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Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity. Specific recommendations were formulated and reported as Level A, B, and C based on whether they were based on "strong", "moderate" or "weak" agreement. The tracheomalacia that exists in the site of the fistula was considered the main abnormality that predisposes to all other respiratory complications due to airway collapse and impaired clearance of secretions. Aspiration due to impaired airway protection reflexes is the main underlying contributing mechanism. Flexible bronchoscopy is the main diagnostic modality, aided by imaging modalities, especially CT scans of the chest. Noninvasive positive airway pressure support, surgical techniques such as tracheopexy and rarely tracheostomy are required for the management of severe tracheomalacia. Regular long-term follow-up by a multidisciplinary team was considered imperative. Specific templates outlining the elements of the clinical respiratory evaluation according to the patients' age were also developed.

Background: reports suggest that congenital esophageal atresia/tracheoesophageal fistula (EA/TEF) is associated with a number of upper airway anomalies [1,2]. Aims: it remains unclear whether vocal cord paresis or paralysis (VCP) is usually a primary anomaly, or is secondary to EA/TEF repair. Methods: a retrospective study was carried out in 105 patients with EA/TEF referred to the Department of Pediatric Medicine, Bambino Gesu Children’s Hospital (BGCH) for multidisciplinary follow-up following EA/TEF repair, between 2010 and 2015. Results: 64 of the 105 patients included in the study underwent EA/TEF repair and had pre-operative bronchoscopy in BGCH; the remaining patients had their initial surgery elsewhere. No patient included in the study had VCP detected pre-operatively. Six patients were diagnosed with VCP during the follow-up period (6/105, 5.7%), of whom 4 were surgically managed at the BGCH, and two were managed elsewhere. Four children with bilateral VCP initially required tracheostomy. The other two patients were treated with posterior cordotomy by CO2 laser, and one received a tracheostomy. Conclusion: we did not observe VCP in pre-operative bronchoscopy in patients undergoing EA/TEF repair, but observed VCP in 5.7% of patients during a post-operative re-evaluation, suggesting that VCP is an acquired lesion in TEF patients. Future studies are needed to determine whether VCP is present in some patients prior to EA/TEF repair, and if so, what the prevalence is. References: 1. Ann Otol Rhinol Laryngol. 2015; 124: 808-13. 2. Laryngoscope. 2015; 125: 469-74.

Canadian Inuit infants suffer the highest rate of lower respiratory tract infections (LRTI's) in the world. The causes of this are incompletely understood. The primary objective of this study was to determine whether there exists an association between respiratory morbidity and oral aspiration in Inuit children. A retrospective chart review was conducted including children from Nunavut who underwent Video Fluoroscopic Swallowing Study between the years of 2001 to 2015. The primary outcome was hospitalization for LRTI. We hypothesized that infants found to have aspiration would experience a higher rate of admissions for LRTI than those with normal swallowing studies. One-hundred and twenty-seven patients were identified, of whom 94 were included. Fifty-six percent of patients had an abnormal swallowing study. Compared with patients with normal swallowing, the incidence rate of LRTI was higher in patients with aspiration (incidence rate ratio [IRR] = 1.51; 95% confidence interval [CI] = 1.23-1.87) and in patients with penetration (IRR = 1.40; 95% CI = 1.11-1.76). Fourteen percent of patients had confirmed laryngeal cleft; patients with confirmed presence of this also had a higher incidence rate of LRTI (IRR = 1.66; 95% CI = 1.32-2.07). The incidence of abnormal swallowing study showed an 11-fold variation across the five regions in Nunavut, with the highest prevalence in west Qikiqtani Region (Baffin Island). We conclude that swallowing dysfunction is not only prevalent amongst Canadian Inuit but clinically significant. This is the first study to demonstrate an association between swallowing dysfunction and respiratory morbidity in this population. Geographic distribution patterns and high rates of laryngeal cleft may point to a potential genetic etiology for what remains at this point, idiopathic swallowing dysfunction.

Following the introduction of 7-valent pneumococcal vaccine (PCV7), while overall rates of invasive pneumococcal disease and pneumococcal pneumonia in children declined, rates of empyema increased. We examined changes in the incidence of hospitalization for pediatric complicated pneumonia (PCOMP) in Eastern Ontario, Canada, particularly since the introduction of the 13-valent vaccine (PCV13). A retrospective chart review was carried out evaluating previously healthy children admitted with PCOMP, which included empyema, parapneumonic effusion, necrotizing pneumonia, and lung abscess between 2002 and 2015. Three-hundred seventy-one children were included. Subjects had a median age of four years, and 188/370 (50.8%) required a chest tube. Admission rates changed markedly during this time period. The number of admissions per year rose most sharply between 2009 and 2012, corresponding to the period following introduction of PCV7 and then the occurrence of pandemic influenza A (H1N1). In children who likely received PCV13, the incidence of PCOMP returned to approximately pre-PCV7 levels. In contrast, rates of PCOMP in older children (who would not have received PCV13) remained elevated during the post-PCV13 time period. While rates of PCOMP, particularly in older children, remain elevated following the introduction of PCV13, this might be expected to resolve with more widespread vaccine coverage with PCV13 and herd immunity.

