Pulmonary hypertension in children and young adults with sickle cell disease: Evidence for familial clustering

Pediatric Blood and Cancer

Volumn 54, Issue 3, 2010, Pages 398-402

  Dahoui, Hanane A ^a   Hayek, Munya N ^a   Nietert, Paul J ^b   Arabi, Mariam T ^a   Muwakkit, Samar A ^a   Saab, Raya H ^a   Bissar, Abdallah N ^c   Jumaa, Nidal M ^d   Farhat, Fadi S ^e   Dabbous, Ibrahim A ^a   Bitar, Fadi F ^a   Abboud, Miguel R ^a  

^a AMERICAN UNIVERSITY OF BEIRUT   (Lebanon)

^b MEDICAL UNIVERSITY OF SOUTH CAROLINA   (United States)

^c Nini Hospital   (Lebanon)

^d Tyre Hospitals   (Lebanon)

^e Hammoud Hospital   (Lebanon)

Author keywords

Familial; Hemolysis; Pulmonary hypertension; Sickle cell disease

Indexed keywords

GLUCOSE 6 PHOSPHATE DEHYDROGENASE; HEMOGLOBIN F; HYDROXYUREA; LACTATE DEHYDROGENASE;

ADOLESCENT; ADULT; ARTICLE; CARDIOVASCULAR RISK; CHILD; CONTROLLED STUDY; DISEASE ASSOCIATION; DRUG DOSE INCREASE; FAMILIAL DISEASE; FEMALE; GLUCOSE 6 PHOSPHATE DEHYDROGENASE DEFICIENCY; HEMOLYSIS; HUMAN; LACTATE DEHYDROGENASE BLOOD LEVEL; LEBANON; MAJOR CLINICAL STUDY; MALE; MEAN CORPUSCULAR VOLUME; PRESCHOOL CHILD; PRIORITY JOURNAL; PULMONARY HYPERTENSION; SCHOOL CHILD; SICKLE CELL ANEMIA; STEADY STATE; TRANSTHORACIC ECHOCARDIOGRAPHY; TRICUSPID VALVE REGURGITATION;

ADOLESCENT; ADULT; ANEMIA, SICKLE CELL; ANTISICKLING AGENTS; CHILD; CHILD, PRESCHOOL; ECHOCARDIOGRAPHY; FEMALE; HUMANS; HYDROXYUREA; HYPERTENSION, PULMONARY; LEBANON; MALE; PEDIGREE; YOUNG ADULT;

EID: 76749114711     PISSN: 15455009     EISSN: 15455017     Source Type: Journal    
DOI: 10.1002/pbc.22306     Document Type: Article

References (34)

