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Lisa R. Young, MD; Timothy J. Vece, MD; R. Paul Guillerman, MD
Author and Funding Information

Dr Vece is currently at University of North Carolina School of Medicine (Chapel Hill, NC).

FINANCIAL/NONFINANCIAL DISCLOSURES: See earlier cited article for author conflicts of interest.

CORRESPONDENCE TO: Lisa R. Young, MD, 2200 Children's Way, Doctor's Office Tower 11215, Pulmonary Medicine, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN 37232


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;149(6):1579-1580. doi:10.1016/j.chest.2016.03.020
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We thank Dr Berteloot and colleagues for their interest in our review article recently published in CHEST. The authors present two interesting cases as a platform to discuss a 2010 report of chest CT scan findings in neuroendocrine cell hyperplasia of infancy (NEHI). Berteloot et al raise concerns about the specificity of chest CT scans by proposing that their cases had imaging findings suggestive of NEHI but did not ultimately have NEHI based on the histologic findings and the clinical response to corticosteroid therapy.

We concur that the images provided for these cases exhibit a NEHI pattern of ground-glass opacities without septal thickening, consolidation, or bronchiectasis. We also agree that perceived steroid responsiveness could reflect an alternate or additional diagnosis. Chronic corticosteroids are not beneficial in most cases of isolated NEHI, in which lung biopsy specimens demonstrate little, if any, inflammation. Thus, we question whether these patients had superimposed inflammatory lung disease. For example, some children with NEHI finally come to the attention of pulmonary consultants when viral illness exacerbates their chronic symptoms, and other cases are complicated by aspiration.,

The landmark study by Brody et al reported the specificity of CT scan findings at 100%, although the sample size was relatively small, and a confidence interval of the probable specificity range was not provided. False-positive results on CT scans are plausible, potentially from a spectrum of small airway diseases such as constrictive bronchiolitis or follicular bronchiolitis. Therefore, we wish to emphasize that the guidance provided in our review and in the American Thoracic Society’s clinical guideline requires a compatible clinical setting to rely on radiologic criteria for diagnosing NEHI without performing a lung biopsy. The clinical presentation and course are essential for a confident diagnosis of NEHI and should inform decisions about whether to proceed to lung biopsy, which is still needed in some cases.

Unfortunately, there are also substantial challenges in the histologic assessment of NEHI. Pulmonary neuroendocrine cells are present in most normal airways, and thus it is puzzling that the cases of Berteloot et al had “no bombesin-positive pulmonary neuroendocrine cells” present. Published data illustrate that the prominence of these cells can be patchy, and sufficient sampling with detailed morphometric analysis may be needed. Berteloot et al classified their cases as desquamative interstitial pneumonia on the basis of accumulation of alveolar macrophages. We contend that this finding is a nonspecific observation noted in a variety of lung disorders, including NEHI. The absence of type II alveolar epithelial cell hyperplasia and interstitial remodeling does not support a diagnosis of desquamative interstitial pneumonia, which is a histologic pattern now most commonly associated with surfactant dysfunction disorders in children.

In summary, the noninvasive diagnosis of NEHI on the basis of corroborative clinical and imaging findings has been a remarkably impactful advance that has obviated the need for lung biopsy for many children with suspected NEHI in recent years. Caution about generalization of findings from small studies is appropriate, and the clinical context remains essential. Our understanding of NEHI and other diffuse lung disease of childhood is continually evolving and is greatly aided by the contributions of such letters, which emphasize the need for further studies.

References

Vece T.J. .Young L.R. . Update on diffuse lung disease in children. Chest. 2016;149:836-845 [PubMed]journal. [CrossRef] [PubMed]
 
Brody A.S. .Guillerman R.P. .Hay T.C. .et al Neuroendocrine cell hyperplasia of infancy: diagnosis with high-resolution CT. AJR Am J Roentgenol. 2010;194:238-244 [PubMed]journal. [CrossRef] [PubMed]
 
Young L.R. .Brody A.S. .Inge T.H. .et al Neuroendocrine cell distribution and frequency distinguish neuroendocrine cell hyperplasia of infancy from other pulmonary disorders. Chest. 2011;139:1060-1071 [PubMed]journal. [CrossRef] [PubMed]
 
O'Connor M.G. .Wurth M. .Young L.R. . Rare becomes more common: recognizing neuroendocrine cell hyperplasia of infancy in everyday pulmonary consultations. Ann Am Thorac Soc. 2015;12:1730-1732 [PubMed]journal. [PubMed]
 
Kurland G. .Deterding R.R. .Hagood J.S. .et al An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med. 2013;188:376-394 [PubMed]journal. [CrossRef] [PubMed]
 

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References

Vece T.J. .Young L.R. . Update on diffuse lung disease in children. Chest. 2016;149:836-845 [PubMed]journal. [CrossRef] [PubMed]
 
Brody A.S. .Guillerman R.P. .Hay T.C. .et al Neuroendocrine cell hyperplasia of infancy: diagnosis with high-resolution CT. AJR Am J Roentgenol. 2010;194:238-244 [PubMed]journal. [CrossRef] [PubMed]
 
Young L.R. .Brody A.S. .Inge T.H. .et al Neuroendocrine cell distribution and frequency distinguish neuroendocrine cell hyperplasia of infancy from other pulmonary disorders. Chest. 2011;139:1060-1071 [PubMed]journal. [CrossRef] [PubMed]
 
O'Connor M.G. .Wurth M. .Young L.R. . Rare becomes more common: recognizing neuroendocrine cell hyperplasia of infancy in everyday pulmonary consultations. Ann Am Thorac Soc. 2015;12:1730-1732 [PubMed]journal. [PubMed]
 
Kurland G. .Deterding R.R. .Hagood J.S. .et al An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med. 2013;188:376-394 [PubMed]journal. [CrossRef] [PubMed]
 
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