About this Journal Submit a Manuscript Table of Contents
BioMed Research International
Volume 2013 (2013), Article ID 604140, 12 pages
http://dx.doi.org/10.1155/2013/604140
Review Article

Asthma Management in Sickle Cell Disease

1Department of Hematology-Oncology, Children’s Hospital & Research Center Oakland, Oakland, CA 94609, USA
2Division of Pediatric Emergency Medicine, Department of Pediatrics, Emory University School of Medicine, 1645 Tullie Circle, NE, Atlanta, GA 30329, USA

Received 24 May 2013; Revised 5 September 2013; Accepted 13 September 2013

Academic Editor: Susanne Saussele

Copyright © 2013 Esteban Gomez and Claudia R. Morris. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Linked References

  1. National Heart, Lung, and Blood Institute, “Disease and conditions index,” in Sickle Cell Anemia: Who Is at Risk? US Department of Health and Human Services, National Institutes of Health, National Heart, Lung, and Blood Institute, Bethesda, Md, USA, 2009.
  2. H. S. Zahran, C. Bailey, and P. Garbe, “Vital signs: asthma prevalence, disease characteristics, and self-management education—United States, 2001–2009,” Morbidity and Mortality Weekly Report, vol. 60, no. 17, pp. 547–552, 2011. View at Scopus
  3. J. H. Boyd, E. A. Macklin, R. C. Strunk, and M. R. DeBaun, “Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia,” Blood, vol. 108, no. 9, pp. 2923–2927, 2006. View at Publisher · View at Google Scholar · View at Scopus
  4. D. M. Mannino, D. M. Homa, L. J. Akinbami, J. E. Moorman, C. Gwynn, and S. C. Redd, “Surveillance for asthma—United States, 1980–1999,” Morbidity and Mortality Weekly Report: Surveillance Summaries, vol. 51, no. 1, pp. 1–13, 2002. View at Scopus
  5. Y. Y. Meng, S. H. Babey, E. Malcolm, E. R. Brown, and N. Chawla, Asthma in California: Findings from the 2001 California Health Interview Survey, UCLA Center for Health Policy Research, Los Angeles, Calif, USA, 2003.
  6. W. Hagar, J. Michlitsch, J. Gardner, E. Vichinsky, and C. R. Morris, “Differences in disease patterns between adults and children with pulmonary hypertension and sickle cell disease,” British Journal of Haematology, vol. 140, pp. 104–112, 2008.
  7. C. R. Morris, “Asthma management: reinventing the wheel in sickle cell disease,” The American Journal of Hematology, vol. 84, no. 4, pp. 234–241, 2009. View at Publisher · View at Google Scholar · View at Scopus
  8. A. C. Koumbourlis, H. J. Zar, A. Hurlet-Jensen, and M. R. Goldberg, “Prevalence and reversibility of lower airway obstruction in children with sickle cell disease,” Journal of Pediatrics, vol. 138, no. 2, pp. 188–192, 2001. View at Publisher · View at Google Scholar · View at Scopus
  9. O. Y. Ozbek, B. Malbora, N. Sen, A. C. Yazici, E. Ozyurek, and N. Ozbek, “Airway hyperreactivity detected by methacholine challenge in children with sickle cell disease,” Pediatric Pulmonology, vol. 42, no. 12, pp. 1187–1192, 2007. View at Publisher · View at Google Scholar · View at Scopus
  10. M. A. Leong, C. Dampier, L. Varlotta, and J. L. Allen, “Airway hyperreactivity in children with sickle cell disease,” Journal of Pediatrics, vol. 131, no. 2, pp. 278–285, 1997. View at Publisher · View at Google Scholar · View at Scopus
  11. N. Sen, I. Kozanoglu, M. Karatasli, H. Ermis, C. Boga, and F. O. Eyuboglu, “Pulmonary function and airway hyperresponsiveness in adults with sickle cell disease,” Lung, vol. 187, no. 3, pp. 195–200, 2009. View at Publisher · View at Google Scholar · View at Scopus
  12. R. C. Strunk, M. S. Brown, J. H. Boyd, P. Bates, J. J. Field, and M. R. DeBaun, “Methacholine challenge in children with sickle cell disease: a case series,” Pediatric Pulmonology, vol. 43, no. 9, pp. 924–929, 2008. View at Publisher · View at Google Scholar · View at Scopus
