Following up on previous findings from the All Our Families (AOF) cohort, the current study investigated the relationship between birthing parent history of adverse childhood experiences (ACEs) and child atopy, including asthma, allergy, and eczema, at five years of age. Potential indirect effects were explored. Participants completed the ACEs scale, validated questionnaires of anxiety and depression symptoms, and reported on their and their children's atopic disease history. Archival analyses of AOF data (N = 3,387) was conducted using logistic regression and path analysis with counterfactually based indirect effects. Birthing parent history of ACEs was associated with an 18% increased risk of child allergy at five years (OR = 1.18, 95% CI: 1.09, 1.20). Exploratory path analyses indicated a significant indirect effect of ACEs through birthing parent history of atopy on child asthma, allergy, and eczema at five years. There were no significant indirect effects through birthing parent symptoms of anxiety or depression during pregnancy, at two or five years postpartum. Birthing parent history of ACEs, combined with birthing parent history of atopy, may elevate the risk of child atopy. This presents an opportunity for early intervention for children at risk of atopic disease.

RATIONALE AND OBJECTIVE: Bronchopulmonary dysplasia (BPD) is defined as the persistent need for oxygen at 36 weeks post menstrual age or 28 days of postnatal life in premature infants. Close to half of all premature infants with BPD are discharged from hospital with supplemental home oxygen and pediatric respirologists are commonly involved in the decision of discontinuing supplemental oxygen use. Our objective was to assess whether there is variability among Canadian pediatric respirologists on when to discontinue supplemental oxygen given the lack of evidence-based guidelines shaping this decision-making process. METHODS: Ethics approval was obtained and a cross sectional survey study was completed. Online and paper surveys included 4 oximetry test results and clinical vignettes provided in both English and French. Descriptive statistics were obtained for quantitative variables and data were analyzed for significance using the STATA statistical analysis software. RESULTS: The survey response rate was 27%. The decision to discontinue home oxygen did vary based on the province of current work; Alberta had the highest rate of respondents choosing to discontinue home oxygen (47.5%) in comparison to British Columbia and Quebec (18 and 20%, respectively; chi-square 3.97, p = 0.403). The years of experience of the physician was associated with a higher use of a guideline in making decisions on supplemental oxygen use (3.1 vs. 1.8, p = 0.167). CONCLUSION: This project allowed us to gather data on current decision making practices on home oxygen use among Canadian pediatric respirologists and informs that there is variability on care that patients receive across the country.

The Tobacco and Vaping Products Act (Canada, 1997) (the "TVPA") aims to protect the health of young persons by restricting access to vaping products. We studied whether the TVPA achieves this goal by sending young 'secret shoppers' to 120 shops in Calgary, Edmonton, and Red Deer to attempt to buy nicotine-based vaping-initiation products, and by asking minors to purchase the same product online. We used three 'improper' shop scenarios: 1. a minor or minors; 2. a young adult with no or invalid identification ("ID"); and 3. a young adult with valid ID but clearly buying for an accompanying minor. Of total vendors, 42.5% (51/120) were willing to sell to the young people (p <.001). Most vendors requested ID in all scenarios (97/120, 80.8%). Of these, 28 vendors (28.9% of those requesting ID), were still willing to sell the product. All vendors who did not request ID (23/120, 19.2%) were willing to sell; vape shops were more likely than convenience stores not to request ID (25.4% v. 13.1%). In five online purchase attempts, 60% of deliverers did not meet the TVPA's ID verification requirements. The TVPA does not require packages to reveal their contents; one parent inadvertently signed for the parcel. To prevent youth access, the TVPA should require: a minimum nicotine product purchase age of 21, positive obligations on vendors to request ID, prohibition of sales to adults buying for minors, and that manufacturers disclose the product on posted or delivered parcels. The TVPA should be strictly enforced.

