The US national registry for childhood interstitial and diffuse lung disease: Report of study design and initial enrollment cohort.

INTRODUCTION: Childhood interstitial and diffuse lung disease (chILD) encompasses a broad spectrum of rare disorders. The Children's Interstitial and Diffuse Lung Disease Research Network (chILDRN) established a prospective registry to advance knowledge regarding etiology, phenotype, natural history, and management of these disorders. METHODS: This longitudinal, observational, multicenter registry utilizes single-IRB reliance agreements, with participation from 25 chILDRN centers across the U.S. Clinical data are collected and managed using the Research Electronic Data Capture (REDCap) electronic data platform. RESULTS: We report the study design and selected elements of the initial Registry enrollment cohort, which includes 683 subjects with a broad range of chILD diagnoses. The most common diagnosis reported was neuroendocrine cell hyperplasia of infancy, with 155 (23%) subjects. Components of underlying disease biology were identified by enrolling sites, with cohorts of interstitial fibrosis, immune dysregulation, and airway disease being most commonly reported. Prominent morbidities affecting enrolled children included home supplemental oxygen use (63%) and failure to thrive (46%). CONCLUSION: This Registry is the largest longitudinal chILD cohort in the United States to date, providing a powerful framework for collaborating centers committed to improving the understanding and treatment of these rare disorders.

Asthma Screening in Pediatric Sickle Cell Disease: A Clinic-Based Program Using Questionnaires and Spirometry.

A clinician diagnosis of asthma is associated with increased morbidity and mortality in people with sickle cell disease (SCD). We hypothesized that a screening program would help identify children with asthma needing referral to pulmonary clinic. We conducted a single-center project to screen patients with SCD for asthma using a previously validated questionnaire (Breathmobile) and for pulmonary function abnormalities with portable spirometry. Participants with a positive questionnaire and/or abnormal spirometry were referred to pediatric pulmonary clinic. We evaluated clinical associations with abnormal spirometry and questionnaire responses. Of the 157 participants, 58 (37%) had a positive asthma screening questionnaire. Interpretable spirometry was available for 105 (83% of those eligible) and of these, 35 (34%) had abnormal results. The asthma questionnaire was 87.5% sensitive [95% confidence interval (CI) 74.8-95.3] and 85.3% specific (95% CI 77.3-91.4) to detect a clinician diagnosis of asthma. Participants with positive questionnaires were older (mean age 12.2 vs. 9.9 years, P = 0.012). Spirometry identified 16 additional participants who had normal asthma questionnaires. Seventy-four participants (47%) were referred to pediatric pulmonary clinic and 25 (34%) of these participants scheduled clinic appointments; however, only 13 (52%) were evaluated in pulmonary clinic. Clinic-based asthma screening and spirometry frequently identified individuals with asthma and pulmonary function abnormalities. Only 22% of those referred were eventually seen in pulmonary clinic. The impact of improved screening and treatment on the pulmonary morbidity in SCD needs to be defined and is an area for future investigation. In addition, case management or multidisciplinary clinics may enhance future screening programs.

Reducing Inpatient Length of Stay Using a Multicollaborative Protocol for Management of Non-Intensive Care Unit Asthmatics.

Maximizing clinical efficiency through the reduction in inpatient length of stay (LOS) using standardized protocols has been a major objective among hospital administrators, most notably in the context of recent healthcare reimbursement changes at statewide levels. The objective of our project was to determine whether a synchronous change in an inpatient asthma protocol that relied on a respiratory therapist (RT)-driven bronchodilator weaning algorithm and bronchodilator therapy given through a metered dose inhaler (MDI) plus valved holding chamber (VHC) could impact clinical and financial outcomes. A pre-post study assessed patients aged 2-21 years of age admitted with a primary diagnosis of status asthmaticus. The effect of the protocol was measured from October 2014 to July 2015. Outcome variables included patient demographics, hospital LOS, all-patient refined diagnosis-related groups (APR-DRGs), and inpatient charges. Outcomes were compared between the preimplementation and postimplementation time periods. Statistical significance was measured using Wilcoxon signed-rank test and bootstrap logistic regression models. Protocol patients (n = 110) had a similar demographic and clinical profile compared with the matched population from the previous nonprotocol fiscal year (n = 150). Use of the protocol resulted in a significantly reduced LOS that maintained significance after adjusting for APR-DRGs weight (P < 0.05). The protocol did not alter the total hospital billing charges. A nonstatistically significant reduction in 30-day readmission rates was observed among those administered the protocol. An RT-led weaning protocol using a quantitative scoring system and MDI+VHC for bronchodilator administration resulted in a significantly reduced LOS.

Respiratory Care of Infants and Children with Congenital Tracheo-Oesophageal Fistula and Oesophageal Atresia.

Despite acute respiratory and chronic respiratory and gastro-intestinal complications, most infants and children with a history of oesophageal atresia / trachea-oesophageal fistula [OA/TOF] can expect to live a fairly normal life. Close multidisciplinary medical and surgical follow-up can identify important co-morbidities whose treatment can improve symptoms and optimize pulmonary and nutritional outcomes. This article will discuss the aetiology, classification, diagnosis and treatment of congenital TOF, with an emphasis on post-surgical respiratory management, recognition of early and late onset complications, and long-term clinical outcomes.