Genotypic and Phenotypic Composition of Sickle Cell Disease in the Arab Population - A Systematic Review

Figures & data

Figure 1 PRISMA flow diagram of the article screening process with the details of included and excluded studies.

Table 1 Baseline Characteristics of the Published Data of Patients with SCD in the Arab Population (N= 44,034)

Characteristic	N (%)
Gender Total	N=13105
Male	6415 (49)
Female	6690 (51)
Country
Algeria	84 (0.19)
Bahrain	12,209 (27.5)
Egypt	1550 (3.5)
Iran	28 (0.06)
Iraq	415 (0.9)
Jordan	2082 (4.7)
Kuwait	1520 (3.4)
Lebanon	453 (1)
Libya	5 (0.01)
Morocco	124 (0.2)
Oman	836 (1.8)
Palestine	151 (0.3)
Qatar	2 (0.004)
Saudi Arabia	22,708 (51.3)
Sudan	476 (1)
Tunisia	1104 (2.5)
UAE	147 (0.3)
Yemen	256 (0.5)
Genotypes
Genotypes reported	14,257 (32.3)
HbSS	10980 (77)
HbSβ^0	1415 (9.9)
HbSβ^+	1037 (7.2)
HbSC	502 (3.5)
HbS/α-Thal	339 (2.3)
HBSD	66 (0.4)
HbSO-Arab	56 (0.3)
Hb Sβ^0 + Alpha Thal	42 (0.3)
HBSE	16 (0.1)
HBS-Oman	1 (0.007)

Table 2 Number of Studies Published per Country (N=184)

Country	No. of Studies	Percentage
Saudi Arabia	61	33.2
Kuwait	21	11.4
Egypt	20	10.9
Oman	15	82
Tunisia	15	82
Bahrain	14	7.6
Sudan	7	3.8
Jordan	6	3.3
Lebanon	6	3.3
Yemen	5	2.7
Morocco	3	1.6
Iran	2	1.1
Iraq	2	1.1
Palestine	2	1.1
UAE	2	1.1
Algeria	1	0.5
Libya	1	0.5
Qatar	1	0.5

Table 3 Complications of Patients with SCD in the Arab Population (N= 44,034)

Complication	Frequency	Population*	Percentage
Pain crisis	1161	2407	48.23
Neurological complications	1526	4560	33.46
Hepatobiliary complications	1087	4258	25.53
Musculoskeletal complications	1277	5163	24.73
Hemolytic anemia	434	1841	23.57
Pulmonary complications	565	3857	14.65
Acute splenic sequestration	335	2826	11.85
Renal complications	163	2117	7.70
Functional asplenia	85	1498	5.67
Hyper viscosity	77	1430	5.38
Transfusion hemosiderosis	50	1920	2.60
Ophthalmologic complications	76	3329	2.28
Dermatological complications	10	1359	0.74

Notes: *Data readily available from the published studies included in the review as the complications were not reported in all the studies identified. Reported from highest to lowest frequency.

Table 4 Treatment Options Used for Patients with SCD in the Arab Population (N= 44,034)

Treatment	Frequency	Population*	Percentage
Blood transfusion	1022	7144	14.3
Deferasirox	30	989	3.03
Hydroxyurea	663	3332	19.9
Stem cell transplantation	0
L-Glutamine
Voxelotor
Crizanlizumab
Gene therapy

Note: *Data readily available from the published studies included in the review as complications were not reported in all the studies identified.