  13. J. J. Field, J. Stocks, F. J. Kirkham et al., “Airway hyperresponsiveness in children with sickle cell anemia,” Chest, vol. 139, no. 3, pp. 563–568, 2011. View at Publisher · View at Google Scholar · View at Scopus
  14. R. W. Hagar, J. G. Michlitsch, J. Gardner, E. P. Vichinsky, and C. R. Morris, “Clinical differences between children and adults with pulmonary hypertension and sickle cell disease,” The British Journal of Haematology, vol. 140, no. 1, pp. 104–112, 2008. View at Publisher · View at Google Scholar · View at Scopus
  15. N. R. Shilo and L. C. Lands, “Asthma and chronic sickle cell lung disease: a dynamic relationship,” Paediatric Respiratory Reviews, vol. 12, no. 1, pp. 78–82, 2011. View at Publisher · View at Google Scholar · View at Scopus
  16. S. Intzes, R. V. Kalpatthi, R. Short, and H. Imran, “Pulmonary function abnormalities and asthma are prevalent in children with sickle cell disease and are associated with acute chest syndrome,” Pediatric Hematology/Oncology. In press.
  17. S. T. Weiss, I. B. Tager, J. W. Weiss, A. Munoz, F. E. Speizer, and R. H. Ingram, “Airways responsiveness in a population sample of adults and children,” The American Review of Respiratory Disease, vol. 129, no. 6, pp. 898–902, 1984. View at Scopus
  18. J. M. Knight-Madden, A. Barton-Gooden, S. R. Weaver, M. Reid, and A. Greenough, “Mortality, asthma, smoking and acute chest syndrome in young adults with sickle cell disease,” Lung, vol. 191, no. 1, pp. 95–100, 2013. View at Publisher · View at Google Scholar
  19. J. H. Boyd, A. Moinuddin, R. C. Strunk, and M. R. DeBaun, “Asthma and acute chest in sickle-cell disease,” Pediatric Pulmonology, vol. 38, no. 3, pp. 229–232, 2004. View at Publisher · View at Google Scholar · View at Scopus
  20. J. M. Knight-Madden, T. S. Forrester, N. A. Lewis, and A. Greenough, “Asthma in children with sickle cell disease and its association with acute chest syndrome,” Thorax, vol. 60, no. 3, pp. 206–210, 2005. View at Publisher · View at Google Scholar · View at Scopus
  21. M. E. Nordness, J. Lynn, M. C. Zacharisen, P. J. Scott, and K. J. Kelly, “Asthma is a risk factor for acute chest syndrome and cerebral vascular accidents in children with sickle cell disease,” Clinical and Molecular Allergy, vol. 3, article 2, 2005. View at Publisher · View at Google Scholar · View at Scopus
  22. J. H. Boyd, E. A. Macklin, R. C. Strunk, and M. R. DeBaun, “Asthma is associated with increased mortality in individuals with sickle cell anemia,” Haematologica, vol. 92, no. 8, pp. 1115–1118, 2007. View at Publisher · View at Google Scholar · View at Scopus
  23. J. H. Boyd, M. R. Debaun, W. J. Morgan, J. Mao, and R. C. Strunk, “Lower airway obstruction is associated with increased morbidity in children with sickle cell disease,” Pediatric Pulmonology, vol. 44, no. 3, pp. 290–296, 2009. View at Publisher · View at Google Scholar · View at Scopus
  24. R. Paul, C. P. Minniti, M. Nouraie et al., “Clinical correlates of acute pulmonary events in children and adolescents with sickle cell disease,” European Journal of Haematology, vol. 91, no. 1, pp. 62–68, 2013. View at Publisher · View at Google Scholar
  25. J. A. Glassberg, J. Wang, R. Cohen, L. D. Richardson, and M. R. DeBaun, “Risk factors for increased ED utilization in a multinational cohort of children with sickle cell disease,” Academic Emergency Medicine, vol. 19, no. 6, pp. 664–672, 2012. View at Publisher · View at Google Scholar
  26. J. A. Glassberg, A. Chow, J. Wisnivesky, R. Hoffman, M. R. Debaun, and L. D. Richardson, “Wheezing and asthma are independent risk factors for increased sickle cell disease morbidity,” The British Journal of Haematology, vol. 159, no. 4, pp. 472–479, 2012. View at Publisher · View at Google Scholar
  27. M. Nouraie, S. Rana, O. Castro et al., “Predictors of mortality in children and adolescents with sickle cell disease: the PUSH study,” Blood, vol. 108, no. 9, pp. 2923–2927, 2011.
