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Abnormal infant pulmonary function in young children with neuroendocrine cell hyperplasia of infancy

Gwendolyn S Kerby1, Brandie D Wagner, Jonathan Popler

  • 1Department of Pediatrics, Section of Pulmonary Medicine, University of Colorado School of Medicine and The Breathing Institute, Children's Hospital Colorado, Aurora, Colorado.

Pediatric Pulmonology
|November 22, 2012
PubMed

Insights

Infant pulmonary function tests (IPFTs) in neuroendocrine cell hyperplasia of infancy (NEHI) are feasible and reveal significant airflow limitation and air trapping. These tests correlate with future oxygen saturation and lung function, aiding NEHI patient care.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine
  • Neonatal Intensive Care

Background:

  • Neuroendocrine cell hyperplasia of infancy (NEHI) is a rare pediatric respiratory condition.
  • Understanding lung function in NEHI is crucial for predicting clinical outcomes.
  • Current data on infant pulmonary function tests (IPFTs) in NEHI and their correlation with long-term outcomes are limited.

Purpose of the Study:

  • To assess the feasibility and diagnostic value of IPFTs in infants with NEHI.
  • To compare IPFT results between NEHI patients and disease control (DC) subjects.
  • To investigate the correlation between NEHI IPFTs and future clinical outcomes, including oxygen saturation and lung function.

Main Methods:

  • A retrospective, single-center study involving infants diagnosed with NEHI (by lung biopsy or clinically as NEHI syndrome) and DC subjects.
  • Infant pulmonary function tests (IPFTs) were performed using raised volume rapid thoracoabdominal compression (RVRTC) and plethysmography.
  • Standard spirometry measures, room air oxygen saturation (RA O2 sat), and weight percentiles were collected during follow-up.

Main Results:

  • IPFTs were successfully performed in 57 subjects (15 NEHI, 22 NEHI syndrome, 20 DC).
  • NEHI and NEHI syndrome groups showed significant airflow limitation and air trapping compared to DCs.
  • Initial IPFT measures correlated with short-term RA O2 sat and long-term FEV1, indicating predictive value.

Conclusions:

  • IPFTs are feasible in infants with NEHI and demonstrate significant airway obstruction and air trapping.
  • IPFTs provide valuable clinical information for managing NEHI patients.
  • These tests correlate with future clinical parameters like oxygen saturation and FEV1, supporting their utility in NEHI care.
Abstract

Keywords:
children's interstitial lung diseasehypoxemiainfant pulmonary functionneuroendocrine cellpediatric diffuse lung disease

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