The Accuracy of Readily Available Consumer-Grade Oxygen Saturation Monitors in Pediatric Patients.

BACKGROUND: Pulse oximetry measurement is ubiquitous in acute health care settings in high-income countries and is familiar to any parent whose child has been treated in such a setting. Oximeters for home use are readily available online and are incorporated in several smartphones and smartwatches. METHODS: We wished to determine how accurate are oximeters available online that are designated for adult and pediatric use, and the saturation monitor integrated in a smartphone, when used in children, compared to reference, hospital-grade oximeters. We evaluated a fingertip oximeter marketed for children purchased online; an adult fingertip oximeter purchased online; the oximeter integrated in a smartphone; and reference, hospital-grade oximeters. Participants were < 18 y of age. Bland-Altman charts were generated, and the estimated root mean square error (EARMS) was calculated. Rates of failure to obtain a measurement, relationship between device and time to successful measurement, relationship between age and time to successful measurement, and relationship between error (vs the reference device) and age were evaluated for each consumer-grade device. RESULTS: We measured SpO2 in 74 children between 0.1-17.0 y of age. Subjects weighing < 30 kg had a median (interquartile range [IQR]) age of 2 (1.0 month-1.4 y) months, and subjects weighing ≥ 30 kg had a median (IQR) age of 14.3 (11.9-16.2) y. Readings could not be obtained in 7.5, 0, and 38.8% of subjects using the pediatric, adult, and smartphone oximeters, respectively. The time to successful reading had a modest negative correlation with age with the inexpensive adult and pediatric oximeters. The inexpensive pediatric oximeter had an overall negative bias, with a mean difference from the reference device of -4.5% (SD 7.9%) and an error that ranged from > 8% to < 33% the reference device. The EARMS was 7.92%. The inexpensive adult oximeter demonstrated no obvious trend in error in the limited saturation range evaluated of 87-99%. The overall mean difference was -0.7% (SD 2.5%). EARMS was 2.5%. The smartphone oximeter underestimated SpO2 at saturations < 94% and overestimated SpO2 for saturations > 94%. Saturations could read as much as > 4%, or < 17%, than the reference oximeter. The mean difference was -2.9% (SD 5.2%). EARMS was 5.1%. CONCLUSIONS: Our findings suggest that the performance of consumer-grade devices varies considerably by both subject age and device. The pediatric fingertip device and smartphone application we tested are poorly suited for use in infants. The adult fingertip device we tested performed quite well in larger children with relatively normal oxygen saturations, and the pediatric fingertip device performed moderately well in subjects > 1 y of age who weighed < 30 kg. Given the vast number of devices available online and ever-changing technology, research to evaluate nonclinical oximeters will continue to be required.

Indoor air quality in remote first nations communities in Ontario, Canada.

A recent study of the health of Indigenous children in four First Nations Communities in remote northwestern Ontario found that 21% of children had been admitted to hospital for respiratory infections before age 2 years. Here we report a detailed analysis of the housing conditions in these communities. We employed a variety of statistical methods, including linear regression, mixed models, and logistic regression, to assess the correlations between housing conditions and loadings of biocontaminants (dust mite allergens, fungal glucan, and endotoxin) and indoor concentrations of PM2.5, CO2, benzene, and formaldehyde. The houses (n = 101) were crowded with an average of approximately 7 people. Approximately 27% of the homes had sustained CO2 concentrations above 1500 ppm. Most homes had more than one smoker. Commercial tobacco smoking and the use of non-electric heating (e.g., wood, oil) were associated with increased fine particle concentrations. Over 90% of the homes lacked working Heat Recovery Ventilators (HRVs), which was associated with increased fine particle concentrations and higher CO2. Of the 101 homes, 12 had mold damage sufficient to increase the relative risk of respiratory disease. This resulted from roof leaks, through walls or around the windows due to construction defects or lack of maintenance. A similar percentage had mold resulting from condensation on windows. Endotoxin loadings were much higher than any previous study in Canada. This work provides evidence for the need for more effort to repair existing houses and to ensure the HRVs are properly installed and maintained.