Approximately 8% of all Canadian births are preterm and one of the most common pulmonary complications associated with prematurity is bronchopulmonary dysplasia (BPD) which is defined as the persistent need for oxygen at 36 weeks post menstrual age or 28 days of postnatal life. Close to half of all premature infants with BPD are discharged from hospital with home oxygen and Pediatric respirologists are commonly involved in the decision of discontinuing home oxygen use after the newborn period. Our aim was to assess whether there is variability among Canadian pediatric respirologists on when to discontinue supplemental oxygen and given the lack of evidence based guidelines we hypothesize that there will be variability in this decision making process. Gathering data on current practices in Canada can help fuel quality improvement projects to help improve standardization and clinical practice guidelines specific to the Canadian population of premature infants with BPD. A cross sectional survey study was completed and anonymous surveys were distributed in both English and French through the Cross Canada respiratory rounds list serve and the Canadian Respiratory conference 2018. Surveys included four oximetry test interpretations, 8 follow up questions on the decision making process and 4 questions on physician demographics. Ethics approval was obtained through the Research and Ethics Board prior to data collection. The survey response rate was 27%. There were responses from 6 provinces, 71% reported having more than 15 patients who required home oxygen use in their practice and 63% had practiced in the field for at least 5 years. The decision to discontinue home oxygen varied amongst Canadian Pediatric respirologists with 29% in favor of discontinuing home oxygen using the 4 provided oximetry tests to 71% against the decision. The decision to discontinue home oxygen did vary based on the province of current work; Alberta had a highest rate of respondents choosing to discontinue home oxygen (47.5%) in comparison to British Columbia and Quebec (18% and 20% respectively). Respirologists who completed their fellowship training in Canada were more likely to discontinue home oxygen compared to respondents who trained in the United States (31% vs 25%). The presence of pulmonary hypertension was rated as being the highest likelihood in affecting the decision to discontinue home oxygen (97%). Close to half of the respondents (42%) reported not being aware of any existing guidelines on supplemental oxygen use and amongst the respondents who did use a guideline, 72% reported using the British Thoracic Society guidelines for home oxygen use in children. This project allowed us to gather data on current practices on home oxygen use decision making amongst Canadian respirologists and informs that there is variability on care that patients receive across the country based on the experience and training of the pediatric respirologists.

A 16-year-old female with a complex medical history, including myelomeningocele and nephrolithiasis, presented with severe chest pain and dyspnea following nephrolithotomy and nephrostomy tube insertion. Chest X-ray and CT scan revealed a unilateral pleural effusion, while laboratory studies revealed elevated levels of creatinine in pleural fluid, thereby establishing the diagnosis of a urinothorax. This is an unusual cause of pleural effusion, caused by either an obstructive uropathy or direct injury to the pleural cavity. Treatment is largely symptomatic, with consideration of thoracentesis for large effusions. Urinothorax should be considered as a cause of pleural effusion in patients.

BACKGROUND:Asthma is the most common chronic condition in children. For many, the disease is inadequately controlled, which can burden the lives of children and their families as well as the health care system. Improved use of the best available scientific evidence by primary care practitioners could reduce the need for hospital care and improve quality of life and asthma control, thereby reducing overall costs to society and families.OBJECTIVE:The Primary Care Pathway for Childhood Asthma aims to improve the management of children with asthma by (1) providing primary care practitioners with an electronic guide (a clinical pathway) incorporated into the patient's electronic medical record, and (2) providing train-the-trainer education to chronic disease management health professionals to promote the provision of asthma education in primary care.METHODS:The research will utilize a pragmatic cluster-controlled design, quantitative and qualitative research methodologies, and economic evaluation to assess the implementation of a pathway and education intervention in primary care. The intervention will be analyzed for effectiveness, and if the results are positive, a strategy will be developed to implement delivery to all primary care practices in Alberta.RESULTS:The research has been successfully funded and ethics approvals have been obtained. Practice recruitment began fall 2015, and we expect all study-related activities to be concluded by March 2018.CONCLUSIONS:The proposed pathway and education intervention has the potential to improve pediatric asthma management in Alberta. The intervention is anticipated to result in better quality of care for equal or lesser cost.CLINICALTRIAL:ClinicalTrials.gov NCT02481037; https://clinicaltrials.gov/ct2/show/NCT02481037 (Archived by WebCite at http://www.webcitation.org/6fPIQ02Ma).

Background. We assessed vitamin D intakes and serum 25(OH) vitamin D levels in pediatric asthma patients on moderate-to-high dose inhaled steroids and compared them to published findings of healthy children in our city. Methods. Parents and/or patients were interviewed to estimate the children’s vitamin D intakes from foods and supplements (using an adapted validated food frequency questionnaire) and asthma duration and management. Vitamin D status: serum 25-hyroxy vitamin D (25(OH)D) was obtained from the medical records. Results. Vitamin D intakes from food and supplements of the asthma patients (n=20, 742 ± 185 IU/day) were significantly higher compared to healthy Canadian children (n=1442, 229 ± 121 IU/day). Despite higher vitamin D intakes, the children had nonsignificantly lower serum 25(OH) vitamin D levels compared to the comparison group. Serum 25(OH)D levels increased by 3.6 nmol/L with each 100 IU of vitamin D intake (95% Confidence interval = 2.0–4.0, R2 = 0.931, and p=0.001). Conclusion. Since adequate vitamin D status in asthma patients is necessary to support bone mineral accretion, it is important to achieve adequate vitamin D status by checking serum 25(OH)D status and supplement accordingly.