  28. J. J. Field, J. Horst, R. C. Strunk, F. V. White, and M. R. DeBaun, “Death due to asthma in two adolescents with sickle cell disease,” Pediatric Blood and Cancer, vol. 56, no. 3, pp. 454–457, 2011. View at Publisher · View at Google Scholar · View at Scopus
  29. M. Newaskar, K. A. Hardy, and C. R. Morris, “Asthma in sickle cell disease,” TheScientificWorldJournal, vol. 11, pp. 1138–1152, 2011. View at Publisher · View at Google Scholar · View at Scopus
  30. J. A. Knight-Madden and I. R. Hambleton, “Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease,” Cochrane Database of Systematic Reviews, no. 3, Article ID CD003733, 2003. View at Scopus
  31. P. Bradding, A. F. Walls, and S. T. Holgate, “The role of the mast cell in the pathophysiology of asthma,” Journal of Allergy and Clinical Immunology, vol. 117, no. 6, pp. 1277–1284, 2006. View at Publisher · View at Google Scholar · View at Scopus
  32. M. Larché, D. S. Robinson, and A. B. Kay, “The role of T lymphocytes in the pathogenesis of asthma,” Journal of Allergy and Clinical Immunology, vol. 111, no. 3, pp. 450–463, 2003. View at Publisher · View at Google Scholar · View at Scopus
  33. N. W. Lukacs, “Role of chemokines in the pathogenesis of asthma,” Nature Reviews Immunology, vol. 1, no. 2, pp. 108–116, 2001. View at Scopus
  34. J. A. Elias, C. G. Lee, T. Zheng, B. Ma, R. J. Homer, and Z. Zhu, “New insights into the pathogenesis of asthma,” Journal of Clinical Investigation, vol. 111, no. 3, pp. 291–297, 2003. View at Publisher · View at Google Scholar · View at Scopus
  35. A. Pathare, S. Al Kindi, S. Daar, and D. Dennison, “Cytokines in sickle cell disease,” Hematology, vol. 8, no. 5, pp. 329–337, 2003. View at Scopus
  36. A. J. Duits, R. C. Pieters, A. W. Saleh et al., “Enhanced levels of soluble VCAM-1 in sickle cell patients and their specific increment during vasoocclusive crisis,” Clinical Immunology and Immunopathology, vol. 81, no. 1, pp. 96–98, 1996. View at Publisher · View at Google Scholar · View at Scopus
  37. S. D. Nandedkar, T. R. Feroah, W. Hutchins et al., “Histopathology of experimentally induced asthma in a murine model of sickle cell disease,” Blood, vol. 112, no. 6, pp. 2529–2538, 2008. View at Publisher · View at Google Scholar · View at Scopus
  38. J. J. Field, J. Krings, N. L. White et al., “Urinary cysteinyl leukotriene e4 is associated with increased risk for pain and acute chest syndrome in adults with sickle cell disease,” The American Journal of Hematology, vol. 84, no. 3, pp. 158–160, 2009. View at Publisher · View at Google Scholar · View at Scopus
  39. J. J. Field, R. C. Strunk, J. E. Knight-Perry, M. A. Blinder, R. R. Townsend, and M. R. DeBaun, “Urinary cysteinyl leukotriene E4 significantly increases during pain in children and adults with sickle cell disease,” The American Journal of Hematology, vol. 84, no. 4, pp. 231–233, 2009. View at Publisher · View at Google Scholar · View at Scopus
  40. J. E. Jennings, T. Ramkumar, J. Mao et al., “Elevated urinary leukotriene E4 levels are associated with hospitalization for pain in children with sickle cell disease,” The American Journal of Hematology, vol. 83, no. 8, pp. 640–643, 2008. View at Publisher · View at Google Scholar · View at Scopus
  41. L. A. Styles, C. G. Schalkwijk, A. J. Aarsman, E. P. Vichinsky, B. H. Lubin, and F. A. Kuypers, “Phospholipase A2 levels in acute chest syndrome of sickle cell disease,” Blood, vol. 87, no. 6, pp. 2573–2578, 1996. View at Scopus
