A case report of chronic granulomatous disease presenting with aspergillus pneumonia in a 2-month old girl.
10.3345/kjp.2010.53.6.722
- Author:
Eun LEE
1
;
Seak Hee OH
;
Ji Won KWON
;
Byoung Ju KIM
;
Jinho YU
;
Chan Jeoung PARK
;
Soo Jong HONG
Author Information
1. Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea. sjhong@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Chronic granulomatous disease;
Aspergillosis;
Pneumonia;
BAL culture;
Amphotericin B;
Voriconazole;
Infant
- MeSH:
Amphotericin B;
Aspergillosis;
Aspergillus;
Bacteria;
Bronchoalveolar Lavage Fluid;
Burkholderia cepacia;
Catalase;
Follow-Up Studies;
Fungi;
Granulomatous Disease, Chronic;
Homicide;
Humans;
Infant;
Invasive Pulmonary Aspergillosis;
Lung;
NADPH Oxidase;
Nocardia;
Phagocytes;
Pneumonia;
Pulmonary Aspergillosis;
Pyrimidines;
Serologic Tests;
Serratia marcescens;
Staphylococcus aureus;
Triazoles
- From:Korean Journal of Pediatrics
2010;53(6):722-726
- CountryRepublic of Korea
- Language:English
-
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-gamma 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.
