Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series
OBJECTIVE: Neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease characterized by tachypnea, retractions, crackles, and hypoxia. The aim of this study was to report and discuss the clinical, imaging, and histopathological findings in a series of NEHI cas...
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| Format: | Article |
| Language: | English |
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Sociedade Brasileira de Pneumologia e Tisiologia
2013-09-01
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| Series: | Jornal Brasileiro de Pneumologia |
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| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132013000500569&lng=en&tlng=en |
| _version_ | 1818357438187831296 |
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| author | Vivianne Calheiros Chaves Gomes Mara Cristina Coelho Silva Jose Holanda Maia Filho Pedro Daltro Simone Gusmao Ramos Alan S. Brody Edson Marchiori |
| author_facet | Vivianne Calheiros Chaves Gomes Mara Cristina Coelho Silva Jose Holanda Maia Filho Pedro Daltro Simone Gusmao Ramos Alan S. Brody Edson Marchiori |
| author_sort | Vivianne Calheiros Chaves Gomes |
| collection | DOAJ |
| description | OBJECTIVE: Neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease characterized by tachypnea, retractions, crackles, and hypoxia. The aim of this study was to report and discuss the clinical, imaging, and histopathological findings in a series of NEHI cases at a tertiary pediatric hospital, with an emphasis on diagnostic criteria and clinical outcomes. METHODS: Between 2003 and 2011, 12 full-term infants were diagnosed with NEHI, based on clinical and tomographic findings. Those infants were followed for 1-91 months. Four infants were biopsied, and the histopathological specimens were stained with bombesin antibody. RESULTS: In this case series, symptoms appeared at birth in 6 infants and by 3 months of age in the remaining 6. In all of the cases, NEHI was associated with acute respiratory infection. The most common initial chest HRCT findings were ground-glass opacities that were in the middle lobe/lingula in 12 patients and in other medullary areas in 10. Air trapping was the second most common finding, being observed in 7 patients. Follow-up HRCT scans (performed in 10 patients) revealed normal results in 1 patient and improvement in 9. The biopsy findings were nonspecific, and the staining was positive for bombesin in all samples. Confirmation of NEHI was primarily based on clinical and tomographic findings. Symptoms improved during the follow-up period (mean, 41 months). A clinical cure was achieved in 4 patients. CONCLUSIONS: In this sample of patients, the diagnosis of NEHI was made on the basis of the clinical and tomographic findings, independent of the lung biopsy results. Most of the patients showed clinical improvement and persistent tomographic changes during the follow-up period, regardless of the initial severity of the disease or type of treatment. |
| first_indexed | 2024-12-13T20:13:06Z |
| format | Article |
| id | doaj.art-30c7f9ca83074efaa810b94321503ce0 |
| institution | Directory Open Access Journal |
| issn | 1806-3756 |
| language | English |
| last_indexed | 2024-12-13T20:13:06Z |
| publishDate | 2013-09-01 |
| publisher | Sociedade Brasileira de Pneumologia e Tisiologia |
| record_format | Article |
| series | Jornal Brasileiro de Pneumologia |
| spelling | doaj.art-30c7f9ca83074efaa810b94321503ce02022-12-21T23:32:53ZengSociedade Brasileira de Pneumologia e TisiologiaJornal Brasileiro de Pneumologia1806-37562013-09-0139556957810.1590/S1806-37132013000500007S1806-37132013000500569Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case seriesVivianne Calheiros Chaves GomesMara Cristina Coelho SilvaJose Holanda Maia FilhoPedro DaltroSimone Gusmao RamosAlan S. BrodyEdson MarchioriOBJECTIVE: Neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease characterized by tachypnea, retractions, crackles, and hypoxia. The aim of this study was to report and discuss the clinical, imaging, and histopathological findings in a series of NEHI cases at a tertiary pediatric hospital, with an emphasis on diagnostic criteria and clinical outcomes. METHODS: Between 2003 and 2011, 12 full-term infants were diagnosed with NEHI, based on clinical and tomographic findings. Those infants were followed for 1-91 months. Four infants were biopsied, and the histopathological specimens were stained with bombesin antibody. RESULTS: In this case series, symptoms appeared at birth in 6 infants and by 3 months of age in the remaining 6. In all of the cases, NEHI was associated with acute respiratory infection. The most common initial chest HRCT findings were ground-glass opacities that were in the middle lobe/lingula in 12 patients and in other medullary areas in 10. Air trapping was the second most common finding, being observed in 7 patients. Follow-up HRCT scans (performed in 10 patients) revealed normal results in 1 patient and improvement in 9. The biopsy findings were nonspecific, and the staining was positive for bombesin in all samples. Confirmation of NEHI was primarily based on clinical and tomographic findings. Symptoms improved during the follow-up period (mean, 41 months). A clinical cure was achieved in 4 patients. CONCLUSIONS: In this sample of patients, the diagnosis of NEHI was made on the basis of the clinical and tomographic findings, independent of the lung biopsy results. Most of the patients showed clinical improvement and persistent tomographic changes during the follow-up period, regardless of the initial severity of the disease or type of treatment.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132013000500569&lng=en&tlng=enDoencas pulmonares intersticiais/diagnosticoDoencas pulmonares intersticiais/terapiaTomografia computadorizada por raios X |
| spellingShingle | Vivianne Calheiros Chaves Gomes Mara Cristina Coelho Silva Jose Holanda Maia Filho Pedro Daltro Simone Gusmao Ramos Alan S. Brody Edson Marchiori Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series Jornal Brasileiro de Pneumologia Doencas pulmonares intersticiais/diagnostico Doencas pulmonares intersticiais/terapia Tomografia computadorizada por raios X |
| title | Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series |
| title_full | Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series |
| title_fullStr | Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series |
| title_full_unstemmed | Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series |
| title_short | Diagnostic criteria and follow-up in neuroendocrine cell hyperplasia of infancy: a case series |
| title_sort | diagnostic criteria and follow up in neuroendocrine cell hyperplasia of infancy a case series |
| topic | Doencas pulmonares intersticiais/diagnostico Doencas pulmonares intersticiais/terapia Tomografia computadorizada por raios X |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132013000500569&lng=en&tlng=en |
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