PEDIATRICS Vol. 117 No. 2 February 2006, pp. 391-400 (doi:10.1542/peds.2004-2832)
Temporal Associations Among Energy Intake, Plasma Linoleic Acid, and Growth Improvement in Response to Treatment Initiation After Diagnosis of Cystic Fibrosis
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 (
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. EN and sustained normal plasma linoleic acid level (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 EN compared with patients whose CF was diagnosed conventionally (73%). However, more screened patients responded to EN and recovered WtzBR (91%) than patients whose CF was diagnosed conventionally (56%), although this difference was of borderline significance. Compared with having neither EN nor pLA, the likelihood of being a responder was greatest with combined EN and pLA, followed by EN only. The positive associations between EN and pLA to treatment responsiveness remained significant after adjustment for neonatal screening status, baseline height and weight status, and indices of pulmonary disease severity.
CONCLUSION. EN and 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 • 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 • pLA—sustained normal plasma linoleic acid • dLA—sustained adequate dietary linoleic acid intake • WtzDX—weight z score at diagnosis • HtzDX—height z score at diagnosis • 
WtzDX-BR—change in weight z score from birth to diagnosis
Accepted May 31, 2005.
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