Skip to main content
NCBI home page
Postgraduate Medical Journal logoLink to Postgraduate Medical Journal
. 2003 Jul;79(933):384–390. doi: 10.1136/pmj.79.933.384

Pulmonary manifestations of sickle cell disease

A Siddiqui 1, S Ahmed 1
PMCID: PMC1742764  PMID: 12897216

Abstract

Pulmonary complications account for significant morbidity and mortality in patients with sickle cell disease. Clinical lung involvement manifests in two major forms: the acute chest syndrome and sickle cell chronic lung disease. Acute chest syndrome is characterised by fever, chest pain, and appearance of a new infiltrate on chest radiograph. Sickle cell chronic lung disease, on the other hand, manifests as radiographic interstitial abnormalities, impaired pulmonary function, and, in its most severe form, by the evidence of pulmonary hypertension. Progress has been made in understanding the pathophysiology and management of these complications. In this review the current knowledge of the mechanism, diagnosis, and treatment of pulmonary complications of sickle cell disease are discussed.

Full Text

The Full Text of this article is available as a PDF (90.9 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Abildgaard C. F., Simone J. V., Schulman I. Factor-8 (antihaemophilic factor) activity in sickle-cell anaemia. Br J Haematol. 1967 Jan;13(1):19–27. doi: 10.1111/j.1365-2141.1967.tb08691.x. [DOI] [PubMed] [Google Scholar]
  2. Aquino S. L., Gamsu G., Fahy J. V., Claster S., Embury S. H., Mentzer W. C., Vichinsky E. P. Chronic pulmonary disorders in sickle cell disease: findings at thin-section CT. Radiology. 1994 Dec;193(3):807–811. doi: 10.1148/radiology.193.3.7972829. [DOI] [PubMed] [Google Scholar]
  3. Atz A. M., Wessel D. L. Inhaled nitric oxide in sickle cell disease with acute chest syndrome. Anesthesiology. 1997 Oct;87(4):988–990. doi: 10.1097/00000542-199710000-00037. [DOI] [PubMed] [Google Scholar]
  4. Ballas S. K., Larner J., Smith E. D., Surrey S., Schwartz E., Rappaport E. F. Rheologic predictors of the severity of the painful sickle cell crisis. Blood. 1988 Oct;72(4):1216–1223. [PubMed] [Google Scholar]
  5. Barst R. J., Rubin L. J., Long W. A., McGoon M. D., Rich S., Badesch D. B., Groves B. M., Tapson V. F., Bourge R. C., Brundage B. H. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1996 Feb 1;334(5):296–301. doi: 10.1056/NEJM199602013340504. [DOI] [PubMed] [Google Scholar]
  6. Bellet P. S., Kalinyak K. A., Shukla R., Gelfand M. J., Rucknagel D. L. Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. N Engl J Med. 1995 Sep 14;333(11):699–703. doi: 10.1056/NEJM199509143331104. [DOI] [PubMed] [Google Scholar]
  7. Berney S. I., Ridler C. D., Stephens A. D., Thomas A. E., Kovacs I. B. Enhanced platelet reactivity and hypercoagulability in the steady state of sickle cell anaemia. Am J Hematol. 1992 Aug;40(4):290–294. doi: 10.1002/ajh.2830400409. [DOI] [PubMed] [Google Scholar]
  8. Bernini J. C., Rogers Z. R., Sandler E. S., Reisch J. S., Quinn C. T., Buchanan G. R. Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. Blood. 1998 Nov 1;92(9):3082–3089. [PubMed] [Google Scholar]
  9. Bunn H. F. Pathogenesis and treatment of sickle cell disease. N Engl J Med. 1997 Sep 11;337(11):762–769. doi: 10.1056/NEJM199709113371107. [DOI] [PubMed] [Google Scholar]
