Perioperative exposure to volatile organic compounds in neonates undergoing cardiac surgery.

OBJECTIVE: Volatile organic compounds (VOCs) are used in the sterilization and manufacture of medical equipment. These compounds have high vapor pressures with low water solubility and are emitted as gases from solids or liquids. They can be mutagenic, neurotoxic, genotoxic, and/or carcinogenic. Safe limits of exposure are not known for neonates. This study examined determinants of exposure in newborns undergoing cardiac surgery. METHODS: Twenty metabolites of 16 VOCs (eg, xylene, cyanide, acrolein, acrylonitrile, N, N-dimethylformamide, 1,3-butadiene, styrene, and benzene) were measured as metabolites in daily urine samples collected from 10 neonates undergoing cardiac operations (n = 150 samples). Metabolites were quantified using reversed-phase ultra-high performance liquid chromatography and electrospray ionization tandem mass spectrometry. Repeated measures analysis of covariance was performed for each metabolite to examine associations with use of medical devices. RESULTS: At least 3 metabolites were detected in every sample. The median number of metabolites detected in each sample was 14 (range, 3-15). In a model controlling for other factors, the use of extracorporeal membrane oxygenation was associated with significantly (P ≤ .05) greater metabolite levels of acrolein, acrylonitrile, ethylene oxide, propylene oxide, styrene, and ethylbenzene. Patients breathing ambient air had greater levels of metabolites of acrolein, xylene, N,N-dimethylformamide, methyl isocyanate, cyanide, 1,3-butadiene (all P ≤ .05). CONCLUSIONS: Exposure to volatile organic compounds is pervasive in newborns undergoing cardiac surgery. Sources of exposure likely include medical devices and inhalation from the air in the intensive care unit. The contribution of VOC exposure during cardiac surgery in newborns to adverse outcomes warrants further evaluation.

Comparison of technical errors in pediatric bitewing radiographs acquired with round vs rectangular collimation.

OBJECTIVE: To compare technical errors in bitewing radiographs acquired with round vs rectangular collimation in a hospital-based pediatric dentistry training program. STUDY DESIGN: A retrospective chart review was conducted of 176 digital bitewing radiographs exposed with round collimation and 106 exposed with rectangular collimation. The number of re-exposures was calculated, and errors in central ray entry (CRE; "cone cuts"), horizontal and vertical positioning, and angulation were measured. RESULTS: There were no greater re-exposures but significantly more CRE errors with rectangular collimation (21.7%; n = 23; 95% confidence interval [CI], 13.9%-30.0%) than with round collimation (3.4%; n = 6; 95% CI, 0.7%-6.1%). CRE error location, horizontal positioning errors, and size of horizontal overlapped contacts were statistically different but not clinically important. CONCLUSIONS: Use of rectangular collimation resulted in increased CRE errors but no other clinically significant problems. This technique should be used to reduce radiation exposure to patients.

Morbidity and cost burden of prenatal myelomeningocele repair.

OBJECTIVE: This study compared the morbidities and financial burden associated with prenatal open myelomeningocele repair versus postnatal repair. MATERIALS AND METHODS: The retrospective study cohort included 23 mother-infant dyads undergoing prenatal repair and 30 with postnatal repair. Financial, demographic, and medical information were obtained for mother-infant dyads from each infant's birth through the first year of life. RESULTS: Infants in the prenatal repair group were significantly affected by sequelae of prematurity, including apnea, bronchopulmonary dysplasia, and retinopathy of prematurity. Importantly, fewer of these infants required ventriculoperitoneal shunt procedures and the overall improved composite outcome of shunting or death. Infants in the postnatal repair group required more hospital readmissions in the first year of life. Financial costs for mother-infant dyads in the prenatal repair group were higher, driven by the length of stay in the neonatal intensive care unit and maternal hospital admissions. Kaplan-Meier curves analyzing the relative contributions of the length of stay and total charges in the population were constructed. The maternal contribution was markedly different between groups due to postoperative hospitalization, readmission, and close fetal surveillance required. Though these differences did not reach statistical significance, it highlights an important burden on families and the medical system. CONCLUSIONS: This is the first study to report the contributions of prematurity in relation to maternal and infant morbidity and financial costs. The benefits of prenatal myelomeningocele repair include lower risk for ventriculoperitoneal shunting and fewer hospital readmissions. The risk of preterm birth and its neonatal sequelae continue to be a significant burden.