The International Network on Oesophageal Atresia (INoEA) consensus guidelines on the transition of patients with oesophageal atresia-tracheoesophageal fistula.

Oesophageal atresia-tracheoesophageal fistula (EA-TEF) is a common congenital digestive disease. Patients with EA-TEF face gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life issues in childhood, adolescence and adulthood. Although consensus guidelines exist for the management of gastrointestinal, nutritional, surgical and respiratory problems in childhood, a systematic approach to the care of these patients in adolescence, during transition to adulthood and in adulthood is currently lacking. The Transition Working Group of the International Network on Oesophageal Atresia (INoEA) was charged with the task of developing uniform evidence-based guidelines for the management of complications through the transition from adolescence into adulthood. Forty-two questions addressing the diagnosis, treatment and prognosis of gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life complications that patients with EA-TEF face during adolescence and after the transition to adulthood were formulated. A systematic literature search was performed based on which recommendations were made. All recommendations were discussed and finalized during consensus meetings, and the group members voted on each recommendation. Expert opinion was used when no randomized controlled trials were available to support the recommendation. The list of the 42 statements, all based on expert opinion, was voted on and agreed upon.

Persistent chylothorax associated with lymphatic malformation type 6 due to biallelic pathogenic variants in PIEZO1.

PIEZO1 is required for lymphatic valve formation, and several lymphatic abnormalities have been reported to be associated with autosomal recessive PIEZO1 pathogenic variants including neonatal hydrops, lymphedema involving various body regions, and chylothorax. Persistent or recurrent chylothorax has been infrequently described in association with pathogenic variants in the PIEZO1 gene. We present a 4-year-old female with bilateral pleural effusions detected prenatally, who was diagnosed with bilateral chylothoraces post-partum. She subsequently had recurrent pleural effusions involving both pleural cavities, which tended to improve with restriction of her fat intake, and, one occasion, subcutaneous octreotide. She also had bilateral calf, and intermittent cheek swelling. Genetic testing revealed two deleterious variants in PIEZO1: c.2330-2_2330-1del and c.3860G > A (p.Trp1287*), both of which were classified as likely pathogenic. This supported a diagnosis of Lymphatic Malformation Type 6 (OMIM 616843), also known as Hereditary Lymphedema Type III. Hereditary Lymphedema type III can be associated with persistent chylothorax that can vary in size over time.

First reports of primary ciliary dyskinesia caused by a shared DNAH11 allele in Canadian Inuit.

BACKGROUND: Primary ciliary dyskinesia (PCD) is typically an autosomal recessive disease characterized by recurrent infections of the lower respiratory tract, frequent and severe otitis media, chronic rhinosinusitis, neonatal respiratory distress, and organ laterality defects. While severe lower respiratory tract infections and bronchiectasis are common in Inuit, PCD has not been recognized in this population. METHODS: We report a case series of seven Inuit patients with PCD identified by genetic testing in three Canadian PCD centers. RESULTS: Patients ranged from 4 to 59 years of age (at time of last evaluation) and originated in the Qikiqtaaluk region (Baffin Island, n = 5), Nunavut, or Nunavik (northern Quebec, n = 2), Canada. They had typical features of PCD, including neonatal respiratory distress (five patients), situs inversus totalis (four patients), bronchiectasis (four patients), chronic atelectasis (six patients), and chronic otitis media (six patients). Most had chronic rhinitis. Genetic evaluation demonstrated that all had homozygous pathogenic variants in DNAH11 at NM_001277115.1:c.4095+2C>A. CONCLUSIONS: The discovery of this homozygous DNAH11 variant in widely disparate parts of the Nunangat (Inuit homelands) suggests this is a founder mutation that may be widespread in Inuit. Thus, PCD may be an important cause of chronic lung, sinus, and middle ear disease in this population. Inuit with chronic lung disease, including bronchiectasis or laterality defects, should undergo genetic testing for PCD. Consideration of including PCD genetic analysis in routine newborn screening should be considered in Inuit regions.