OBJECTIVES: To prospectively study infants with an inconclusive diagnosis of cystic fibrosis (CF) identified by newborn screening (NBS; “CF screen positive, inconclusive diagnosis” [CFSPID]) for disease manifestations. METHODS: Infants with CFSPID and CF based on NBS from 8 CF centers were prospectively evaluated and monitored. Genotype, phenotype, repeat sweat test, serum trypsinogen, and microbiology data were compared between subjects with CF and CFSPID and between subjects with CFSPID who did (CFSPID→CF) and did not (CFSPID→CFSPID) fulfill the criteria for CF during the first 3 years of life. RESULTS: Eighty-two subjects with CFSPID and 80 subjects with CF were enrolled. The ratio of CFSPID to CF ranged from 1:1.4 to 1:2.9 in different centers. CFTR mutation rates did not differ between groups; 96% of subjects with CFSPID and 93% of subjects with CF had 2 mutations. Subjects with CFSPID had significantly lower NBS immunoreactive trypsinogen (median [interquartile range]:77 [61–106] vs 144 [105–199] μg/L; P Pseudomonas aeruginosa and Stenotrophomonas maltophilia were isolated in 12% and 5%, respectively, of subjects with CFSPID. CF was diagnosed in 9 of 82 (11%) subjects with CFSPID (genotype and abnormal sweat chloride = 3; genotype alone = 4; abnormal sweat chloride only = 2). Sweat chloride was abnormal in CFSPID→CF patients at a mean (SD) age of 21.3 (13.8) months. CFSPID→CF patients had significantly higher serial sweat chloride ( P P = .009) levels than did CFSPID→CFSPID patients. CONCLUSIONS: A proportion of infants with CFSPID will be diagnosed with CF within the first 3 years. These findings underscore the need for clinical monitoring, repeat sweat testing at age 2 to 3 years, and extensive genotyping.

Background . Food insecurity, vitamin D deficiency and lower respiratory tract infections are highly prevalent conditions among Inuit children. However, the relationship between these conditions has not been examined in this population. Objective . The objective of this study was to examine the relationship between food insecurity and severe respiratory infections before age 2 years and health centre visits for a respiratory problem in the past year. We also explored the relationship between serum vitamin D status and respiratory outcomes in this population. Design . We included children aged 3–5 years who participated in a cross-sectional survey of the health of preschool Inuit children in Nunavut, Canada, from 2007 to 2008 (n=388). Parental reports of severe respiratory infections in the first 2 years of life and health care visits in the past 12 months were assessed through a questionnaire. Child and adult food security were assessed separately and serum 25-hydroxyvitamin D3 levels were measured in a subgroup of participants (n=279). Multivariate logistic regression was performed to assess the association between food security, vitamin D and each of the 2 respiratory outcomes. Results . Child and adult food insecurity measures were not significantly associated with adverse respiratory outcomes. Household crowding [odds ratio (OR)=1.51, 95% confidence interval (CI) 1.09–2.09, p=0.01 for the child food security model] and higher birth weight (OR=1.21, 95% CI: 1.02–1.43, p=0.03) were associated with reported severe chest infections before age 2 years while increasing age was associated with decreased odds of reported health care visits for a respiratory problem (OR=0.66, 95% CI: 0.48–0.91, p=0.02). Neither vitamin D insufficiency nor deficiency was associated with these respiratory outcomes. Conclusions . Using a large cross-sectional survey of Inuit children, we found that household crowding, but not food security or vitamin D levels, was associated with adverse respiratory outcomes. Further studies are warranted to examine the impact of decreasing household crowding on the respiratory health of these children. Keywords: food security; vitamin D; Inuit; bronchiolitis; lower respiratory tract infections; crowding (Published: 15 February 2016) Citation: Int J Circumpolar Health 2016, 75: 29954 - http://dx.doi.org/10.3402/ijch.v75.29954

