EDITORIAL URRENT C OPINION

Pediatric pulmonary medicine: progress at the intersections David N. Cornfield

This section of Current Opinion in Pediatrics highlights current opportunities and challenges in pediatric pulmonary medicine. In many ways, the field of pulmonary medicine serves as a window to view the more general, and considerable, progress of pediatric medicine over the past many decades. As is the case in medicine in general, over the past 100 years the major advances of pulmonary medicine have occurred when disciplines previously thought to be distinct were brought together. Particularly, explicit examples of such progress include the march from discovery of the tubercle bacillus (TB) by Koch in 1882 to the initial clinical use of streptomycin, isolated from a fungus, to address TB in 1946. Diagnosis and treatment of cystic fibrosis have evolved steadily while drawing upon disciplines as varied as engineering, protein chemistry, ethics, nutrition, public policy, and molecular diagnostics. The past 40 years have brought remarkable progress in the area of neonatal lung disease. Techniques and therapeutic strategies that are currently routinely applied were painstakingly developed and refined. Blood gas analysis, positive-pressure ventilation, surfactant protein biology and replacement therapy, and inhaled nitric oxide each have contributed to rapidly advance the frontiers of neonatal lung disease. The articles included in this edition of Current Opinion in Pediatrics, I hope, build upon this tradition and illustrate the critically important relationship between science and medicine. The initial contribution, from Baker and Alvira, offers insight into the fundamental mechanisms that underlie postnatal alveolarization. With enhanced neonatal care and survival, the clinical implications of these insights have never been more palpably apparent. By building upon the existing knowledge surrounding lung development, the authors offer insights into the physiologic consequences of disrupted postnatal alveolarization, the new bronchopulmonary dysplasia, as well as potential strategies to unlock the secrets of lung regeneration and growth. Imaging technology has advanced rapidly over the past several decades. With increasingly clear and www.co-pediatrics.com

detailed in-utero images, therapeutic dilemmas have emerged as lung abnormalities of uncertain clinical importance are being revealed. In the second contribution of the edition, Wall and Coates carefully consider how best to approach congenital lung malformations, especially in asymptomatic infants and children. Which lesions warrant intervention is no longer certain. The issue is increasingly important as imaging technology is likely to improve still further, thereby presenting even more diagnostic quandaries, for infants, children, and even young adults. General pediatricians will certainly encounter these issues as curious and anxious parents seek counsel. The contribution of Kuo and Young addresses the rapidly evolving area of childhood interstitial lung disease (ILD). The inclusion of a review on ILD is particularly propitious given the relatively recent descriptions of several distinct forms of childhood ILD such as neuroendocrine cell hyperplasia of infancy and surfactant protein abnormalities. Childhood ILD encompasses lung abnormalities that range from self-limited and relatively benign to pathological states that are almost certainly fatal. The optimal diagnosis and management of childhood ILD entail use of genetic, clinical, and imaging tools, thereby emphasizing the need to understand the information that lies at the confluence of science and medicine. While contemporary medicine has created new knowledge and standards that ought to be specifically applied to certain illnesses, patient care continues to be confounded by an inability to broadly translate care standards into practice. Bronchiolitis, perhaps, represents the best example of this problem, as a good many therapeutic interventions Division of Pulmonary Medicine, Department of Pediatrics, Stanford University School of Medicine, Stanford, California, USA Correspondence to David N. Cornfield, MD, Stanford University School of Medicine Center for Excellence in Pulmonary Biology, 770, Welch Road, Suite 350, Stanford, CA 94305-5162, USA. Tel: +1 650 723 8235; fax: +1 650 725 0171; e-mail: [email protected] Curr Opin Pediatr 2014, 26:304–305 DOI:10.1097/MOP.0000000000000099 Volume 26  Number 3  June 2014

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Pediatric pulmonary medicine Cornfield

persist despite considerable evidence indicating an absence of therapeutic benefit. As medical science creates new and durable knowledge, it is essential that physicians incorporate these insights into the care of children. In the context of bronchiolitis, a remarkably common childhood illness, hospitalized children are treated with a host of therapies that increase cost, anxiety, and complexity of care, but provide no meaningful benefit. Indeed, if the promise of evidence-based medicine is to be realized, then clinicians must carefully distinguish between indicated and nonindicated therapeutic interventions. In the final piece of this edition, the state of lung transplantation for children is considered. It is fitting, I think, to end the edition with a review of lung transplantation. Lung transplantation has moved, rapidly, from a relatively experimental and infrequently employed strategy for children to a more frequently applied therapeutic intervention for an ever-widening circle of indications. Contingent upon the clinical circumstance, infants, children, and adolescents can meaningfully benefit from lung transplantation. While cystic fibrosis remains the most common cause of end-stage lung disease that requires lung transplantation, increasing numbers of children with neonatal lung disease because of surfactant protein abnormalities or pulmonary vascular disease are undergoing lung transplantation. However, the success of lung

transplantation is mitigated by long-term compromise in lung function due to the development of bronchiolitis obliterans. Greater understanding of the cause and potential of bronchiolitis obliterans is needed if lung transplantation is to realize its full promise. Hopefully, the present collection of articles provides a window into the progress and promise of pediatric pulmonary medicine. Certainly, the clinical issues highlighted in this edition each represent significant clinical challenges that ought to be addressed with diligence and care at the bedside of each affected child. On a more global level, and arguably just as significant, it is the imperative to wed science with clinical care in order to ensure that tomorrow’s care is better than today’s. New diagnostic categories superimposed on novel therapeutic strategies highlight the importance of understanding the physiology of lung development as well as lung disease. If we, collectively, continue to mindfully seek novel approaches to lung development and disease, then the steep and steady trajectory of progress can be sustained over the next many decades and presentday fears can be replaced by true hope. Acknowledgements None. Conflicts of interest There are no conflicts of interest.

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Pediatric pulmonary medicine: progress at the intersections.

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