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Korean J Pediatr 2010 June;53(6) :722-726.
A case report of chronic granulomatous disease presenting with aspergillus pneumonia in a 2-month old girl
Eun Lee (Lee E)1, Seak Hee Oh (Oh SH)1, Ji Won Kwon (Kwon JW)2, Byoung Ju Kim (Kim BJ)2, Jinho Yu (Yu Jh)2, Chan Jeoung Park (Park CJ)1, Soo Jong Hong (Hong SJ)2
1Department of Pediatrics, Asan Medical Center Children`s Hospital, University of Ulsan College of Medicine, Seoul, Korea
2Department of Pediatrics, Childhood Asthma Atopy Center, Asan Medical Center Children`s Hospital, University of Ulsan College of Medicine, Seoul, Korea
Corresponding Author: Soo Jong Hong ,Email:
Copyright © 2010 by The Korean Pediatric Society
Chronic granulomatous disease (CGD) is an uncommon inherited disorder caused by mutations in any of the genes encoding subunits of the superoxide-generating phagocyte NADPH oxidase system, which is essential for killing catalase producing bacteria and fungi, such as Aspergillus species, Staphylococcus aureus, Serratia marcescens, Nocardia species and Burkholderia cepacia . In case of a history of recurrent or persistent infections, immune deficiency should be investigated. Particularly, in the case of uncommon infections such as aspergillosis in early life, CGD should be considered. We describe here a case of CGD that presented with invasive pulmonary aspergillosis in a 2-month-old girl. We confirmed pulmonary aspergillosis noninvasively through a positive result from the culture of bronchial alveolar lavage fluid, positive serological test for Aspergillus antigen and radiology results. She was successfully treated with Amphotericin B and recombinant IFN- initially. Six weeks later after discharge, she was readmitted for pneumonia. Since there were infiltrates on the right lower lung, which were considered as residual lesions, voriconazole therapy was initiated. She showed a favorable response to the treatment and follow-up CT showed regression of the pulmonary infiltrates.
Keywords: Chronic granulomatous disease | Aspergillosis | Pneumonia | BAL culture | Amphotericin B | Voriconazole | Infant
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