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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. 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. We report the study design and some 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 (NEHI), 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%). This Registry is the largest longitudinal chILD cohort in the U.S. to date, providing a powerful framework for collaborating centers committed to improving the understanding and treatment of these rare disorders.

OBJECTIVE: Cardiopulmonary exercise testing (CPET) is used prior to bariatric surgery in adolescents with obesity to assess surgical risk factors. Lack of normative equations for peak oxygen consumption (pVO2) for this population limits CPET interpretation. We aimed to use lean body weight (LBW) to predict pVO and developed novel predictive equations for use in this population. METHODS: 446 participants with obesity ages 9-20 underwent CPET prior to bariatric surgery from January 1, 2006 to December 31, 2019. Bioelectrical impedance analysis helped calculate LBW. Achieving peak heart rate > 90% predicted and RER of >1.1 were considered satisfactory effort. RESULTS: 107 CPET studies (29%) were satisfactory. Mean weight was 127 ± 21 kg, and BF% 49.7 ± 6.8, and LBW 63 ± 10, kg. Mean pVO was 22.2 ± 3.2 mL/kg/min. The mean pVO using LBW was 44.8 ± 8.7 mL/kg/min. Total body weight (TBW) estimated pVO to be 50.7 ± 7.0 % predicted[(1)](#ref-0001), while LBW estimated it at 102.3 ± 17.6 % predicted. Linear regression yielded reference equations pVO =1571.6+12.2*TBW (males) and pVO2=1301.8+10.6*TBW (females). CONCLUSION: When pVO is corrected for LBW, adolescents with obesity demonstrate normal pVO . A novel set of equations were developed to predict absolute pVO using TBW.