Long-term follow-up and prognosis of chronic granulomatous disease in yugoslavia: Is there a role for early bone marrow transplantation?

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...

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Bibliographic Details
Published in:Journal of clinical immunology 2003, Vol.23 (1), p.55-61
Main Authors: PASIC, Srdjan, MINIC, Aleksandra, MINIC, Predrag, VELJKOVIC, Dobrila, LILIC, Desa, SLAVKOVIC, Bojana, PEJNOVIC, Nada, ABINUN, Mario
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aspergillus - pathogenicity
Biological and medical sciences
Bone Marrow Transplantation
Child
Child, Preschool
Female
Granulomatous Disease, Chronic - microbiology
Granulomatous Disease, Chronic - physiopathology
Granulomatous Disease, Chronic - therapy
Hematologic and hematopoietic diseases
Humans
Infant
Male
Medical sciences
Other diseases. Hematologic involvement in other diseases
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Yugoslavia - epidemiology
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Summary: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.
ISSN:0271-9142
1573-2592
DOI:10.1023/A:1021952315651