Comparing the Use of Centers for Disease Control and Prevention and World Health Organization Growth Charts in Children with Cystic Fibrosis through 2 Years of Age.

OBJECTIVE: To examine differences between use of World Health Organization (WHO) and Centers for Disease Control and Prevention (CDC) growth reference in children with cystic fibrosis (CF) up to 2 years of age. STUDY DESIGN: Growth from 1-24 months in 2587 children, born 2003-2006 and recorded in the US CF Foundation Registry, was evaluated using WHO and CDC references. RESULTS: In both boys and girls with CF aged 1-24 months, use of WHO charts resulted in ∼8 percentile lower length-for-age and ∼13% higher short stature rate (length-for-age <5th percentile). WHO weight-for-age was ∼9 percentile lower prior to age 6 months, crossed at 6-7 months, and remained ∼14 percentile higher at 8-24 months. WHO weight-for-length (WFL) percentile (WFLp) was similar before 12 months but ∼10 percentile higher at 12-24 months compared with CDC. When using WHO charts, 9% of children had underweight (WFLp <50th) classified differently and this rate varied with age: 4% in the first year, 7% at 12, 13% at 15, and 16% at 18 months, respectively. Weight status assessed by WHO body mass index (BMI) charts was different from WHO WFL charts. At 24 months when switching back to CDC, 26% of children with normal WFLp on WHO charts appeared underweight on CDC charts. A 70th percentile of WHO BMI percentile was equivalent to the 50th percentile CDC BMI percentile. CONCLUSIONS: Growth status in children with CF differed when using WHO and CDC references, particularly during the second year of life. These differences need to be considered for all uses of growth assessment in CF.

Association Between Alcohol Use Among College Students and Alcohol Outlet Proximity and Densities.

BACKGROUND: Alcohol consumption is common on college campuses and is associated with negative consequences. Factors associated with availability of alcohol are not completely understood. OBJECTIVE: To describe how proximity and density of alcohol outlets are associated with any drinking and binge drinking in students at the University of Wisconsin-Madison. METHODS: Participants were full-time students enrolled in the Young Adults Eating and Active for Health, a multisite, randomized intervention that assessed a variety of health behaviors. Geographic information systems were used to calculate proximity and enumerate alcohol outlet densities. Participants were categorized as "drinkers" or "nondrinkers" based on self-reported alcohol consumption. Binge drinking was categorized as "non-binge drinker," "frequent binge drinker," and "excessive binge drinker." Analysis included regression, t tests, and chi-square tests. RESULTS. Among the 166 participants, 126 (76%) were drinkers. Among drinkers, 80 (63%) were either frequent or excessive binge drinkers. Drinkers lived closer to an alcohol outlet than non-drinkers (0.18 +/- 0.15 vs. 0.61 +/- 1.59 miles, respectively, P=0.005). Within a 1-mile walking radius, there were 47% more establishments for drinkers (153 +/- 47 compared to 104 +/- 55 outlets for nondrinkers, P<0.0001). At distances of 0.10-0.25 and 0.25-0.50 miles, twice as many outlets were available to drinkers (19 +/- 19 and 43 +/- 25, respectively) compared to nondrinkers (7 +/- 11 and 20 +/- 22, respectively), P<0.001. Proximity and density were hot associated with binge drinking frequency. CONCLUSION: Drinkers lived closer to alcohol outlets and had significantly more outlets available at a distance of up to 1 mile. Municipal and college administrators could consider limiting alcohol license distributions in municipalities with high alcohol consumption.

Nutritional status is associated with health-related quality of life in children with cystic fibrosis aged 9-19 years.

BACKGROUND: The impact of improved nutritional status on health-related quality of life (HRQOL) is unknown for children with cystic fibrosis (CF). METHODS: Associations between nutritional status and HRQOL were examined over 2 years in 95 children, aged 9-19 years, who were followed in the Wisconsin Newborn Screening Project. HRQOL was assessed using the Cystic Fibrosis Questionnaire (CFQ). Associations between height z-score (HtZ), BMI z-score (BMIZ) and seven CFQ dimensions were evaluated. RESULTS: Mean values of at least 80 were observed for all CFQ dimensions except respiratory symptoms and treatment burden. Treatment burden was significantly worse in patients with meconium ileus (57) compared to pancreatic insufficient (65) and sufficient (78) subjects, p<0.0001. HtZ and BMIZ were positively associated with physical functioning and body image (p<0.05). CONCLUSIONS: Better nutritional status was associated with increased HRQOL scores. Early diagnosis through newborn screening and improved nutrition provides an opportunity to enhance quality of life and body image perception.