  42. L. A. Styles, M. Abboud, S. Larkin, M. Lo, and F. A. Kuypers, “Transfusion prevents acute chest syndrome predicted by elevated secretory phospholipase A2,” The British Journal of Haematology, vol. 136, no. 2, pp. 343–344, 2007. View at Publisher · View at Google Scholar · View at Scopus
  43. D. L. Bowton, M. C. Seeds, M. B. Fasano, B. Goldsmith, and D. A. Bass, “Phospholipase A2 and arachidonate increase in bronchoalveolar lavage fluid after inhaled antigen challenge in asthmatics,” The American Journal of Respiratory and Critical Care Medicine, vol. 155, no. 2, pp. 421–425, 1997. View at Scopus
  44. S. O. Shaheen, R. B. Newson, S. M. Ring, M. J. Rose-Zerilli, J. W. Holloway, and A. J. Henderson, “Prenatal and infant acetaminophen exposure, antioxidant gene polymorphisms, and childhood asthma,” Journal of Allergy and Clinical Immunology, vol. 126, no. 6, pp. 1141.e7–1148.e7, 2010. View at Publisher · View at Google Scholar · View at Scopus
  45. N. E. King, M. E. Rothenberg, and N. Zimmermann, “Arginine in asthma and lung inflammation,” Journal of Nutrition, vol. 134, pp. 2830S–2836S, 2004.
  46. C. R. Morris, M. Poljakovic, L. Lavrisha, L. Machado, F. A. Kuypers, and S. M. Morris Jr., “Decreased arginine bioavailability and increased serum arginase activity in asthma,” The American Journal of Respiratory and Critical Care Medicine, vol. 170, no. 2, pp. 148–153, 2004. View at Scopus
  47. A. Lara, S. B. Khatri, Z. Wang et al., “Alterations of the arginine metabolome in asthma,” The American Journal of Respiratory and Critical Care Medicine, vol. 178, no. 7, pp. 673–681, 2008. View at Publisher · View at Google Scholar · View at Scopus
  48. H. Maarsingh, T. Pera, and H. Meurs, “Arginase and pulmonary diseases,” Naunyn-Schmiedeberg's Archives of Pharmacology, vol. 378, no. 2, pp. 171–184, 2008. View at Publisher · View at Google Scholar · View at Scopus
  49. H. Maarsingh, B. E. Bossenga, I. S. T. Bos, H. H. Volders, J. Zaagsma, and H. Meurs, “L-arginine deficiency causes airway hyperresponsiveness after the late asthmatic reaction,” European Respiratory Journal, vol. 34, no. 1, pp. 191–199, 2009. View at Publisher · View at Google Scholar · View at Scopus
  50. H. Maarsingh, J. Zaagsma, and H. Meurs, “Arginase: a key enzyme in the pathophysiology of allergic asthma opening novel therapeutic perspectives,” The British Journal of Pharmacology, vol. 158, no. 3, pp. 652–664, 2009. View at Publisher · View at Google Scholar · View at Scopus
  51. R. Benson, K. A. Hardy, and K. A. C. R M, “Arginase and arginine dysregulation in asthma,” Journal of Allergy, vol. 2011, Article ID 736319, 12 pages, 2011. View at Publisher · View at Google Scholar
  52. C. R. Morris, F. A. Kuypers, S. Larkin, E. P. Vichinsky, and L. A. Styles, “Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome,” Journal of Pediatric Hematology/Oncology, vol. 22, no. 6, pp. 515–520, 2000. View at Scopus
  53. C. R. Morris, G. J. Kato, M. Poljakovic et al., “Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease,” The Journal of the American Medical Association, vol. 294, no. 1, pp. 81–90, 2005. View at Publisher · View at Google Scholar · View at Scopus
  54. C. R. Morris, “Hemolysis-associated pulmonary hypertension in sickle cell disease: global disruption of the arginine-nitric oxide pathway,” Current Hypertension Reviews, vol. 3, no. 3, pp. 223–230, 2007. View at Publisher · View at Google Scholar · View at Scopus
  55. D. K. Kaul, X. Zhang, T. Dasgupta, and M. E. Fabry, “Arginine therapy of transgenic-knockout sickle mice improves microvascular function by reducing non-nitric oxide vasodilators, hemolysis, and oxidative stress,” The American Journal of Physiology—Heart and Circulatory Physiology, vol. 295, no. 1, pp. H39–H47, 2008. View at Publisher · View at Google Scholar · View at Scopus
