title: Invasive mold infections in chronic granulomatous disease: a 25-year retrospective survey.
abstract: BACKGROUND
  Invasive fungal infection (IFI) represents a life-threatening condition for patients with chronic granulomatous disease (CGD) and causes one-third of deaths in this population. This study offers a descriptive review of invasive mold infection (mIFI) in children with CGD over an extended period of time.
  
  
  METHODS
  In a cohort of patients with CGD registered in the French National database for Primary Immunodeficiency, we performed a retrospective review of proven mIFI episodes (European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group 2008 criteria) occurring from 1984 through 2009.
  
  
  RESULTS
  Twenty-nine proven mIFIs were identified in 24 patients. Thirteen (54%) of 24 children were receiving itraconazole prophylaxis. Seven episodes were caused by Aspergillus fumigatus, 10 by Aspergillus nidulans, 2 by Aspergillus species, and 6 by other opportunistic molds (4 patients only had positive pathological examination findings). First proven mIFI occurred later in the group that received itraconazole than in the group without (median time to mIFI, 10 vs 4 years; P < .01), with a higher proportion of infections due to A. nidulans and other opportunistic molds (P < .05). Course of IFI was complex, with the median duration of therapy and hospitalization reaching 446 and 153 days, respectively. Combined antifungal therapy was commonly used. Four patients received geno-identical hematopoietic stem cell transplantation as salvage therapy. Global cure rate among the cohort reached 75%, but sequelae were frequent. Prognosis has improved over time (43% mortality during 1985-1990 vs 6% thereafter; P = .06). Mortality tended to be lower in the group that recieved itraconazole prophylaxis but at the cost of a longer duration of therapy among cured patients.
  
  
  CONCLUSIONS
  Management of mIFI remains challenging in patients with CGD, but significant improvements have been made over the past decade.
authors.affiliation: Immunology and Haematology Unit, Hôpital Necker Enfants Malades, Paris, France. sophie.blumental@ulb.ac.be
authors.forename: Sophie
authors.initials: S
authors.lastname: Blumental
authors.forename: Richard
authors.initials: R
authors.lastname: Mouy
authors.forename: Nizar
authors.initials: N
authors.lastname: Mahlaoui
authors.forename: Marie-Elisabeth
authors.initials: ME
authors.lastname: Bougnoux
authors.forename: Marianne
authors.initials: M
authors.lastname: Debré
authors.forename: Julien
authors.initials: J
authors.lastname: Beauté
authors.forename: Olivier
authors.initials: O
authors.lastname: Lortholary
authors.forename: Stéphane
authors.initials: S
authors.lastname: Blanche
authors.forename: Alain
authors.initials: A
authors.lastname: Fischer
mesh_terms: D000293:Adolescent; D000935:Antifungal Agents / Q000008:administration & dosage; D001230:Aspergillus; D001232:Aspergillus fumigatus; D001233:Aspergillus nidulans; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination / Q000379:methods; D005260:Female; D005602:France / Q000453:epidemiology; D005658:Fungi / Q000145:classification* / Q000302:isolation & purification*; D006105:Granulomatous Disease, Chronic / Q000150:complications*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D009181:Mycoses / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000935:Antifungal Agents