European Journal of Pediatrics

, Volume 166, Issue 1, pp 83–84 | Cite as

Invasive aspergillosis in chronic granulomatous disease: report of 7 cases

  • Setareh Mamishi
  • Nima Parvaneh
  • Ali Salavati
  • Sina Abdollahzadeh
  • Mehdi Yeganeh
Short Report
Chronic granulomatous disease (CGD) is an inherited disorder of the NADPH oxidase complex characterized by recurrent bacterial and fungal infections. The underlying defect is an inability to generate reactive oxygen intermediates and activation of intracellular proteases, which are key elements in host defense [ 5]. The incidence of fungal infections in CGD has been reported to be 20%, with Aspergillus spp accounting for 78% of them [ 2]. We report seven CGD patients with invasive aspergillosis (IA) and describe their clinical picture (Table  1). The diagnostic criteria for CGD were abnormal nitroblue tetrazolium test (NBT), and/or dihydrorhodamin 123 (DHR) assay. The X-linked form of CGD was suspected by the pattern of inheritance, abnormal NBT or mosaic DHR pattern in mothers.
Table 1

Characteristics of CGD patients affected with invasive aspergillosis



Age of infection

Involved organ

Isolated fungi

ESR (mm/hr)

Prophylaxis before infection


Duration of treatment...


Chronic granulomatous disease CGD Invasive aspergillosis 


  1. 1.
    Almyroudis NG, Holland SM, Segal BH (2005) Invasive aspergillosis in primary immunodeficiencies. Med Mycol 43(Suppl 1):S247–S259PubMedGoogle Scholar
  2. 2.
    Cohen MS, Isturiz RE, Malech HL, Root RK, Wilfert CM, Gutman L, Buckley RH (1981) Fungal infection in chronic granulomatous disease. The importance of the phagocyte in defense against fungi. Am J Med 71:59–66PubMedCrossRefGoogle Scholar
  3. 3.
    Gallin JI, Alling DW, Malech HL, Wesley R, Koziol D, Marciano B, Eisenstein EM, Turner ML, DeCarlo ES, Starling JM, Holland SM (2003) Itraconazole to prevent fungal infections in chronic granulomatous disease. N Engl J Med 348:2416–2422PubMedCrossRefGoogle Scholar
  4. 4.
    Herbrecht R, Denning DW, Patterson TF, Bennett JE, Greene RE, Oestmann JW, Kern WV, Marr KA, Ribaud P, Lortholary O, Sylvester R, Rubin RH, Wingard JR, Stark P, Durand C, Caillot D, Thiel E, Chandrasekar PH, Hodges MR, Schlamm HT, Troke PF, de Pauw B (2002) Voriconazole versus amphotericin B for primary therapy of invasive aspergillosis. N Engl J Med 347:408–415PubMedCrossRefGoogle Scholar
  5. 5.
    Reeves EP, Lu H, Jacobs HL, Messina CG, Bolsover S, Gabella G, Potma EO, Warley A, Roes J, Segal AW (2002) Killing activity of neutrophils is mediated through activation of proteases by K+flux. Nature 416:291–297PubMedCrossRefGoogle Scholar
  6. 6.
    Segal BH, Barnhart LA, Anderson VL, Walsh TJ, Malech HL, Holland SM (2005) Posaconazole as salvage therapy in patients with chronic granulomatous disease and invasive filamentous fungal infection. Clin Infect Dis 40:1684–1688PubMedCrossRefGoogle Scholar
  7. 7.
    Segal BH, DeCarlo ES, Kwon-Chung KJ, Malech HL, Gallin JI, Holland SM (1998) Aspergillus nidulans infection in chronic granulomatous disease. Medicine 77:345–354PubMedCrossRefGoogle Scholar
  8. 8.
    Segal BH, Leto TL, Gallin JI, Malech HL, Holland SM (2000) Genetic, biochemical, and clinical features of chronic granulomatous disease. Medicine 79:170–200PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Setareh Mamishi
    • 1
  • Nima Parvaneh
    • 1
  • Ali Salavati
    • 2
  • Sina Abdollahzadeh
    • 2
  • Mehdi Yeganeh
    • 3
  1. 1.Department of Pediatrics, Infectious Disease Research Center, Children’s Medical CenterTehran University of Medical SciencesTehranIran
  2. 2.Student’s Scientific Research CenterTehran University of Medical SciencesTehranIran
  3. 3.Immunology, Asthma and Allergy Research InstituteTehran University of Medical SciencesTehranIran

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