Children with asthma who do not respond to first-line therapy may need inhaled corticosteroid long-acting beta agonist combination (ICS-LABA) therapy. Adrenal insufficiency (AI) due to adrenal suppression is a recognized but relatively uncommon side effect of ICS. An increase in suspected cases of AI associated with 1 particular ICS-LABA, mometasone-formoterol (Zenhale) was observed at a tertiary care asthma clinic over a 6-month period.

The authors report a case involving a child with chronic respiratory symptoms, who did not respond to conventional treatment. Low serum immunoglobin levels and pathological findings on lung biopsy revealed an unusual diagnosis for his age group. A specific treatment led to clinical improvement.

The treatment of children with asthma has historically relied upon expert opinion using data extrapolated from adult studies. Over the past few years, landmark studies have been completed providing healthcare professionals with evidence on which a reasonable approach can be made for children suffering from this common and serious disease. Asthmatic phenotype in children, unlike adults, tends to differ according to age, which must be taken into account as well as triggers, severity, and level of control. The care of the child with asthma is complex, but accumulating data have demonstrated that we are on the right path for optimizing control while reducing the burden of side effects. The newest Global Initiative for Asthma (GINA) guidelines, as well as recent updates from the landmark CAMP (Childhood Asthma Management Program) study and information from the PACT (Pediatric Asthma Control Trial) and budesonide/formoterol controller and reliever studies, along with recent comparisons of higher dose inhaled corticosteroids (ICS), and ICS/long-acting β 2 -adrenoceptor agonist (LABA) combination and leukotriene receptor antagonist (LTRA) therapies in children have clarified a few of the big questions in pediatric asthma. For children with asthma aged 5 years and older, the CAMP trial demonstrated that regular use of ICS reduces the frequency of symptoms; however, height was adversely affected and there is no evidence for altering the natural history of asthma. In patients aged 6 years and over whose asthma is uncontrolled on ICS alone, combination therapy with ICS and a LABA has been recently compared with the use of higher dose ICS and the addition of an LTRA in pediatric patients. The addition of a LABA statistically will be of most benefit; however, some children will have optimal control with doubling the baseline dose of ICS or addition of an LTRA. Use of budesonide/formoterol as a controller and reliever therapy extends the time to first exacerbation versus contemporary use of this medication in patients aged 4 years and older. Ciclesonide, a newer ICS, has demonstrated acceptable efficacy but has the added benefit of not affecting growth. Certainly, with mounting evidence, the care-map in pediatric asthma control is becoming clearer.

Achalasia is characterized by obstruction of the distal esophagus and subsequent dilation of the proximal esophagus, and is considered to be a rare disorder in children. Patients commonly present with gastrointestinal (GI) symptoms such as dysphagia; however, pulmonary symptoms may also occur. Rare pulmonary symptoms due to achalasia are dyspnea and wheeze due to tracheal compression. The authors describe an 11-year-old boy who was referred to a pediatric respiratory clinic for asthma that was not responsive to inhaled medications. The child presented with a one-year history of dyspnea on exertion, cough and wheeze. He also complained of chronic dyspepsia. The presence of GI symptoms, in addition to abnormalities on chest radiograph and spirometry, suggested the presence of achalasia. The diagnosis was confirmed and the patient subsequently underwent surgical myotomy that relieved his GI and pulmonary symptoms, and normalized spirometry. The present article is an illustrative case report to remind pediatricians to consider other diagnoses when a patient does not respond to asthma medications.