OBJECTIVE:To assess the effectiveness of nurse-initiated administration of oral corticosteroids before physician assessment in moderate to severe acute asthma exacerbations in the pediatric ED.METHODS:A time-series controlled trial evaluated nurse initiation of treatment with steroids before physician assessment in children with Pediatric Respiratory Assessment Measure score ≥4. One-to-one periods (physician-initiated and nurse-initiated) were analyzed from September 2009 through May 2010. In both phases, triage nurses initiated bronchodilator therapy before physician assessment, per Pediatric Respiratory Assessment Measure score. We reviewed charts of 644 consecutive children aged 2 to 17 years for the following outcomes: admission rate; times to clinical improvement, steroid receipt, mild status, and discharge; and rate of return ED visit and subsequent admission.RESULTS:Nurse-initiated phase children improved earlier compared to physician-initiated phase (median difference: 24 minutes; 95% confidence interval [CI]: 1–50; P = .04). Admission was less likely if children received steroids at triage (odds ratio = 0.56; 95% CI: 0.36–0.87). Efficiency gains were made in time to steroid receipt (median difference: 44 minutes; 95% CI: 39–50; P < .001), time to mild status (median difference: 51 minutes; 95% CI: 17–84; P = .04), and time to discharge (median difference: 44 minutes; 95% CI: 17–68; P = .02). No differences were found in return visit rate or subsequent admission.CONCLUSIONS:Triage nurse initiation of oral corticosteroid before physician assessment was associated with reduced times to clinical improvement and discharge, and reduced admission rates in children presenting with moderate to severe acute asthma exacerbations.

Inuit children in Nunavut, Canada, have high rates of lower respiratory tract infection (LRTI) early in life. Whether this commonly results in chronic respiratory symptoms later in life is unknown.A cross-sectional survey of 3- to 5-years-old Inuit children was conducted in all three regions of Nunavut, as part of the "Qanuippitali, what about us, how are we?" survey.Reported chronic cough and wheezing were common in preschool Inuit children, although reported asthma diagnosed by a healthcare professional was uncommon. The presence of smokers in the home tended to be associated with severe LRTI in the first 2 years of life. Reported wheezing as well as reported bronchitis or pneumonia in the previous 12 months was significantly associated with severe LRTI in the first 2 years of life. Reported wheezing was also strongly associated with reported bronchitis or pneumonia in the past 12 months. The prevalence of chronic moist cough could not be clearly assessed, due to limitations in the questionnaire.Severe LRTI in the first 2 years of life was associated with ongoing respiratory morbidity in preschool Inuit children, although symptoms appeared to lessen in severity over time.

Inuit infants have high rates of reported hospitalization for respiratory infection, associated with overcrowding and reduced ventilation. We performed a randomized, double-blind, placebo controlled trial to determine whether home heat recovery ventilators (HRV) would improve ventilation and reduce the risk of respiratory illnesses in young Inuit children. Inuit children under 6 years of age living in several communities in Nunavut, Canada were randomized to receive an active or placebo HRV. We monitored respiratory symptoms, health center encounters, and indoor air quality for 6 months. HRVs were placed in 68 homes, and 51 houses could be analyzed. Subjects had a mean age of 26.8 months. Active HRVs brought indoor carbon dioxide con- centrations to within recommended concentrations. Relative humidity was also reduced. Use of HRV, compared with placebo, was associated with a progressive fall in the odds ratio for reported wheeze of 12.3% per week (95%CI 1.9-21.6%, P = 0.022). Rates of reported rhinitis were significantly lower in the HRV group than the placebo group in month 1 (odds ratio 0.20, 95%CI 0.058-0.69, P = 0.011) and in month 4 (odds ratio 0.24, 95%CI 0.054-0.90, P = 0.035). There were no significant reductions in the number of health center encounters, and there were no hospitalizations. Use of HRVs was associated with in improvement in air quality and reductions in reported respiratory symptoms in Inuit children.

The fractional concentration of exhaled nitric oxide (Feno) is a useful indicator of airway inflammation in children and adults with asthma.We determined the range of Feno concentrations and the factors affecting it in a large sample of healthy school children attending grades 4 through 6, in Windsor, ON, Canada.Feno was measured in 657 children between 9.1 and 12.9 years of age. The range of Feno concentrations in healthy school children was 12.7 parts per billion (ppb) [95% confidence interval (CI), 11.8 to 13.7 ppb] in whites and 22.8 ppb [95% CI, 17.9 to 27.7 ppb] in Asian-Canadian children (p0.001). Feno values also appeared to be higher in African-Canadian children than in whites, although the CI was wide because of the small number of African-Canadian children sampled. Feno rose slightly but significantly with age (p = 0.007) and with height (p = 0.023). Body mass index and gender did not significantly alter the measured Feno. FVC had a nonsignificant effect on Feno. Participation in physical activity during the same day had a borderline-significant effect on measured Feno, but a reported history of a respiratory tract infection in the preceding 2 weeks did not.Feno concentrations in healthy school-aged children appeared to be affected by race, and, to a lesser extent, by age and height. These factors should be taken into consideration when interpreting clinical results.

