Published ahead of print on October 9, 2003, doi:10.1164/rccm.200304-558OC
© 2004 American Thoracic Society Airway Inflammation and Infection in Congenital Bilateral Absence of the Vas DeferensDepartment of Respiratory Medicine and Allergology, Sahlgrenska University Hospital, Göteborg University, Göteborg, Sweden; Division of Respirology, Department of Medicine, University of Sherbrooke, Sherbrooke, Quebec; Adult Cystic Fibrosis Centre, and Diagnostic Laboratories and Pathobiology, St Michael's Hospital; Toronto General Hospital Research Institute; Research Institute, Hospital for Sick Children; and Division of Respirology, Department of Medicine, Department of Pediatrics, and Department of Medical and Molecular Genetics, University of Toronto, Toronto, Ontario, Canada Correspondence and requests for reprints should be addressed to Elizabeth Tullis, M.D., St. Michael's Hospital, 30 Bond Street, Toronto, Ontario, Canada M5B 1W8. E-mail: tullise{at}smh.toronto.on.ca
In cystic fibrosis (CF), airway disease begins early in life. Bacteria and elevated levels of neutrophils and inflammatory mediators have been detected in bronchoalveolar lavage (BAL) fluid from infants with CF. Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) are common in men with congenital bilateral absence of the vas deferens (CBAVD) and it has been suggested that this syndrome represents a mild form of CF. We hypothesized that men with CBAVD also have subclinical pulmonary disease. Bronchoscopy with BAL, viral and quantitative bacterial cultures, and analyses of total and differential cell count, cytokines, and free neutrophil elastase was performed in eight men with CBAVD, who had mutations in the CFTR and intermediate or elevated sweat chloride levels, and in four healthy control subjects. There was light growth of Staphylococcus aureus in one of eight men with CBAVD, and small numbers of opportunistic gram-negative bacteria in six of eight men with CBAVD and in one control subject. BAL cell counts and neutrophil elastase were within the normal range. Interleukin-8 and tumor necrosis factor-
Key Words: bronchoalveolar lavage • cystic fibrosis • cystic fibrosis transmembrane conductance regulator • infection • inflammation This article has been cited by other articles:
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levels were higher for men with CBAVD than for control subjects. These data suggest that mutations in the CFTR in men with CBAVD, in addition to causing infertility, lead to subclinical bacterial pulmonary infection and inflammation consistent with mild CF. 
