Chronic Granulomatous Disease: a Cohort of 173 Patients-10-Years Single Center Experience from Egypt.


Journal

Journal of clinical immunology
ISSN: 1573-2592
Titre abrégé: J Clin Immunol
Pays: Netherlands
ID NLM: 8102137

Informations de publication

Date de publication:
11 2023
Historique:
received: 21 02 2023
accepted: 19 06 2023
medline: 21 11 2023
pubmed: 12 7 2023
entrez: 11 7 2023
Statut: ppublish

Résumé

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. 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. One hundred seventy-three confirmed CGD patients were included. AR-CGD was diagnosed in 132 patients (76.3%) including 83 patients (48%) with p47 AR-CGD is predominant in Egypt; CGD must always be ruled out in any patient presenting with typical or atypical mycobacterial or BCG-disease.

Identifiants

pubmed: 37433991
doi: 10.1007/s10875-023-01541-4
pii: 10.1007/s10875-023-01541-4
pmc: PMC10661789
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1799-1811

Informations de copyright

© 2023. The Author(s).

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Auteurs

Dalia Abd Elaziz (D)

Pediatric Department, Faculty of Medicine, Cairo University, Cairo, Egypt. dalia.abdelaziz@kasralainy.edu.eg.

Rabab El Hawary (R)

Clinical Pathology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Safa Meshaal (S)

Clinical Pathology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Radwa Alkady (R)

Pediatric Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Sohilla Lotfy (S)

Pediatric Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Alia Eldash (A)

Clinical Pathology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Aya Erfan (A)

Clinical Pathology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Engy Chohayeb (E)

Pediatric Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Mai Saad (M)

Pediatric Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Jeannette Boutros (J)

Pediatric Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Nermeen Galal (N)

Pediatric Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

Aisha Elmarsafy (A)

Pediatric Department, Faculty of Medicine, Cairo University, Cairo, Egypt.

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