  10. Castele R. J., Strohl K. P., Chester C. S., Brittenham G. M., Harris J. W. Oxygen saturation with sleep in patients with sickle cell disease. Arch Intern Med. 1986 Apr;146(4):722–725. [PubMed] [Google Scholar]
  11. Castro O. Systemic fat embolism and pulmonary hypertension in sickle cell disease. Hematol Oncol Clin North Am. 1996 Dec;10(6):1289–1303. doi: 10.1016/s0889-8588(05)70401-9. [DOI] [PubMed] [Google Scholar]
  12. Charache S., Terrin M. L., Moore R. D., Dover G. J., Barton F. B., Eckert S. V., McMahon R. P., Bonds D. R. 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. 1995 May 18;332(20):1317–1322. doi: 10.1056/NEJM199505183322001. [DOI] [PubMed] [Google Scholar]
  13. Collins F. S., Orringer E. P. Pulmonary hypertension and cor pulmonale in the sickle hemoglobinopathies. Am J Med. 1982 Dec;73(6):814–821. doi: 10.1016/0002-9343(82)90763-x. [DOI] [PubMed] [Google Scholar]
  14. Davies S. C., Luce P. J., Win A. A., Riordan J. F., Brozovic M. Acute chest syndrome in sickle-cell disease. Lancet. 1984 Jan 7;1(8367):36–38. doi: 10.1016/s0140-6736(84)90193-4. [DOI] [PubMed] [Google Scholar]
  15. Emre U., Miller S. T., Gutierez M., Steiner P., Rao S. P., Rao M. Effect of transfusion in acute chest syndrome of sickle cell disease. J Pediatr. 1995 Dec;127(6):901–904. doi: 10.1016/s0022-3476(95)70025-0. [DOI] [PubMed] [Google Scholar]
  16. Faller D. V. Endothelial cell responses to hypoxic stress. Clin Exp Pharmacol Physiol. 1999 Jan;26(1):74–84. doi: 10.1046/j.1440-1681.1999.02992.x. [DOI] [PubMed] [Google Scholar]
  17. Farber M. D., Koshy M., Kinney T. R. Cooperative Study of Sickle Cell Disease: Demographic and socioeconomic characteristics of patients and families with sickle cell disease. J Chronic Dis. 1985;38(6):495–505. doi: 10.1016/0021-9681(85)90033-5. [DOI] [PubMed] [Google Scholar]
  18. Femi-Pearse D., Gazioglu K. M., Yu P. N. Pulmonary function studies in sickle cell disease. J Appl Physiol. 1970 May;28(5):574–577. doi: 10.1152/jappl.1970.28.5.574. [DOI] [PubMed] [Google Scholar]
  19. Francis R. B., Jr, Johnson C. S. Vascular occlusion in sickle cell disease: current concepts and unanswered questions. Blood. 1991 Apr 1;77(7):1405–1414. [PubMed] [Google Scholar]
  20. Gaine S. Pulmonary hypertension. JAMA. 2000 Dec 27;284(24):3160–3168. doi: 10.1001/jama.284.24.3160. [DOI] [PubMed] [Google Scholar]
  21. Galiè Nazzareno, Humbert Marc, Vachiéry Jean-Luc, Vizza Carmine Dario, Kneussl Meinhard, Manes Alessandra, Sitbon Olivier, Torbicki Adam, Delcroix Marion, Naeije Robert. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol. 2002 May 1;39(9):1496–1502. doi: 10.1016/s0735-1097(02)01786-2. [DOI] [PubMed] [Google Scholar]
  22. Gelfand M. J., Daya S. A., Rucknagel D. L., Kalinyak K. A., Paltiel H. J. Simultaneous occurrence of rib infarction and pulmonary infiltrates in sickle cell disease patients with acute chest syndrome. J Nucl Med. 1993 Apr;34(4):614–618. [PubMed] [Google Scholar]
  23. Gladwin M. T., Schechter A. N. Nitric oxide therapy in sickle cell disease. Semin Hematol. 2001 Oct;38(4):333–342. doi: 10.1016/s0037-1963(01)90027-7. [DOI] [PubMed] [Google Scholar]
  24. Gladwin M. T., Schechter A. N., Shelhamer J. H., Ognibene F. P. The acute chest syndrome in sickle cell disease. Possible role of nitric oxide in its pathophysiology and treatment. Am J Respir Crit Care Med. 1999 May;159(5 Pt 1):1368–1376. doi: 10.1164/ajrccm.159.5.9810094. [DOI] [PubMed] [Google Scholar]
