Lung function and airway hyperresponsiveness in adult patients with sickle cell disease

• Published in: The American journal of the medical sciences Vol. 332; no. 2; p. 68
• Main Authors: Vendramini, Elisa Cristina, Vianna, Elcio O, De Lucena Ðngulo, Ivan, De Castro, Flavia Bueno, Martinez, José A B, Terra-Filho, João
• Format: Journal Article
• Language: English
• Published: United States 01-08-2006
• Subjects: Adolescent; Adult; Age Factors; Anemia, Sickle Cell - complications; Anemia, Sickle Cell - mortality; Anemia, Sickle Cell - physiopathology; Bronchial Hyperreactivity - etiology; Bronchial Hyperreactivity - mortality; Bronchial Hyperreactivity - physiopathology; Bronchoconstrictor Agents - administration & dosage; Female; Humans; Lung Diseases, Obstructive - etiology; Lung Diseases, Obstructive - mortality; Lung Diseases, Obstructive - physiopathology; Male; Methacholine Chloride - administration & dosage; Middle Aged; Respiratory Function Tests - methods; Respiratory Hypersensitivity - etiology; Respiratory Hypersensitivity - mortality; Respiratory Hypersensitivity - physiopathology; Sex Factors
• ISSN: 0002-9629
• DOI: 10.1097/00000441-200608000-00003

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.
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.
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%).
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.