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Chronic granulomatous disease presenting as fulminant Aspergillus pneumonitis: A lethal combination?

Lookup NU author(s): Dr Arun Gupta, Dr Michael McKean, Dr Christopher Wright, Dr Terence Flood, Katherine Gould, Julian Smith, Dr Fraser Charlton, Dr Mario Abinun


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Objective: Case reports of two patients with unusually late initial presentation of chronic granulomatous disease with fulminant Aspergillus pneumonia. Data Sources and Extraction: Medical notes; retrospective study. Study Selection: Identical pattern of clinical presentation in two patients referred for support with extracorporeal membrane oxygenation (ECMO). Our Institutional Review Board waived the need for consent. Data Synthesis: Two school-aged boys presented with features of, and were initially treated, for community-acquired pneumonia. However, the disease course was rapidly progressive to fulminant respiratory failure and because both failed conventional intensive care management, they were referred to ECMO support. Although both died of evolving multiorgan failure, ECMO support allowed open lung biopsy leading to diagnosis of invasive Aspergillus pneumonia and chronic granulomatous disease. Conclusions: Failure of adequate therapy for acute community-acquired pneumonia and rapid progression to respiratory failure should lead to the possibility of fungal etiology. Congenital immunodeficiency may present for the first time late in life, so acute invasive pulmonary aspergillosis in the absence of known risk factors should lead to consideration of chronic granulomatous disease regardless of patient age. © 2009 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.

Publication metadata

Author(s): Gupta A, McKean M, Haynes S, Wright C, Barge D, Flood T, Gould K, Smith J, Charlton F, Mannikar S, Abinun M, Cassidy J

Publication type: Article

Publication status: Published

Journal: Pediatric Critical Care Medicine

Year: 2009

Volume: 10

Issue: 4

Pages: e43-e45

ISSN (print): 1529-7535

ISSN (electronic): 1947-3893

Publisher: Lippincott Williams & Wilkins


DOI: 10.1097/PCC.0b013e318198b22a

PubMed id: 19584634


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