Does glucose-6-phosphate dehydrogenase deficiency worsen the clinical features of sickle cell disease? A multi-hospital-based cross-sectional study

Figures & data

Table 1. Characteristics of the study participants.

Characteristics	Overall	Sickle cell disease patients	Non-sickle cell participants
	N = 333	N = 122	N = 211
	n(%)	n(%)	n(%)
Sex
Female	51(15.3)	43(35,2)	8(3,2)
Male	282(84,7)	79(64,8)	203(96,2)
Age(years)
≤ 5	9(2.7)	9(7.4)	0(0)
> 5	324(97.3)	113(92.6)	211(100)
Mean age ± SD		13,79 ± 6.77	26,12 ± 8.16
Hemoglobin phenotypes
AA	174(52.3)	0(0)	174(82.5)
AS	37(11.1)	0(0)	37(17.5)
SS	122(36.6)	122(100)	0(0)
History of G-6-PD screening
Yes	0(0)	0(0)	0(0)
No	333(100)	122(100)	211(100)
History of SCD screening
Yes	122(100)	122(100)	0(0)
No	211(100)	0(0)	211(100)

N, total number; n, number; SD, standard deviation; G-6-PD, glucose-6-phosphate dehydrogenase, SCD, Sickle cell disease.

Table 2. Prevalence of G-6-PD deficiency and its distribution according to the characteristics of the study participants.

Variables	Overall	G-6-PD deficiency	Non- G-6-PD deficiency	p-value
	n(%)	n(%)	n(%)
Overall prevalence	333(100)	114(34.2)	219(65.8)
SCD status				.767^1
SCD patients	122(100)	43(35.2)	79(64.8)
Non-SCD participants	211(100)	71(33.6)	140(66.4)
Sex				.038^1
Female	51(100)	11(21.6)	40(78.4)
Male	282(100)	103(36.5)	179(63.5)
Age				.498^2
≤ 5	9(100)	4(44.4)	5(55.5)
> 5	324(100)	110(33.9)	214(66.1)
Hb phenotypes				.214^1
AA	174(100)	54(31)	120(69)
AS	37(100)	17(45.9)	20(54.1)
SS	122(100)	43(35.2)	79(64.8)

G-6-PD: Glucose-6-phosphate dehydrogenase, n: Number; SCD: Sickle cell disease.

¹ Chi-squared test.

² Fisher's exact test.

Table 3. Clinical characteristics of sickle cell disease patients according to their G-6-PD status.

Characteristics	G-6-PD deficiency	Number of subjects	Mean ± SD	Mean difference	95% CI of difference	p-value^1
Hemoglobin level, g/dL	yes	43	5.605 ± 2.172	-0.3953	−1.156–0.366	.964
	no	79	6.000 ± 1.687
Number of blood transfusions received in the last 6 months	yes	43	0.65 ± 0.897	0.411	0.116–0.706	.004
	no	79	0.24 ± 0.486
Number of hospitalizations in the last 12 months for fever, anemia or painful crisis	yes	43	1.42 ± 1.451	0.659	0.152–1.166	.007
	no	79	0.76 ± 1.112
Frequency of major vaso-occlusive crises* in the last 12 months	yes	43	1.37 ± 1.092	0.524	0.133–0.915	.005
	no	79	0.85 ± 1.014
Frequency of anemia requiring blood transfusion in the last 12 months	yes	43	0.74 ± 0.902	0.364	0.044–0.685	.007
	no	79	0.38 ± 0.739

G-6-PD: Glucose-6-phosphate dehydrogenase; SD: Standard deviation; CI: Confidence interval.

*Vaso-occlusive crisis requiring care in a health facility.

¹ Student t-test.