Barbara A. Chini

Neuropsychological effects of pediatric obstructive sleep apnea.

Obstructive sleep apnea (OSA) is a fairly common nocturnal breathing disorder, affecting 2-4% of individuals. Although OSA is associated with medical morbidity, its most functionally disruptive effects in adults appear to be neuropsychological in nature. Research on the neuropsychological effects of pediatric OSA has been limited. This study compared the neuropsychological functioning of school-aged children with OSA to that of healthy children. The primary goal was to clarify the presence and pattern of neuropsychological morbidity associated with pediatric OSA. Sleep was assessed with parent-report questionnaires and laboratory sleep studies. Neuropsychological functioning was assessed by formal tests and parent- and teacher-report questionnaires. Data indicated OSA-related cognitive and behavioral impairment that was particularly marked on measures of behavior regulation and some aspects of attention and executive functioning. Minimal effects were observed on measures of intelligence, verbal memory, or processing speed. Exploratory analyses failed to indicate any clear relationship between neuropsychological functioning and objective indexes of hypoxia or sleep disruption, though the sample was small. These data add to a growing literature which suggests that significant neuropsychological deficits are associated with pediatric OSA. Findings suggest a pattern of neuropsychological morbidity that is similar but not identical to that seen in adult OSA.

Neonates with Tongue-Based Airway Obstruction A Systematic Review

Objective. In this systematic review, the authors summarize the current evidence in the literature regarding diagnosis, treatment, and long-term outcomes in neonates with tongue-based airway obstruction (TBAO) and assess the level of evidence of included studies. Data Sources. The terms Pierre Robin syndrome/sequence, micrognathia, retrognathia, and cleft palate were combined with airway obstruction, treatment, tongue-lip plication, and osteogenesis distraction to perform an Ovid literature search, yielding 341 references. The authors excluded references containing patients with isolated choanal/nasal obstruction, patients older than 12 months, and expert opinion papers, yielding 126 articles. Review Methods. The authors searched 3 electronic databases and reference lists of existing reviews from 1980 to October 2010 for articles pertaining to the diagnosis, treatment, and outcomes of TBAO. Reviewers assigned a level of evidence score based on Oxford’s Centre for Evidence Based Medicine scoring system and recorded relevant information. Results. Most studies were case studies and single-center findings. The lack of standardization of diagnostic and treatment protocols and the heterogeneity of cohorts both within and between studies precluded a meta-analysis. There was little evidence beyond expert opinion and single-center evaluation regarding diagnosis, treatment, and long-term outcomes of neonates with TBAO. Conclusions. The variability in the phenotype of the cohorts studied and the absence of standardized indications for intervention preclude deriving any definitive conclusions regarding diagnostic tools to evaluate this patient population, treatment choices, or long-term outcomes. A coordinated multicenter study with a standardized diagnostic and treatment algorithm is recommended to develop evidence for the diagnosis and treatment of neonates with TBAO.

The impact of transforming healthcare delivery on cystic fibrosis outcomes: a decade of quality improvement at Cincinnati Children’s Hospital

Background In 2001, Cincinnati Childrens Hospital embarked on a journey to improve healthcare delivery to patients with cystic fibrosis (CF). Data from the Cystic Fibrosis Foundation National Patient Registry revealed our below-average clinical outcomes, prompting us to initiate improvement interventions. Objective To improve clinical outcomes for patients with CF through a comprehensive quality-improvement approach directed at increasing patient centredness and improving healthcare delivery. Interventions In 2001, we shared our below-average outcomes with patients, families and care providers. We instituted a quality-improvement steering committee with parental and hospital leadership, and our data-management support was restructured to provide real-time clinical data to monitor our progress. In 2002, our weekly chart conference changed to a prospective planning session and individualised daily schedules were created for inpatients. In 2003, an influenza vaccination campaign was initiated and our infection-control practices were redesigned. In 2005, best-practice guidelines were developed for airway-clearance therapy. In 2007, evidence-based clinical algorithms were designed and implemented and key care-team members were added. Measurements Primary outcome measures were median forced expiratory volume in 1 s per cent predicted (age range 6–17 years) and median body mass index percentile (age range 2–20 years). Results From 2000 to 2010, median forced expiratory volume in 1 s increased from 81.7% to 100.1% predicted and median body mass index increased from the 35th to the 55th centile. Discussion By focusing on specific outcomes, empowering families and patients, effectively using data, and standardising care processes, we transformed the culture and delivery of care for our patients with CF and learned valuable lessons potentially translatable to other chronic-care providers.

Decision Making for Children with Obstructive Sleep Apnea without Tonsillar Hypertrophy

Objective Evidence-based medicine is the gold standard practice model for patient management. Our aim was to determine whether decisions made by pediatric subspecialists regarding management of obstructive sleep apnea in children without tonsillar hypertrophy adhered to this model or were based on clinical experiences. Study Design Single-institution prospective study. Setting Multidisciplinary upper airway center in an academic pediatric hospital. Subjects and Methods Twelve pediatric subspecialists representing 8 specialties participating in upper airway clinics and management conferences. Real-time decisions made in treatment conferences and upper airway clinics were collected. Physicians were queried regarding the basis of their decisions, and these decisions were then classified into 10 categories. Results Over 13 days (10 case conferences, 3 half-day clinics), 324 decisions were made for 58 patients (mean age = 8.9 ± 7.4 years, mean body mass index percentile = 75 ± 29); 34% (n = 108) of decisions were evidence based; 59% (n = 193) were nonevidence based; and 7% (n = 23) were based on parental preference. Providers were able to cite specific studies for <20% of these decisions. There was no significant increase in the proportion of evidence-based decisions made over time. Conclusions We deemed 34% of decisions regarding the management of obstructive sleep apnea in children without tonsillar hypertrophy to be evidence based and found that sharing the basis for decisions did not improve the percentage of evidence-based decisions over time. These findings reflect significant evidence gaps and highlight the need for a systematic literature evaluation to identify best practice in managing this population. We recommend that these evidence gaps be further characterized and incorporated into an agenda for future research.

Relationship between exercise capacity and glucose tolerance in cystic fibrosis

Improved exercise capacity (EC) and normal glucose tolerance (NGT) are independently associated with favorable outcomes in CF, however, little information on this relationship exists in patients with CF.

211 Vitamin D insufficiency is associated with pulmonary exacerbations in children with cystic fibrosis

Objectives To investigate whether vitamin D status and vitamin D insufficiency were associated with CF pulmonary exacerbations in our local CF Center. Methods Data was used for patients ages 6–18 years during the years 2000–2010 from our local CF database. Models were created to investigate the association of Vitamin D 25-OH levels and IV exacerbation frequency. Vitamin D 25-OH levels were introduced in different models as either continuous or categorical variables (≥30 vs. Pseudomonas and MRSA. Results are reported as odds-ratios and 95% confidence intervals. Results The number of Vitamin D measurements included was 958. The number of unique patients included was 212. Covariates associated with pulmonary exacerbations requiring IV therapy were female sex, older age, lower BMI z-score, chronic Pseudomonas infection, and Vitamin D 25-OH status. For every 1 point decrease in Vitamin D 25-OH level, the odds of having a subsequent pulmonary IV exacerbation within 1 year increased by 1.019 times (95% CI 1.001–1.037; p 0.03). With Vitamin D insufficiency ( Conclusion Vitamin D insufficiency is associated with pulmonary exacerbations requiring IV therapy in children with cystic fibrosis.