1. Chronic Granulomatous Disease: a Cohort of 173 Patients—10-Years Single Center Experience from Egypt.
- Author
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Abd Elaziz, Dalia, EL Hawary, Rabab, Meshaal, Safa, Alkady, Radwa, Lotfy, Sohilla, Eldash, Alia, Erfan, Aya, Chohayeb, Engy, Saad, Mai, Boutros, Jeannette, Galal, Nermeen, and Elmarsafy, Aisha
- Subjects
CHRONIC granulomatous disease ,PRIMARY immunodeficiency diseases ,CHILDREN'S hospitals ,BACTERIAL diseases ,MYCOSES ,BURULI ulcer - Abstract
Purpose: Chronic granulomatous disease (CGD) is an inherited primary immunodeficiency disorder of phagocytes, characterized by recurrent fungal and bacterial infections. Our aim is to describe the different clinical presentations, non-infectious auto-inflammatory features, types and sites of infections, and to estimate the mortality among our large cohort. Methods: This is a retrospective study conducted at the Pediatric Department of Cairo University Children's Hospital in Egypt, including cases with a confirmed CGD diagnosis. Results: One hundred seventy-three confirmed CGD patients were included. AR-CGD was diagnosed in 132 patients (76.3%) including 83 patients (48%) with p47
phox defect, 44 patients (25.4%) with p22phox defect, and 5 patients (2.9%) with p67phox defect. XL-CGD was diagnosed in 25 patients (14.4%). The most common recorded clinical manifestations were deep-seated abscesses and pneumonia. Gram-negative bacteria and Aspergillus were the most frequently isolated species. Regarding the outcome, 36 patients (20.8%) were lost from follow-up. Among patients with known outcome, 94/137 patients (68.6%) are living, while 43/137 patients (31.4%) died. Conclusion: AR-CGD is predominant in Egypt; CGD must always be ruled out in any patient presenting with typical or atypical mycobacterial or BCG-disease. [ABSTRACT FROM AUTHOR]- Published
- 2023
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