Risk and outcomes of pulmonary fungal infection after pediatric lung transplantation.

BACKGROUND: Prospective studies to determine associated risk factors and related outcomes for pulmonary fungal infection (PFI) after pediatric lung transplant (PLT) are lacking. METHODS: NIH-sponsored Clinical Trials in Organ Transplantation in Children enrolled PLT candidates, collecting data prospectively for 2 years post-transplant. Demographics, signs/symptoms, radiology, pathology and microbiology were collected. Analyses evaluated for PFI-related risks and outcomes. RESULTS: In 59 PLT, pre-transplant fungal colonization occurred in 6 donors and 15 recipients. Cystic fibrosis (CF) was associated with pre-transplant colonization (P < .01). Twenty-five (42%) PLT had 26 post-transplant colonizations (median = 67 days, range = 0-750 days) with Candida (13), Aspergillus (4), mold (6) or yeast (3). Post-PLT colonization was not associated with CF, age, or pre-PLT colonization. Thirteen PFIs occurred in 10 (17%) patients, 3 proven (Candida species) and 10 probable (Candida [3], Aspergillus [3], Penicillium [3], and mold [1]). Pulmonary fungal infection was preceded by post-PLT colonization with the same organism in 4 of 13 PFI, but post-PLT colonization did not predict subsequent PFI (P = .87). Older age at transplant was a risk for PFI (P < .01). No mortality was attributed to PFI. Prophylaxis use was not associated with decreased post-PLT colonization (P = .60) or PFI (P = .48). CONCLUSION: In PLT, PFI and fungal colonization are common but without associated mortality. Post-PLT colonization did not predict PFI. Optimal prevention strategies require additional study.

Epstein-Barr viral loads do not predict post-transplant lymphoproliferative disorder in pediatric lung transplant recipients: A multicenter prospective cohort study.

Prediction of PTLD after pediatric lung transplant remains difficult. Use of EBV VL in WB has been poorly predictive, while measurement of VL in BAL fluid has been suggested to have enhanced utility. The NIH-sponsored Clinical Trials in Organ Transplantation in Children (CTOTC-03) prospectively obtained serial quantitative measurements of EBV PCR in both WB and BAL fluid after pediatric lung transplantation. Descriptive statistics, contingency analyses, and Kaplan-Meier analyses evaluated possible association between EBV and PTLD. Of 61 patients, 34 (56%) had an EBV+PCR (at least once in WB or BAL). EBV donor (D)+patients more often had a positive PCR (D+/recipient (R)-: 13/18; D+/R+: 14/23) compared to EBV D- patients (6/17). Several D-/R- (5/12) patients developed EBV, but none developed PTLD. All four PTLD patients were D+/R- with EBV+PCR. Neither the time to first EBV+PCR nor the CT for PCR positivity in BAL or WB was statistically different between those with and without PTLD. Having an EBV-seropositive donor was associated with increased risk of EBV+PCR in WB. EBV load in BAL was not predictive of PTLD.

Temporal trends in inpatient pediatric sleep apnea surgery: 1993-2010.