  25. Godeau B., Schaeffer A., Bachir D., Fleury-Feith J., Galacteros F., Verra F., Escudier E., Vaillant J. N., Brun-Buisson C., Rahmouni A. Bronchoalveolar lavage in adult sickle cell patients with acute chest syndrome: value for diagnostic assessment of fat embolism. Am J Respir Crit Care Med. 1996 May;153(5):1691–1696. doi: 10.1164/ajrccm.153.5.8630622. [DOI] [PubMed] [Google Scholar]
  26. Griffin T. C., McIntire D., Buchanan G. R. High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease. N Engl J Med. 1994 Mar 17;330(11):733–737. doi: 10.1056/NEJM199403173301101. [DOI] [PubMed] [Google Scholar]
  27. Hammerman S. I., Klings E. S., Hendra K. P., Upchurch G. R., Jr, Rishikof D. C., Loscalzo J., Farber H. W. Endothelial cell nitric oxide production in acute chest syndrome. Am J Physiol. 1999 Oct;277(4 Pt 2):H1579–H1592. doi: 10.1152/ajpheart.1999.277.4.H1579. [DOI] [PubMed] [Google Scholar]
  28. Hammerman S. I., Kourembanas S., Conca T. J., Tucci M., Brauer M., Farber H. W. Endothelin-1 production during the acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 1997 Jul;156(1):280–285. doi: 10.1164/ajrccm.156.1.9611085. [DOI] [PubMed] [Google Scholar]
  29. Hargrave Darren R., Wade Angie, Evans Jane P. M., Hewes Deborah K. M., Kirkham Fenella J. Nocturnal oxygen saturation and painful sickle cell crises in children. Blood. 2002 Sep 12;101(3):846–848. doi: 10.1182/blood-2002-05-1392. [DOI] [PubMed] [Google Scholar]
  30. Hasuda T., Satoh T., Shimouchi A., Sakamaki F., Kyotani S., Matsumoto T., Goto Y., Nakanishi N. Improvement in exercise capacity with nitric oxide inhalation in patients with precapillary pulmonary hypertension. Circulation. 2000 May 2;101(17):2066–2070. doi: 10.1161/01.cir.101.17.2066. [DOI] [PubMed] [Google Scholar]
  31. Haupt H. M., Moore G. W., Bauer T. W., Hutchins G. M. The lung in sickle cell disease. Chest. 1982 Mar;81(3):332–337. doi: 10.1378/chest.81.3.332. [DOI] [PubMed] [Google Scholar]
  32. Haynes J., Jr, Allison R. C. Pulmonary edema. Complication in the management of sickle cell pain crisis. Am J Med. 1986 May;80(5):833–840. doi: 10.1016/0002-9343(86)90624-8. [DOI] [PubMed] [Google Scholar]
  33. Hoeper Marius M., Galiè Nazzareno, Simonneau Gerald, Rubin Lewis J. New treatments for pulmonary arterial hypertension. Am J Respir Crit Care Med. 2002 May 1;165(9):1209–1216. doi: 10.1164/rccm.200110-028PP. [DOI] [PubMed] [Google Scholar]
  34. Hofstra T. C., Kalra V. K., Meiselman H. J., Coates T. D. Sickle erythrocytes adhere to polymorphonuclear neutrophils and activate the neutrophil respiratory burst. Blood. 1996 May 15;87(10):4440–4447. [PubMed] [Google Scholar]
  35. Hosenpud J. D., Bennett L. E., Keck B. M., Fiol B., Novick R. J. The Registry of the International Society for Heart and Lung Transplantation: fourteenth official report--1997. J Heart Lung Transplant. 1997 Jul;16(7):691–712. [PubMed] [Google Scholar]
  36. Johnson C. S., Giorgio A. J. Arterial blood pressure in adults with sickle cell disease. Arch Intern Med. 1981 Jun;141(7):891–893. [PubMed] [Google Scholar]
  37. Kaur K., Brown B., Lombardo F. Prostacyclin for secondary pulmonary hypertension. Ann Intern Med. 2000 Jan 18;132(2):165–165. doi: 10.7326/0003-4819-132-2-200001180-00019. [DOI] [PubMed] [Google Scholar]
