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Lookup NU author(s): Dr Desa Lilic, Dr Mario Abinun
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We report the long-term follow-up of 12 pediatric-aged patients with chronic granulomatous disease (CGD). The mean age at the onset of infections was 5 months with a median delay in diagnosis of 2.5 years. Bacille Calmette-Guérin lymphadenitis was the most common presenting infection (6) followed by suppurative lymphadenitis (4), liver abscess (1), or Salmonella sepsis (1). Prophylaxis with cotrimoxazole was recommended to all patients. During the mean follow-up of 10 years (range, 4-23 years) pneumonitis was the most prevalent infection (91%) followed by lymphadenitis (83%), aphtous stomatitis (58%), and liver abscesses (25%). Seven (58%) patients developed chronic lung disease due to grossly delayed diagnosis (3) or poor compliance with antimicrobial prophylaxis (4). Five (41%) patients died during the second decade of life of aspergillosis (3) or chronic lung disease (2). Probability of survival into the third decade of life was estimated to be only 19%. We argue that HLA-identical bone marrow transplantation (BMT), if possible, should be attempted at early age because of significant morbidity and mortality in adolescence. BMT also should be considered in patients who suffer severe infections despite antimicrobial prophylaxis or patients with evidence of chronic lung disease. Possibility of elective BMT from unrelated donors remains to be carefully evaluated.
Author(s): Pasic S, Minic A, Minic P, Veljkovic D, Lilic D, Slavkovic B, Pejnovic N, Abinun M
Publication type: Article
Publication status: Published
Journal: Journal of Clinical Immunology
Year: 2003
Volume: 23
Issue: 1
Pages: 55-61
Print publication date: 01/01/2003
ISSN (print): 0271-9142
ISSN (electronic): 1573-2592
Publisher: Springer
URL: http://dx.doi.org/10.1023/A:1021952315651
DOI: 10.1023/A:1021952315651
PubMed id: 12645860
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