Skin morbidity in Indigenous children in relation to housing conditions in remote communities in Northwestern Ontario, Canada.

BACKGROUND: Elevated rates of eczema and skin infections in Canadian First Nation (FN) communities are of concern to families, community leaders and healthcare professionals. AIM: To determine whether skin morbidity was associated with indoor environmental quality factors in Canadian FN children living in remote communities. METHODS: We quantified indoor environmental quality (IEQ) in the homes of FN children aged < 4 years of age living in four remote communities in the Sioux Lookout region of Northwestern Ontario, Canada. We conducted a quantitative housing inspection, including measuring surface area of mould (SAM), and monitored air quality for 5 days in each home, including carbon dioxide and relative humidity and quantified endotoxin in settled floor dust. We reviewed the medical charts of participating children for skin conditions and administered a health questionnaire. Relationships between IEQ and skin infections or eczema were evaluated using multivariable regression. RESULTS: In total, 98 children were included in the descriptive analyses, of whom 86 had complete data and were evaluated in multivariate analyses for dermatological outcomes (mean age 1.6 years). Of these 86 children, 55% had made ≥ 1 visits to the local health centre (HC) for skin and soft tissue infections and 25.5% for eczema. Unexpectedly, annualized eczema visits were inversely associated with SAM (RR = 0.14; 95% CI 0.01-0.93). There was a trend suggesting an inverse relationship between endotoxin and HC encounters for eczema and skin and soft tissue infections. CONCLUSION: Skin infections were common in this population of FN children. IEQ did not appear to be associated with skin infections or eczema. Mould exposure appeared to be inversely associated with HC encounters for eczema, possibly related to complex microorganism-host interactions occurring early in life.

Housing conditions and respiratory morbidity in Indigenous children in remote communities in Northwestern Ontario, Canada.

BACKGROUND: Rates of lower respiratory tract infection (LRTI) among First Nations (FN) children living in Canada are elevated. We aimed to quantify indoor environmental quality (IEQ) in the homes of FN children in isolated communities and evaluate any associations with respiratory morbidity. METHODS: We performed a cross-sectional evaluation of 98 FN children (81 with complete data) aged 3 years or younger, living in 4 FN communities in the Sioux Lookout region of Northern Ontario. We performed medical chart reviews and administered questionnaires. We performed a housing inspection, including quantifying the interior surface area of mould (SAM). We monitored air quality for 5 days in each home and quantified the contaminant loading of settled floor dust, including endotoxin. We analyzed associations between IEQ variables and respiratory conditions using univariable and multivariable analyses. RESULTS: Participants had a mean age of 1.6 years and 21% had been admitted to hospital for respiratory infections before age 2 years. Houses were generally crowded (mean occupancy 6.6 [standard deviation 2.6, range 3-17] people per house). Serious housing concerns were frequent, including a lack of functioning controlled ventilation. The mean SAM in the occupied space was 0.2 m2. In multivariable modelling, there was evidence of an association of LRTI with log endotoxin (p = 0.07) and age (p = 0.02), and for upper respiratory tract infections, with SAM (p = 0.07) and age (p = 0.03). Wheeze with colds was associated with log endotoxin (p = 0.03) and age (p = 0.04). INTERPRETATION: We observed poor housing conditions and an association between endotoxin and wheezing in young FN children living in Northern Ontario.

Care recommendations for the respiratory complications of esophageal atresia-tracheoesophageal fistula.

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.

Variation in lung function and nutritional decline in cystic fibrosis by genotype: An analysis of the Canadian cystic fibrosis registry.

