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Undiagnosed Chronic Granulomatous Disease, Burkholderia cepacia complex Pneumonia, and Acquired Hemophagocytic Lymphohistiocytosis : A Deadly Association
Joint Authors
Maignan, Maxime
Verdant, Colin
Berthiaume, Yves
Van Spall, Michael
Bouvet, Guillaume F.
Source
Issue
Vol. 2013, Issue 2013 (31 Dec. 2013), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2013-08-24
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
Background.
Chronic granulomatous disease is a rare inherited disorder of the phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase.
The clinical course of the disease is marked by recurrent infections, including Burkholderia cepacia complex infection.
Case Report.
Here we report the case of a 21-year-old male hospitalized for a Burkholderia cepacia complex pneumonia.
Despite the broad spectrum antibiotic treatment, fever continued and patient’s condition worsened.
Anemia and thrombocytopenia developed together with hypofibrinogenemia.
The patient died of multiple organ dysfunction 17 days after his admission.
Autopsy revealed hemophagocytosis, suggesting the diagnosis of acquired hemophagocytic lymphohistiocytosis.
DNA analysis showed a deletion in the p47phox gene, confirming the diagnosis of autosomal recessive chronic granulomatous disease.
Discussion.
In addition to chronic granulomatous disease, recent findings have demonstrated that Burkholderia cepacia complex can decrease activity of the NADPH oxidase.
Interestingly, hemophagocytic lymphohistiocytosis is characterized by an impaired function of the T-cell mediated inflammation which is partly regulated by the NADPH oxidase.
Physicians should therefore pay particular attention to this deadly association.
American Psychological Association (APA)
Maignan, Maxime& Verdant, Colin& Bouvet, Guillaume F.& Van Spall, Michael& Berthiaume, Yves. 2013. Undiagnosed Chronic Granulomatous Disease, Burkholderia cepacia complex Pneumonia, and Acquired Hemophagocytic Lymphohistiocytosis : A Deadly Association. Case Reports in Pulmonology،Vol. 2013, no. 2013, pp.1-5.
https://search.emarefa.net/detail/BIM-505271
Modern Language Association (MLA)
Maignan, Maxime…[et al.]. Undiagnosed Chronic Granulomatous Disease, Burkholderia cepacia complex Pneumonia, and Acquired Hemophagocytic Lymphohistiocytosis : A Deadly Association. Case Reports in Pulmonology No. 2013 (2013), pp.1-5.
https://search.emarefa.net/detail/BIM-505271
American Medical Association (AMA)
Maignan, Maxime& Verdant, Colin& Bouvet, Guillaume F.& Van Spall, Michael& Berthiaume, Yves. Undiagnosed Chronic Granulomatous Disease, Burkholderia cepacia complex Pneumonia, and Acquired Hemophagocytic Lymphohistiocytosis : A Deadly Association. Case Reports in Pulmonology. 2013. Vol. 2013, no. 2013, pp.1-5.
https://search.emarefa.net/detail/BIM-505271
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-505271