TURKIYE KLINIKLERI TIP BILIMLERI DERGISI, cilt.29, sa.6, ss.1724-1728, 2009 (SCI-Expanded)
The management of chronic respiratory failure and Pseudomonas aeruginosa infections may be difficult in patients with Kartagener's syndrome (KS), characterized by the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus. We reported an adult patient with KS suffering from severe disease course complicated with chronic respiratory failure and frequent acute infectious exacerbations due to P. aeruginosa and treated with inhaled tobramycin and home mechanical ventilation. These patients are difficult to treat and to follow-up and the treatment strategies are not evidence based. Long-term ventilatory assistance at home (non-invasive mechanical ventilation, positive pressure ventilation via tracheostomy) is required for end-stage patients and inhaled antibiotics can be an alternative option for controlling acute infectious exacerbations due to resistant microorganisms without systemic toxicities. In this case report, the possible treatment alternatives for severe KS patients were discussed mainly based on the literature of cystic fibrosis experiences.