Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: evaluation of various criteria used to identify malnutrition.

OBJECTIVES: The objectives of this study were to determine growth status and to identify malnutrition with various anthropometric indicators in children with cystic fibrosis (CF) based on cross-sectional analysis of the 1993 National CF Patient Registry data. METHODS: Heights and weights of 13,116 children with CF were evaluated with percentile, percent of reference median, Z-score, and percent ideal weight-for-height based on National Center for Health Statistics/Centers for Disease Control growth references. Malnutrition was defined by four criteria: (1) height-for-age <5th percentile ("stunting") or weight-for-age <5th percentile ("wasting") (2) height-for-age <90% of reference median or weight-for-age <80% of reference median, (3) height-for-age <5th percentile or percent ideal weight-for-height <85%, and (4) height-for-age <90% of reference median or weight-for-height <85% of reference median. RESULTS: Mean and median height- and weight-for-age were found to be at the 30th and 20th percentiles in children with CF. Malnutrition (height- or weight-for-age <5th percentile) was particularly pronounced in infants (47%) and adolescents (34%) and patients with newly diagnosed CF (44%). A significant sex difference (p < 0.01) in the occurrence of stunting (height-for-age <5th percentile) was observed during adolescence: boys 11 to 14 years of age showed lower occurrence of stunting (19%) compared with girls (29%), whereas the opposite trend was observed at 15 to 18 years (34% in male patients vs 28% in female patients). CONCLUSION: Twenty percent of all children in the 1993 National CF Patient Registry were <5th percentile for height- or weight-for-age. A significant discrepancy was found when different criteria were used to distinguish "stunting" versus "wasting" in malnourished children with CF.

Oral supplementation with a high-fat, high-energy product improves nutritional status and alters serum lipids in patients with cystic fibrosis.

OBJECTIVE: To assess the tolerance and acceptability of a nutrition supplement in patients with cystic fibrosis (CF), to monitor changes in dietary intake, and to evaluate nutritional status. DESIGN: Subjects were their own controls for this 3-month, prospective, open study. Acceptability and tolerance questionnaires and 3-day food records were completed at baseline and monthly intervals. Compliance and nutritional status were also assessed. SETTING: This study was conducted at the University of Wisconsin Hospital and Clinics Cystic Fibrosis Center, Madison. SUBJECTS: Patients with CF older than 4 years of age were recruited during clinic or hospital visits if they met specific weight or growth criteria (n = 19). INTERVENTION: Subjects were asked to consume the supplement at a maximum of 30% their estimated daily energy requirements. MAIN OUTCOME MEASURES: Responses to acceptability ratings of and tolerance questions about the supplement were obtained along with anthropometric data and biochemical measurements of serum albumin, plasma retinol, alpha-tocopherol, and fatty acid levels. STATISTICAL ANALYSES PERFORMED: Data were analyzed using Minitab and Statistical Analysis Software. Paired and unpaired t tests and nonparametric sign tests were used, as well as regression and Pearson correlations. A significance level of .05 was used for all tests. RESULTS: All subjects tolerated the supplement, although 12 reported mild symptoms of fullness, nausea, and/or bloating, which were resolved when intake was distributed throughout the day. Mean compliance was 69% of recommended intake. Weight gain in children was strongly correlated with compliance (r = .98). Linoleic acid intake increased significantly (P = .0003) as did plasma linoleic acid in the phospholipid fraction (P = .03). CONCLUSION: The supplement studied would be a beneficial addition to the supplementation choices available to patients with CF.

Nutritional status of infants with cystic fibrosis associated with early diagnosis and intervention.

The purpose of this study was to characterize the nutritional status of infants diagnosed with cystic fibrosis (CF) through neonatal screening and to determine if they would achieve normal nutrition when managed with early intervention. In addition, nutrient intake was assessed to determine energy and macronutrient-consumption patterns. Evaluation of growth revealed that normal patterns could be achieved with mean energy intake values at ages 6 and 12 mo of 481 and 426 kJ/kg body wt (115 and 102 kcal/kg body wt), respectively. Biochemical assessment demonstrated low alpha-tocopherol and linoleic acid values at diagnosis in the majority of infants whereas one-third had abnormal indices of protein nutriture. Essential fatty acid deficiency was also demonstrated at diagnosis by abnormal triene-tetraene ratio values in 27% of screened infants. With predigested formula and dietary supplementation, there was improvement in all indices of nutritional status and only a low percentage of patients showed mild biochemical abnormalities at age 12 mo.

Nutritional assessment of infants with cystic fibrosis diagnosed through screening.

Presymptomatic infants diagnosed through neonatal screening for cystic fibrosis can have biochemical evidence of malnutrition. With aggressive dietary management and treatment with pancreatic enzymes, normal biochemical indices of nutrition can be achieved at 12 months of life in most cases. Males with cystic fibrosis appear to be more at risk than females for abnormal growth and biochemical indices of nutrition in the first year of life. This may be related to the observed decrease in fat intake when compared to females. Males, especially, should be carefully observed for development of nutritional abnormalities based on this data. Careful attention should be paid to vitamin E and essential fatty acid status in all CF infants. The numbers in this study are small and the long-term consequences of early nutritional intervention await the conclusion of the randomized, controlled study on-going in Wisconsin.

Predigested formula for infants with cystic fibrosis.

The object of this study was to assess the growth rates of patients with cystic fibrosis fed various diets and test the hypothesis that the weight of infants could be normalized by 1 year of age if they were placed on predigested formula before age 6 months. A group of 19 newly diagnosed patients placed on Pregestimil were compared with a group who were fed standard formula. At 1 year of age, the Pregestimil group showed significantly greater length and weight and a twofold higher average weight percentile. Growth velocity determined for the period between diagnosis and 12 months of age was better (p less than .001) for babies raised on Pregestimil (556 compared with 423 gm/month). Using weight percentile as the major growth index and values less than the fifth percentile as abnormal at age 12 months, we found that all 19 babies were normal in the group fed Pregestimil, whereas nine were below the fifth percentile in the group fed regular formula (p = .0006).

The role of diet in managing diabetes in children.

The steps used to construct an appropriate food plan for the diabetic child and to educate the patient and his family in the goals and implementation of the plan are outlined. Factors that should be considered in constructing a flexible and workable plan include age, family situation, activity level, degree of motivation, extent of knowledge, and dietary history.