Table 4.
α-Thalassemia and pulmonary and cardiac complications in hemoglobin SS subjects 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 |
|
| αα/αα1 | 14.2% | 33.5%% | 1.3% | 25.2% | 8.3 (0.1) | 8.5 (0.1) | 498 (478–518) | 459 (446–473) |
| αα/α-2 | 23.8% | 29.1% | 0% | 16.4% | 8.9 (0.1) | 9.0 (0.1) | 433 (407–459) | 407 (392–424) |
| α-/α-3 | 22.2% | 33.3% | 0% | 0% | 9.3 (0.5) | 9.1 (0.5) | 392 (324–478) | 302 (260–351) |
| P | 0.041 | 0.5 | 0.24 | 0.008 | <0.001 | 0.012 | 0.07 | 0.006 |
| 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 |
|
| αα/αα1 | 47.6% | 67.0% | 12.9% | 25.7% | 13.1% | 16.6% | 28.4% | 28.9% |
| αα/α-2 | 58.4% | 59.6% | 7.0% | 13.2% | 9.7% | 9.6% | 27.6% | 19.0% |
| α-/α-3 | 44.4% | 40.0% | 0% | 0% | 0% | 0% | 0% | 20.0% |
| P | 0.17 | 0.035 | 0.07 | 0.003 | 0.19 | 0.022 | 0.3 | 0.034 |
| 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 |
|
| αα/αα1 | 1.5 (0.4–2.5) | 34 (29–39) | 90 (73–109) | 109 (91–131) | 64 (61–67) | 62 (58–66) | 6.4 (5.5–7.6) | 6.4 (5.1–8.0) |
| αα/α-2 | 0.9 (0.1–1.8) | 34 (30–38) | 77 (64–97) | 103 (83–122) | 64 (61–67) | 61 (58–65) | 6.3 (5.8–7.0) | 6.4 (5.2–7.9) |
| α-/α-3 | 0.3 (−0.4–1.2) | 34 (30–34) | 77 (64–80) | 97 (83–129) | 62 (61–66) | 63 (58–66) | 6.1 (5.5–6.8) | 6.5 (6.0–6.9) |
| P | <0.001 | 0.4 | <0.001 | 0.004 | 0.8 | 0.6 | 0.12 | 0.8 |
1
PUSH: N = 234 children; Walk-PHaSST: N = 306 predominantly adults
2
PUSH:N = 106 children; Walk-PHaSST: N = 147 predominantly adults
3
PUSH: N = 9 children; Walk-PHaSST: N = 10 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