  38. Kerstein D., Levy P. S., Hsu D. T., Hordof A. J., Gersony W. M., Barst R. J. Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation. 1995 Apr 1;91(7):2028–2035. doi: 10.1161/01.cir.91.7.2028. [DOI] [PubMed] [Google Scholar]
  39. Key N. S., Slungaard A., Dandelet L., Nelson S. C., Moertel C., Styles L. A., Kuypers F. A., Bach R. R. Whole blood tissue factor procoagulant activity is elevated in patients with sickle cell disease. Blood. 1998 Jun 1;91(11):4216–4223. [PubMed] [Google Scholar]
  40. Klings E. S., Christman B. W., McClung J., Stucchi A. F., McMahon L., Brauer M., Farber H. W. Increased F2 isoprostanes in the acute chest syndrome of sickle cell disease as a marker of oxidative stress. Am J Respir Crit Care Med. 2001 Oct 1;164(7):1248–1252. doi: 10.1164/ajrccm.164.7.2101020. [DOI] [PubMed] [Google Scholar]
  41. Knight J., Murphy T. M., Browning I. The lung in sickle cell disease. Pediatr Pulmonol. 1999 Sep;28(3):205–216. doi: 10.1002/(sici)1099-0496(199909)28:3<205::aid-ppul8>3.0.co;2-z. [DOI] [PubMed] [Google Scholar]
  42. Koumbourlis A. C., Zar H. J., Hurlet-Jensen A., Goldberg M. R. Prevalence and reversibility of lower airway obstruction in children with sickle cell disease. J Pediatr. 2001 Feb;138(2):188–192. doi: 10.1067/mpd.2001.111824. [DOI] [PubMed] [Google Scholar]
  43. Kress J. P., Pohlman A. S., Hall J. B. Determination of hemoglobin saturation in patients with acute sickle chest syndrome: a comparison of arterial blood gases and pulse oximetry. Chest. 1999 May;115(5):1316–1320. doi: 10.1378/chest.115.5.1316. [DOI] [PubMed] [Google Scholar]
  44. Leong M. A., Dampier C., Varlotta L., Allen J. L. Airway hyperreactivity in children with sickle cell disease. J Pediatr. 1997 Aug;131(2):278–283. doi: 10.1016/s0022-3476(97)70166-5. [DOI] [PubMed] [Google Scholar]
  45. Maddern B. R., Reed H. T., Ohene-Frempong K., Beckerman R. C. Obstructive sleep apnea syndrome in sickle cell disease. Ann Otol Rhinol Laryngol. 1989 Mar;98(3):174–178. doi: 10.1177/000348948909800302. [DOI] [PubMed] [Google Scholar]
  46. Maitre B., Habibi A., Roudot-Thoraval F., Bachir D., Belghiti D. D., Galacteros F., Godeau B. Acute chest syndrome in adults with sickle cell disease. Chest. 2000 May;117(5):1386–1392. doi: 10.1378/chest.117.5.1386. [DOI] [PubMed] [Google Scholar]
  47. Marwick C. Trial halted as sickle cell treatment proves itself. JAMA. 1995 Feb 22;273(8):611–611. [PubMed] [Google Scholar]
  48. Miller G. J., Serjeant G. R. An assessment of lung volumes and gas transfer in sickle-cell anaemia. Thorax. 1971 May;26(3):309–315. doi: 10.1136/thx.26.3.309. [DOI] [PMC free article] [PubMed] [Google Scholar]
  49. Miller S. T., Wright E., Abboud M., Berman B., Files B., Scher C. D., Styles L., Adams R. J., STOP Investigators Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia. J Pediatr. 2001 Dec;139(6):785–789. doi: 10.1067/mpd.2001.119593. [DOI] [PubMed] [Google Scholar]
  50. Needleman J. P., Franco M. E., Varlotta L., Reber-Brodecki D., Bauer N., Dampier C., Allen J. L. Mechanisms of nocturnal oxyhemoglobin desaturation in children and adolescents with sickle cell disease. Pediatr Pulmonol. 1999 Dec;28(6):418–422. doi: 10.1002/(sici)1099-0496(199912)28:6<418::aid-ppul6>3.0.co;2-d. [DOI] [PubMed] [Google Scholar]