Background: Inuit infants have the highest reported rate of hospital admissions because of lower respiratory tract infections in the world. We evaluated the prevalence of reduced ventilation in houses in Nunavut, Canada, and whether this was associated with an increased risk of these infections among young Inuit children. Methods: We measured ventilation in 49 homes of Inuit children less than 5 years of age in Qikiqtaaluk (Baffin) Region, Nunavut. We identified the occurrence of lower respiratory tract infections using a standardized questionnaire. Associations between ventilation measures and lower respiratory tract infection were evaluated using multiple logistic regression models. Results: The mean number of occupants per house was 6.1 people. The mean ventilation rate per person was 5.6 L/s (standard deviation [SD] 3.7); 80% (37/46) of the houses had ventilation rates below the recommended rate of 7.5 L/s per person. The mean indoor carbon dioxide (CO 2 ) concentration of 1358 (SD 531) ppm was higher than the recommended target level of 1000 ppm. Smokers were present in 46 homes (94%). Of the 49 children, 27 (55%) had a reported history of lower respiratory tract infection. Reported respiratory infection was significantly associated with mean CO 2 levels (odds ratio [OR] 2.85 per 500-ppm increase in mean indoor CO 2 , 95% confidence interval [CI] 1.23–6.59) and occupancy (OR 1.81 for each additional occupant, 95% CI 1.14–2.86). Interpretation: Reduced ventilation and crowding may contribute to the observed excess of lower respiratory tract infection among young Inuit children. The benefits of measures to reduce indoor smoking and occupancy rates and to increase ventilation should be studied.

Infants with bronchopulmonary dysplasia (BPD) are at risk for numerous complications following discharge from the Neonatal Intensive Care Unit (NICU). Few studies have evaluated risk factors for adverse events (AE). This retrospective study provided an initial evaluation of the use of capillary carbon dioxide (PCO2) tension as a predictor of infants with BPD at increased risk for AE. PCO2 was compared in patients who suffered, or avoided, severe AE, defined as pulmonary hypertension, death, or subsequent reintubation or tracheostomy for respiratory illness. One hundred twelve consecutive patients followed at the BPD clinic were evaluated, and data from 104 subjects were suitable for analysis. Mean PCO2, obtained shortly before or after discharge from NICU, was 47.2 mmHg (range, 31-83). PCO2 was significantly higher in patients who required reintubation and ventilation (54.7 vs. 46.7, p < 0.04). No cutoff value of PCO2 clearly distinguished patients with subsequent AE. PCO2 was not significantly higher in the group of patients who had a severe AE than in the group of patients who did not have a severe AE, but logistic regression showed a significant association between PCO2 and risk of both severe AE (p = 0.018), and readmission to hospital (p = 0.038). An elevated PCO2 is associated with an increased risk of AE, including reintubation, and readmission to hospital, in infants with BPD. Patients with an elevated discharge PCO2 may require closer monitoring during followup. Prospective studies will be needed to confirm these observations.

These are commentaries on a Cochrane review, published in the issue of EBCH, first published as: Sauni R, Uitti J, Jauhiainen M, Kreiss K, Sigsgaard T, Verbeek JH. Remediating buildings damaged by dampness and mould for preventing or reducing respiratory tract symptoms, infections and asthma. Cochrane Database of Systematic Reviews 2011, Issue 9. Art. No.: CD007897. DOI: 10.1002/14651858.CD007897.pub2.

The aim of this study was to describe the changes in respiratory system compliance and other measures of respiratory mechanics associated with peritoneal insufflation (12 mmHg pressure) with carbon dioxide (PNP12) and 20° Trendelenburg positioning (TDG20) in pediatric patients undergoing laparoscopic surgery for abdominal cryptorchidism. Twelve subjects with abdominal cryptorchidism undergoing orchiopexy were enrolled in the study. General anesthesia was conducted with sevoflurane/O2/air, fentanyl, and rocuronium. Pressure-controlled ventilation with a peak inspiratory pressure (PIP) of 10-15 cm H2O and a positive end-expiratory pressure of 5 cm H2O was titrated to achieve a tidal volume (VT/kg) of 6-10 mL·kg−1 and end-tidal carbon dioxide (EtCO2) of 35-40 mmHg. Adjustments of PIP and respiratory rate (RR) were made to maintain the initial VT/kg and EtCO2

Background. Adrenal suppression secondary to high-dose inhaled corticosteroid use has been reported in children. Methods. The authors report the use of ciclesonide to reverse adrenal suppression secondary to inhaled fluticasone use in four pediatric patients. Results. In these four children, hypothalamic-pituitary-adrenal axis function normalized after the patients were changed to ciclesonide, while good asthma control was maintained. Conclusions. Ciclesonide should be considered for the reversal of adrenal suppression secondary to the use of fluticasone, and perhaps other older inhaled corticosteroids as well.

