Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study

PLoS ONE

Volumn 8, Issue 11, 2013, Pages

  Darbari, Deepika S ^a,b   Wang, Zhengyuan ^b   Kwak, Minjung ^b   Hildesheim, Mariana ^b   Nichols, James ^b   Allen, Darlene ^b   Seamon, Catherine ^b   Peters Lawrence, Marlene ^b   Conrey, Anna ^b   Hall, Mary K ^c   Kato, Gregory J ^b   Taylor VI, James G ^b  

^a CHILDREN'S NATIONAL MEDICAL CENTER   (United States)

^b NATIONAL HEART LUNG AND BLOOD INSTITUTE   (United States)

^c NATIONAL INSTITUTES OF HEALTH   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

FERRITIN; HIGH DENSITY LIPOPROTEIN CHOLESTEROL; HYDROXYUREA;

ADULT; ARTERY OCCLUSION; ARTICLE; CHOLESTEROL BLOOD LEVEL; COHORT ANALYSIS; CONTROLLED STUDY; DISEASE SEVERITY; EMERGENCY WARD; FEMALE; FERRITIN BLOOD LEVEL; GLOMERULUS FILTRATION RATE; HEALTH CARE UTILIZATION; HEMATOCRIT; HIGH RISK PATIENT; HOSPITALIZATION; HUMAN; KIDNEY FUNCTION; MAJOR CLINICAL STUDY; MALE; MORTALITY; PAIN; RISK FACTOR; SICKLE CELL ANEMIA; TRICUSPID VALVE REGURGITATION; VEIN OCCLUSION;

ADULT; ANEMIA, SICKLE CELL; CHOLESTEROL, HDL; FEMALE; FERRITINS; HEMATOCRIT; HUMANS; KAPLAN-MEIER ESTIMATE; LOGISTIC MODELS; MALE; MIDDLE AGED; PROPORTIONAL HAZARDS MODELS; RISK FACTORS;

EID: 84892430495     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0079923     Document Type: Article

References (31)

