Table 3. Clinical features of patients... | Rockefeller University Press

Table 3.

Clinical features of patients with CGD from the MENA region compared with those from the USA and Europe

	MENA (2025)	USA (2000) (47)	Europe (2009) (48)
Number of patients	322	368	429
Sex	Males 64.3%	Males 85.9%	Males 81.8%
Age at onset of symptoms	Mean 20.2 mo Median 6.0 mo	N/A	N/A
Age at diagnosis	Mean 40.1 mo Median 20.0 mo	Mean XL-CGD 3.0 years AR-CGD 7.8 years	Mean XL-CGD 4.9 years AR 8.8 years
Diagnosis delay	17 mo	N/A	N/A
Consanguinity	60.2%	N/A	N/A
CGD diagnosis	Genetic diagnosis 30.4% XL-CGD 15.7% AR-CGD 84.3%	XL-CGD 69.8% AR-CGD 22%	XL-CGD 67% AR-CGD 33%
Genotype	gp91phox 16/98 (16.3%) p47phox 44/98 (44.9%) p67phox 27/98 (27.6%) p22phox 9/98 (9.2%) p40phox 2/98 (2.0%)	gp91phox 259/368 (70.4%) p47phox 45/81 (56%) p67phox 10/81 (12%) p22phox 7/81 (8%)	gp91phox 290 (67%) p47phox 69/139 (49%) p67phox 11/139 (8%) p22phox 22/139 (16%)
Common infections	Pneumonia 58.7% Lymphadenitis 49% Skin abscess 41%	Pneumonia 79% Abscess 68% Adenitis 53%	Lung 66% Skin 53% Lymph nodes 50%
Common microorganisms	BCG adenitis 28.6% Staphylococcus spp. 22% Aspergillus spp. 19.3% Disseminated BCG 12.1%	Aspergillus spp. 41% Staphylococcus spp. 41.2% Serratia spp. 24.5%	S. aureus 30% Aspergillus spp. 26% Salmonella spp. 16% BCGitis 8%
Immune dysregulation	AIHA 16.8% ITP 6.2% Colitis 5.6% SLE 1.6%	Lupus syndromes 3.3% Colitis 17.4% Granuloma 26.4% ITP 1.4%	N/A
Management	Cotrimoxazole 67% Itraconazole 69.6% IFN-g 14% Granulocyte Tx. 1 patient HSCT 11.8%	Antibiotics 89% IFN-g 73% Granulocyte Tx. 15% HSCT 2 patients	Antibiotics 71% Antifungal 53% IFN-g 33% Granulocyte Tx. 7% HSCT 6%
Mortality and survival	16.5% Median survival age XL-CGD 4.0 years AR-CGD 14.0 years 10-year survival XL-CGD 49.6% AR-CGD 88.0%	17.6% 5-year follow-up XL-CGD 76% AR-CGD 88%	20% Median death age XL-CGD 8.8 years AR-CGD 10.4 years

	MENA (2025)	USA (2000) (47)	Europe (2009) (48)
Number of patients	322	368	429
Sex	Males 64.3%	Males 85.9%	Males 81.8%
Age at onset of symptoms	Mean 20.2 moMedian 6.0 mo	N/A	N/A
Age at diagnosis	Mean 40.1 moMedian 20.0 mo	MeanXL-CGD 3.0 yearsAR-CGD 7.8 years	MeanXL-CGD 4.9 yearsAR 8.8 years
Diagnosis delay	17 mo	N/A	N/A
Consanguinity	60.2%	N/A	N/A
CGD diagnosis	Genetic diagnosis 30.4%XL-CGD 15.7%AR-CGD 84.3%	XL-CGD 69.8%AR-CGD 22%	XL-CGD 67%AR-CGD 33%
Genotype	gp91phox 16/98 (16.3%)p47phox 44/98 (44.9%)p67phox 27/98 (27.6%)p22phox 9/98 (9.2%)p40phox 2/98 (2.0%)	gp91phox 259/368 (70.4%)p47phox 45/81 (56%)p67phox 10/81 (12%)p22phox 7/81 (8%)	gp91phox 290 (67%)p47phox 69/139 (49%)p67phox 11/139 (8%)p22phox 22/139 (16%)
Common infections	Pneumonia 58.7%Lymphadenitis 49%Skin abscess 41%	Pneumonia 79%Abscess 68%Adenitis 53%	Lung 66%Skin 53%Lymph nodes 50%
Common microorganisms	BCG adenitis 28.6%Staphylococcus spp. 22%Aspergillus spp. 19.3%Disseminated BCG 12.1%	Aspergillus spp. 41%Staphylococcus spp. 41.2%Serratia spp. 24.5%	S. aureus 30%Aspergillus spp. 26%Salmonella spp. 16%BCGitis 8%
Immune dysregulation	AIHA 16.8%ITP 6.2%Colitis 5.6%SLE 1.6%	Lupus syndromes 3.3%Colitis 17.4%Granuloma 26.4%ITP 1.4%	N/A
Management	Cotrimoxazole 67%Itraconazole 69.6%IFN-g 14%Granulocyte Tx. 1 patientHSCT 11.8%	Antibiotics 89%IFN-g 73%Granulocyte Tx. 15%HSCT 2 patients	Antibiotics 71%Antifungal 53%IFN-g 33%Granulocyte Tx. 7%HSCT 6%
Mortality and survival	16.5%Median survival ageXL-CGD 4.0 yearsAR-CGD 14.0 years10-year survivalXL-CGD 49.6%AR-CGD 88.0%	17.6%5-year follow-upXL-CGD 76%AR-CGD 88%	20%Median death ageXL-CGD 8.8 yearsAR-CGD 10.4 years

AR-CGD, autosomal recessive chronic granulomatous disease; XL-CGD, X-linked chronic granulomatous disease; BCG, bacillus Calmette–Guérin; AIHA, autoimmune hemolytic anemia; ITP, immune thrombocytopenic purpura; SLE, systemic lupus erythematosus; IFN-g, interferon γ; Tx., transfusion; HSCT, hematopoietic stem cell transplantation.

[ViewLarge]

Sharing Unavailable