16 Hospital Physician Board Review Manual www.turner-white.com
study. Br J Haematol 2006;134:109–15.
91. Ataga KI, Sood N, De Gent G, et al. Pulmonary hypertension in sickle cell disease. Am J Med 2004;117:665–9.
92. Reiter CD, Wang X, Tanus-Santos JE, et al. Cell-free
hemoglobin limits nitric oxide bioavailability in sickle-cell
disease. Nat Med 2002;8:1383–9.
93. Rybicki AC, Benjamin LJ. Increased levels of endothelin- 1
in plasma of sickle cell anemia patients [letter]. Blood
94. Hammerman SI, Kourembanas S, Conca TJ, et al. Endothelin- 1 production during the acute chest syndrome in sickle
cell disease. Am J Respir Crit Care Med 1997;156:280– 5.
95. Machado RF, Anthi A, Steinberg MH, et al; MSH Investigators. N-terminal pro-brain natriuretic peptide levels and risk
of death in sickle cell disease. JAMA 2006;296:310–8.
96. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary
hypertension. A national prospective study. Ann Intern Med
97. Giaid A, Saleh D. Reduced expression of endothelial nitric
oxide synthase in the lungs of patients with pulmonary
hypertension. N Engl J Med 1995;333:214–21.
98. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in
patients with primary pulmonary hypertension. Results
from a national prospective registry. Ann Intern Med
99. Minniti CP, Machado RF, Coles WA, et al. Endothe-
lin receptor antagonists for pulmonary hypertension in
adult patients with sickle cell disease. Br J Haematol
100. Barst RJ, Mubarak KK, Machado RF, et al. Exercise capac-
ity and haemodynamics in patients with sickle cell disease
with pulmonary hypertension treated with bosentan: results
of the ASSET studies. Br J Haematol 2010;149:426–35.
101. Klings ES, Wyszynski DF, Nolan VG, Steinberg MH. Abnormal pulmonary function in adults with sickle cell anemia. Am J Respir Crit Care Med 2006;173:1264–9.
102. Miller GJ, Serjeant GR. An assessment of lung volumes and gas transfer in sickle-cell anaemia. Thorax
103. Delclaux C, Zerah-Lancner F, Bachir D, et al. Factors associated with dyspnea in adult patients with sickle cell
disease. Chest 2005;128:3336–44.
104. Kaleyias J, Mostofi N, Grant M, et al. Severity of obstructive sleep apnea in children with sickle cell disease. J Pediatr Hematol Oncol 2008;30:659–65.
105. Needleman JP, Franco ME, Varlotta L, et al. Mechanisms
of nocturnal oxyhemoglobin desaturation in children and
adolescents with sickle cell disease. Pediatr Pulmonol
106. Hargrave DR, Wade A, Evans JP, et al. Nocturnal oxygen
saturation and painful sickle cell crises in children. Blood