title: Fungal infection and increased mortality in patients with chronic granulomatous disease.
abstract: OBJECTIVE
  Fungal 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).
  
  
  METHODS
  We 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.
  
  
  RESULTS
  We 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.
  
  
  CONCLUSIONS
  Invasive 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.
authors.affiliation: Medical Mycology Department, Pasteur Institute of Iran, Pasteur Street, number 69, P.O. Box: 1316943551, Tehran, Iran. basiri@pasteur.ac.ir
authors.forename: S
authors.initials: S
authors.lastname: Bassiri-Jahromi
authors.forename: A
authors.initials: A
authors.lastname: Doostkam
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000401:mortality*; D006801:Humans; D007492:Iran / Q000453:epidemiology; D008171:Lung Diseases / Q000150:complications / Q000401:mortality; D008206:Lymphatic Diseases / Q000150:complications / Q000401:mortality; D008297:Male; D009181:Mycoses / Q000150:complications / Q000401:mortality*; D010019:Osteomyelitis / Q000150:complications / Q000401:mortality; D012189:Retrospective Studies