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Table 5 Characteristics of the patients followed in Mayotte according to the HbF level

From: High fetal hemoglobin level is associated with increased risk of cerebral vasculopathy in children with sickle cell disease in Mayotte

ProfileHbF ≥ 10%HbF < 10%OR (95%CI)PP*
Age, Mean (SD)8,6 (±0,5)12,1 (±0,6) < 0,0010.09
Hemoglobin sickle cell genotype n (%)   0.1870.2
HbSS58 (68,2)60 (76,9)   
HbS/β°Thalassemia18 (21,2)8 (10,3)   
HbS/β+Thalassemia9 (10,5)10 (12,8)   
Haplotypes n (%)
Bantu/−79 (97,5)62 (93,9) 0.410.45
Bantu/Bantu48 (59,3)47 (71,2) 0.140.18
Benin/−27 (69,2)12 (18,2)2,25 [1,03-4,9]0.040.4
Benin/Benin1 (1,2)3 (4,5) 0.330.5
Alpha thalassemia n(%)56 (60,2)42 (58,3) 0.870.8
 G6PD deficiency n(%)     
G6PD-Deficiency5 (6,4)7 (10,3) 0.020.2
Heterozygote4 (5,1)12 (17,6)   
UGT1A1 mutation n (%)12 (14)11 (15,1) 11
SNP n (%)
BCL11A or rs467139320 (19,8)7 (8)3,13 [1,1-8,89}0.0470.2
BCL11A rs1188686828 (63,6)9 (29)4,28 [1,6-11,5]0.0050.06
BCL11A rs142740728 (41,8)10 (15,2)4 [1,75-9,22]0.0010.051
HMIP rs939913712 (14,3)2 (2,7)5,92 [1,28-27,4]0.010.24
Xmn1 or rs78421446 (7,2)4 (5,7) 0.760.74
BCL11A rs1018985764 (79)55 (77,5) 0.850.8
HMIP rs2838451330 (73,2)23 (74,2) 11
HMIP rs6665037131 (41,3)22 (33,3) 0.390.4
HMIP rs48954417 (17,1)2 (6,5) 0.280.3
Hydroxyurea treatment11 (11)19 (22,1) 0.0470.5
Osteonecrosis (n, %)1 (1,3)8 (11,1)   
Number of hospitalization (n, %)   0.010.1
No21 (21)14 (16,5)   
1 à 2 per year77 (77)60 (70,6)   
≥ 3 per year2 (2)11 (12,9)   
  1. P* obtained after a multivariate analysis