Faculty Bibliography

OBJECTIVE: Sleep disordered breathing (SDB) has not been well studied in urban adolescents with asthma in community settings. Nor has the association of SDB symptoms and asthma severity been studied. We characterized self-reported symptoms suggesting SDB and investigated the association of SDB symptoms, probable asthma, and asthma severity. METHODS: 9,565 adolescents from 21 inner-city high schools were screened for an asthma intervention study. Students reported on symptoms suggesting SDB using questions from the 2007 NHANES, if they were ever diagnosed with asthma, and on asthma symptoms. Using generalized linear mixed models with logit link with school as a random intercept and adjusting for age, gender, and race/ethnicity, we examined associations of SDB symptoms, and demographic characteristics, probable asthma, and asthma severity. RESULTS: 12% reported SDB symptoms. Older and bi-racial participants (compared to Caucasian) had higher odds of symptoms suggesting SDB (p<.001). Compared to those without probable asthma, adolescents with probable asthma had 2.63 greater odds of reporting SDB symptoms (p<.001). Among those with probable asthma, the odds of reporting SDB symptoms increased with asthma severity. When exploring daytime severity and severity due to night wakening separately, results were similar. All results remained significant when controlling for age, gender, and ethnicity. CONCLUSIONS: In a large urban community cohort of predominately ethnic minority adolescents, self-reported SDB symptoms were associated with probable asthma and increased asthma severity. This study highlights the importance of SDB as a modifiable co-morbidity of asthma.

OBJECTIVE: To examine the association of adolescent asthma-related anxiety, social anxiety, separation anxiety, and caregiver asthma-related anxiety, with asthma care by urban adolescents. METHODS: Participants were 386 ethnic minority adolescents (mean age=12.8) with persistent asthma and their caregivers. Adolescents reported what they do to prevent asthma symptoms and to manage acute symptoms, and if they or their caregiver is responsible for their asthma care. Adolescents completed the Youth Asthma-related Anxiety Scale, and the social and separation anxiety subscales of the SCARED; caregivers completed the Parent Asthma-related Anxiety Scale. Linearity of the associations was assessed via Generalized Additive Models (GAM). When there was no evidence for non-linearity, linear mixed effects models were employed to evaluate the effects of the predictors. RESULTS: Adolescents asthma-related anxiety had a strong curvilinear relationship with symptom prevention (P<0.001). Adolescents took more prevention steps as their anxiety increased, with a plateau at moderate anxiety. There was a linear relationship of adolescent asthma-related anxiety to symptom management (beta=0.03, P=.021) and to asthma responsibility (beta=0.11, P=.015), and of caregiver asthma-related anxiety to adolescent symptom prevention (beta=0.04, P=.001). Adolescent social and separation anxiety had weak-to-no relationship with asthma care. Results remained consistent when controlling for each of the other anxieties. CONCLUSIONS: Asthma-related anxiety plays an important, independent role in asthma care. When low, adolescents may benefit from increased support from caregivers and awareness of the consequences of uncontrolled asthma. When elevated, health providers should ensure the adolescents are not assuming responsibility for asthma care prematurely.

Ultrastructural examination of cilia is the "gold standard" for diagnosing primary ciliary dyskinesia. There is little evidence suggesting the most effective method of procuring a ciliary biopsy and scant benchmark data on rates of conclusive biopsies or on the diagnostic impact of such biopsies. To critically assess rates of inconclusive, positive, and negative ciliary biopsies and to identify clinical factors associated with conclusive results, we reviewed ciliary biopsies submitted for electron microscopy from 2006 to 2011, noting whether specimens were adequate for analysis and whether the ciliary structure was normal. The biopsy site, method used, procedurist's specialty, and clinical diagnoses were determined. Biopsy findings were categorized by diagnostic impact. Over 5 years, 187 patients had 211 biopsies. Conclusive results were obtained on 133/211 biopsies (63%); the remainder were insufficient. The rate of inconclusive biopsies did not vary significantly (P > 0.05; Fisher's exact) among sampling methods. Abnormal results were identified in 8/133 (6.0%) of the adequate specimens. Forceps compared to brush biopsies (abnormal in 4/12 versus 4/121 of the adequate specimens, P = 0.002), along with multiple biopsy samples (taken on same or different days) compared with a single biopsy sample (abnormal in 3/12 versus 1/110 of the adequate specimens, P = 0.01), were more likely to yield an abnormal result. Only 63% of pediatric ciliary biopsies provide adequate morphology for analysis, the large majority of these samples showing normal ciliary anatomy. The method of obtaining biopsies did not significantly affect result conclusiveness. Understanding the diagnostic impact of ultrastructural analysis is important as new diagnostic algorithms are developed for primary ciliary dyskinesia.