  56. C. R. Morris, “Mechanisms of vasculopathy in sickle cell disease and thalassemia,” Hematology/the Education Program of the American Society of Hematology, pp. 177–185, 2008. View at Scopus
  57. F. Holguin, S. A. Comhair, S. L. Hazen et al., “An association between L-arginine/asymmetric dimethyl arginine balance, obesity, and the age of asthma onset phenotype,” The American Journal of Respiratory and Critical Care Medicine, vol. 187, no. 2, pp. 153–159, 2013. View at Publisher · View at Google Scholar
  58. J. A. Scott, M. L. North, M. Rafii et al., “Asymmetric dimethylarginine is increased in asthma,” The American Journal of Respiratory and Critical Care Medicine, vol. 184, no. 7, pp. 779–785, 2011. View at Publisher · View at Google Scholar · View at Scopus
  59. G. J. Kato, Z. Wang, R. F. MacHado, W. C. Blackwelder, J. G. Taylor VI, and S. L. Hazen, “Endogenous nitric oxide synthase inhibitors in sickle cell disease: abnormal levels and correlations with pulmonary hypertension, desaturation, haemolysis, organ dysfunction and death,” The British Journal of Haematology, vol. 145, no. 4, pp. 506–513, 2009. View at Publisher · View at Google Scholar · View at Scopus
  60. P. P. Landburg, T. Teerlink, F. A. J. Muskiet, A. J. Duits, and J. J. B. Schnog, “Plasma concentrations of asymmetric dimethylarginine, an endogenous nitric oxide synthase inhibitor, are elevated in sickle cell patients but do not increase further during painful crisis,” The American Journal of Hematology, vol. 83, no. 7, pp. 577–579, 2008. View at Publisher · View at Google Scholar · View at Scopus
  61. J. B. Schnog, T. Teerlink, F. P. L. van der Dijs, A. J. Duits, and F. A. J. Muskiet, “Plasma levels of asymmetric dimethylarginine (ADMA), an endogenous nitric oxide synthase inhibitor, are elevated in sickle cell disease,” Annals of Hematology, vol. 84, no. 5, pp. 282–286, 2005. View at Publisher · View at Google Scholar · View at Scopus
  62. L. R. DeNicola, N. Kissoon, L. J. Duckworth, K. V. Blake, S. P. Murphy, and P. E. Silkoff, “Exhaled nitric oxide as an indicator of severity of asthmatic inflammation,” Pediatric Emergency Care, vol. 16, no. 4, pp. 290–295, 2000. View at Publisher · View at Google Scholar · View at Scopus
  63. R. E. Girgis, M. A. Qureshi, J. Abrams, and P. Swerdlow, “Decreased exhaled nitric oxide in sickle cell disease: relationship with chronic lung involvement,” The American Journal of Hematology, vol. 72, no. 3, pp. 177–184, 2003. View at Publisher · View at Google Scholar · View at Scopus
  64. K. J. Sullivan, N. Kissoon, L. J. Duckworth et al., “Low exhaled nitric oxide and a polymorphism in the NOS I gene is associated with acute chest syndrome,” The American Journal of Respiratory and Critical Care Medicine, vol. 164, no. 12, pp. 2186–2190, 2002. View at Scopus
  65. D. K. Radhakrishnan, G. N. Bendiak, D. Mateos-Corral et al., “Lower airway nitric oxide is increased in children with sickle cell disease,” Journal of Pediatrics, vol. 160, no. 1, pp. 93–97, 2012. View at Publisher · View at Google Scholar · View at Scopus
  66. C. R. Morris, E. P. Vichinsky, J. van Warmerdam et al., “Hydroxyurea and arginine therapy: impact on nitric oxide production in sickle cell disease,” Journal of Pediatric Hematology/Oncology, vol. 25, no. 8, pp. 629–634, 2003. View at Publisher · View at Google Scholar · View at Scopus
  67. H. Meurs, H. Maarsingh, and J. Zaagsma, “Arginase and asthma: novel insights into nitric oxide homeostasis and airway hyperresponsiveness,” Trends in Pharmacological Sciences, vol. 24, no. 9, pp. 450–455, 2003. View at Publisher · View at Google Scholar · View at Scopus