1. Kauf TL, Coates TD, Huazhi L, Mody-Patel N, Hartzema AG (2009) The cost of health care for children and adults with sickle cell disease. Am J Hematol 84: 323-327. doi:10.1002/ajh.21408. PubMed: 19358302.
2. Steinberg MH (1999) Management of sickle cell disease. N Engl J Med 340: 1021-1030. doi:10.1056/NEJM199904013401307. PubMed: 10099145.
3. Brousseau DC, Owens PL, Mosso AL, Panepinto JA, Steiner CA (2010) Acute care utilization and rehospitalizations for sickle cell disease. JAMA 303: 1288-1294. doi:10.1001/jama.2010.378. PubMed: 20371788.
4. Platt OS, Thorington BD, Brambilla DJ, Milner PF, Rosse WF et al. (1991) Pain in sickle cell disease. Rates and risk factors. N Engl J Med 325: 11-16. doi:10.1056/NEJM199107043250103. PubMed: 1710777.
5. Powars DR, Chan LS, Hiti A, Ramicone E, Johnson C (2005) Outcome of sickle cell anemia: a 4-decade observational study of 1056 patients. Medicine (Baltimore) 84: 363-376. doi:10.1097/01.md.0000189089.45003.52. PubMed: 16267411.
6. Smith WR, Penberthy LT, Bovbjerg VE, McClish DK, Roberts JD et al. (2008) Daily assessment of pain in adults with sickle cell disease. Ann Intern Med 148: 94-101. doi: 10.7326/0003-4819-148-2-200801150-00004. PubMed: 18195334.
7. Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O et al. (1994) Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med 330: 1639-1644. doi:10.1056/NEJM199406093302303. PubMed: 7993409.
8. Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E et al. (1998) Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med 339: 5-11. doi:10.1056/NEJM199807023390102. PubMed: 9647873.
9. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB et al. (1995) Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 332: 1317-1322. doi:10.1056/NEJM199505183322001. PubMed: 7715639.
10. Gaston MH, Verter JI, Woods G, Pegelow C, Kelleher J et al. (1986) Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial. N Engl J Med 314: 1593-1599. doi:10.1056/NEJM198606193142501. PubMed: 3086721.
11. Steinberg MH, McCarthy WF, Castro O, Ballas SK, Armstrong FD et al. (2010) The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up. Am J Hematol 85: 403-408. PubMed: 20513116.
12. Miller ST, Wright E, Abboud M, Berman B, Files B et al. (2001) Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia. J Pediatr 139: 785-789. doi:10.1067/mpd.2001.119593. PubMed: 11743502.
13. Castro O, Hoque M, Brown BD (2003) Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood 101: 1257-1261. doi:10.1182/blood-2002-03-0948. PubMed: 12393669.
14. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF et al. (2004) Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 350: 886-895. doi:10.1056/NEJMoa035477. PubMed: 14985486.
15. Parent F, Bachir D, Inamo J, Lionnet F, Driss F et al. (2011) A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med 365: 44-53. doi:10.1056/NEJMoa1005565. PubMed: 21732836.
16. Machado RF, Barst RJ, Yovetich NA, Hassell KL, Kato GJ et al. (2011) Hospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacity. Blood 118: 855-864. doi:10.1182/blood-2010-09-306167. PubMed: 21527519.
17. Minniti CP, Machado RF, Coles WA, Sachdev V, Gladwin MT et al. (2009) Endothelin receptor antagonists for pulmonary hypertension in adult patients with sickle cell disease. Br J Haematol 147: 737-743. doi: 10.1111/j.1365-2141. 2009.07906.x. PubMed: 19775299.
18. Taylor JG, Ackah D, Cobb C, Orr N, Percy MJ et al. (2008) Mutations and polymorphisms in hemoglobin genes and the risk of pulmonary hypertension and death in sickle cell disease. Am J Hematol 83: 6-14. doi:10.1002/ajh.21035. PubMed: 17724704.
19. Liu YT, Old JM, Miles K, Fisher CA, Weatherall DJ et al. (2000) Rapid detection of alpha-thalassaemia deletions and alpha-globin gene triplication by multiplex polymerase chain reactions. Br J Haematol 108: 295-299. doi:10.1046/j.1365-2141.2000.01870.x. PubMed: 10691858.
20. Taylor JG, Nolan VG, Mendelsohn L, Kato GJ, Gladwin MT et al. (2008) Chronic hyper-hemolysis in sickle cell anemia: association of vascular complications and mortality with less frequent vasoocclusive pain. PLOS ONE 3: e2095. doi:10.1371/journal.pone.0002095. PubMed: 18461136.
21. Levey AS, Bosch JP, Lewis JB, Greene T, Rogers N et al. (1999) A more accurate method to estimate glomerular filtration rate from serum creatinine: a new prediction equation. Modification of Diet in Renal Disease Study Group. Ann Intern Med 130: 461-470. doi: 10.7326/0003-4819-130-6-199903160-00002. PubMed: 10075613.
22. Nagel RL, Fabry ME, Steinberg MH (2003) The paradox of hemoglobin SC disease. Blood Rev 17: 167-178. doi:10.1016/S0268-960X(03)00003-1. PubMed: 12818227.
23. Billett HH, Kim K, Fabry ME, Nagel RL (1986) The percentage of dense red cells does not predict incidence of sickle cell painful crisis. Blood 68: 301-303. PubMed: 3719102.
24. Ballas SK, Larner J, Smith ED, Surrey S, Schwartz E et al. (1988) Rheologic predictors of the severity of the painful sickle cell crisis. Blood 72: 1216-1223. PubMed: 3167204.
25. Shalev H, Kapelushnik J, Moser A, Knobler H, Tamary H (2007) Hypocholesterolemia in chronic anemias with increased erythropoietic activity. Am J Hematol 82: 199-202. doi:10.1002/ajh.20804. PubMed: 17039515.
26. Zorca S, Freeman L, Hildesheim M, Allen D, Remaley AT et al. (2010) Lipid levels in sickle-cell disease associated with haemolytic severity, vascular dysfunction and pulmonary hypertension. Br J Haematol 149: 436-445. doi:10.1111/j.1365-2141.2010.08109.x. PubMed: 20230401.
27. Sasaki J, Waterman MR, Buchanan GR, Cottam GL (1983) Plasma and erythrocyte lipids in sickle cell anaemia. Clin Lab Haematol 5: 35-44. PubMed: 6851435.
28. Bean CJ, Boulet SL, Ellingsen D, Pyle ME, Barron-Casella EA et al. (2012) Heme oxygenase-1 gene promoter polymorphism is associated with reduced incidence of acute chest syndrome among children with sickle cell disease. Blood 120: 3822-3828. doi:10.1182/blood-2011-06-361642. PubMed: 22966170.
29. Charache S, Terrin ML, Moore RD, Dover GJ, McMahon RP et al. (1995) Design of the multicenter study of hydroxyurea in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea. Control Clin Trials 16: 432-446. doi:10.1016/S0197-2456(95)00098-4. PubMed: 8925656.
30. Hoots WK, Shurin SB (2012) Future directions of sickle cell disease research: the NIH perspective. Pediatr Blood Cancer 59: 353-357. doi: 10.1002/pbc.24180. PubMed: 22517801.
31. Lettre G (2012) The Search for Genetic Modifiers of Disease Severity in the beta-Hemoglobinopathies. Cold Spring Harbor Perspectives Med 2.