Fungal infection and increased mortality in patients with chronic granulomatous disease

SummaryObjectiveFungal infection presents a serious risk to individuals with compromised immune systems. Chronic granulomatous disease is a primary immunodeficiency with X-linked or autosomal recessive inheritance. Patients with CGD are predisposed to bacterial and fungal infections. The aim of this study was to determine the incidence of fungal infections, identify the most common fungal pathogens, and determine the risk factors associated with fungal infections and mortality in patients with chronic granulomatous disease (CGD).Material and methodsWe reviewed retrospectively 12 patients with CGD in the period of 1995–2008. All of the patients were suspected to fungal infections. The data was gathered from the medical records of all patients as having CGD. Twelve patients had adequate medical records to enter the study. The diagnostic of fungal infections were confirmed by histopathology and direct preparation, culture techniques, histopathology of surgical biopsies, and radiological examination of the affected site.ResultsWe evaluated 12 cases of chronic granulomatosis. Patients that are susceptible to recurrent, sever infections. Patients consisted of seven males and five females. The median age of patients at the time of the study was 11.66 years (3 to18). Neutrophil oxidative burst were absent (NBT = 0) in all patients. Fungal infections were confirmed in five patients (41/7%) by histology and mycological methods. The most common isolated fungi in this study were Aspergillus spp. Out of five cases of fungal infections identified, tree were Aspergillus spp, and two Fusarium spp. The most common manifestations of CGD due to fungal infections (in descending order) were osteomyelitis (42.8%), pulmonary infections (28.6%), lymphadenopathy (14.3%) and skin involvement (14.3%) during their illness.ConclusionInvasive fungal infections are a frequent and life-threatening complication in CGD patients. The lungs and skeletal, were the most commonly affected organ; however, lymphatic, and skin involvement have also been described. Our present study showed that fusariosis also is a threat to CGD patients.

RésuméObjectifDéterminer l’incidence des infections fongiques, identifier les champignons responsables et étudier les facteurs de risque et la mortalité chez des patients atteints de granulomatose chronique (CGD).Matériel et méthodesNous avons examiné 12 patients atteints de granulomatose chronique au cours des années 1995 à 2008. Tous ces patients étaient suspectés d’infection fongique. Ce diagnostic a été confirmé par examen histopathologique et mycologique des biopsies et par radiographie des sites infectés.RésultatsParmi les 12 patients examinés il y avait sept hommes et cinq femmes. Leur âge médian était de 11,66 ans (3 à 18). Le burst oxydatif des neutrophiles était absent chez tous les patients. L’infection fongique a été confirmée par des méthodes histologique et mycologique chez cinq patients. Elle était due à Aspergillus sp. pour trois cas et à Fusarium sp. pour deux cas. Les manifestations les plus communes de CGD avec infection fongique étaient l’ostéomyélite (42,8 %), les infections pulmonaires (28,6 %), une lymphadénopathie (14,3 %) et des affections cutanées (14,3 %).ConclusionL’infection fongique invasive est une complication fréquente et mettant en jeu le pronostic vital chez les patients atteints de CGD. Les poumons et le squelette sont les organes les plus affectés ; l’implication des organes lymphatiques et de la peau a été aussi décrite. Notre étude montre que la fusariose est aussi une menace chez ces patients.