Differences in the course of pulmonary disease in cystic fibrosis (CF) may be altered by different treatment strategies in different CF centers. The Copenhagen clinic uses scheduled, regular and very aggressive treatment of lung infection. The Toronto clinic treats pulmonary infection with oral, inhaled, or intravenous antibiotics, and has emphasized aggressive nutritional therapy. This study compared the clinical status of CF patients treated in the two centers (Toronto, Canada, n=302, and Copenhagen, Denmark, n=214) using a cross-sectional design in terms of Pseudomonas aeruginosa (PA) and Burkholderia cepacia (BC) lung infections, pulmonary function, and levels of PA and BC precipitating antibodies (precipitins). Median ages were similar, but the age distribution was significantly different, with a higher proportion of patients under 10 and > or = 25 years in Toronto, and higher proportion of patients 11-24 years of age in Copenhagen. A higher number of female patients was observed in Copenhagen than in Toronto. Seventy-nine percent of Copenhagen patients, and 52% of Toronto patients were deltaF508 homozygous. Of all the patients, 20.1% of Copenhagen patients and 38% of Toronto patients were deltaF508 heterozygous. Ten percent of Toronto patients had two uncommon mutations. Pulmonary function and nutritional status in both groups were similar despite varying treatment strategies. The prevalence of PA was lower in Danish children and higher in Danish adults than in Canada. These differences are probably due to cohort isolation, which was introduced in Copenhagen in 1981. The prevalence of BC was higher in Toronto than in Copenhagen patients at all ages. In both centers, the number of PA and BC precipitins increased with age in patients chronically infected with PA and BC, respectively, and the number of both PA and BC precipitins rose with declining lung function. This study suggests that the clinic populations had similar pulmonary and nutritional statuses despite differing clinic antibiotic treatment strategies. Microbial colonization seemed to differ, at least in part, because of differences in cohort isolation strategies. Early, aggressive anti-pseudomonal chemotherapy may have reduced pseudomonal colonization among younger patients in Copenhagen. Future studies will be required to assess the impact of this on this cohort's outcome.

Asthma is a serious health problem for First Nations and Inuit children. In children younger than one year of age, asthma needs to be distinguished from viral bronchiolitis, which is unusually common in Canadian Aboriginal children. In children younger than six years of age, the diagnosis depends on the presence of typical symptoms, the absence of atypical features and the documentation of response to therapy - particularly a rapid, transient response to bronchodilators. In older children, the presence of reversible airway obstruction should be determined using spirometry whenever feasible to confirm the diagnosis. Environmental triggers should be evaluated and corrected whenever possible. Regular use of inhaled steroids is the most important measure for maintaining good asthma control in children with asthma. Clients and their families should receive asthma education. Control should be regularly reassessed at follow-up visits in health centres, with therapy adjusted to the lowest level capable of maintaining good control.L’asthme est un grave problème de santé pour les enfants inuits et des Premières nations. Chez les enfants de moins d’un an, il faut distinguer l’asthme de la bronchiolite virale, anormalement fréquente chez les enfants autochtones du Canada. Chez les enfants de moins de six ans, le diagnostic dépend de la présence de symptômes classiques, de l’absence de caractéristiques atypiques et de la consignation de la réponse au traitement, notamment la réponse rapide et transitoire aux bronchodilatateurs. Chez les enfants plus âgés, il faut, dans la mesure du possible, déterminer la présence d’une obstruction réversible des voies aériennes par spirométrie afin de confirmer le diagnostic ainsi qu’évaluer et corriger les déclencheurs environnementaux. L’utilisation régulière de corticoïdes en aérosol est la principale mesure à prendre pour maintenir un bon contrôle de l’asthme chez les enfants asthmatiques. Les clients et leur famille devraient recevoir une formation sur l’asthme. Il faut réévaluer régulièrement le contrôle aux visites de suivi dans des centres de santé et rajuster le traitement à la dose la plus basse possible pour le maintien de ce contrôle.

A nine-year-old girl presented to the emergency department in the fall of 2009 with a one-week history of cough, neck swelling, sore throat and mild diarrhea, but no fever or vomiting. She had a history of asthma, which was treated with montelukast. Her physical examination showed subcutaneous

Billy, who is 2 years old, presents to the physician’s office with his fourth episode of wheezing in the past 18 months. He was admitted to hospital at 6 months of age with bronchiolitis caused by respiratory syncytial virus. At 9 months, he was seen in the local emergency department with a cold

BACKGROUND: The fractional concentration of exhaled nitric oxide (FeNO) appears to be a good marker for airway inflammation in children with asthma.OBJECTIVE: To evaluate the effect of environmental exposures on exhaled nitric oxide in a community sample of children.METHODS: The relationship among exhaled nitric oxide, underlying disease and home environmental exposures was examined using questionnaire data and measurement of exhaled nitric oxide in a cross-sectional study of 1135 children that included healthy children, and children with allergies and/or asthma who were attending grades 4 through 6 in Windsor, Ontario.RESULTS: Among healthy children, there was a positive association between FeNO and occupancy (PCONCLUSIONS: The type of heating system, but not previously reported environmental tobacco smoke or mold exposure appears to affect exhaled nitric oxide in children. Exposure to different types of pets may have disparate effects on airway inflammation.

