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Chronic granulomatous disease: long-term follow-up and prognosis in Serbia = Kronična granulomatozna bolezen: dolgoročno sledenje in napoved izida v SrbijiPašić, Srdjan ...We report on the long-term follow-up of 16 paediatric patients with chronic granulomatous disease diagnosed between 1975 and 2004. Their mean age at the onset of symptoms was 5 months, while the ... median delay in diagnosis was 2.5 years. Bacillus Calmette-Guerin lymphadenitis was the most common presenting infection (7) followed by suppurative lymphadenitis (5), lung infections (1), skin infection (1), liver abscess (1) and Salmonella sepsis (1). Prophylaxis with co-trimoxazole was recommended for all patients, and after 1996 itraconazole was used as antifungal prophylaxis in 8 patients. During the meanfollow-up of 13 years (range, 3 - 30 years) pneumonitis was the most prevalent infection, followed by BCG or suppurative lymphadenitis, skin infections, aphthous stomatitis and liver abscesses. Six (37%) patients died, and in 5 patients lethal outcomes occurred during the second decade of life (4Aspergillus infection, 2 chronic lung disease). The probability of survival into the third decade was estimated to be only 19%. However, in eight surviving patients with documented strict use of antibacterial and antifungal prophylaxis, better survival into adulthood may be expected. In view of the poor long-term prognosis, early haematopoetic stem cell transplantation shouldbe considered, especially in patients with a suitable HLA-identical donor.Source: Slovenska pediatrija : revija Združenja pediatrov Slovenije in Združenja specialistov šolske in visokošolske medicine Slovenije. - ISSN 1318-4423 (Letn. 15, št. 1, 2008, str. 87-92)Type of material - article, component partPublish date - 2008Language - englishCOBISS.SI-ID - 24524249
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source: Slovenska pediatrija : revija Združenja pediatrov Slovenije in Združenja specialistov šolske in visokošolske medicine Slovenije. - ISSN 1318-4423 (Letn. 15, št. 1, 2008, str. 87-92)
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Pašić, Srdjan | ![]() |
Bonači-Nikolić, Branka | ![]() |
Nikolić, Miloš M. | ![]() |
Veljković, Danijela | ![]() |
Slavković, B. | ![]() |
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