2 years after diagnosis of CF.

METHODS. A total of 71 CF children who had pancreatic insufficiency but not meconium ileus and were enrolled in the Wisconsin CF Neonatal Screening Project were studied. Responders were defined by having achieved adequate weight gain, as indicated by a recovery of weight z score (Wtz) comparable to Wtz at birth (WtzBR) within 2 years of diagnosis. {uparrow}EN and sustained normal plasma linoleic acid level ({uparrow}pLA) were defined by achieving energy intake ≥120% of estimated requirement for ≥75% of the time and maintaining plasma LA ≥26% of total fatty acids for ≥75% of the time, respectively.

RESULTS. Thirty-two (68%) screened patients and 13 (54%) patients whose CF was diagnosed conventionally recovered WtzBR within 2 years of diagnosis. Screened patients responded at significantly younger ages (mean/median: 6.3/4.3 months) than patients whose CF was diagnosed conventionally (mean/median: 15.8/11.8 months). Proportionately fewer screened patients (33%) achieved {uparrow}EN compared with patients whose CF was diagnosed conventionally (73%). However, more screened patients responded to {uparrow}EN and recovered WtzBR (91%) than patients whose CF was diagnosed conventionally (56%), although this difference was of borderline significance. Compared with having neither {uparrow}EN nor {uparrow}pLA, the likelihood of being a responder was greatest with combined {uparrow}EN and {uparrow}pLA, followed by {uparrow}EN only. The positive associations between {uparrow}EN and {uparrow}pLA to treatment responsiveness remained significant after adjustment for neonatal screening status, baseline height and weight status, and indices of pulmonary disease severity.

CONCLUSION. {uparrow}EN and {uparrow}pLA are critical in promoting adequate weight gain in children with newly diagnosed CF.

Key Words: cystic fibrosis • diet • energy intake • growth • height • linoleic acid • malnutrition • neonatal screening • prospective study • weight

Abbreviations: CF—cystic fibrosis • RDA—recommended dietary allowance • LA—linoleic acid • {uparrow}EN—sustained high energy intake • PI—pancreatic insufficiency • MI—meconium ileus • WtzBR—weight z score at birth • Wtz—weight z score • Htz—height z score • EER—estimated energy requirement • DRI—Dietary Reference Intakes • {uparrow}pLA—sustained normal plasma linoleic acid • {uparrow}dLA—sustained adequate dietary linoleic acid intake • WtzDX—weight z score at diagnosis • HtzDX—height z score at diagnosis • {Delta}WtzDX-BR—change in weight z score from birth to diagnosis

Accepted May 31, 2005.