title: Pulmonary function in thalassaemia major and its correlation with body iron stores.
abstract: This study compared pulmonary function tests (PFTs) with cardiac, pancreatic and liver iron in 76 thalassemia major (TM) patients. Restrictive lung disease was observed in 16%, hyperinflation in 32% and abnormal diffusing capacity in 3%. While no patients met Global Initiative for Chronic Lung Disease criteria for airways obstruction, there were indicators of small airways disease and air trapping. PFTs did not correlate with somatic iron burden, blood counts or haemolysis. Restrictive lung disease was associated with inflammation. We conclude that TM patients have pulmonary abnormalities consistent with small airways obstruction. Restrictive disease and impaired diffusion are less common.
authors.affiliation: Divisions of Pediatric Pulmonology Pediatric Cardiology Hematology, Children's Hospital Los Angeles, 4650 Sunset Blvd, Los Angeles, CA 90027, USA.
authors.forename: Eugene Y
authors.initials: EY
authors.lastname: Sohn
authors.forename: Leila J
authors.initials: LJ
authors.lastname: Noetzli
authors.forename: Aakanksha
authors.initials: A
authors.lastname: Gera
authors.forename: Roberta
authors.initials: R
authors.lastname: Kato
authors.forename: Thomas D
authors.initials: TD
authors.lastname: Coates
authors.forename: Paul
authors.initials: P
authors.lastname: Harmatz
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Keens
authors.forename: John C
authors.initials: JC
authors.lastname: Wood
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D007501:Iron / Q000378:metabolism*; D019190:Iron Overload / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology; D008099:Liver / Q000378:metabolism; D008168:Lung / Q000503:physiopathology; D008171:Lung Diseases / Q000209:etiology* / Q000378:metabolism / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism; D010179:Pancreas / Q000378:metabolism; D029424:Pulmonary Disease, Chronic Obstructive / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology; D012129:Respiratory Function Tests / Q000379:methods; D015656:Respiratory Mechanics / Q000502:physiology; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications* / Q000378:metabolism / Q000503:physiopathology
chemical_list: D007501:Iron