OBJECTIVES/HYPOTHESIS: We sought to characterize changes in the patterns of inpatient surgical sleep care over time and ascertain if these changes were consistent with previously reported trends in adult surgical sleep care. STUDY DESIGN: Repeated cross-sectional study. METHODS: Discharge data from the U.S. Nationwide Inpatient Sample for 125,691 nasal, palatal, or hypopharyngeal procedures in children for sleep-disordered breathing or obstructive sleep apnea (OSA) from 1993 to 2010 were analyzed using cross-tabulations and multivariate regression modeling. RESULTS: Inpatient surgical sleep procedures increased from 45,671 performed in 1993 to 2000 (study period 1) to 80,020 in 2001 to 2010 (study period 2). Overall, patients were commonly male (61.3%), privately insured (46.8%), 2 to 6 years old (39.4%), and white (36.4%). Obesity was reported in 4.6% and 6.7% of children during study periods 1 and 2, respectively (P < .0001). Tonsillectomy (with and without adenoidectomy) was the most commonly performed procedure in both study periods. With the exception of uvulopalatopharyngoplasty and tracheostomy, all sleep procedures increased over time; however, multilevel surgery did not significantly increase (P = .28). Children who underwent sleep surgery during study period 2 were more likely to receive a supraglottoplasty (P = .0125) and to undergo procedures at high-volume hospitals (P = .0311), and less likely to undergo a tracheostomy (P < .0001). CONCLUSIONS: These data reflect changing trends in the surgical management of pediatric OSA, with significant increases in nasal and hypopharyngeal procedures, particularly lingual tonsillectomy and supraglottoplasty. Unlike the trend in adults, multilevel surgery in children with sleep disordered breathing or OSA has not yet been integrated into routine clinical practice. LEVEL OF EVIDENCE: 2C Laryngoscope, 127:1235-1241, 2017.

Pulmonary Morbidity in Infancy after Exposure to Chorioamnionitis in Late Preterm Infants.

RATIONALE: Chorioamnionitis is an important cause of preterm birth, but its impact on postnatal outcomes is understudied. OBJECTIVES: To evaluate whether fetal exposure to inflammation is associated with adverse pulmonary outcomes at 6 to 12 months' chronological age in infants born moderate to late preterm. METHODS: Infants born between 32 and 36 weeks' gestational age were prospectively recruited (N = 184). Chorioamnionitis was diagnosed by placenta and umbilical cord histology. Select cytokines were measured in samples of cord blood. Validated pulmonary questionnaires were administered (n = 184), and infant pulmonary function testing was performed (n = 69) between 6 and 12 months' chronological age by the raised volume rapid thoracoabdominal compression technique. MEASUREMENTS AND MAIN RESULTS: A total of 25% of participants had chorioamnionitis. Although infant pulmonary function testing variables were lower in infants born preterm compared with historical normative data for term infants, there were no differences between infants with chorioamnionitis (n = 20) and those without (n = 49). Boys and black infants had lower infant pulmonary function testing measurements than girls and white infants, respectively. Chorioamnionitis exposure was associated independently with wheeze (odds ratio [OR], 2.08) and respiratory-related physician visits (OR, 3.18) in the first year of life. Infants exposed to severe chorioamnionitis had increased levels of cord blood IL-6 and greater pulmonary morbidity at age 6 to 12 months than those exposed to mild chorioamnionitis. Elevated IL-6 was associated with significantly more respiratory problems (OR, 3.23). CONCLUSIONS: In infants born moderate or late preterm, elevated cord blood IL-6 and exposure to histologically identified chorioamnionitis was associated with respiratory morbidity during infancy without significant changes in infant pulmonary function testing measurements. Black compared with white and boy compared with girl infants had lower infant pulmonary function testing measurements and worse pulmonary outcomes.

Using Polysomnography and Airway Evaluation to Predict Successful Decannulation in Children.

