title: Lung function and airway hyperresponsiveness in adult patients with sickle cell disease.
abstract: BACKGROUND
  Lung disease is a major cause of morbidity and death in sickle cell disease. Although airway hyperresponsiveness has been noted in children, there are no studies in adult sickle cell patients. The aim of this study was to investigate the prevalence of airway hyperresponsiveness in adult sickle cell patients.
  
  
  METHODS
  Twenty-six patients with sickle cell disease (10 HbSC, 9 HbSS, and 7 HbSbeta) were compared with 28 normal control subjects. Pulmonary function tests, including spirometry, measurements of single-breath diffusing capacity and the methacholine challenge test were performed.
  
  
  RESULTS
  There were no significant differences in age, gender, or height between groups. Restrictive ventilatory defect was observed in six patients (24%) in the sickle cell disease group. Obstructive ventilatory defect and reduced diffusing lung DLCO capacity was observed in all sickle cell disease subgroups. A positive methacholine challenge test was obtained in eight (31%) sickle cell patients and in two of the 28 controls (7%).
  
  
  CONCLUSIONS
  These features suggest that there is a high prevalence of airway hyperresponsiveness in adult patients with sickle cell disease without a history of reactive airway disease.
authors.affiliation: Department of Medicine, University of S. Paulo Medical School at Ribeirão Preto.. Electronic address: elisavn@hotmail.com.
authors.forename: Elisa Cristina
authors.initials: EC
authors.lastname: Vendramini
authors.affiliation: Department of Medicine, University of S. Paulo Medical School at Ribeirão Preto.
authors.forename: Elcio O
authors.initials: EO
authors.lastname: Vianna
authors.affiliation: Department of Medicine, University of S. Paulo Medical School at Ribeirão Preto.
authors.forename: Ivan
authors.initials: I
authors.lastname: De Lucena Ðngulo
authors.affiliation: Department of Medicine, University of S. Paulo Medical School at Ribeirão Preto.
authors.forename: Flavia Bueno
authors.initials: FB
authors.lastname: De Castro
authors.affiliation: Department of Medicine, University of S. Paulo Medical School at Ribeirão Preto.
authors.forename: José A B
authors.initials: JAB
authors.lastname: Martinez
authors.affiliation: Department of Medicine, University of S. Paulo Medical School at Ribeirão Preto.
authors.forename: João
authors.initials: J
authors.lastname: Terra-Filho
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000150:complications / Q000401:mortality / Q000503:physiopathology*; D016535:Bronchial Hyperreactivity / Q000209:etiology / Q000401:mortality / Q000503:physiopathology*; D016085:Bronchoconstrictor Agents / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008173:Lung Diseases, Obstructive / Q000209:etiology / Q000401:mortality / Q000503:physiopathology*; D008297:Male; D016210:Methacholine Chloride / Q000008:administration & dosage; D008875:Middle Aged; D012129:Respiratory Function Tests / Q000379:methods; D012130:Respiratory Hypersensitivity / Q000209:etiology / Q000401:mortality / Q000503:physiopathology*; D012737:Sex Factors
chemical_list: D016085:Bronchoconstrictor Agents; D016210:Methacholine Chloride