  68. M. L. North, N. Khanna, P. A. Marsden, H. Grasemann, and J. A. Scott, “Functionally important role for arginase 1 in the airway hyperresponsiveness of asthma,” The American Journal of Physiology—Lung Cellular and Molecular Physiology, vol. 296, no. 6, pp. L911–L920, 2009. View at Publisher · View at Google Scholar · View at Scopus
  69. H. Li, C. J. Meininger, J. R. Hawker Jr. et al., “Regulatory role of arginase I and II in nitric oxide, polyamine, and proline syntheses in endothelial cells,” The American Journal of Physiology—Endocrinology and Metabolism, vol. 280, no. 1, pp. E75–E82, 2001. View at Scopus
  70. W. Durante, F. K. Johnson, and R. A. Johnson, “Arginase: a critical regulator of nitric oxide synthesis and vascular function,” Clinical and Experimental Pharmacology and Physiology, vol. 34, no. 9, pp. 906–911, 2007. View at Publisher · View at Google Scholar · View at Scopus
  71. C. R. Morris, M. T. Gladwin, and G. J. Kato, “Nitric oxide and arginine dysregulation: a novel pathway to pulmonary hypertension in hemolytic disorders,” Current Molecular Medicine, vol. 8, no. 7, pp. 620–632, 2008. View at Publisher · View at Google Scholar · View at Scopus
  72. C. O. Enwonwu, “Increased metabolic demand for arginine in sickle cell anaemia,” Medical Science Research, vol. 17, no. 23, pp. 997–998, 1989. View at Scopus
  73. B. L. Lopez, A. A. Kreshak, C. R. Morris, L. Davis-Moon, S. K. Ballas, and X. Ma, “L-arginine levels are diminished in adult acute vaso-occlusive sickle cell crisis in the emergency department,” The British Journal of Haematology, vol. 120, no. 3, pp. 532–534, 2003. View at Publisher · View at Google Scholar · View at Scopus
  74. C. R. Morris, F. A. Kuypers, L. Lavrisha et al., “A randomized, placebo-control trial of arginine therapy for the treatment ofchildren with sickle cell disease hospitalized with vaso-occlusive pain episodes,” Haematologica, vol. 98, no. 9, pp. 1375–1382, 2013. View at Publisher · View at Google Scholar
  75. G. J. Kato, V. McGowan, R. F. Machado et al., “Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease,” Blood, vol. 107, no. 6, pp. 2279–2285, 2006. View at Publisher · View at Google Scholar · View at Scopus
  76. K. A. Pritchard Jr., T. R. Feroah, S. D. Nandedkar et al., “Effects of experimental asthma on inflammation and lung mechanics in sickle cell mice,” The American Journal of Respiratory Cell and Molecular Biology, vol. 46, no. 3, pp. 389–396, 2012. View at Publisher · View at Google Scholar · View at Scopus
  77. V. R. Gordeuk, C. P. Minniti, M. Nouraie et al., “Elevated tricuspid regurgitation velocity and decline in exercise capacity over 22 months of follow up in children and adolescents with sickle cell anemia,” Haematologica, vol. 96, no. 1, pp. 33–40, 2011. View at Publisher · View at Google Scholar · View at Scopus
  78. G. J. Kato, M. T. Gladwin, and M. H. Steinberg, “Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes,” Blood Reviews, vol. 21, no. 1, pp. 37–47, 2007. View at Publisher · View at Google Scholar · View at Scopus
  79. R. P. Hebbel, “Reconstructing sickle cell disease: a data-based analysis of the “hyperhemolysis paradigm” for pulmonary hypertension from the perspective of evidence-based medicine,” The American Journal of Hematology, vol. 86, no. 2, pp. 123–154, 2011. View at Publisher · View at Google Scholar · View at Scopus
  80. G. Wu and S. M. Morris Jr., “Arginine metabolism: nitric oxide and beyond,” Biochemical Journal, vol. 336, no. 1, pp. 1–17, 1998. View at Scopus