BACKGROUND: This study aimed to improve our understanding of the natural history of cystic fibrosis (CF) by comparing lung function and body mass index z-score (zBMI) between patients with different genotypes and identify a genotype with outcomes most comparable to homozygous ΔF508 patients. METHODS: Data was obtained from the Canadian CF Registry between January 1st 2007-January 1st 2016. Patients were categorized into one of five groups based on their genotype. A mixed-effects model was conducted with adjustments for age, sex, age of diagnosis, and baseline clinical measures. RESULTS: Among 2612 patients, those with non-mild forms of CF, and particularly adult patients with the same functional allele classification were found to have a lung function trajectory comparable to individuals with the homozygous ∆F508 genotype (annual change in percent predicted forced expiratory volume in 1 s of -0.83, 95% CI: -0.93, -0.73). The rates of zBMI change over the study period were not significantly different between the genotype groups. CONCLUSION: This population-based study of Canadian CF patients provides adjusted rates of lung function decline and zBMI over ten years. The finding that there are different genotypes with comparable rates of lung function decline to patients of the homozygous ∆F508 group supports the use of multiple comparison groups to assess the long-term efficacy of emerging CF therapies.

Oral aspiration, type 1 laryngeal cleft, and respiratory tract infections in canadian inuit children.

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.

Vocal cord paralysis appears to be an acquired lesion in children with repaired esophageal atresia/tracheoesophageal fistula.

OBJECTIVES: Determine whether vocal cord paresis or paralysis (VCP/P) following surgical repair of congenital esophageal atresia/tracheoesophageal fistula (EA/TEF) is generally a primary anomaly, or is secondary to EA/TEF repair. METHODS: We carried out a retrospective study based on a recently published protocol, which included the systematic performance of a laryngo-tracheo-bronchoscopy before and after EA/TEF repair. RESULTS: There were 105 patients with EA/TEF referred for multidisciplinary follow-up, between 2010 and 2015. Sixty-four of the 105 patients included in the study underwent EA/TEF repair at the referral center and had pre-operative bronchoscopy; the others had their initial surgery elsewhere. No included patient had VCP/P detected pre-operatively. Six patients (4 initially managed at the referral center) were diagnosed with VCP/P during the follow-up period (6/105, 5.7%). CONCLUSION: VCP appears to be an acquired lesion in this population.

Impact of home remediation and household education on indoor air quality, respiratory visits and symptoms in Alaska Native children.

Alaska Native children experience high rates of lower respiratory tract infections (LRTIs) and lung conditions, which are associated with substandard indoor air quality (IAQ). We conducted an intervention of home remediation and education to assess the impact on IAQ, respiratory symptoms and LRTI visits. We enrolled households of children 1-12 years of age with lung conditions. Home remediation included improving ventilation and replacing leaky woodstoves. We provided education about IAQ and respiratory health. We monitored indoor airborne particles (PM2.5), CO2, relative humidity and volatile organic compounds (VOCs), and interviewed caregivers about children's symptoms before, and for 1 year after intervention. We evaluated the association between children's respiratory visits, symptoms and IAQ indicators using multiple logistic regression. A total of 60 of 63 homes completed the study. VOCs decreased (coefficient = -0.20; p < 0.001); however, PM2.5 (coeff. = -0.010; p = 0.89) did not decrease. Burning wood for heat, VOCs and PM2.5 were associated with respiratory symptoms. After remediation, parents reported decreases in runny nose, cough between colds, wet cough, wheezing with colds, wheezing between colds and school absences. Children had an age-adjusted decrease in LRTI visits (coefficient = -0.33; p = 0.028). Home remediation and education reduced respiratory symptoms, LRTI visits and school absenteeism in children with lung conditions.

Aspiration Risk and Respiratory Complications in Patients with Esophageal Atresia.