Objective tools are needed to assess the response to treatment in pediatric narcolepsy. This article presents a single-center experience documenting the use of the maintenance of wakefulness test (MWT) in a pediatric series. This study reviewed the charts of children with narcolepsy who had an MWT performed between January 2008 and June 2012. A cutoff was used for mean sleep latency: <8 minutes for inadequate control of hypersomnia, and >20 minutes to indicate adequate control on medications. Thirteen tests were performed on 10 children (median age 15.8 years, range 8.7-20.3 years) with narcolepsy, of which six had cataplexy and three were boys. Comorbid conditions included Prader-Willi syndrome, bipolar affective disorder, and epilepsy (n = 1 each). The median mean sleep latency for all studies was 16 minutes (range 5.8-40 minutes). Sleep-onset rapid eye movement sleep events were seen in three of 13 studies. In seven patients, findings from the MWT resulted in changes in management. These data suggest that the MWT may be a useful and feasible test for assessing response to treatment in children with narcolepsy. Future research is needed to obtain normative MWT data on children with and without narcolepsy.

BACKGROUND: : The role of adenotonsillectomy in the treatment of obstructive sleep apnea in patients with craniosynostosis is not well established. METHODS: : A retrospective review was conducted of all children with syndromic craniosynostosis (Apert, Crouzon, and Pfeiffer syndromes) seen at Boston Children's Hospital from January 2001 through April 2011. The primary outcome measure was the apnea/hypopnea index before and after adenotonsillectomy. RESULTS: : There were 47 patients (66 percent Apert syndrome, 19 percent Pfeiffer syndrome, and 15 percent Crouzon syndrome) less than 21 years of age (mean, 1.2 +/- 3.3 years at first visit) who were followed for a mean of 14.6 +/- 8.6 years. Of children with at least one polysomnogram, 83 percent had obstructive sleep apnea (42 percent severe, 19 percent moderate, 22 percent mild). Adenotonsillectomy was performed in 62 percent of patients. Preoperative and postoperative polysomnography was performed in 45 percent of children undergoing adenotonsillectomy. Following adenotonsillectomy, the mean apnea/hypopnea index was not significantly different, and obstructive sleep apnea persisted in 11 of 13 children. CONCLUSIONS: : Obstructive sleep apnea is commonly present in children with syndromic craniosynostosis and is a complex disease with airway obstruction at several different levels. Adenotonsillectomy often does not improve the severity of obstructive sleep apnea in this population. CLINICAL QUESTION/LEVEL OF EVIDENCE: : Therapeutic, IV.

Rationale, aims and objectives Early adopters of electronic health records (EHRs) are transitioning from older to newer EHRs to satisfy meaningful use requirements. Facilitators and barriers to satisfaction after transitioning are important to understand as provider satisfaction is linked with improvement in health care. Method We conducted a cross-sectional survey of providers who transitioned from an older to a newer EHR at six academic, urban ambulatory medical practices. A novel survey was developed to assess: (1) satisfaction with the EHR implementation; (2) patterns of information technology use; (3) work perceptions; (4) methods for completing clinical tasks; and (5) demographic characteristics. We analysed the results using bivariate and multivariate analyses. Results The response rate was 64% (n = 197). A small majority were satisfied with the new EHR (64%, n = 120). Providers who reported satisfaction with overall quality of work life, their workload and the transition were more likely to be satisfied with the new EHR (P < 0.01). Providers who reported using the Internet at least daily were also more likely to be satisfied with the new EHR (P < 0.05). In a multivariate model, satisfaction with the transition was a strong predictor of satisfaction with the new EHR (P < 0.01). Barriers to satisfaction include dissatisfaction with: maintaining problem and medication lists, tracking health maintenance information, referring to clinical practice guidelines and ordering laboratory and radiology tests (P < 0.01). Conclusion This study provides groundwork for understanding the facilitators and barriers to provider satisfaction after the transition between EHRs and provides insight into areas requiring attention by entities undergoing similar transitions.

OBJECTIVE: To evaluate practitioners' expectations of, and satisfaction with, older and newer electronic health records (EHRs) after a transition. MATERIAL AND METHODS: Pre- and post-transition survey administered at six academic-affiliated ambulatory care practices from 2006 to 2008. Four practices transitioned to one commercial EHR and two practices to another. We compared respondents' expectations of, and satisfaction with, the newer EHR. RESULTS: 523 subjects were eligible: 217 were available before transition and 306 after transition. 162 pre-transition and 197 post-transition responses were received, yielding 75% and 64% response rates, respectively. Practitioners were more satisfied with the newer EHRs (64%) compared with the older (56%) (p=0.15) and a small majority (58%) were satisfied with the transition. Practitioners' satisfaction with the older EHRs for completing clinical tasks was high. The newer EHRs exceeded practitioner expectations regarding remote access (61% vs 74%; p=0.03). However, the newer EHRs did not meet practitioners' expectations regarding their ability to perform clinical tasks, or more globally, improve medication safety (81% vs 61%; p<0.001), efficiency (70% vs 44%; p<0.001), and quality of care (77% vs 67%; p=0.04). DISCUSSION: Most practitioners had favorable opinions about EHRs and reported overall improved satisfaction with the newer EHRs. However, practitioners' high expectations of the newer EHRs were often unmet regarding facilitation of specific clinical tasks or for improving quality, safety, and efficiency. CONCLUSION: To ensure practitioners' expectations, for instance regarding improvements in medication safety, are met, vendors should develop and implement refinements in their software as practices upgrade to newer, certified EHRs.