Right lung agenesis with multi-drug-resistant Cepacia syndrome: A case report

Lung agenesis (LA) is a rare congenital anomaly that refers to an absence of lung tissue and bronchi. <i>Burkholderia cepacia</i> complex (BCC) causes a fatal acute necrotising pneumonia called Cepacia syndrome. The unique concomitant association of both these rare diseases has not been highlighted in the literature to the best of our knowledge. Here, we present a case of association of right-sided lung agenesis along with Cepacia syndrome. In our case, the <i>Burkholderia cepacia</i> complex was found to be a multi-drug-resistant organism. Debilitating deterioration in the oxygen saturation was observed and initiation of treatment after culture sensitivity report yielded in remission of the disease. However, due to underlying congenital agenesis, therapeutic intervention with steroids and bronchodilators led to stabilising the patient by developing oxygenation. Underlying pulmonary congenital defects predispose individuals to infections. The implementation of antimicrobial therapy must be aided with culture and sensitivity reports for better outcomes.

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Publication Details

Journal
Journal of Family Medicine and Primary Care
Published
2025-02-01
DOI
https://doi.org/10.4103/jfmpc.jfmpc_1424_24
Primary Topic
Congenital Diaphragmatic Hernia Studies
Type
article
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article

Right lung agenesis with multi-drug-resistant Cepacia syndrome: A case report

Nikhil Sarangdhar, Anant Patil, Mohit Kondisetti, Priyavardhan Mishra et al.
Journal of Family Medicine and Primary Care
Congenital Diaphragmatic Hernia Studies
article

Right lung agenesis with multi-drug-resistant Cepacia syndrome: A case report

Nikhil Sarangdhar, Anant Patil, Mohit Kondisetti, Priyavardhan Mishra, Sankeerth Chanamolu
article en

Abstract

Lung agenesis (LA) is a rare congenital anomaly that refers to an absence of lung tissue and bronchi. <i>Burkholderia cepacia</i> complex (BCC) causes a fatal acute necrotising pneumonia called Cepacia syndrome. The unique concomitant association of both these rare diseases has not been highlighted in the literature to the best of our knowledge. Here, we present a case of association of right-sided lung agenesis along with Cepacia syndrome. In our case, the <i>Burkholderia cepacia</i> complex was found to be a multi-drug-resistant organism. Debilitating deterioration in the oxygen saturation was observed and initiation of treatment after culture sensitivity report yielded in remission of the disease. However, due to underlying congenital agenesis, therapeutic intervention with steroids and bronchodilators led to stabilising the patient by developing oxygenation. Underlying pulmonary congenital defects predispose individuals to infections. The implementation of antimicrobial therapy must be aided with culture and sensitivity reports for better outcomes.

Journal of Family Medicine and Primary CareVol. 14(2)
D.Y. Patil University (IN)
Openalex Percentile: Top 95%
Congenital Diaphragmatic Hernia Studies
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