Growth and pulmonary outcomes during the first 2 y of life of breastfed and formula-fed infants diagnosed with cystic fibrosis through the Wisconsin Routine Newborn Screening Program.

BACKGROUND: The optimal feeding (breast milk, formula, or a combination) for infants with cystic fibrosis (CF) is unknown. Recommendations from the CF Foundation are based on limited data. OBJECTIVE: We compared growth and pulmonary outcomes between breastfed and formula-fed infants through the age of 2 y. DESIGN: A total of 103 CF infants born in 1994-2006 and diagnosed through newborn screening in Wisconsin were studied. Breastfed infants were classified by the duration of exclusive breastfeeding (ExBF). Exclusive formula-feeding (ExFM) was classified by the formula's caloric density (ie, standard [0.67 kcal/mL (20 kcal/oz) (ExFM20)] throughout infancy or high density [≥0.74 kcal/mL (22 kcal/oz) (ExFM22+)] for some duration of infancy). RESULTS: Fifty-three infants (51% of infants) were breastfed and 50 infants (49% of infants) were ExFM. In breastfed infants, the duration of ExBF was <1 mo (53% of infants), 1-1.9 mo (21% of infants), 2-3 mo (17% of infants), and 4-9 mo (9% of infants). In ExFM infants, 23 infants (46%) received a formula with a high caloric density; approximately half (n = 13) of the ExFM infants received the formula by 6 mo of age. Proportionately more infants with pancreatic sufficiency (n = 9) were ExBF ≥1 mo (44% of infants), and none of the infants were ExFM22+, compared with infants with meconium ileus (n = 24; 13% of infants were ExBF ≥1 mo, and 38% of infants were ExFM22+) or pancreatic insufficiency (n = 70; 25% of infants were ExBF ≥1 mo, and 20% of infants were ExFM22+) (P = 0.02). In infants with pancreatic insufficiency, weight z scores declined from birth to 6 mo (P < 0.0001) in infants who were ExBF ≥2 mo, and the number of Pseudomonas aeruginosa infections through the age of 2 y was fewer in breastfed than in ExFM infants (P = 0.003) but did not differ by the duration of ExBF. CONCLUSION: For infants with CF, ExBF <2 mo does not compromise growth and is associated with a respiratory benefit.

Incorporating genetic potential when evaluating stature in children with cystic fibrosis.

OBJECTIVE: The 2002 Cystic Fibrosis Foundation (CFF) practice guidelines recommend adjusting for genetic potential when evaluating height status in children with CF. However, there is paucity of data to support this recommendation. We compared three methods of classifying short stature: unadjusted height percentile <10th, Himes adjusted height percentile <10th, and unadjusted height below the CFF target height lower bound. PATIENTS AND METHODS: Data from 3306 children with parental heights documented in the 1986-2005 CFF Patient Registry were analyzed. RESULTS: Mean height percentile of CF children (33rd) was lower than their parents' (mothers' 53rd, fathers' 57th), and 80% of CF children were below the average of their parental height percentiles. In children with short parents, Himes adjusted height percentile was significantly higher than unadjusted height percentile (27th vs. 8th), whereas the opposite was found in children with tall parents (Himes adjusted at 18th vs. unadjusted at 49th). Consequently, the prevalence of short stature decreased from 52% to 22% in children with short parents and increased from 8% to 34% in children with tall parents after Himes adjustment. In children with discrepant classification on short stature before and after Himes adjustment, percent predicted forced expiratory volume in one second was negatively associated with unadjusted height percentile but positively associated with Himes adjusted height percentile. In children with short parents, the CFF method underestimated the prevalence of short stature (9%) compared to the Himes method (22%). CONCLUSION: Without adjustment of genetic potential, the prevalence of short stature is underestimated and the association between height and lung function is biased.

Recovery of birth weight z score within 2 years of diagnosis is positively associated with pulmonary status at 6 years of age in children with cystic fibrosis.

