Kartegener Syndrome Presenting With Acute Bronchiectasis Exacerbation Secondary to Achromobacter Xyloxidians Infection

Document Type

Conference Proceeding

Publication Date

5-2024

Publication Title

American Journal of Respiratory and Critical Care Medicine

Abstract

Introduction: Kartagener syndrome is a subgroup of primary ciliary dyskinesia (PCD), characterized by situs inversus, chronic sinusitis, and bronchiectasis. It has a prevalence of around 1 in 20,000 to 40,000 individuals. Approximately 66% of patients with PCD have defects in the outer dynein arm of the cilia. Despite having a distinct pathophysiology from cystic fibrosis, which results from electrolyte imbalance related to the CFTR protein, there is microbiological overlap between the two conditions based on the common grounds of defective mucociliary clearance. Common respiratory pathogens for both conditions include Staphylococcus aureus, Pseudomonas aeruginosa, and Haemophilus influenzae. However, certain organisms like Achromobacter xylosoxidans,Burkholderia cepacia, and Ralstonia species are more common in cystic fibrosis, with a prevalence of less than 5% in PCD. Case Report:A 43-year-old female with a past medical history of Kartagener syndrome presented with chief complaints of fever,chills, productive cough, and hemoptysis for 3 days. Additionally, she had malaise, body aches, left-sided chest pressure, and shortness of breath that increased when lying down. She had a fever of 101°F and was tachypneic with a respiratory rate ranging between 20-25. Laboratory tests revealed neutrophilic leukocytosis (WBC: 14.4bil/l). She had a history of recurrent lower respiratory tract infections since childhood; she took Azithromycin 250 mg once daily and inhaled bronchodilators during these flares. CT scan of the chest showed interval worsening of left reticulonodular opacities along with left lower lobe bronchiectasis and bronchiolitis. Pulmonary function tests showed restrictive physiology (FVC: 65%, FEV1: 67%, FEV/FVC: 84%, DLCO: 75%).Sputum culture grew Achromobacter xylosoxidans. She was treated with IV meropenem based on culture sensitivity. The fever and hemoptysis resolved by day 2 and the cough improved by day 4. She was discharged on a 14-day course of Bactrim. Discussion: The microbiology in primary ciliary dyskinesia is much less characterized compared to cystic fibrosis. Consequently, there is a lack of guidelines for primary, secondary, and tertiary management of infections and exacerbations caused by rare organisms like Achromobacter xylosoxidians. The mainstay of treatment is regular microbiological surveillance; BEAT-PCD network suggests routine screening and treatment for Pseudomonas aeruginosa, Burkholderia cepacia, and Nontuberculous Mycobacteria. Maintenance therapy with Azithromycin has been shown to reduce the number of exacerbations. Lung resection inpatients with localized disease has been explored; however, lung decline seems to progress in lobectomized individuals.

Volume

209

Issue

Suppl

First Page

A2406

Comments

International Conference of the American Thoracic Society, May 17 -22, 2024, San Diego, CA

DOI

10.1164/ajrccm-conference.2024.209.1_MeetingAbstracts.A2406

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