Avtor/Urednik		Pašić, S; Bonači-Nikolić, B; Nikolić, M; Veljković, D; Slavković, B
Naslov		Chronic granulomatous disease: long-term follow-up and prognosis in Serbia
Prevedeni naslov		Kronična granulomatozna bolezen: dolgoročno sledenje in napoved izida v Srbiji
Tip		članek
Vir		Slov Pediatr
Vol. in št.		Letnik 15, št. 1
Leto izdaje		2008
Obseg		str. 87-92
Jezik		eng
Abstrakt		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 mean follow-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 (4 Aspergillus 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 should be considered, especially in patients with a suitable HLA-identical donor.
Deskriptorji		GRANULOMATOUS DISEASE, CHRONIC FOLLOW-UP STUDIES