Predictors and outcome of low initial forced expiratory volume in 1 second measurement in children with cystic fibrosis.

OBJECTIVE: To identify the characteristics of children with cystic fibrosis with low initial forced expiratory volume in 1 second (FEV1) % predicted and to investigate their outcome. STUDY DESIGN: Patients were categorized into low or high initial FEV1 groups using cluster analysis. Comparisons of the demographic and clinical data were performed between the 2 groups. RESULTS: From 122 children, 21 clustered into the low and 101 into the high FEV1 group. The mean FEV1 was 69% ± 12% predicted for the low and 95% ± 12% predicted for the high FEV1 group (P < .001). The low FEV1 group had lower body mass index percentiles (P = .003), were hospitalized more frequently (P = .001), and had been on dornase alfa longer (P = .006). Low FEV1 group had more patients with positive cultures for Pseudomonas aeruginosa (P = .002) and Stenotrophomonas maltophilia (P < .001) and had more total number of cultures positive for mucoid P. aeruginosa (P = .009) and methicillin resistant Staphylococcus aureus + P. aeruginosa (P = .005). The low FEV1 group continued to have low FEV1 measurements, their FEV1 declined slower, required more hospitalizations per year (P = .01), and had more cultures for mucoid (P = .003) and nonmucoid P. aeruginosa (P = .02) ± methicillin resistant S. aureus (P = .002) in comparison with the high FEV1 group. Poor adherence was associated with lower initial FEV1 values in females, and early, rapid decline of FEV1 in males. CONCLUSIONS: Some children with cystic fibrosis may present with poor lung function early in life and continue to have subnormal lung function associated with reduced body mass index, more frequent hospitalization, and higher rates of infection. Such children may benefit from careful evaluation and close follow-up.

The use of neoprene as a scar management modality.

PURPOSE: Scar formation is one of the most functionally and cosmetically debilitating results of thermal injuries. Burn team members continuously search for new, cost effective, ways to prevent and treat hypertrophic scar formation. This study is a retrospective review of one facility's use of neoprene based products as an adjunct to, or in place of, traditional scar management techniques. METHODS: Records of all patients treated with neoprene patches or neoprene splints from March 2008 through April 2011 were retrospectively reviewed. Vancouver Scar Scores (VSS) were collected, photographs were reviewed and any documented problems reported by the patients were noted. RESULTS: Mean VSS scores were significantly lower at follow-up (5.3, SD 2.8) than at initial appointment (11.7, SD 1.4) with an estimated mean difference of 6.3 (P=0.0001). A 95% confidence interval for this mean difference is 3.9-8.7. CONCLUSIONS: In the reviewed cases, the use of neoprene inserts or splints resulted in a statistically significant reduction in hypertrophic scars with no secondary complications.

Periodic limb movements during sleep in children with narcolepsy.

STUDY OBJECTIVES: In adults with narcolepsy, periodic limb movements of sleep (PLMS) occur more frequently than in control population, and presence of increased PLMS is associated with greater sleep disruption and shorter mean sleep latency. This study was performed to determine whether PLMS are common in children with narcolepsy, and whether the presence of PLMS is associated with greater sleep disruption. DESIGN: Demographic and polysomnographic information were collected from consecutive patients diagnosed with narcolepsy identified retrospectively by diagnosis-based search. Descriptive data were compiled, and sleep characteristics of children with and without PLMS were compared. SETTING: Sleep disorders center in a children's hospital. PATIENTS: 44 patients, 6-19 years old (mean 13 years, SD 3.57), were identified. Twenty-eight were African American. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Four patients had a PLMS index (PLMI) ≥ 5/h (considered abnormal in literature). Sixteen (36%) had "any PLMS" (PLMI > 0/h). The mean PLMI was 1.3/h (SD 2.5). Sleep was significantly more disrupted, and the mean sleep latency was shorter in patients with "any PLMS" as compared to those with no PLMS. There was no correlation between the PLMI and other diagnostic criteria for narcolepsy. "Any PLMS" were present equally in children of African American and Caucasian heritage, 35.7% vs. 37.5%. CONCLUSIONS: As in adults, children with PLMS and narcolepsy have more sleep disruption and shorter mean sleep latencies than those with narcolepsy but without PLMS. Our findings also suggest that the use of adult criteria for diagnosis of "significant" PLMS in children may not be sufficiently sensitive.

Body satisfaction, nutritional adherence, and quality of life in youth with cystic fibrosis.

Children with cystic fibrosis (CF) typically have similar nutritional intake as healthy peers, despite recommendations to consume more calories and fat. Body satisfaction may play a role in nutritional intake: females may be content with their smaller size despite recommendations for weight gain, while males may desire to be larger and more muscular, which is more congruent with medical advice. Females are especially at risk, given their propensity to desire a smaller body size, tendency for lower HRQOL, steeper trajectory of health decline and shorter life expectancy than males. This study evaluated body satisfaction in relation to nutritional adherence and HRQOL in youth with CF. Fifty-four individuals with CF (age 9-17) completed the Cystic Fibrosis Questionnaire-Revised (CFQ-R), the Figure Rating Scale, and a 24-hr diet recall interview with their caregiver. Twenty-four percent of youth were non-adherent with caloric goals, and 40.7% did not obtain the minimum recommendation for fat intake. Youth were classified as inconsistent with treatment goals (TI) if they desired a smaller body size or were content with their current size despite a BMI less than the 50th percentile; 44.8% of females were classified as TI, compared to only 8% of males. Statistical analyses were performed to evaluate the impact of gender and body satisfaction on HRQOL in youth with CF. Linear multiple regression models were fit; TI females had Emotional HRQOL scores 23 points lower than males. Results suggest that improving body satisfaction, especially for females, may help to improve overall quality of life and potentially impact nutritional adherence.