  81. C. R. Morris, “Mechanisms of vasculopathy in sickle cell disease and thalassemia,” Hematology/the Education Program of the American Society of Hematology, pp. 177–185, 2008. View at Publisher · View at Google Scholar
  82. R. P. Rother, L. Bell, P. Hillmen, and M. T. Gladwin, “The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease,” The Journal of the American Medical Association, vol. 293, no. 13, pp. 1653–1662, 2005. View at Publisher · View at Google Scholar · View at Scopus
  83. S. I. Zharikov and E. R. Block, “Association of L-arginine transporters with fodrin: implications for hypoxic inhibition of arginine uptake,” The American Journal of Physiology—Lung Cellular and Molecular Physiology, vol. 278, no. 1, pp. L111–L117, 2000. View at Scopus
  84. P. Gräf, U. Förstermann, and E. I. Closs, “The transport activity of the human cationic amino acid transporter hCAT-1 is downregulated by activation of protein kinase C,” The British Journal of Pharmacology, vol. 132, no. 6, pp. 1193–1200, 2001. View at Scopus
  85. C. D. Reiter, X. Wang, J. E. Tanus-Santos et al., “Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease,” Nature Medicine, vol. 8, no. 12, pp. 1383–1389, 2002. View at Publisher · View at Google Scholar · View at Scopus
  86. P. M. Dias-Da-Motta, V. R. Arruda, M. N. Muscará et al., “The release of nitric oxide and superoxide anion by neutrophils and mononuclear cells from patients with sickle cell anaemia,” The British Journal of Haematology, vol. 93, no. 2, pp. 333–340, 1996. View at Scopus
  87. M. Aslan, T. M. Ryan, B. Adler et al., “Oxygen radical inhibition of nitric oxide-dependent vascular function in sickle cell disease,” Proceedings of the National Academy of Sciences of the United States of America, vol. 98, no. 26, pp. 15215–15220, 2001. View at Publisher · View at Google Scholar · View at Scopus
  88. M. Aslan and B. A. Freeman, “Oxidant-mediated impairment of nitric oxide signaling in sickle cell disease—mechanisms and consequences,” Cellular and Molecular Biology, vol. 50, no. 1, pp. 95–105, 2004. View at Scopus
  89. S. K. Das and R. C. Nair, “Superoxide dismutase, glutathione peroxidase, catalase and lipid peroxidation of normal and sickled erythrocytes,” The British Journal of Haematology, vol. 44, no. 1, pp. 87–92, 1980. View at Scopus
  90. L. Schacter, J. A. Warth, E. M. Gordon, A. Prasad, and B. L. Klein, “Altered amount and activity of superoxide dismutase in sickle cell anemia,” FASEB Journal, vol. 2, no. 3, pp. 237–243, 1988. View at Scopus
  91. K. C. Wood, R. P. Hebbel, D. J. Lefer, and D. N. Granger, “Critical role of endothelial cell-derived nitric oxide synthase in sickle cell disease-induced microvascular dysfunction,” Free Radical Biology and Medicine, vol. 40, no. 8, pp. 1443–1453, 2006. View at Publisher · View at Google Scholar · View at Scopus
  92. L. L. Hsu, H. C. Champion, S. A. Campbell-Lee et al., “Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability,” Blood, vol. 109, no. 7, pp. 3088–3098, 2007. View at Publisher · View at Google Scholar · View at Scopus
  93. Y. Xia, V. L. Dawson, T. M. Dawson, S. H. Snyder, and J. L. Zweier, “Nitric oxide synthase generates superoxide and nitric oxide in arginine-depleted cells leading to peroxynitrite-mediated cellular injury,” Proceedings of the National Academy of Sciences of the United States of America, vol. 93, no. 13, pp. 6770–6774, 1996. View at Scopus
  94. NHLBI, Expert Panel Report 3: Guidelines for the Diagnosis and Management of Asthma, National Heart, Lung, and Blood Institute National Asthma Education and Prevention Program, 2007.