OBJECTIVE: Decannulation readiness is approached in several ways and is tailored to the individual patient. While microlaryngoscopy and bronchoscopy evaluate airway patency at all levels, polysomnography assesses sleep-related upper airway physiology. There are limited data in the literature on the utility of these tools. Thus, the main objectives of this study were (1) to identify sleep and respiratory parameters associated with successful decannulation and (2) to determine the agreement between microlaryngoscopy and bronchoscopy and polysomnography. STUDY DESIGN: Case series with chart review. SETTING: Quaternary care pediatric hospital. SUBJECTS AND METHODS: A retrospective review of medical records, microlaryngoscopy and bronchoscopy, and polysomnographs was performed on subjects preparing for decannulation from 2005 to 2010. Fifty-nine subjects who underwent overnight polysomnography with a tracheostomy capping trial and microlaryngoscopy and bronchoscopy were included. Prediction of successful decannulation from polysomnography parameters was determined using logistic regression analysis. RESULTS: Fifty-nine subjects with a total of 78 polysomnographs were subdivided into 2 groups: 42 polysomnographs were classified as successfully decannulated, and 36 polysomnographs belonged to the group that did not decannulate. Logistic regression analysis determined that the Apnea Hypopnea Index (P = .0423) and maximal end-tidal CO2 (P = .046) were significant predictors for successful decannulation. CONCLUSION: Airway evaluation by microlaryngoscopy and bronchoscopy is an essential tool in the assessment of decannulation readiness. Polysomnography is an important additional tool for children with complex airway problems. Our results indicate that certain polysomnographic parameters such as Apnea Hypopnea Index and maximal end-tidal CO2 are valuable in predicting successful tracheostomy decannulation.

Concentration of fractional excretion of nitric oxide (FENO): A potential airway biomarker of restored CFTR function.

BACKGROUND: Lower airway biomarkers of restored cystic fibrosis transmembrane conductance regulator (CFTR) function are limited. We hypothesized that fractional excretion of nitric oxide (FENO), typically low in CF patients, would demonstrate reproducibility during CFTR-independent therapies, and increase during CFTR-specific intervention (ivacaftor) in patients with CFTR gating mutations. METHODS: Repeated FENO and spirometry measurements in children with CF (Cohort 1; n=29) were performed during hospital admission for acute pulmonary exacerbations and routine outpatient care. FENO measurements before and after one month of ivacaftor treatment (150 mg every 12h) were completed in CF patients with CFTR gating mutations (Cohort 2; n=5). RESULTS: Cohort 1: Mean forced expiratory volume in 1s (FEV1 % predicted) at enrollment was 72.3% (range 25%-102%). Mean FENO measurements varied minimally over the two inpatient and two outpatient measurements (9.8-10.9 ppb). There were no clear changes related to treatment of pulmonary exacerbations, gender, genotype or microbiology, and weak correlation with inhaled corticosteroid use (P<0.05). Between the two inpatient measurements, FEV1 % predicted increased by 7.3% (P<0.03) and FENO did not change. In Cohort 2, mean FENO increased from 6.6 ppb (SD=2.19) to 11.8 ppb (SD=4.97) during ivacaftor treatment. Mean sweat chloride dropped by 58 mM and mean FEV1 % predicted increased by 10.2%. CONCLUSIONS: Repeated FENO measurements were stable in CF patients, whereas FENO increased in all patients with CFTR gating mutations treated with ivacaftor. Acute changes in FENO may serve as a biomarker of restored CFTR function in the CF lower airway during CFTR modulator treatment.

Insulin secretion abnormalities in exocrine pancreatic sufficient cystic fibrosis patients.

BACKGROUND: The aim of this study is to assess insulin secretion in pediatric cystic fibrosis (CF) patients with exocrine pancreatic sufficiency. METHODS: Glucose and insulin responses during an oral glucose tolerance test (OGTT) were measured in 146 CF patients. Patients were divided into exocrine sufficient (CF-PS) and insufficient (CF-PI) groups based on pancreatic enzyme usage and fecal elastase. A reference group included healthy, non-diabetic subjects. RESULTS: All CF groups showed reduced insulin secretion as measured by insulinogenic index. The CF-PS patients had normal glucose tolerance. There was a direct correlation between BMI z-score and insulin area under the curve. CONCLUSION: Patients with CF have reduced insulin secretion during an OGTT regardless of exocrine pancreatic status. The abnormal insulin secretion in all CF patients may predispose them for glucose intolerance, particularly when challenged by inflammation, infection, or nutritional deficiency. In addition, the diminished insulin secretion may contribute to increased catabolism. Lastly, the CF-related diabetes (CFRD) screening guidelines should be followed by all CF patients regardless of pancreatic status.

