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.

Wheezing is a common outcome of preterm birth. This article will review the mechanisms, epidemiology, and treatment of wheezing in preterm children with and without a history of bronchopulmonary dysplasia

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but serious, sequala of acute pulmonary embolism. Symptoms can be subtle and non-specific and the prognosis is poor if severe pulmonary hypertension (PH) and right ventricular dysfunction are present. While PH-targeted therapies are often used, there is only one FDA approved therapy, and only for disease that is deemed inoperable. The greatest chance for potential cure and long-term survival is surgical pulmonary endarterectomy. We report a 6-year-old male with a history of asthma and two unprovoked deep venous thromboses who presented with syncope. Chest x-ray showed cardiomegaly and an echocardiogram showed severe PH with severely decreased right ventricular (RV) function. Ventilation-perfusion scan showed mismatched perfusion defects involving the right lower lobe, and CT of the chest showed right lower lobe subsegmental pulmonary thrombus, suspicious for chronic thromboembolism. Given his clinical presentation and the severity of his right ventricular dysfunction, he was started on ambrisentan, sildenafil and subcutaneous treprostinil. Bilateral pulmonary endarterectomy was performed with resection of level 2 to 3 disease and he was successfully weaned off all PH therapy. Four months post-op, he is clinically asymptomatic and his echo shows normal RV function without PH. In conclusion, CTEPH is a rare but likely underdiagnosed disease process in pediatrics. Clinicians should have a high index of suspicion for at risk patients with unexplained dyspnea. Even if right ventricular dysfunction and severe PH are present, surgical pulmonary endarterectomy can be performed successfully in young children.