  51. Needleman J. P., Setty B. N., Varlotta L., Dampier C., Allen J. L. Measurement of hemoglobin saturation by oxygen in children and adolescents with sickle cell disease. Pediatr Pulmonol. 1999 Dec;28(6):423–428. doi: 10.1002/(sici)1099-0496(199912)28:6<423::aid-ppul7>3.0.co;2-c. [DOI] [PubMed] [Google Scholar]
  52. Norris S. L., Johnson C., Haywood L. J. Left ventricular filling pressure in sickle cell anemia. J Assoc Acad Minor Phys. 1992;3(1):20–23. [PubMed] [Google Scholar]
  53. Olschewski Horst, Simonneau Gerald, Galiè Nazzareno, Higenbottam Timothy, Naeije Robert, Rubin Lewis J., Nikkho Sylvia, Speich Rudolf, Hoeper Marius M., Behr Jürgen. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002 Aug 1;347(5):322–329. doi: 10.1056/NEJMoa020204. [DOI] [PubMed] [Google Scholar]
  54. Oppenheimer E. H., Esterly J. R. Pulmonary changes in sickle cell disease. Am Rev Respir Dis. 1971 Jun;103(6):858–859. doi: 10.1164/arrd.1971.103.6.858. [DOI] [PubMed] [Google Scholar]
  55. Platt O. S., Brambilla D. J., Rosse W. F., Milner P. F., Castro O., Steinberg M. H., Klug P. P. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994 Jun 9;330(23):1639–1644. doi: 10.1056/NEJM199406093302303. [DOI] [PubMed] [Google Scholar]
  56. Platt O. S. Sickle cell anemia as an inflammatory disease. J Clin Invest. 2000 Aug;106(3):337–338. doi: 10.1172/JCI10726. [DOI] [PMC free article] [PubMed] [Google Scholar]
  57. Poncz M., Kane E., Gill F. M. Acute chest syndrome in sickle cell disease: etiology and clinical correlates. J Pediatr. 1985 Dec;107(6):861–866. doi: 10.1016/s0022-3476(85)80176-1. [DOI] [PubMed] [Google Scholar]
  58. Pond G. D., Ovitt T. W., Capp M. P. Comparison of conventional pulmonary angiography with intravenous digital subtraction angiography for pulmonary embolic disease. Radiology. 1983 May;147(2):345–350. doi: 10.1148/radiology.147.2.6340154. [DOI] [PubMed] [Google Scholar]
  59. Powars D. R. Sickle cell anemia and major organ failure. Hemoglobin. 1990;14(6):573–598. doi: 10.3109/03630269009046967. [DOI] [PubMed] [Google Scholar]
  60. Powars D., Weidman J. A., Odom-Maryon T., Niland J. C., Johnson C. Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine (Baltimore) 1988 Jan;67(1):66–76. [PubMed] [Google Scholar]
  61. Quinn C. T., Buchanan G. R. The acute chest syndrome of sickle cell disease. J Pediatr. 1999 Oct;135(4):416–422. doi: 10.1016/s0022-3476(99)70162-9. [DOI] [PubMed] [Google Scholar]
  62. Rich S., Kaufmann E., Levy P. S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992 Jul 9;327(2):76–81. doi: 10.1056/NEJM199207093270203. [DOI] [PubMed] [Google Scholar]
  63. Rich S., Seidlitz M., Dodin E., Osimani D., Judd D., Genthner D., McLaughlin V., Francis G. The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest. 1998 Sep;114(3):787–792. doi: 10.1378/chest.114.3.787. [DOI] [PubMed] [Google Scholar]
  64. Rickles F. R., O'Leary D. S. Role of coagulation system in pathophysiology of sickle cell disease. Arch Intern Med. 1974 Apr;133(4):635–641. [PubMed] [Google Scholar]
  65. Rubin Lewis J., Badesch David B., Barst Robyn J., Galie Nazzareno, Black Carol M., Keogh Anne, Pulido Tomas, Frost Adaani, Roux Sebastien, Leconte Isabelle. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002 Mar 21;346(12):896–903. doi: 10.1056/NEJMoa012212. [DOI] [PubMed] [Google Scholar]
