Lori A. Hunter

TL;DR: Pulmonary hypertension appears to be a complication of chronic hemolysis, is resistant to hydroxyurea therapy, and confers a high risk of death in adults with sickle cell disease.

TL;DR: In patients with SCD, administration of sildenafil, a phosphodiesterase-5 inhibitor that potentiates NO-dependent signaling, reduced platelet activation, supports a role for NO-based therapeutics for SCD vasculopathy.

TL;DR: Patients with SCD-associated PH have both pulmonary arterial and venous PH associated with severe limitations in exercise capacity, likely compounded by interstitial lung fibrosis and severe anemia, and the use of the six-minute-walk distance as an index of PH and cardiopulmonary function is supported.

TL;DR: Sickle cell disease patients with anaemia and pulmonary hypertension have significant exercise limitation and the 6‐min walk distance may be a valid endpoint in this population; therapy with sildenafil appears safe and improves pulmonary hypertension and exercise capacity.

TL;DR: It is found that higher levels of soluble vascular cell adhesion molecule‐1 (sVCAM‐1) were associated with markers indicating renal dysfunction and hepatic impairment, and increased soluble adhesion molecules expression correlated with severity of pulmonary hypertension, a clinical manifestation of endothelial dysfunction.