title: A case report of chronic granulomatous disease presenting with aspergillus pneumonia in a 2-month old girl.
abstract: 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-γ 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.
authors.affiliation: Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: Eun
authors.initials: E
authors.lastname: Lee
authors.forename: Seak Hee
authors.initials: SH
authors.lastname: Oh
authors.forename: Ji Won
authors.initials: JW
authors.lastname: Kwon
authors.forename: Byoung Ju
authors.initials: BJ
authors.lastname: Kim
authors.forename: Jinho
authors.initials: J
authors.lastname: Yu
authors.forename: Chan Jeoung
authors.initials: CJ
authors.lastname: Park
authors.forename: Soo Jong
authors.initials: SJ
authors.lastname: Hong