Objective To test the association between reported allergy and allergic diseases, respiratory symptoms, and the fractional concentration of exhaled nitric oxide (FeNO), in a community sample of school aged children. Methodology We administered a respiratory questionnaire and measured FeNO in a cross-sectional study of 1,135 children. Results FeNO was significantly greater in children with reported asthma (20.3 (standard deviation (SD) 21.3) parts per billion (ppb)) or allergies (18.1 (SD 18.0) ppb) than in healthy children (14.0 (SD 13.4) ppb). It was greater in children with asthma and reported allergies (22.8 (SD 23.6) ppb), than in children with asthma but no allergies (15.8 (SD 15.6) ppb) (overall P-value between disease groups = 0.002). FeNO was not related to respiratory symptoms in healthy children. Eczema was associated with an elevated FeNO concentration, even in the absence of respiratory symptoms. Some children with reported allergies but not asthma who had respiratory symptoms suggestive of asthma had elevated FeNO concentrations, and the proportion of healthy children with reported bronchitis or pneumonia in the past year who had an abnormally high FeNO concentration was significantly elevated. Conclusions In a community sample of children, FeNO concentrations appear to reflect allergic conditions, including allergic asthma, reported allergies, and eczema, rather than just asthma, particularly since asthma in children may be non-allergic. FeNO is similarly elevated in school aged children with reported asthma or reported allergies. FeNO is higher in children with asthma and allergies than in children with asthma alone. However, an elevated FeNO may help alert the clinician to the possibility of undiagnosed asthma. Pediatr. Pulmonol. 2008; 43:1198–1205. © 2008 Wiley-Liss, Inc.

Inuit infants have extremely high rates of lower respiratory tract infection (LRTI), but the causes for this are unclear. The aims of this study were to assess, in young Inuit children in Baffin Region, Nunavut, the feasibility of an epidemiologic study of the association between indoor air quality (IAQ) and respiratory health; to obtain data on IAQ in their housing; and to identify and classify risk factors for LRTI. Twenty houses in Cape Dorset, Nunavut with children below 2 years of age, were evaluated using a structured housing inspection and measurement of IAQ parameters, and a respiratory health questionnaire was administered. Twenty-five percent of the children had, at some time, been hospitalized for chest illness. Houses were very small, and had a median of six occupants per house. Forty-one percent of the houses had a calculated natural air change rate0.35 air changes per hour. NO(2) concentrations were within the acceptable range. Smokers were present in at least 90% of the households, and nicotine concentrations exceeded 1.5 microg/m(3) in 25% of the dwellings. Particulates were found to be correlated closely with nicotine but not with NO(2) concentrations, suggesting that their main source was cigarette smoking rather than leakage from furnaces. Mattress fungal levels were markedly increased, although building fungal concentrations were low. Dust-mites were virtually non-existent. Potential risk factors related to IAQ for viral LRTI in Inuit infants were observed in this study, including reduced air exchange and environmental tobacco smoke exposure. Severe lower respiratory tract infection is common in Inuit infants. We found reduced air change rates and high occupancy levels in houses in Cape Dorset, which may increase the risk of respiratory infections. This suggests the measures to promote better ventilation or more housing may be beneficial. Further health benefits may be obtained by reducing bed sharing by infants and greater turnover of mattresses, which were found to have high levels of fungi.

We describe a case of horseshoe lung in an infant with facio-auriculo-vertebral (FAV) sequence that included mild hemifacial microsomia, ear anomalies, a missing left rib, left hemivertebrae (T2-T4), and complex congenital heart disease. Of the approximately 40 cases of horseshoe lung described since 1962, most are reported in association with scimitar syndrome, and only four reported cases were associated with left lung hypoplasia. None of these cases included malformations consistent with a diagnosis of FAV sequence.

Background: Although guidelines for the diagnosis and management of asthma have been published over the last 15 years, there has been little focus on issues relating to asthma in childhood. Since the last revision of the 1999 Canadian asthma consensus report, important new studies, particularly in children, have highlighted the need to incorporate this new information into asthma guidelines. Objectives: To review the literature on asthma published between January 2000 and June 2003 and to evaluate the influence of new evidence on the recommendations made in the Canadian Asthma Consensus Report, 1999 and its 2001 update with a major focus on pediatric issues. Methods: Diagnosis of asthma in young children, prevention strategies, pharmacotherapy, inhalation devices, immunotherapy and asthma education were selected for review by small expert resource groups. In June 2003, the reviews were discussed at a meeting under the auspices of the Canadian Network For Asthma Care and the Canadian Thoracic Society. Data published up to December 2004 were subsequently reviewed by the individual expert resource groups. Results: This report evaluates early life prevention strategies and focuses on treatment of asthma in children. Emphasis is placed on the importance of an early diagnosis and prevention therapy, the benefits of additional therapy and the essential role of asthma education. Conclusion: We generally support previous recommendations and focus on new issues, particularly those relevant to children and their families. This guide for asthma management is based on the best available published data and the opinion of health care professionals including asthma experts and educators.