  66. Rubler S., Fleischer R. A. Sickle cell states and cardiomyopathy. Sudden death due to pulmonary thrombosis and infarction. Am J Cardiol. 1967 Jun;19(6):867–873. doi: 10.1016/0002-9149(67)90510-3. [DOI] [PubMed] [Google Scholar]
  67. Rucknagel D. L., Kalinyak K. A., Gelfand M. J. Rib infarcts and acute chest syndrome in sickle cell diseases. Lancet. 1991 Apr 6;337(8745):831–833. doi: 10.1016/0140-6736(91)92525-7. [DOI] [PubMed] [Google Scholar]
  68. Salzman Steve H. Does splinting from thoracic bone ischemia and infarction contribute to the acute chest syndrome in sickle cell disease? Chest. 2002 Jul;122(1):6–9. doi: 10.1378/chest.122.1.6. [DOI] [PubMed] [Google Scholar]
  69. Samuels M. P., Stebbens V. A., Davies S. C., Picton-Jones E., Southall D. P. Sleep related upper airway obstruction and hypoxaemia in sickle cell disease. Arch Dis Child. 1992 Jul;67(7):925–929. doi: 10.1136/adc.67.7.925. [DOI] [PMC free article] [PubMed] [Google Scholar]
  70. Santoli F., Zerah F., Vasile N., Bachir D., Galacteros F., Atlan G. Pulmonary function in sickle cell disease with or without acute chest syndrome. Eur Respir J. 1998 Nov;12(5):1124–1129. doi: 10.1183/09031936.98.12051124. [DOI] [PubMed] [Google Scholar]
  71. Setty B. N., Stuart M. J. Vascular cell adhesion molecule-1 is involved in mediating hypoxia-induced sickle red blood cell adherence to endothelium: potential role in sickle cell disease. Blood. 1996 Sep 15;88(6):2311–2320. [PubMed] [Google Scholar]
  72. Sidman J. D., Fry T. L. Exacerbation of sickle cell disease by obstructive sleep apnea. Arch Otolaryngol Head Neck Surg. 1988 Aug;114(8):916–917. doi: 10.1001/archotol.1988.01860200100028. [DOI] [PubMed] [Google Scholar]
  73. Simmons B. E., Santhanam V., Castaner A., Rao K. R., Sachdev N., Cooper R. Sickle cell heart disease. Two-dimensional echo and Doppler ultrasonographic findings in the hearts of adult patients with sickle cell anemia. Arch Intern Med. 1988 Jul;148(7):1526–1528. doi: 10.1001/archinte.148.7.1526. [DOI] [PubMed] [Google Scholar]
  74. Simonneau Gerald, Barst Robyn J., Galie Nazzareno, Naeije Robert, Rich Stuart, Bourge Robert C., Keogh Anne, Oudiz Ronald, Frost Adaani, Blackburn Shelmer D. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002 Mar 15;165(6):800–804. doi: 10.1164/ajrccm.165.6.2106079. [DOI] [PubMed] [Google Scholar]
  75. Solovey A., Gui L., Key N. S., Hebbel R. P. Tissue factor expression by endothelial cells in sickle cell anemia. J Clin Invest. 1998 May 1;101(9):1899–1904. doi: 10.1172/JCI1932. [DOI] [PMC free article] [PubMed] [Google Scholar]
  76. Solovey A., Lin Y., Browne P., Choong S., Wayner E., Hebbel R. P. Circulating activated endothelial cells in sickle cell anemia. N Engl J Med. 1997 Nov 27;337(22):1584–1590. doi: 10.1056/NEJM199711273372203. [DOI] [PubMed] [Google Scholar]
  77. Stuart M. J., Setty B. N. Acute chest syndrome of sickle cell disease: new light on an old problem. Curr Opin Hematol. 2001 Mar;8(2):111–122. doi: 10.1097/00062752-200103000-00009. [DOI] [PubMed] [Google Scholar]
  78. Stuart M. J., Setty B. N. Sickle cell acute chest syndrome: pathogenesis and rationale for treatment. Blood. 1999 Sep 1;94(5):1555–1560. [PubMed] [Google Scholar]
