Premium
Small airways dysfunction in long‐term survivors of pediatric stem cell transplantation
Author(s) -
Uhlving Hilde Hylland,
Mathiesen Sidsel,
Buchvald Frederik,
Green Kent,
Heilmann Carsten,
Gustafsson Per,
Müller Klaus,
Nielsen Kim Gjerum
Publication year - 2015
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.23058
Subject(s) - medicine , spirometry , hematopoietic stem cell transplantation , pulmonary function testing , nitrogen washout , respiratory system , plethysmograph , lung volumes , cardiology , airway , ventilation (architecture) , transplantation , lung , surgery , asthma , functional residual capacity , mechanical engineering , engineering
Summary Background Chronic graft‐versus‐host disease (cGvHD) in the lungs is a life‐threatening complication of allogeneic hematopoietic stem cell transplantation (HSCT). Pulmonary cGvHD is initiated in the peripheral airways, and diagnosis may be delayed by low sensitivity of standard pulmonary function tests. Multiple breath nitrogen washout (MBW N2 ) is a promising, sensitive method to assess small airways function. This is the first report on MBW N2 in survivors of pediatric HSCT. Methods This cross‐sectional study undertaken 3–10 years post‐HSCT, included 64 patients and 64 matched controls who all performed spirometry, whole‐body plethysmography and MBW N2 . From MBW N2 the lung clearance index (LCI) and indices reflecting ventilation inhomogeneity arising close to the acinar lung zone (S acin ) and in the conductive airway zone (S cond ) were derived. Subjective respiratory morbidity was assessed using the St. George Respiratory Questionnaire. Results LCI, S acin , and S cond were significantly higher in HSCT‐patients compared with controls. Despite few reported symptoms and normal forced expiratory volume in 1 sec (FEV 1 ) in 91%, LCI, S cond , and S acin were abnormal in 34%, 52%, and 25% of HSCT‐patients, respectively. LCI and S cond correlated weakly with spirometric findings in HSCT‐patients, but not in controls. S cond was abnormal in 82% (9/11) of patients with evidence of cGvHD, and was associated with cGvHD in the multivariate analysis (r 2 = 0.26, P = 0.001). Conclusions Small airways dysfunction as measured by MBW N2 was a common finding at long term follow‐up of children after allogeneic HSCT and was associated with cGvHD. The majority of these subjects had normal spirometric values and did not report any respiratory symptoms. Prospective studies are required to evaluate the long term clinical consequences of these signs of small airway disease and the value of MBW N2 as an early marker of pulmonary cGvHD. Pediatr Pulmonol. 2015; 50:704–712. © 2014 Wiley Periodicals, Inc.