Small airways dysfunction in long‐term survivors of pediatric stem cell transplantation

Abstract

Small airways dysfunction as measured by MBWN2 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 MBWN2 as an early marker of pulmonary cGvHD.

The single breath nitrogen test and mortality – A 38 years follow up

“…In cystic fibrosis, Gustafsson et al [32] have shown that peripheral airways are indeed involved. Similarly, in stem cell transplanted children [33] and in obliterating bronchiolitis after lung transplantation [34], peripheral lung function is affected. Thus, peripheral lung units are involved in fundamentally different lung diseases.…”

Pulmonary function and fitness years after treatment for hypersensitivity pneumonitis during childhood

“…Green et al examined a group of primary ciliary dyskinesia patients of whom nearly all, including those with normal FEV 1 , had abnormal LCI 2.5 . Similar observations were also found in long‐term survivors of pediatric stem cell transplantation …”

“…However, PCD and CF differ considerably from HP according to basic pathology, infectious mucus retention/clearance and life‐long chronicity. Uhlving et al studied MBW N2 in pediatric stem cell recipients and found that one‐third of the patients had abnormal LCI 2.5 , despite normal spirometry in 90%. These findings are consistent with ours and in contrast to studies in asthmatic children and adolescents, where LCI 2.5 seems to be within or close to the normal range …”

“…The overall inhomogeneity is reflected in the lung clearance index (LCI 2.5 ) whereas regional inhomogeneties are reflected in the conductive and acinar airway ventilation heterogeneity indices (Scond and Sacin, respectively) . Studies in children with various types of diffuse airway diseases have shown that MBW can detect peripheral airway impairment despite normal spirometry . Measurement of peak oxygen uptake (VO 2peak ), reflecting aerobic physical fitness of the individual, is an important determinant of endurance capacity and is of interest given the possible impairment of diffusing capacity, but may also reflect an overall evaluation of the patient's general physical health.…”

Lung clearance index for early detection of pulmonary complications after allo‐HSCT in children

“…In a cross‐sectional study of pediatric HSCT‐recipients, we have previously demonstrated abnormal LCI in 34% of patients at 3 to 10 years follow‐up, despite normal spirometry in a majority of patients . Nyilas et al found cGvHD score to be closely correlated to LCI value.…”