Introduction Pulmonary toxicity of delayed onset is a rare complication of B-lymphocyte depleting antibody therapy and has been almost exclusively reported in older patients with B-cell malignancies. Aims To describe a pediatric patient with rituximab-associated lung injury (RALI), to systematically analyze previous reports of pulmonary complications, and to summarize common clinico-pathological features, treatment, and outcome. Results A teenage boy with focal segmental glomerulosclerosis (FSGS) presented with progressive dyspnea, fever, hypoxemia and fatigue 18 days after the completion of a second course of rituximab infusions for calcineurin inhibitor-dependent nephrotic syndrome. Respiratory symptoms started while he received high-dose prednisone for persistent proteinuria. Bilateral, diffuse ground-glass infiltrates corresponded to the presence of inflammatory cells in the bronchioalveolar lavage fluid. Empiric antibiotic treatment including clarithromycin was given, but the microbiological work-up remained negative. Serum IgE, C3, and C4 concentrations were normal. He recovered within 3 weeks after onset. We systematically reviewed 23 reports describing 30 additional cases of rituximab-associated lung disease. Twenty eight patients had received rituximab for B-cell malignancies, one for graft-versus-host disease and one for immune thrombocytopenia. Median age was 64 years (interquartile range [IQR] 58-69 years). Seventy one percent received concomitant chemotherapy Time to onset from the last rituximab dose was 14 days (IQR 11-22 days). Eleven of 31 patients required mechanical ventilation, and 9 died (29%). Ventilation was a significant predictor of fatal outcome (odds ratio 46.7; confidence interval 9.5-229.9). High dose glucocorticoid therapy did not improve survival or prevent severe lung disease or death. Conclusions With the expanding use of rituximab for novel indications, additional cases of RALI affecting younger age groups are expected to emerge. Mechanical ventilation predicts poor outcome. Glucocorticoids may not be protective. Pediatr Pulmonol. 2009;44:922-934. (C) 2009 Wiley-Liss, Inc.

Background. Fetal tracheal occlusion (TO) has been investigated as a treatment option for lung hypoplasia secondary to congenital diaphragmatic hernia (CDH). TO increases lung size, but it is unclear whether TO stimulates mature lung growth or simply induces alveolarization without concomitant bronchial development. In this study, we characterize bronchial branch development in fetal rats with CDH with or without TO through conventional histological and morphometric analysis as well as lung casting.Materials and methods. Rat dams were gavaged nitrofen at gestational day 9.5, and 3 to 4 fetuses per dam underwent fetal TO on gestational day 19 (term = 22 days). Fetuses were sacrificed on day 21, the presence of CDH was confirmed, and the lung weight to body weight ratio (LW/BW) was calculated. Lung casts of all research groups were created using liquid silicon and bronchial branches were quantified from lung periphery to carina.Results. CDH fetuses had smaller LW/BW ratios and a lesser percentage (%) of airspace when compared to controls, and manifested less lung branching than controls. Fetuses treated by TO had a greater LW/BW ratio and % airspace, but did not have a different number of branch iterations. Fetuses with CDH and TO demonstrated a restoration in LW/BW ratio to control levels (P = 0.42), but the number of bronchial branch iterations remained less than control animals.Conclusion. The results of this study suggest that TO in this animal model at gestational day 19 promotes distal airway proliferation but does not reverse the under-development of bronchial branching seen in lung hypoplasia due to CDH. (C) 2008 Elsevier Inc. All rights reserved.

Pleuropulmonary blastoma (PPB) is a rare and aggressive intrathoracic malignant tumor that can be associated with cystic lung lesions in children. This neoplasm is histologically characterized by primitive blastoma and a malignant mesenchymal stroma. The authors describe a 3-year-old boy who presented with a history of fever and cough. Radiological imaging demonstrated a large cystic lesion replacing the left lower lobe. The patient underwent thoracoscopic resection of the lesion. Interestingly, the histopathology demonstrated a type 1 PPB. Type 1 lesions are usually observed in young infants, whereas older infants and children tend to present with type 2 or 3 PPB, which carry a poorer prognosis and higher risk of recurrence. Thus, the presence of large or peripherally based lung cysts should raise the suspicion of PPB. Resection is warranted for all such lesions.

9:10 AM *Muscle Protein Synthesis Regulation in Neonates Pamela MJ O’Connor, MB, BCh, BAO, Agus Suryawan, PhD, Jill A. Bush, PhD Hanh V. Nguyen, BSc, Scot R. Kimball, PhD, and Teresa A. Davis, PhD. USDA/ARS Children’s Nutrition Research Center, Pediatrics, Neonatology, Baylor College of Medicine, Houston, TX; and Cellular and Molecular Physiology, Pennsylvania State University College of Medicine, Hershey, PA

We report on two brothers with short stature, severe developmental delay and unusual appearance. Several conditions including the Russell-Silver, Dubowitz, Floating-Harbour and Cockayne syndromes were considered in the differential diagnosis, but subsequently rejected. These two cases are likely to represent a new autosomal recessive or X-linked recessive syndrome.