Congenital esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) are common congenital anomalies. Respiratory and GI complications occur frequently, and may persist lifelong. Late complications of EA/TEF include tracheomalacia, a recurrence of the TEF, esophageal stricture, and gastroesophageal reflux. These complications may lead to a brassy or honking-type cough, dysphagia, recurrent pneumonia, obstructive and restrictive ventilatory defects, and airway hyperreactivity. Aspiration should be excluded in children and adults with a history of EA/TEF who present with respiratory symptoms and/or recurrent lower respiratory infections, to prevent chronic pulmonary disease.

Background: Infections caused by herpes virus, in particular, Epstein-Barr virus (EBV), remain a major challenge in solid organ transplantation. Little is known about the significance of tissue EBV load. Methods: Twenty-three tissue biopsy specimens (19 kidney, 3 gastrointestinal, and 1 tonsil specimen) and 2 bronchoalveolar lavage specimens from 14 pediatric transplant recipients (10 kidney, 3 liver, 1 combined transplant) were subject to tissue EBV polymerase chain reaction (PCR) semiquantitative analysis and enzyme-linked immunosorbent assay (ELISA) methods. Results of biopsies were correlated with clinical data. Results: Five of 14 patients had clinically diagnosed EBV disease: 2 patients presented with a septic picture with multiorgan failure and pneumonitis; 1 patient had mononucleosis; 1 patient had an increase in serum creatinine level, lymphadenopathy, and chronic fatigue; and 1 patient had EBV nephritis. These 5 patients underwent 12 biopsies at the time of clinically active infection; 8 biopsies had positive results (up to 111 copies/10 μL of extracted DNA). Conversely, 1 of the remaining 13 tissue biopsy specimens from asymptomatic patients had positive results on ELISA, but undetectable viral load, whereas 8 patients had a positive EBV immunoglobulin G titer with historic evidence of EBV replication in the blood. No patient without evidence of EBV had positive EBV tissue PCR results. Conclusion: Increased EBV load was found in more than 50% of patients, pointing to a previously underrecognized importance of EBV detection in tissues from transplant recipients. The presence of EBV in tissue correlated with the presence of viremia, whereas tissue PCR had 100% specificity. EBV load should be included in biopsy evaluation. Am J Kidney Dis 41:212-219. © 2003 by the National Kidney Foundation, Inc.

The objective of the present document is to review the impact of new information on the recommendations made in the last (1999) Canadian Asthma Consensus Guidelines. It includes relevant published studies and observations or comments regarding what are considered to be the main issues in asthma management in children and adults in office, emergency department, hospital and clinical settings. Asthma is still insufficiently controlled in a large number of patients, and practice guidelines need to be integrated better with current care. This report re-emphasises the need for the following: objective measures of airflow obstruction to confirm the diagnosis of asthma suggested by the clinical evaluation; identification of contributing factors; and the establishment of a treatment plan to rapidly obtain and maintain optimal asthma control according to specific criteria. Recent publications support the essential role of asthma education and environmental control in asthma management. They further support the role of inhaled corticosteroids as the mainstay of anti-inflammatory therapy of asthma, and of both long acting beta2-agonists and leukotriene antagonists as effective means to improve asthma control when inhaled corticosteroids are insufficient. New developments, such as combination therapy, and recent major trials, such as the Children’s Asthma Management Project (CAMP) study, are discussed.

Plasma carnitine concentrations were measured in 43 children and adults with cystic fibrosis (CF), and values were compared with those from normal controls. Clinically significant abnormalities of plasma carnitine concentration were not found in CF patients. The concentration of free carnitine was slightly but significantly elevated in CF patients, and the acylcarnitine concentration and acylcarnitine/free-carnitine ratio were slightly but significantly lower. Total carnitine concentrations were similar to those of controls. The CF patients did not have abnormal urinary acylcarnitines. Altered concentrations of free and esterified carnitine were not associated with nutritional status or with liver or pulmonary function.

