title: Abnormal pulmonary function and associated risk factors in children and adolescents with sickle cell anemia.
abstract: Obstructive and restrictive pulmonary changes develop in children with sickle cell disease, but reports conflict as to the type of change that predominates. We prospectively performed spirometry, plethysmography, and lung diffusing capacity in 146 children aged 7 to 20 years with hemoglobin SS or Sβ(0)-thalassemia. Nineteen percent of the patients had obstructive physiology as defined according to guidelines of the American Thoracic Society. In addition, 9% had restrictive physiology and 11% had abnormal but not categorized physiology. Increasing age, patient-reported or family-reported history of asthma or wheezing, and higher lactate dehydrogenase concentration were independent predictors of obstruction as reflected in lower forced expiratory volume in the first second/forced vital capacity. In conclusion, abnormal pulmonary function, most often obstructive, is common in children with hemoglobin SS and Sβ(0)-thalassemia. Full pulmonary function testing should be performed in children with hemoglobin SS or Sβ(0)-thalassemia, especially with history of asthma or wheezing and accentuated elevations in hemolytic markers.
authors.affiliation: *Department of Medicine, University of Michigan, Ann Arbor, MI †Center for Sickle Cell Disease, Howard University ‡Department of Pediatrics, Children's National Medical Center, Washington, DC §Hematology Branch, National Heart, Lung and Blood Institute, Bethesda, MD ∥Department of Medicine, University of Pittsburgh, Pittsburgh, PA ¶Sickle Cell Center, University of Illinois, Chicago, IL.
authors.forename: Manuel
authors.initials: M
authors.lastname: Arteta
authors.forename: Andrew
authors.initials: A
authors.lastname: Campbell
authors.forename: Mehdi
authors.initials: M
authors.lastname: Nouraie
authors.forename: Sohail
authors.initials: S
authors.lastname: Rana
authors.forename: Onyinye C
authors.initials: OC
authors.lastname: Onyekwere
authors.forename: Gregory
authors.initials: G
authors.lastname: Ensing
authors.forename: Craig
authors.initials: C
authors.lastname: Sable
authors.forename: Niti
authors.initials: N
authors.lastname: Dham
authors.forename: Deepika
authors.initials: D
authors.lastname: Darbari
authors.forename: Lori
authors.initials: L
authors.lastname: Luchtman-Jones
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Kato
authors.forename: Mark T
authors.initials: MT
authors.lastname: Gladwin
authors.forename: Oswaldo L
authors.initials: OL
authors.lastname: Castro
authors.forename: Caterina P
authors.initials: CP
authors.lastname: Minniti
authors.forename: Victor R
authors.initials: VR
authors.lastname: Gordeuk
mesh_terms: D000293:Adolescent; D000328:Adult; D000402:Airway Obstruction / Q000209:etiology* / Q000473:pathology; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000473:pathology; D001249:Asthma / Q000209:etiology* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D011379:Prognosis; D011446:Prospective Studies; D012129:Respiratory Function Tests; D012143:Respiratory Physiological Phenomena; D012307:Risk Factors; D055815:Young Adult