Ground glass and fibrotic change in children with surfactant protein C dysfunction mutations.

INTRODUCTION: Therapeutics exist to treat fibrotic lung disease in adults, but these have not been investigated in children. Defining biomarkers for pediatric fibrotic lung disease in children is crucial for clinical trials. Children with surfactant protein C (SFTPC) dysfunction mutations develop fibrotic lung disease over time. We evaluated chest computed tomography (CT) changes over time in children with SFTPC dysfunction mutations. METHODS: We performed an institutional review board-approved retrospective review of children with SFTPC dysfunction mutations. We collected demographic and clinical information. Chest CT scans were evaluated using visual and computerized scores. Chest CT scores and pulmonary function tests were reviewed. RESULTS: Eleven children were included. All children presented in infancy and four children suffered from respiratory failure requiring mechanical ventilation. Those who performed pulmonary function tests had stable forced vital capacities over time by percent predicted, but increased forced vital capacity in liters. CT findings evolved over time in most patients with earlier CT scans demonstrating ground glass opacities and later CT scans with more fibrotic features. In a pilot analysis, data-driven textural analysis software identified fibrotic features in children with SFTPC dysfunction that increased over time and correlated with visual CT scores. DISCUSSION: We describe 11 children with SFTPC dysfunction mutations. Increases in forced vital capacity over time suggest that these children experience lung growth and that therapeutic intervention may maximize lung growth. Ground glass opacities are the primary early imaging findings while fibrotic features dominate later. CT findings suggest the development of and increases in fibrotic features that may serve as potential biomarkers for antifibrotic therapeutic trials.

The use of light's criteria in hospitalized children with a pleural effusion of unknown etiology.

OBJECTIVE: Pleural effusions are common in pediatrics. When the etiology of a pleural effusion remains unknown, adult literature recommends the use of Light's criteria to differentiate a transudate from an exudate. Pediatricians may rely on adult literature for the diagnostic management of pleural effusions as Light's criteria has not been validated in children. The purpose of this study was to review the use of Light's criteria in hospitalized children with a pleural effusion of unknown etiology. METHODS: Retrospective review was performed on children hospitalized with a pleural effusion requiring chest tube placement or thoracentesis between January 1, 2016 to January 1, 2017 at Children's Hospital Colorado. Charts were reviewed for primary team, use of Light's criteria, pleural effusion diagnosis, and 30-day recurrence of repeat intervention or fluid analysis. RESULTS: Sixty-eight patients were hospitalized with a pleural effusion of unknown etiology requiring intervention. Only 16 pleural effusions (24%) were classified using Light's criteria. In those patients for whom Light's criteria was used, a diagnosis or change in management occurred in 10 of 16 patients (63%). Pleural effusions were most common on the cardiology service (26/68). Use of Light's criteria was most frequent on the oncology service (7/8). Thirty-day need for repeat intervention was lower in those with Light's criteria (13%) compared to those without (27%). CONCLUSIONS: Light's criteria were utilized infrequently in hospitalized children with a pleural effusion of unknown etiology at a single institution. There was considerable practice variation among provider teams. When utilized, Light's criteria assisted in making a diagnosis or changing management in many patients, and may lead to a reduction in 30-day recurrence requiring repeat intervention.