Chronic, long-term respiratory morbidity (CRM) is common in patients with a history of repaired congenital esophageal atresia, typically associated with tracheoesophageal fistula (EA/TEF). EA/TEF patients are at high risk of having aspiration, and retrospective studies have associated CRM with both recurrent aspiration and atopy. However, studies evaluating the association between CRM in this population and either aspiration or atopy have reported conflicting results. Furthermore, CRM in this population may be due to other related conditions as well, such as tracheomalacia and/or recurrent infections. Aspiration is difficult to confirm, short of lung biopsy. Moreover, even within the largest evidence base assessing the association between CRM and aspiration, which has evaluated the potential relationship between gastroesophageal reflux and asthma, findings are contradictory. Studies attempting to relate CRM to prior aspiration events may inadequately estimate the frequency and severity of previous aspiration episodes. There is convincing evidence documenting that chronic, massive aspiration in patients with repaired EA/TEF is associated with the development of bronchiectasis. While chronic aspiration is likely associated with other CRM in patients with repaired EA/TEF, this does not appear to have been confirmed by the data currently available. Prospective studies that systematically evaluate aspiration risk and allergic disease in patients with repaired EA/TEF and document subsequent CRM will be needed to clarify the causes of CRM in this population. Given the prevalence of CRM, patients with repaired EA/TEF should ideally receive regular follow-up by multidisciplinary teams with expertise in this condition, throughout both childhood and adulthood.

Association Between Chronic Aspiration and Chronic Airway Infection with Pseudomonas aeruginosa and Other Gram-Negative Bacteria in Children with Cerebral Palsy.

PURPOSE: Children with cerebral palsy (CP) are at an increased risk for aspiration, and subsequent pneumonia or pneumonitis. Pneumonia is a common cause of hospital admission, intensive care unit (ICU) admission, and death in patients with CP, and may disproportionately contribute to mortality. The role of respiratory microflora is unknown. This study examined the relationship between respiratory infections with Gram-negative bacteria (GNB), particularly Pseudomonas aeruginosa, and the frequency/severity of pneumonia hospitalization. METHODS: Retrospective chart review of 69 patients with CP and hospitalization for pneumonia. Eligible patients required hospitalization for bacterial pneumonia, at least one respiratory culture, and fulfillment of Bax definition of CP. Group assignment was based on respiratory culture. Charts were analyzed for comorbid illness, hospitalization demographics, and disease severity. RESULTS: Children with isolation of P. aeruginosa or other GNB had increased frequency of ICU admission (77.4, 65.1, vs. 26.9 %, respectively, p < 0.01), intubation (45.2, 39.5 vs. 11.5 %, p = 0.02, p = 0.03 respectively), and large pleural effusions (37.5, vs. 0 %) than children without GNB. Children with isolation of GNB had more prolonged hospitalizations and were more likely to have multiple hospitalizations than those without GNB. CONCLUSION: Colonization with P. aeruginosa and other Gram-negative organisms in children with CP is associated with increased morbidity, prolonged hospitalization, and severity of pneumonia including need for PICU admission and intervention. Further research is required to determine causality, the role of antimicrobials active against Gram negative in pneumonia treatment, and the role of GNB eradication therapy in children with CP.

The impact of pneumoperitoneum and Trendelenburg positioning on respiratory system mechanics during laparoscopic pelvic surgery in children: a prospective observational study.

PURPOSE: 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. METHODS: 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 < 50 mmHg. Measurements of weight-corrected dynamic compliance (Cdyn/kg), VT/kg, and EtCO2 were recorded at baseline, after PNP12, at TDG20, and again after deflation and return to the level position. RESULTS: Adjustments in PIP were required in all subjects to maintain the target VT/kg. The Cdyn/kg decreased 42% (95% confidence interval [CI]: 30 to 51; P < 0.001) after PNP12, and it remained below baseline until deflation. The TDG20 caused only minimal additional reductions in Cdyn/kg (10% decrease; 95% CI: 0 to 19; P = 0.048). The VT/kg decreased 42% (95% CI: 31 to 52; P = 0.048) with PNP12, and after TDG20, it decreased a further 10% (95% CI: 4 to 19; P = 0.038). After deflation, the VT/kg increased by 56% (95% CI: 28 to 90; P = 0.001) and was then adjusted back to the initial values. CONCLUSION: The PNP12 significantly decreases Cdyn/kg and VT/kg in pediatric patients. The use of TDG20 produces a relatively minor additional impact in respiratory mechanics. This study emphasizes the need to adjust ventilator settings to maintain normal gas exchange during this procedure.