Obstructive lung disease in children with idiopathic scoliosis.

OBJECTIVE: To measure the prevalence of obstructive lung disease (OLD) among patients undergoing preoperative pulmonary assessment for idiopathic scoliosis. STUDY DESIGN: This was a retrospective, descriptive review from clinical data in a tertiary care pediatric hospital in the US. Patients (n = 176) with idiopathic scoliosis with Cobb angles of ≥ 40 degrees who performed acceptable and repeatable preoperative pulmonary function testing were included. The primary outcome measure was the forced expiratory volume in the first second (FEV1)/forced vital capacity (FVC) ratio. RESULTS: The prevalence of OLD (low FEV1/FVC ratio) was 39% (68/176 patients). In multivariate modeling, radiographic measures were poor predictors of pulmonary function outcomes of FVC (r(2) 0.06), FEV1 (r(2) 0.05), FEV1/FVC ratio (r(2) 0.08), and total lung capacity (r(2) 0.06). CONCLUSIONS: OLD is common in patients with idiopathic scoliosis. We recommend preoperative pulmonary function testing for patients with idiopathic scoliosis under consideration for spinal fusion surgery.

Inhaled tobramycin effectively reduces FEV1 decline in cystic fibrosis. An instrumental variables analysis.

RATIONALE: The efficacy of inhaled tobramycin on chronic Pseudomonas aeruginosa infections in patients with cystic fibrosis (CF) has been established in clinical trials. However, little is known about its clinical effectiveness on lung function outside randomized controlled trial settings; conventional analysis of existing registry data has heretofore been confounded by treatment selection bias. OBJECTIVE: To determine effectiveness of inhaled tobramycin on FEV1 decline in patients with chronic P. aeruginosa infections using observational data from the Cystic Fibrosis Foundation Patient Registry. METHODS: Patient-level tobramycin use was measured at first chronic P. aeruginosa infection (n = 13,686 patients; age, 6-21 yr). Decline in FEV1 2 years after infection was estimated for patients treated with tobramycin and compared with untreated patients. Multiple linear regressions with confounder adjustment and propensity scores were used to estimate mean FEV1 decline for each group. Because care is organized by centers, we used center-specific prescription rates as an instrument to reduce treatment-by-condition bias. MEASUREMENTS AND MAIN RESULTS: Using center-level prescribing rates, instrumental variables analysis showed less FEV1 decline for patients who received tobramycin when first eligible compared with those who did not receive tobramycin (difference, 2.55% predicted; 95% confidence interval, 0.16-4.94; P = 0.0366). CONCLUSIONS: Inhaled tobramycin is effective in reducing lung function decline among patients 6 to 21 years of age with CF. Because CF care is organized by center, using center-specific prescription rates as an instrumental variable is a feasible approach to using the Cystic Fibrosis Foundation Patient Registry to determine treatment effectiveness. More generally, this approach can correct for treatment-by-condition bias arising from observational studies.

Baroreflex sensitivity after adenotonsillectomy in children with obstructive sleep apnea during wakefulness and sleep.