OBJECTIVE: We recently reported that 60% of children newly diagnosed with cystic fibrosis who had pancreatic insufficiency responded to treatment initiation and achieved catch-up weight gain to a level comparable with their birth weight z score within 2 years of diagnosis ("responders"), whereas the remaining 40% failed to do so ("nonresponders"). The present study examined the impact of this early weight recovery on subsequent growth pattern and pulmonary status at 6 years of age. PATIENTS AND METHODS: Sixty-three children with cystic fibrosis who had pancreatic insufficiency but no meconium ileus, and were enrolled in the Wisconsin Cystic Fibrosis Neonatal Screening Project, were studied. Responders were defined by a recovery of weight z score comparable with that at birth within 2 years of diagnosis. From ages 2 to 6, growth was measured by both height and BMI. Pulmonary status was evaluated by symptoms, spirometry, quantitative chest radiography, and respiratory microbiology. RESULTS: The majority (71%) of the responders maintained their early weight recovery through 6 years of age, whereas only 32% of the nonresponders achieved substantial growth improvement from 2 to 6 years of age. Proportionately fewer responders reported cough symptoms (10% daytime cough; 22% nighttime cough) compared with nonresponders (41% daytime cough; 45% nighttime cough) at age 6. The percentage of predicted forced expiratory volume in 1 second at age 6 was 11% higher in responders (99.5% +/- 13.9%) compared with nonresponders (88.3% +/- 18.5%). Responders had significantly better Brasfield (20.1 +/- 1.4) and Wisconsin chest radiograph (8.3 +/- 3.3) scores compared with nonresponders (Brasfield: 18.9 +/- 1.8; Wisconsin: 12.3 +/- 8.3). Respiratory microbiology results were not significantly different. Multiple regression analyses indicated that the positive association between responder and percent predicted forced expiratory volume in 1 second at 6 years of age remained statistically significant after controlling for infections with Pseudomonas aeruginosa and Staphylococcus aureus and chest radiograph scores. Growth patterns from 2 to 6 years of age were not associated with pulmonary measures at age 6. CONCLUSIONS: Patients with cystic fibrosis with pancreatic insufficiency who achieved early growth recovery within 2 years of diagnosis had fewer cough symptoms, higher lung function, and better chest radiograph scores at 6 years of age.

Classification of malnutrition in cystic fibrosis: implications for evaluating and benchmarking clinical practice performance.

BACKGROUND: In 2005, the Cystic Fibrosis Foundation (CFF) revised the nutrition classification guidelines to eliminate the use of percentage of ideal body weight (%IBW) to define "nutritional failure"; the CFF also recommended that children with cystic fibrosis maintain a body mass index percentile (BMIp) > or = 50th. OBJECTIVE: We assessed the effect of the 2005 CFF nutrition classification guidelines on evaluating the performance of nutritional care practices. DESIGN: Data from 14,702 children reported to the 2002 CFF Patient Registry were analyzed to compare malnutrition rates in 113 cystic fibrosis centers in the United States. Nutritional failure was defined according to the 2002 CFF criteria--ie, height < 5th percentile, %IBW < 90%, or BMIp < 10th. "Below BMI goal" was defined according to the 2005 CFF criterion, ie BMIp < 50th. RESULTS: Eliminating %IBW resulted in a 6% reduction (from 33% to 27%) in the nutritional failure rate in the United States. The use of BMIp < 50th led to the classification of 57% of children as below the BMI goal. Misclassification of nutritional failure according to %IBW ranged from 1% to 16% among 113 centers and was greater in the centers with a larger proportion of tall patients. After the elimination of %IBW, one-third of centers changed to a different tertile ranking for nutritional failure rates (kappa = 0.50, moderate-to-poor agreement). More than half the centers changed to a different tertile ranking, from nutritional failure to below BMI goal (kappa = 0.22, poor agreement). CONCLUSION: Eliminating misclassification by %IBW and implementing the new BMI goal led to profound and unequal changes in malnutrition rates across cystic fibrosis centers.

Temporal associations among energy intake, plasma linoleic acid, and growth improvement in response to treatment initiation after diagnosis of cystic fibrosis.

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 (increased 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. Increased EN and sustained normal plasma linoleic acid level (increased pLA) were defined by achieving energy intake > or =120% of estimated requirement for > or =75% of the time and maintaining plasma LA > or =26% of total fatty acids for > or =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 increased EN compared with patients whose CF was diagnosed conventionally (73%). However, more screened patients responded to increased EN and recovered WtzBR (91%) than patients whose CF was diagnosed conventionally (56%), although this difference was of borderline significance. Compared with having neither increased EN nor increased pLA, the likelihood of being a responder was greatest with combined increased EN and increased pLA, followed by increased EN only. The positive associations between increased EN and increased pLA to treatment responsiveness remained significant after adjustment for neonatal screening status, baseline height and weight status, and indices of pulmonary disease severity. CONCLUSION: Increased EN and increased pLA are critical in promoting adequate weight gain in children with newly diagnosed CF.