TY  - JOUR
TI  - The Efficacy of Long-Term Voriconazole Prophylaxis on
Seven Patients with Chronic Granulomatous Disease and
Review of the Literature
AB  - Objective: Chronic granulomatous disease (CGD) a primary immunodeficiency
disease that affects the innate immune system, and characterized
by life-threatening and/or recurrent infections caused by bacteria
and fungi. Invasive fungal diseases (IFDs) are the most common causes
of death among CGD patients. Itraconazole is usually used for prophylaxis
in CGD patients at high risk of IFD. However, patient compliance with
oral itraconazole use is low due to gastrointestinal side effects. We aim
to report the efficacy of long-term voriconazole prophylaxis for IFD on
our CGD patients.
Material and Methods: This retrospective study includes seven CGD
patients. All data were recorded from their hospital registries. CGD was
diagnosed with both functional and genetic analysis. Patients who had
used voriconazole prophylaxis for six months or more after clinical and/
or laboratory diagnosis of IFD were included in this study. Invasive fungal
diseases were classified as proven, probable, or possible according to the
revised criteria of the guideline.
Results: All seven patients were male. Two had an X-linked recessive subtype
of CGD (X-CGD), and the others had autosomal recessive subtypes
of CGD. The median age at diagnosis was 7 (4.5-19) years. Aspergillus spp
was showed in three of patients. The median age at which first IFD oc-curred was 10 (4.5-19) years. The median follow-up period was 6 (3-16)
years. After voriconazole prophylaxis, 6 patients did not suffer from IFD
second time. Only one patient, who has X-CGD, suffered from multiple
fungal osteomyelitis and recurrent IFD despite undergoing voriconazole
prophylaxis. Only one patient developed a lupus-like lesion in the cheek,
which resolved after discontinuation of voriconazole prophylaxis.
Conclusion: Long-term voriconazole prophylaxis may be an alternative
option in CGD patients with IFD in terms of patient compliance, efficacy
and side effects.
AU  - Kökcü karadağ, Şefika ilknur
AU  - BIRINCI, ASUMAN
AU  - ŞENSOY, S. Gülnar
AU  - koker, Mustafa Yavuz
AU  - Kutluğ, Şeyhan
AU  - YILDIRAN, ALISAN
DO  - 10.5578/ced.20219601
PY  - 2021
JO  - Çocuk Enfeksiyon Dergisi
VL  - 15
IS  - 4
SN  - 1307-1068
SP  - 203
EP  - 209
DB  - TRDizin
UR  - http://search/yayin/detay/514417
ER  -