STUDY OBJECTIVES: Children with obstructive sleep apnea have blunted baroreflex sensitivity and increased blood pressure variability. The aim of the study was to test the hypothesis that treatment of sleep apnea by adenotonsillectomy results in significant improvement of baroreflex sensitivity, lowering of blood pressure and blood pressure variability and increase vagal heart rate modulation. STUDY DESIGN: One hundred ninety-four children aged 9.6 ± 2.3 years were enrolled; 133 had obstructive sleep apnea and 61 were healthy controls. For children with sleep apnea, polysomnography with 3-lead electrocardiography and continuous blood pressure was performed before adenotonsillectomy, then 6 weeks and 6 months postoperatively. Controls underwent the same assessment at study entry and 6 months later. Spontaneous baroreflex sensitivity was measured in the time and frequency domains. Data analyses were performed for available and complete cases. RESULTS: Children with sleep apnea experienced postoperatively an increase in baroreflex sensitivity and decrease in blood pressure variability during wakefulness and sleep. A decrease in blood pressure during sleep and in heart rate during wakefulness was also measured. The improvement in baroreflex sensitivity was predicted by the change in the apnea-hypopnea and arousal indices. A normal pattern of rising baroreflex sensitivity during the night was restored in children with severe apnea after surgery. However, baroreceptor sensitivity did not completely normalize after treatment. CONCLUSION: Treatment of obstructive sleep apnea in children by adenotonsillectomy is associated with gradual improvement in known risk factors for cardiovascular disease. Complete normalization of baroreceptor sensitivity was not achieved 6 months postoperatively.

Bronchiectasis in chronic pulmonary aspiration: risk factors and clinical implications.

INTRODUCTION: Bronchiectasis is a well-known sequela of chronic pulmonary aspiration (CPA) that can result in significant respiratory morbidity and death. However, its true prevalence is unknown because diagnosis requires high resolution computed tomography which is not routinely utilized in this population. This study describes the prevalence, time course for development, and risk factors for bronchiectasis in children with CPA. MATERIALS AND METHODS: Using a cross-sectional design, medical records were reviewed for all patients with swallow study or airway endoscopy-confirmed aspiration in our airway center over a 21 month period. All patients underwent rigid and flexible bronchoscopy, and high resolution chest computed tomography. Prevalence, distribution, and risk factors for bronchiectasis were identified. RESULTS: One hundred subjects age 6 months to 19 years were identified. Overall, 66% had bronchiectasis, including 51% of those less than 2 years old. The youngest was 8 months old. Severe neurological impairment (OR 9.45, P<0.004) and history of gastroesophageal reflux (OR 3.36, P=0.036) were identified as risk factors. Clinical history, exam, and other co-morbidities did not predict bronchiectasis. Sixteen subjects with bronchiectasis had repeat chest computed tomography with 44% demonstrating improvement or resolution. DISCUSSION: Bronchiectasis is highly prevalent in children with CPA and its presence in young children demonstrates that it can develop rapidly. Early identification of bronchiectasis, along with interventions aimed at preventing further airway damage, may minimize morbidity and mortality in patients with CPA.

Improving evidence-based care in cystic fibrosis through quality improvement.

OBJECTIVE: To increase clinician adherence to prescribing guidelines for pulmonary medications in children with cystic fibrosis (CF). DESIGN: Quality improvement project with multiple time series design. SETTING: The CF center at a tertiary care pediatric hospital in the United States. PATIENTS: Children with CF who were eligible to receive oral azithromycin, nebulized dornase alfa, or inhaled tobramycin sulfate based on prescribing guidelines for CF lung disease. INTERVENTION: Evidence-based prescribing guidelines were designed by a local committee to reflect consensus recommendations from the CF Foundation. Clinicians and families were educated about guidelines. Adherence to prescribing guidelines was tracked using a local CF database and record reviews. Weekly meetings were used to highlight adherence failures and promote clinician accountability. MAIN OUTCOME MEASURE: The rate of clinician adherence to prescribing guidelines. RESULTS: One hundred seventy patients with CF were included. At the start of the project, the rate of clinician adherence to prescribing guidelines was 62%. After 3 months of the project, the rate of clinician adherence to prescribing guidelines was 87% (odds ratio = 4.6; 95% confidence interval, 3.0-7.0). The improvements in adherence to prescribing guidelines were sustained for 21 months of follow-up. CONCLUSIONS: Educating clinicians about prescribing guidelines, sharing guidelines with families, and monitoring clinician adherence improve prescribing adherence to evidence-based recommendations.

Inhibition of PI3K by PX-866 prevents transforming growth factor-alpha-induced pulmonary fibrosis.

