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Correspondence |

Anything that Looks Like a Neuroendocrine Cell Hyperplasia of Infancy Is Not Necessarily a Neuroendocrine Cell Hyperplasia of Infancy FREE TO VIEW

Laureline Berteloot, MD; Louise Galmiche-Rolland, MD, PhD; Rola Abou-Taam, MD
Author and Funding Information

FINANCIAL/NONFINANCIAL DISCLOSURES: None declared.

CORRESPONDENCE TO: Laureline Berteloot, MD, Hôpital Universitaire Necker-Enfants Malades, 149 rue de Sèvres, 75015 Paris, France


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


Chest. 2016;149(6):1578-1579. doi:10.1016/j.chest.2016.02.683
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Published online

We read with interest the article titled “Update on Diffuse Lung Disease in Children” from Vece and Young in a recent issue of CHEST (March 2016). We particularly appreciated their clear approach and recommendations about this heterogeneous and complex group of rare conditions. The exhaustive description of diagnosis, evaluations, and treatments is helpful, as most institutions will only manage a few cases per year and can be confused when faced with childhood interstitial lung disease (chILD).

We would, however, like to raise concerns regarding the accuracy of CT scanning for the diagnosis of neuroendocrine cell hyperplasia of infancy (NEHI). This topic, which has also been mentioned in the European recommendations, mainly comes from the article of Brody et al, in which images from 23 children with biopsy-proven NEHI were compared with six children with other chILD.

In our cohort, we have had examples which contradict the fact that the CT scan pattern of “ground glass opacities in the right middle lobe, lingula, and medial segments of the upper and lower lobes, in the absence of other significant parenchymal abnormalities” is pathognomonic for NEHI. We have managed some patients with clinical chILD and with the same CT scan pattern (Fig 1) as described in NEHI. One case was a 2-year-old girl; another case involved a 6-month-old boy. They also presented with air-trapping, which is another sign of NEHI, both on CT scan and in pulmonary function test results. Both patients improved over years, as expected in NEHI; however, they required corticosteroid therapy, which is unusual in NEHI. In addition, lung biopsy specimens from both patients at diagnosis exhibited a pattern of desquamative interstitial pneumonia with no bombesin-positive pulmonary neuroendocrine cells; this finding is contrary to the diagnosis of NEHI. The patients were both subjected to sequencing of SFTPC, SFTPB, and ABCA3 proteins of surfactant. No genetic mutation was found in the 2-year-old girl, and a single mutation in the ABCA3 protein was found in the 6-month-old boy.

Figure Jump LinkFigure 1 A-D, CT scan of the 2-year-old girl in (A) axial and (B) coronal planes showing ground-glass opacities in the right middle lobe, lingula, and medial segments of the upper and lower lobes. (C) A control CT scan at the age of 8 years showed a slight improvement with lower density and extension of ground-glass opacities. (D) Lung biopsy performed on the 2-year-old girl showing intra-alveolar and bronchiolar accumulation of macrophages without remodeling of lung architecture. No significant interstitial inflammation was observed.Grahic Jump Location

Correct diagnosis of NEHI is of critical importance because, unlike other types of chILD, NEHI exhibits a poor response to corticosteroid therapy, requires other treatments, and has a better prognosis. On the basis of the two aforementioned examples, we think that the characteristic CT scan pattern of NEHI is not to be considered as pathognomonic. Protein surfactant mutations must be searched in all cases, and lung biopsies should still be considered for differential diagnosis.

References

Vece T.J. .Young L.R. . Update on diffuse lung disease in children. Chest. 2016;149:836-845 [PubMed]journal. [CrossRef] [PubMed]
 
Bush A. .Cunningham S. .de Blic J. . chILD-EU Collaborationet al European protocols for the diagnosis and initial treatment of interstitial lung disease in children. Thorax. 2015;70:1078-1084 [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]
 
Nathan N. .Taam R.A. .Epaud R. .et al A national internet-linked based database for pediatric interstitial lung diseases: the French network. Orphanet J Rare Dis. 2012;7:40- [PubMed]journal. [CrossRef] [PubMed]
 
Deterding R.R. .Pye C. .Fan L.L. .Langston C. . Persistent tachypnea of infancy is associated with neuroendocrine cell hyperplasia. Pediatr Pulmonol. 2005;40:157-165 [PubMed]journal. [CrossRef] [PubMed]
 
Rice A. .Tran-Dang M.A. .Bush A. .Nicholson A.G. . Diffuse lung disease in infancy and childhood: expanding the chILD classification. Histopathology. 2013;63:743-755 [PubMed]journal. [CrossRef] [PubMed]
 

Figures

Figure Jump LinkFigure 1 A-D, CT scan of the 2-year-old girl in (A) axial and (B) coronal planes showing ground-glass opacities in the right middle lobe, lingula, and medial segments of the upper and lower lobes. (C) A control CT scan at the age of 8 years showed a slight improvement with lower density and extension of ground-glass opacities. (D) Lung biopsy performed on the 2-year-old girl showing intra-alveolar and bronchiolar accumulation of macrophages without remodeling of lung architecture. No significant interstitial inflammation was observed.Grahic Jump Location

Tables

References

Vece T.J. .Young L.R. . Update on diffuse lung disease in children. Chest. 2016;149:836-845 [PubMed]journal. [CrossRef] [PubMed]
 
Bush A. .Cunningham S. .de Blic J. . chILD-EU Collaborationet al European protocols for the diagnosis and initial treatment of interstitial lung disease in children. Thorax. 2015;70:1078-1084 [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]
 
Nathan N. .Taam R.A. .Epaud R. .et al A national internet-linked based database for pediatric interstitial lung diseases: the French network. Orphanet J Rare Dis. 2012;7:40- [PubMed]journal. [CrossRef] [PubMed]
 
Deterding R.R. .Pye C. .Fan L.L. .Langston C. . Persistent tachypnea of infancy is associated with neuroendocrine cell hyperplasia. Pediatr Pulmonol. 2005;40:157-165 [PubMed]journal. [CrossRef] [PubMed]
 
Rice A. .Tran-Dang M.A. .Bush A. .Nicholson A.G. . Diffuse lung disease in infancy and childhood: expanding the chILD classification. Histopathology. 2013;63:743-755 [PubMed]journal. [CrossRef] [PubMed]
 
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