Twenty-eight children with high-risk acute lymphocytic leukemia underwent monthly serum lactate dehydrogenase (LDH) and LDH isoenzyme fraction determinations to examine whether LDH isoenzyme fractions change with an increase in the body burden of tumor cells. The 9 patients who relapsed and 5 patients who presented with leukemia during the study period had a slightly lower mean LDH-1 isoenzyme fraction. When the period from 3 months before to 3 months after relapse was examined, significant increases in the LDH-3 isoenzyme fraction and decreases in the LDH-1 and LDH-2 isoenzyme fractions were seen at the time of relapse. These results were highly significant when patients with non-T-cell and T-cell leukemia were combined and when bone marrow and central nervous system relapse was included. The changes at relapse appeared to revert with intensification of chemotherapy. The changes at relapse were not different in magnitude from random variation occurring in patients who remained in remission throughout the study. Although changes in LDH isoenzymes appeared to occur at the time of relapse compared with the periods immediately before and after relapse, these changes were not specific for relapse. LDH isoenzymes do not appear to be useful in predicting relapse in children with leukemia.

The relationship between passive exposure to cigarette smoking and objective measures of health was examined in 340 patients with cystic fibrosis attending a large hospital-based clinic. Patients who came from households with smokers did not differ from those living in smoke-free households in terms of nutritional status, clinical scores, spirometry, or colonization with Pseudomonas. The number of cigarettes smoked in the household was not significantly related to nutritional status, clinical score, spirometry, or hospitalization. Similar results were found when children 6 to 11 yr of age were analyzed separately, except that height percentile was negatively related to the number of cigarettes smoked in the household. The effects of household exposure to cigarette smoke were further evaluated by analyzing changes in nutritional status, clinical score, and spirometry over a 15-yr period among patients whose families never, always, stopped, or started smoking during this time. Height percentile increased slightly during this interval among those whose households never smoked, whereas no change occurred among patients whose households always smoked, and a decline was seen among patients whose households quit. These differences were statistically significant. Patients whose households never smoked had consistently higher pulmonary function measurements than did patients whose families always smoked, although the differences were not statistically significant. The rates of decline were similar in these two groups. Patients whose households stopped smoking had significantly lower pulmonary functions at the end of the study than did subjects whose households never smoked.(ABSTRACT TRUNCATED AT 250 WORDS)

Nebulized ipratropium bromide is thought to be synergistic with albuterol in therapy for acute childhood asthma. Because the efficacy of ipratropium in bronchiolitis is uncertain and some infants with bronchiolitis do not respond to nebulized albuterol alone, the following study was undertaken. In this double-blind, placebo-controlled trial, 69 infants between 6 weeks and 24 months of age who exhibited the first episode of acute bronchiolitis were randomly assigned to receive either nebulized albuterol (0.15 mg/kg per dose) and ipratropium bromide (250 µg per dose) (group A, n = 36) or nebulized albuterol and normal saline (placebo) (group B, n = 33) for two doses, 1 hour apart. The two groups were comparable at baseline. Both therapies resulted in clinically significant improvement. However, the addition of ipratropium resulted in no additional benefit with respect to decrease in the respiratory rate (mean ↓ 10.6/min vs ↓ 8.6/min, P = .86), accessory muscle score (range 0 through 3) (↓ 0.92 vs ↓ 0.82, z = –0.44), wheeze score (range 0 through 3) (↓, 0.94 vs 0.85, z –0.20), oxygen saturation (↑ 0.25% vs ↑ –0.33%, P = .86), or hospitalization rate (17 vs 10). The number of "nonresponders" and "clear responders" was also very similar in both groups. No toxicity was noted. The increase in heart rate was mild and similar in both groups (↑ 6.7 vs ↑ 11.1). The power of the study to detect a difference between the two treatment groups in the respiratory rate change ≥ 8/min is greater than 90%. It is concluded that the addition of nebulized ipratropium bromide appears to offer no further clinical benefit compared with albuterol alone in moderately severe acute bronchiolitis.

In general, angiotensin converting enzyme (ACE) inhibitors should be discontinued in pregnancy, as they can induce an ACE fetopathy. For the treatment of the latter, early peritoneal dialysis is recommended for in utero exposure to captopril and enalapril, although the outcome is poor. Early peritoneal dialysis has not previously been reported for lisinopril induced multiorgan failure. A case is reported in which treatment was given on postnatal day 3. The patient recovered from oligoanuria to almost normal renal function, and heart, brain, and musculoskeletal injury was reversible. This is despite relatively poor clearance of the drug through peritoneal dialysis. Analysis of the pharmacokinetic data suggests that haemodialysis or haemofiltration would be more efficacious for removal of the drug, and these treatments should be performed if available.

We present a child with Down's syndrome, bilateral lower lobe bronchiectasis, sinusitis, and severe ear disease who was found to have a novel ciliary defect, with a frequent, partial absence of the walls of the A subunits of some peripheral doublets. The defect caused the A subunits to be "U-shaped" rather than "O-shaped." A nuclear nasal mucociliary transport study confirmed that this defect was associated with abnormal mucociliary transport. The ciliary defect was not observed in a biopsy performed in a second patient who had Down's syndrome.