Transforming growth factor-alpha (TGFalpha) is a ligand for the epidermal growth factor receptor (EGFR). EGFR activation is associated with fibroproliferative processes in human lung disease and animal models of pulmonary fibrosis. EGFR signaling activates several intracellular signaling pathways including phosphatidylinositol 3'-kinase (PI3K). We previously showed that induction of lung-specific TGFalpha expression in transgenic mice caused progressive pulmonary fibrosis over a 4-week period. The increase in levels of phosphorylated Akt, detected after 1 day of doxycycline-induced TGFalpha expression, was blocked by treatment with the PI3K inhibitor, PX-866. Daily administration of PX-866 during TGFalpha induction prevented increases in lung collagen and airway resistance as well as decreases in lung compliance. Treatment of mice with oral PX-866 4 weeks after the induction of TGFalpha prevented additional weight loss and further increases in total collagen, and attenuated changes in pulmonary mechanics. These data show that PI3K is activated in TGFalpha/EGFR-mediated pulmonary fibrosis and support further studies to determine the role of PI3K activation in human lung fibrotic disease, which could be amenable to targeted therapy.

Improvements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic pseudomonas aeruginosa infections, and dornase alfa use.

OBJECTIVE: To compare lung function and nutritional outcomes in cystic fibrosis (CF) for 2 birth cohorts in our CF center. STUDY DESIGN: Patients with CF born between 1985 and 2000 treated in our CF center before age 5 years were included. The patients were divided into 2 equal birth cohorts for comparison: birth cohort 1 (born between 1985 and 1992) and birth cohort 2 (born between 1993 and 2000). To compare lung function, we used forced expiratory volume in the first second (FEV(1))% predicted and FEV(1)% predicted slope from age 6 to 12 years. We hypothesized that we would find significant improvements in lung function and nutritional outcomes in our patients with CF. RESULTS: The patients born between 1993 and 2000 (birth cohort 2) had better lung function, a slower rate of decline in lung function, and better nutritional outcomes compared with those born between 1985 and 1992 (birth cohort 1). Factors associated with a slower rate of decline in lung function in both groups were a higher baseline body mass index (BMI)%, a slower BMI% rate of decline, absence of chronic Pseudomonas aeruginosa respiratory infection, and initiation of dornase alfa (Pulmozyme) therapy before age 9 years. CONCLUSION: Our results demonstrate dramatically improved lung function and nutritional outcomes in the children with CF in our center. The improvements in lung function outcomes are associated with better nutrition, fewer chronic P aeruginosa infections, and dornase alfa therapy.

Growth velocity predicts recurrence of sleep-disordered breathing 1 year after adenotonsillectomy.

RATIONALE: Adenotonsillectomy, the first line of treatment of sleep-disordered breathing (SDB), is the most commonly performed pediatric surgery. Predictors of the recurrence of SDB after adenotonsillectomy and its impact on cardiovascular risk factors have not been identified. OBJECTIVES: Demonstrate that gain velocity in body mass index (BMI) defined as unit increase in BMI/year confers an independent risk for the recurrence of SDB 1 year after adenotonsillectomy. METHODS: Children with SDB and hypertrophy of the tonsils and a comparison group of healthy children were followed prospectively for 1 year. MEASUREMENTS AND MAIN RESULTS: Serial polysomnographies, BMI, and blood pressure were obtained before adenotonsillectomy and 6 weeks, 6 months, and 1 year postoperatively. Gain velocity in BMI, BMI and being African American (odds ratios, 4-6/unit change/yr; 1.4/unit and 15, respectively) provided equal amounts of predictive power to the risk of recurrence of SDB. In the group that experienced recurrence, systolic blood pressure at 1 year was higher than at baseline and higher than in children who did not experience recurrence. CONCLUSIONS: Three clinical parameters confer independent increased risk for high recurrence of SDB after adenotonsillectomy: gain velocity in BMI, obesity, and being African American. A long-term follow-up of children with SDB and monitoring of gain velocity in BMI are essential to identifying children at risk for recurrence of SDB and in turn at risk for hypertension.