School-entry health assessment of children previously diagnosed with neuroendocrine hyperplasia of infancy. CRC chILD-EU

<bold>Background:</bold> Neuroendocrine hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease. Clinical improvement is observed over time; however, follow-up data are relatively scarce. <bold>Aims:</bold> This study aimed to evaluate NEHI patients (pts) at school-entry age. <bold>Methods:</bold> Children previously diagnosed with NEHI who underwent a school-entry health assessment were included in the study. Clinical evaluation and additional tests were performed. <bold>Results:</bold> 35 pts aged 6y±6mo were enrolled. The median age for NEHI diagnosis was 11mo (IQR 7-18). At the time of diagnosis, 97% of children had tachypnoea (median 60 breaths/min), 100% chest retractions, 97% crackles on auscultation, and 77% had abnormal oxygen saturation during sleep. At school-entry age, tachypnea resolved in all, and dyspnoea was observed in 3 (9%) pts. The median age of dyspnoea resolution was 24mo (IQR 24-30). Hypoxemia at sleep was observed in 6 (17%) pts. The median time of oxygen weaning was 36mo (IQR 23-40). Crackles were still observed in 23 (66%) cases. 12 (34%) children reported worse exercise tolerance than their peers. 31 children had lung USG, and in 25 (81%) cases, abnormalities such as B-line artefacts and pleura irregularities were observed. Out of 32 tested pts, 11 (34%) had spirometry abnormalities, mostly low FVC and FEV1. Two children had an obstructive ventilatory pattern. Nine out of 10 pts who performed body plethysmography had air trapping, and one had high sReff. LCI was over 7.0 in 14 of 16 tested children, with a median of 9.3. <bold>Conclusion:</bold> Despite the resolution of the main symptoms at school age, abnormalities in physical examination and additional tests persist in NEHI patients.

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Published
2024-09-14
DOI
https://doi.org/10.1183/13993003.congress-2024.oa4694
Primary Topic
Neuroendocrine Tumor Research Advances
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article
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article

School-entry health assessment of children previously diagnosed with neuroendocrine hyperplasia of infancy. CRC chILD-EU

Honorata Marczak, Katarzyna Krenke, Magdalena Grochowska, Katarzyna Solarska‐Rydz
Neuroendocrine Tumor Research Advances
article

School-entry health assessment of children previously diagnosed with neuroendocrine hyperplasia of infancy. CRC chILD-EU

Honorata Marczak, Katarzyna Krenke, Magdalena Grochowska, Katarzyna Solarska‐Rydz
article en

Abstract

<bold>Background:</bold> Neuroendocrine hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease. Clinical improvement is observed over time; however, follow-up data are relatively scarce. <bold>Aims:</bold> This study aimed to evaluate NEHI patients (pts) at school-entry age. <bold>Methods:</bold> Children previously diagnosed with NEHI who underwent a school-entry health assessment were included in the study. Clinical evaluation and additional tests were performed. <bold>Results:</bold> 35 pts aged 6y±6mo were enrolled. The median age for NEHI diagnosis was 11mo (IQR 7-18). At the time of diagnosis, 97% of children had tachypnoea (median 60 breaths/min), 100% chest retractions, 97% crackles on auscultation, and 77% had abnormal oxygen saturation during sleep. At school-entry age, tachypnea resolved in all, and dyspnoea was observed in 3 (9%) pts. The median age of dyspnoea resolution was 24mo (IQR 24-30). Hypoxemia at sleep was observed in 6 (17%) pts. The median time of oxygen weaning was 36mo (IQR 23-40). Crackles were still observed in 23 (66%) cases. 12 (34%) children reported worse exercise tolerance than their peers. 31 children had lung USG, and in 25 (81%) cases, abnormalities such as B-line artefacts and pleura irregularities were observed. Out of 32 tested pts, 11 (34%) had spirometry abnormalities, mostly low FVC and FEV1. Two children had an obstructive ventilatory pattern. Nine out of 10 pts who performed body plethysmography had air trapping, and one had high sReff. LCI was over 7.0 in 14 of 16 tested children, with a median of 9.3. <bold>Conclusion:</bold> Despite the resolution of the main symptoms at school age, abnormalities in physical examination and additional tests persist in NEHI patients.

Medical University of Warsaw (PL)
Openalex Percentile: Top 70%
Neuroendocrine Tumor Research Advances
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