Kartagener’s syndrome (KS) is a variant of primary ciliary dyskinesia (PCD). It is an autosomal recessive disease characterized by situs inversus, sinusitis and bronchiectasis. Patients with KS develop chronic and recurrent infections, typically suffering from bronchitis, pneumonia, hemoptysis, sinusitis, and infertility. We present a case of KS with lung abscess caused by Pseudomonas aeruginosa (P. aeruginosa), Mycobacterium avium intracellulare complex (MAC) and Nocardia species (spp).
A 38-year-old white male with established KS presented to the outpatient pulmonary clinic for hemoptysis for nine months and left sided pleuritic chest pain of two weeks duration. He complained of mild dyspnea and anorexia but denied constitutional symptoms. He had received multiple courses of antibiotics and oral steroids over the last 9 months for worsening cough and productive sputum production. The patient was non-adherent with airway clearance measures. A carpenter by profession he had remained a non-smoker all his life. On physical examination enlarged nasal turbinates were observed and bibasilar crackles heard on lung auscultation. Pulmonary function testing was consistent with obstructive ventilatory impairment (FEV1 = 2.52 L (57%) and FEV1/FVC = 55%). A computerized tomography (CT) scan of the chest demonstrated a large left middle lobe cavitary lesion with an air-fluid level and background bronchiectasis with thoraco-abdominal situs inversus. Worsening infiltrates, bronchiectasis, lymphadenopathy and a small left pleural effusion were also noticed. The patient underwent a bronchoscopy with bronchoalveolar lavage (BAL) and a protected specimen bronchial brush. P. aeruginosa, E.coli, Nocardia spp. and MAC were cultures from the specimens. The patient was treated with multiple antimicrobials (see table) with resolution of the abscess. He continues to be on treatment for Nocardia spp. and MAC.
KS is a rare genetic disorder. Patients with KS develop respiratory infections due to ciliary dysfunction and stasis of secretions in the respiratory tract. A systematic study of the microbiology of airway secretions in KS is lacking. Common infectious organisms affecting children with PCD are Haemophilus influenza and Staphylococcus aureus, with P. aeruginosa being more common in adults. Some subjects cultured more than one organism in the same sample: for example, smooth P. aeruginosa with another organism, most commonly nontuberculous mycobacteria .Although pneumonia is frequently seen, lung abscess is rare in KS . Only two previous cases have been reported in medical literature. Of them, one abscess was caused by Streptococcus pneumoniae while the other was secondary to P. aeruginosa. In our patient, the lung abscess involved multiple organisms, namely, P. aeruginosa, MAC and Nocardia spp. To the best of our knowledge, this is the first such case report in English language literature. Nontuberculous mycobacterial infection is considered pathogenic and requires an aggressive multi-drug regimen for eradication. Pulmonary nocardiosis is typically regarded as an opportunistic infection and occurs mostly in immune compromised hosts or patients with underlying lung disease. However, approximately 50% of patients with nocardiosis do not have any predisposing disease. The isolation of Nocardia spp. from the respiratory tract of KS patients in itself, is an unusual finding. The clinical features of these cases are summarized in the table.
Lung abscess infrequently complicates KS. A case of lung abscess with P. aeruginosa, MAC and Nocardia spp. in a patient with KS is presented here. Evaluation of sputum and BAL is often needed to identify organisms. Prompt and appropriate treatment of respiratory infections can minimize irreversible damage in such cases.
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