Skip to main content
. Author manuscript; available in PMC: 2015 Mar 1.
Published in final edited form as: Paediatr Respir Rev. 2013 Nov 15;15(1):4–12. doi: 10.1016/j.prrv.2013.11.003

Table 3.

Sickle cell disease genotype and pulmonary and cardiac complications in the PUSH and Walk-PHaSST cohorts

General clinical manifestations
>3 pain episodes
PUSH		 >3 pain episodes
Walk-PHaSST		 Leg ulcers
PUSH		 Leg ulcers
Walk-PHaSST		 Hb g/dL*
PUSH		 Hb g/dL*
Walk-PHaSST		 LDH U/L**
PUSH		 LDH U/L*
Walk-PHaSST
Hb SS1 16.8% 31.3% 1.1% 22.0% 8.5 (0.1) 8.6 (0.1) 473 (459–488) 437 (428–446)
Hb SC2 10.1% 27.9% 0% 9.0% 11.5 (0.1) 11.6 (0.2) 279 (260–299) 245 (235–255)
Hb Sβ+-thal3 17.6% 25.9% 0% 11.1% 10.7 (0.3) 11.1 (0.4) 308 (276–344) 245 (213–268)
P 0.3 0.4 0.3 0.002 <0.001 <0.001 <0.001 <0.001
Pulmonary complications
Acute chest
syndrome
PUSH 		Acute chest
syndrome
Walk-PHaSST 		O2 sat
<95%
PUSH 		O2 sat <95%
Walk-PHaSST 		TRV≥2.6
m/sec
PUSH 		TRV≥3.0
m/sec
Walk-PHaSST 		BNP
>160 ng/L
PUSH 		BNP
>160 ng/L
Walk-PHaSST
Hb SS1 51.3% 65.3% 11.1% 21.6% 11.7% 15.0% 27.7% 25.8%
Hb SC2 42.7% 52.7% 0% 8.3% 1.2% 8.2% 7.0% 20.5%
Hb Sβ+-thal3 44.4% 64.0% 0% 0% 7.1% 0% 16.7% 8.3%
P 0.17 0.09 <0.001 <0.001 0.013 0.007 <0.001 0.036
Left Ventricular Size and Function
LV Diastolic
Dimension
Z score
PUSH 		LV Diastolic
Area (cm2)
Walk-PHaSST 		LV Mass
Index
(g/m2)
PUSH 		LV Mass
Index
(g/m2)
Walk-pHaSST 		Ejection
Fraction
PUSH 		Ejection
Fraction
Walk-PHaSST 		Mitral
E/Etdi
PUSH 		LV Lateral E/Ea
Walk-PHaSST
Hb SS1 1.6 (0.6–2.5) 35 (31–40) 94 (78–109) 113 (95–133) 64 (61–67) 61 (58–65) 6.5 (5.7–7.6) 6.4 (5.2–8.1)
Hb SC2 1.0 (−0.5– 0.7) 29 (26–33) 67 (60–78) 87 (73–108) 64 (61–66) 61 (57–66) 6.1 (5.5–6.9) 6.4 (4.9–7.6)
Hb Sβ+-thal3 0.4 (−0.4– 1.4) 30 (28–33) 70 (64–90) 84 (69–97) 63 (59–66) 65 (60–68) 5.9 (4.9–6.5) 5.9 (4.8–8.2)
P <0.001 <0.001 <0.001 <0.001 0.046 0.2 0.004 0.7
1

PUSH: N = 381 children; Walk-PHaSST: N = 505 predominantly adults

2

PUSH: N = 90 children; Walk-PHaSST: N = 122 predominantly adults

3

PUSH: N = 18 children; Walk-PHaSST: N = 27 predominantly adults

*

In subjects without recent blood transfusion; adjusted for hydroxyurea

**

In subjects without recent blood transfusion; adjusted for hydroxyurea, age and study site

***

In subjects without recent blood transfusion; adjusted for hydroxyurea and study site