  79. Styles L. A., Schalkwijk C. G., Aarsman A. J., Vichinsky E. P., Lubin B. H., Kuypers F. A. Phospholipase A2 levels in acute chest syndrome of sickle cell disease. Blood. 1996 Mar 15;87(6):2573–2578. [PubMed] [Google Scholar]
  80. Sullivan K. J., Goodwin S. R., Evangelist J., Moore R. D., Mehta P. Nitric oxide successfully used to treat acute chest syndrome of sickle cell disease in a young adolescent. Crit Care Med. 1999 Nov;27(11):2563–2568. doi: 10.1097/00003246-199911000-00039. [DOI] [PubMed] [Google Scholar]
  81. Sutton L. L., Castro O., Cross D. J., Spencer J. E., Lewis J. F. Pulmonary hypertension in sickle cell disease. Am J Cardiol. 1994 Sep 15;74(6):626–628. doi: 10.1016/0002-9149(94)90760-9. [DOI] [PubMed] [Google Scholar]
  82. Thomas A. N., Pattison C., Serjeant G. R. Causes of death in sickle-cell disease in Jamaica. 1982 Aug 28-Sep 4Br Med J (Clin Res Ed) 285(6342):633–635. doi: 10.1136/bmj.285.6342.633. [DOI] [PMC free article] [PubMed] [Google Scholar]
  83. Trant C. A., Jr, Casey J. R., Hansell D., Cheifetz I., Meliones J. N., Ungerleider R. M., Browning I., Greeley W. J. Successful use of extracorporeal membrane oxygenation in the treatment of acute chest syndrome in a child with severe sickle cell anemia. ASAIO J. 1996 May-Jun;42(3):236–239. [PubMed] [Google Scholar]
  84. Vichinsky E. P., Neumayr L. D., Earles A. N., Williams R., Lennette E. T., Dean D., Nickerson B., Orringer E., McKie V., Bellevue R. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000 Jun 22;342(25):1855–1865. doi: 10.1056/NEJM200006223422502. [DOI] [PubMed] [Google Scholar]
  85. Vichinsky E. P., Styles L. A., Colangelo L. H., Wright E. C., Castro O., Nickerson B. Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease. Blood. 1997 Mar 1;89(5):1787–1792. [PubMed] [Google Scholar]
  86. Vichinsky E., Styles L. Pulmonary complications. Hematol Oncol Clin North Am. 1996 Dec;10(6):1275–1287. doi: 10.1016/s0889-8588(05)70400-7. [DOI] [PubMed] [Google Scholar]
  87. Vichinsky E., Williams R., Das M., Earles A. N., Lewis N., Adler A., McQuitty J. Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia. Blood. 1994 Jun 1;83(11):3107–3112. [PubMed] [Google Scholar]
  88. Wayne A. S., Kevy S. V., Nathan D. G. Transfusion management of sickle cell disease. Blood. 1993 Mar 1;81(5):1109–1123. [PubMed] [Google Scholar]
  89. Weil J. V., Castro O., Malik A. B., Rodgers G., Bonds D. R., Jacobs T. P. NHLBI Workshop Summary. Pathogenesis of lung disease in sickle hemoglobinopathies. Am Rev Respir Dis. 1993 Jul;148(1):249–256. doi: 10.1164/ajrccm/148.1.249. [DOI] [PubMed] [Google Scholar]
  90. Young R. C., Jr, Rachal R. E., Reindorf C. A., Armstrong E. M., Polk O. D., Jr, Hackney R. L., Jr, Scott R. B. Lung function in sickle cell hemoglobinopathy patients compared with healthy subjects. J Natl Med Assoc. 1988 May;80(5):509–514. [PMC free article] [PubMed] [Google Scholar]
  91. Yung G. L., Channick R. N., Fedullo P. F., Auger W. R., Kerr K. M., Jamieson S. W., Kapelanski D. P., Moser K. M. Successful pulmonary thromboendarterectomy in two patients with sickle cell disease. Am J Respir Crit Care Med. 1998 May;157(5 Pt 1):1690–1693. doi: 10.1164/ajrccm.157.5.9710032. [DOI] [PubMed] [Google Scholar]

Articles from Postgraduate Medical Journal are provided here courtesy of BMJ Publishing Group

RESOURCES