Nutritional status of patients with cystic fibrosis with meconium ileus: a comparison with patients without meconium ileus and diagnosed early through neonatal screening.
OBJECTIVE
This study was pursued as an extension of a randomized clinical investigation of neonatal screening for cystic fibrosis (CF). The objective was to determine if CF patients with meconium ileus (MI) were more likely to be malnourished compared with those without MI who were diagnosed during early infancy through neonatal screening.


METHODS
Nutritional status was evaluated from early infancy to 13 years of age based on anthropometric, biochemical, and dietary assessments.


RESULTS
MI patients (n = 32) were smaller at birth (3117 g compared with 3413 g) and were shorter (22nd percentile compared with 48th percentile) and thinner (24th percentile compared with 49th percentile) compared with non-MI early diagnosed patients (n = 50) up to 13 years of age. Poor growth was particularly evident in 26 MI patients who required surgery for MI (height and weight at the 20th percentile), whereas those treated without surgery (n = 6) showed better height (45th percentile) and weight (37th percentile). Abnormal essential fatty acid profiles were significantly more prevalent in MI compared with non-MI early-diagnosed patients before 3 years of age. Daily intakes of calorie (130% compared with 111% recommended dietary allowances) and protein (339% compared with 279% recommended dietary allowances) were higher but the percentage of fat (37% compared with 38%) and linoleic acid (4.5% compared with 4.7%) in the diet were similar between the two groups.


CONCLUSIONS
These results demonstrated a clear association of MI with malnutrition in CF. The observed poor growth among our MI patients was not because of poor dietary intakes, but was related to surgical treatment for MI and poor essential fatty acid status. These findings present new challenges regarding the optimal medical treatment and nutritional intervention for CF patients with MI.
Lai
H C
HC
Departments of Pediatrics and Biostatistics, University of Wisconsin School of Medicine, Madison, Wisconsin 53792, USA. lai@biostat.wisc.edu
Kosorok
M R
MR
Laxova
A
A
Davis
L A
LA
FitzSimmon
S C
SC
Farrell
P M
PM
D016022:Case-Control Studies; D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis*; D004041:Dietary Fats / Q000008:administration & dosage; D005228:Fatty Acids, Essential / Q000378:metabolism; D005260:Female; D006128:Growth; D006801:Humans; D007223:Infant; D007415:Intestinal Obstruction / Q000175:diagnosis* / Q000209:etiology; D008297:Male; D008470:Meconium; D015997:Neonatal Screening; D009748:Nutrition Disorders / Q000175:diagnosis* / Q000209:etiology; D009752:Nutritional Status; D012042:Registries
D004041:Dietary Fats; D005228:Fatty Acids, Essential