1. Gladwin MT, Sachdev V, Jison ML, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004;350:886-895.
2. Nelson SC, Adade BB, McDonough EA, et al. High prevalence of pulmonary hypertension in children with sickle cell disease. J Pediatr Hematol Oncol 2007;29:334-337.
3. Onyekwere OC, Campbell A, Teshome M, et al. Pulmonary hypertension in children and adolescents with sickle cell disease. Pediatr Cardiol 2008;29:309-312.
4. Liem RI, Young LT, Thompson AA. Tricuspid regurgitant jet velocity is associated with hemolysis in children and young adults with sickle cell disease evaluated for pulmonary hypertension. Haematologica 2007;92:1549-1552.
5. Pashankar FD, Carbonella J, Bazzy-Asaad A, et al. Prevalence and risk factors of elevated pulmonary artery pressures in children with sickle cell disease. Pediatrics 2008;121:777-782.
6. Kato GJ, McGowan V, Machado RF, et al. Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulcer, pulmonary hypertension, and death in patients with sickle cell disease. Blood 2006;107:2279-2285.
7. Morris CR, Kato GJ, Poljakovic M, et al. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease. JAm Med Assoc 2005;294:81-90.
8. Hsu LL, Champion HC, Campbell-Lee SA, et al. Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability. Blood 2007;109:3088-3098.
9. Hoeper MM, Niedermeyer J, Hoffmeyer F, et al. Pulmonary hypertension after splenectomy? Ann Intern Med 2007;130:506-509.
10. El-Hazmi MA, Warsy AS, Bahakim H, et al. Blood proteins C and S in sickle cell disease. Acta Haematol 1993;90:114-119.
11. Austin E, Loyd J. Genetics and mediators in pulmonary arterial hypertension. Clin Chest Med 2007;28:43.
12. Walters M, Patience M, Leisenring W, et al. Bone marrow transplantation in sickle cell disease.N Engl J Med 1996;335: 369-376.
13. Suell MN, Bezold LI, Okcu MF, et al. Increased pulmonary artery pressures among adolescents with sickle cell disease. J Pediatr Hematol Oncol 2005;27:654-658.
14. Ambrusko SJ, Gunawardena S, sakara A, et al. Elevation of tricuspid jet velocity, a marker of pulmonary hypertension in children with sickle cell disease. Pediatr Blood Cancer 2006;47: 907-913.
15. Vichinsky EP. Pulmonary hypertension in sickle cell disease. N Engl J Med 2004;350:857-859.
16. Nagel RL. Sickle cell anemia is a multigene disorder: Sickle painful crises, a case in point. Am J Hematol 1993;42:96-101.
17. Ashley-Koch A, Elliott L, Kail M, et al. Identification of genetic polymorphisms associated with risk for pulmonary hypertension in sickle cell disease. Blood 2008;111:5721-5726.
18. Kaul DK, Nagel RL, Chen D, et al. Sickle erythrocyte-endothelial interactions in microcirculation: The role of von Willebrand factor and implications for vasoocclusion. Blood 1993;1:2429-2438.
19. Sugihara K, Sugihara T, Mohandras N, et al. Thrombospondin mediates CD36+ sickle reticulocytes to endothelial cells. Blood 1992;80:2634-2642.
20. Gee BE, Platt O. Sickle reticulocytes adhere to VCAM-1. Blood 1995;85:268-274.
21. Kaul DK, Tsai HM, Liu XD, et al. Monoclonal antibodies to alphaVbeta3 (7E3 and LM609) inhibit sickle red blood cell-endothelium interactions induced by platelet activating factor. Blood 2000;95:368-374.
22. Belcher JD, Marker PH, Weber RP, et al. Activated monocytes in sickle cell disease: Potential role in the activation of vascular endothelium and vasoocclsion. Blood 2000;96:2451-2459.
23. French JA, Kenny D, Scott JP, et al. Mechanisms of stroke in sickle cell disease: Sickle erythrocytes decrease cerebral blood flow in rats after nitric oxide synthase inhibition. Blood 1997;89:4591-4596.
24. Kohler HP, Grant PJ. Plasminogen-activator inhibitor type 1 and coronary artery disease. N Engl J Med 2000;342:1792-1801.
25. Gimbone MA. Endothelial dysfunction, hemodynamic forces and atherosclerosis. Thromb Haemost 1999;82:722-726.
26. Khneisser I, Adib SM, Loiselet J, et al. Cost-benefit analysis of G6PD screening in Lebanese newborn males. J Med Liban 2007; 55:29-132.
27. Daher R, Beaini M, Mahfouz R, et al. A neonatal screening in Lebanon: Results of five years' experience. Ann Saudi Med 2003; 23:16-19.
28. Cappellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet 2008;371:64-74.
29. Steinberg M, West MS, Gallagher D, et al. Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia. Blood 1992;71:748-752.
30. Saad ST, Costa FF. Glucose-6-phosphate deficiency and sickle cell disease in Brazil. Hum Hered 1992;42:125-128.
31. Reiter CD, Wang X, Tanus-Santos JE, et al. Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease. Nat Med 2002;8:1383-1389.
32. Souza R, Jardim C, Julio Cesar Fernandes C, et al. NT-proBNP as a tool to stratify disease severity in pulmonary arterial hypertension. Respir Med 2007;101:69-75.
33. Voskaridou E, Tsetsos G, Tsoutsias A, et al. Pulmonary hypertension in patients with sickle cell/beta thalassemia: Incidence and correlation with serum N-terminal pro-brain natriuretic peptide concentrations. Haematologica 2007;92:738-743.
34. Hagar RW, Michlitsch JG, Gardner J, et al. Clinical differences between children and adults with pulmonary hypertension and sickle cell disease. Br J Haematol 2008;140:104-112.