Chronic tracheostomy care of ventilator-dependent and -independent children: Clinical practice patterns of pediatric respirologists in a publicly funded (Canadian) healthcare system.

Objectives: To describe the current clinical practice patterns of Canadian pediatric respirologists at pediatric tertiary care institutions regarding chronic tracheostomy tube care and management of home invasive ventilation.

Methods: A pediatric respirologist/pediatrician with expertise in tracheostomy tube care and home ventilation was identified at each Canadian pediatric tertiary care center to complete a 59-item survey of multiple choice and short answer questions. Domains assessed included tracheostomy tube care, caregiver competency and home monitoring, speaking valves, medical management of tracheostomy complications, decannulation, and long-term follow-up.

In support of point-of-care social needs screening: The effects of five social determinants on the health of children with chronic diseases in British Columbia.

Background: Prior to introducing social needs screening into our subspecialty clinics, we first wanted to understand the health effects of the major social challenges facing children with chronic diseases in British Columbia.

Methods: Using a strict prospective methodology, avoiding use of health databases and proxy end points, we studied the effects of five social health determinants (distance from care, family income, gender, ethnicity, caregiver education), on health outcomes in three groups of children with chronic diseases: cystic fibrosis (CF), type 1 diabetes (T1D), chronic kidney disease (CKD). Social determinant data were collected at a face-to-face interview during a clinic visit.

A review of congenital lung malformations with a simplified classification system for clinical and research use.

Purpose: Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system.

The importance of considering monogenic causes of autoimmunity: A somatic mutation in KRAS causing pediatric Rosai-Dorfman syndrome and systemic lupus erythematosus.

Objectives: Clinicians need to be aware of the growing list of defined monogenic etiologies of autoimmune diseases. This is particularly relevant when evaluating children, as these rare monogenic forms of autoimmunity tend to present very early in life.

Methods And Results: By harnessing the transformative power of next generation sequencing, we made the unifying diagnosis of RAS-associated autoimmune leukoproliferative disease (RALD), caused by the somatic gain-of-function p.

The quality of life of home-ventilated children and their primary caregivers plus the associated social and economic burdens: a prospective study.

Background: Despite the obvious challenges faced by families caring for children on home ventilation, there is surprisingly little research into the details of their daily lives. In particular, little is known about the quality of life of the child and caregiver plus the associated social and economic burdens of care.

Methods: We prospectively studied 90 families enrolled in a paediatric home ventilation service in British Columbia.

Predictive Accuracy of Chest Radiographs in Diagnosing Tachypneic Children.

Objective: To test the predictive accuracy and reporting reproducibility of digital chest radiographs under low-resource conditions.

Methods: One hundred thirty four tachypneic children who presented to two Indian hospitals were enrolled. Based on review of 16 variables recorded in the Emergency Room (ER) by a senior pediatrician, children were given one of the four clinical diagnoses: pneumonia, wheezy disease, mixed and non-respiratory.

Can we distinguish pneumonia from wheezy diseases in tachypnoeic children under low-resource conditions? A prospective observational study in four Indian hospitals.

Background: Acute respiratory infections are the commonest cause of mortality and morbidity in children worldwide. A quarter of all deaths occur in India alone. In order to reduce this disease burden, there is a need for better diagnostic criteria, particularly ones allowing early detection of high-risk children.

Long-term ventilation in children: longitudinal trends and outcomes.

Background: Cross-sectional studies have suggested a rapid expansion in paediatric long-term ventilation (LTV) over the last 20 years but information on longitudinal trends is limited.

Methods: Data were collected prospectively on all patients receiving LTV over a 15-year period (1.1.

Bronchial balloon occlusion in children with complex pulmonary air leaks.

Pulmonary air leaks in children are most commonly due to infection or barotrauma. While cases of severe barotrauma are falling because of advances in neonatal care, the incidence of necrotising pneumonia is rising. The majority of air leaks can be managed conservatively, but more severe cases pose a significant challenge to the clinician.

Superior herniation of the mediastinum presenting as an anterior neck mass on straining.

We report an infant with an anterior neck mass present only on straining. Airway fluoroscopy revealed this to be due to superior mediastinal herniation. Neck masses apparent only during Valsalva maneuver are rare and awareness of the differential diagnosis is important to avoid unnecessary investigation or surgery.

Fifty years of pediatric asthma in developed countries: how reliable are the basic data sources?

Given the difficulties in diagnosing, or even defining, asthma in children, claims of a pediatric asthma epidemic in Canada and other developed countries are accepted with surprisingly little critical examination. We reviewed a broad range of data sources to understand how the epidemic evolved during the last 50 years and also to assess the reliability of the conclusions drawn from that data. We obtained Canadian National and Provincial data from Statistics Canada National Population Health Survey, and the British Columbia Ministry of Health respiratory database.

Predicting major adverse events after cardiac surgery in children.

Objectives: To develop a reliable predictor of major adverse events after pediatric cardiac surgery, with the aim of reducing mortality of cardiac extracorporeal life support through earlier, more accurate patient selection.

Design: Prospective observational study.

Setting: Tertiary level pediatric intensive care unit.

Wilson-Mikity syndrome: updated diagnostic criteria based on nine cases and a review of the literature.

Background: Nearly 50 years ago, Wilson and Mikity described a syndrome (WMS) of chronic lung disease (CLD) in premature infants, characterized by early development of cystic interstitial emphysema (PIE), despite minimal ventilatory support. The validity of the diagnosis is currently unclear; now considered either an anachronism, part of BPD spectrum or included within various poorly defined diagnoses such as chronic pulmonary insufficiency of prematurity (CPIP).

Objectives: To define clinically useful diagnostic criteria for WMS so its position in the spectrum of CLD of infancy can be established.

Life-threatening human metapneumovirus pneumonia requiring extracorporeal membrane oxygenation in a preterm infant.

We present the first report in the literature of a child with human metapneumovirus pneumonia who required extracorporeal membrane oxygenation for survival. This was a 3-month-old premature boy from British Columbia, Canada, who developed severe respiratory failure, experienced failure of high-frequency oscillatory mechanical ventilation, and required extracorporeal membrane oxygenation support for 10 days. This case illustrates the importance of including this newly discovered pathogen among the causes of childhood pneumonia.

Citrate anticoagulation in pediatric continuous venovenous hemofiltration.

Regional citrate anticoagulation has become a common alternative to systemic heparinization in adult continuous venovenous hemofiltration (CVVH) practice. We report our experience with the technique in critically ill children. We carried out a retrospective chart review of a 22-bed pediatric intensive care unit.