Abnormal infant pulmonary function in young children with neuroendocrine cell hyperplasia of infancy
Rationale Lung function in children with neuroendocrine cell hyperplasia of infancy (NEHI) and correlations with future clinical outcomes are needed to guide clinical management. Objective To compare results of infant pulmonary function tests (IPFTs) in children with NEHI to disease control (DC) sub...
Saved in:
Published in | Pediatric pulmonology Vol. 48; no. 10; pp. 1008 - 1015 |
---|---|
Main Authors | , , , , , , , , , |
Format | Journal Article |
Language | English |
Published |
United States
Blackwell Publishing Ltd
01.10.2013
Wiley Subscription Services, Inc |
Subjects | |
Online Access | Get full text |
Cover
Loading…
Summary: | Rationale
Lung function in children with neuroendocrine cell hyperplasia of infancy (NEHI) and correlations with future clinical outcomes are needed to guide clinical management.
Objective
To compare results of infant pulmonary function tests (IPFTs) in children with NEHI to disease control (DC) subjects and to correlate NEHI IPFTs with future outcomes.
Methods
We performed a retrospective, single center study of IPFT in subjects diagnosed by lung biopsy (NEHI) or clinically (NEHI syndrome) and in DC subjects evaluated for cancer or pre‐hematopoietic stem cell transplantation (HSCT). Raised volume rapid thoracoabdominal compression (RVRTC) and plethysmography were performed on all infants and evaluated for quality. Standard spirometry measures, room air oxygen saturations (RA O2 sat), and weight percentiles were collected during follow up.
Measurements and Main Results
Fifty‐seven IPFTs were performed in 15 NEHI, 22 NEHI syndrome, and 20 DC subjects. RVRTC and FRC measurements were obtained in 85% or more of subjects in all groups. Significant airflow limitation (FEV0.5 P‐value ≤ 0.01) and air trapping (FRC P‐value ≤ 0.01) were seen in NEHI and NEHI syndrome subjects compared to DCs. No significant correlations were found between IPFT, oxygen use, RA O2 sat, and weight at the time of the IPFTs. Initial FEV0.5 and FRC z‐scores correlated with RA O2 sat (r = 0.60 and −0.49) at short‐term follow up (6–12 months). Most measurements of RVRTC correlated with FEV1 (n = 5) measured 4–5 years later (r > 0.50).
Conclusions
IPFTs in NEHI subjects are feasible, demonstrate significant obstruction and air trapping, and correlate with future RA O2 sat and FEV1. IPFTs may provide valuable clinical information when caring for NEHI patients. Pediatr Pulmonol. 2013; 48:1008–1015. © 2012 Wiley Periodicals, Inc. |
---|---|
Bibliography: | none reported istex:AC7446BBA0367EF7BFEA4360C6E81A12F2436580 ArticleID:PPUL22718 ark:/67375/WNG-T1R533CW-D ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 |
ISSN: | 8755-6863 1099-0496 |
DOI: | 10.1002/ppul.22718 |