title: Effectiveness of long-term routine pulmonary function surveillance following pediatric hematopoietic stem cell transplantation.
abstract: BACKGROUND
  Pulmonary complications following hematopoietic stem cell transplantation (HSCT) are common and often subclinical. Thus, periodic pulmonary function testing (PFT) is mandatory. This study sought to evaluate the effectiveness of long-term PFT surveillance for children undergoing HSCT and identify potential risk factors.
  
  
  METHODS
  We reviewed long-term PFT for HSCT patients at a tertiary pediatric center. Inclusion criteria were PFT prior to and at least once following HSCT.
  
  
  RESULTS
  Fifty-seven patients performed 202 spirometry and 193 plethysmographic maneuvers; 41 were tested during the first year after HSCT, but only 29 were evaluated consistently long term (2-12 years). FVC and FEV(1) decreased gradually suggesting a restrictive ventilatory defect: FVC % predicted [mean ± SD] dropped from 91 ± 14% to 85 ± 17% after 0-24 months and 80 ± 19% beyond 2 years (P = 0.01) whereas FEV(1) dropped from 95 ± 16% to 88 ± 19% and 82 ± 20%, respectively (P = 0.002). A slight reduction in TLC was observed. Those undergoing allogeneic HSCT had a greater decline in FVC (P = 0.025) and FEV(1) (P = 0.025) as did those conditioned with radiation, regarding both FVC (P = 0.003) and FEV(1) (P = 0.002). Decline occurred earlier (≤2 years) after chemotherapy compared with radiation. Seven children had severe irreversible obstruction at >2 years despite therapeutic intervention.
  
  
  CONCLUSIONS
  Most survivors of childhood HSCT maintain almost normal pulmonary function although mild restrictive lung disease may develop, particularly following allogeneic HSCT and conditioning with radiation. Severe airways obstruction developed in a small minority. The surveillance protocol for PFT needs to be followed more stringently to enable intervention possibly before early subclinical changes progress and become irreversible.
authors.affiliation: Pulmonary Institute, Schneider Children's Medical Center of Israel, Petach Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Dario
authors.initials: D
authors.lastname: Prais
authors.forename: Moran Marx
authors.initials: MM
authors.lastname: Sinik
authors.forename: Jerry
authors.initials: J
authors.lastname: Stein
authors.forename: Meir
authors.initials: M
authors.lastname: Mei-Zahav
authors.forename: Huda
authors.initials: H
authors.lastname: Mussaffi
authors.forename: Guy
authors.initials: G
authors.lastname: Steuer
authors.forename: Shai
authors.initials: S
authors.lastname: Hananya
authors.forename: Aviva
authors.initials: A
authors.lastname: Krauss
authors.forename: Isaac
authors.initials: I
authors.lastname: Yaniv
authors.forename: Hannah
authors.initials: H
authors.lastname: Blau
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005541:Forced Expiratory Volume; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008171:Lung Diseases / Q000209:etiology / Q000503:physiopathology; D008297:Male; D010991:Plethysmography; D012307:Risk Factors; D013147:Spirometry; D014184:Transplantation, Homologous; D014797:Vital Capacity; D055815:Young Adult
keywords: bone marrow transplantation; hematopoietic stem cell; lung function; spirometry