  95. E. P. Vichinsky, L. A. Styles, L. H. Colangelo, E. C. Wright, O. Castro, and B. Nickerson, “Acute chest syndrome in sickle cell disease: clinical presentation and course,” Blood, vol. 89, no. 5, pp. 1787–1792, 1997. View at Scopus
  96. C. Morris, E. Vichinsky, and L. Styles, “Clinician assessment for acute chest syndrome in febrile patients with sickle cell disease: is it accurate enough?” Annals of Emergency Medicine, vol. 34, no. 1, pp. 64–69, 1999. View at Publisher · View at Google Scholar · View at Scopus
  97. K. L. Phillips, P. An, J. H. Boyd et al., “Major gene effect and additive familial pattern of inheritance of asthma exist among families of probands with sickle cell anemia and asthma,” The American Journal of Human Biology, vol. 20, no. 2, pp. 149–153, 2008. View at Publisher · View at Google Scholar · View at Scopus
  98. J. J. Field, E. A. Macklin, Y. Yan, R. C. Strunk, and M. R. DeBaun, “Sibling history of asthma is a risk factor for pain in children with sickle cell anemia,” The American Journal of Hematology, vol. 83, no. 11, pp. 855–857, 2008. View at Publisher · View at Google Scholar · View at Scopus
  99. A. L. Jani and D. L. Hamilos, “Current thinking on the relationship between rhinosinusitis and asthma,” Journal of Asthma, vol. 42, no. 1, pp. 1–7, 2005. View at Publisher · View at Google Scholar · View at Scopus
  100. M. Teodorescu, F. B. Consens, W. F. Bria et al., “Predictors of habitual snoring and obstructive sleep apnea risk in patients with asthma,” Chest, vol. 135, no. 5, pp. 1125–1132, 2009. View at Publisher · View at Google Scholar · View at Scopus
  101. J. S. Debley, E. R. Carter, and G. J. Redding, “Prevalence and impact of gastroesophageal reflux in adolescents with asthma: a population-based study,” Pediatric Pulmonology, vol. 41, no. 5, pp. 475–481, 2006. View at Publisher · View at Google Scholar · View at Scopus
  102. K. P. Sylvester, R. A. Patey, P. Milligan et al., “Pulmonary function abnormalities in children with sickle cell disease,” Thorax, vol. 59, no. 1, pp. 67–70, 2004. View at Scopus
  103. J. M. Knight-Madden, T. S. Forrester, N. A. Lewis, and A. Greenough, “The impact of recurrent acute chest syndrome on the lung function of young adults with sickle cell disease,” Lung, vol. 188, no. 6, pp. 499–504, 2010. View at Publisher · View at Google Scholar · View at Scopus
  104. E. S. Klings, D. F. Wyszynski, V. G. Nolan, and M. H. Steinberg, “Abnormal pulmonary function in adults with sickle cell anemia,” The American Journal of Respiratory and Critical Care Medicine, vol. 173, no. 11, pp. 1264–1269, 2006. View at Publisher · View at Google Scholar · View at Scopus
  105. J. E. MacLean, E. Atenafu, M. Kirby-Allen et al., “Longitudinal decline in lung volume in a population of children with sickle cell disease,” The American Journal of Respiratory and Critical Care Medicine, vol. 178, no. 10, pp. 1055–1059, 2008. View at Publisher · View at Google Scholar · View at Scopus
  106. M. C. Walters, K. Hardy, S. Edwards et al., “Pulmonary, gonadal, and central nervous system status after bone marrow transplantation for sickle cell disease,” Biology of Blood and Marrow Transplantation, vol. 16, no. 2, pp. 263–272, 2010. View at Publisher · View at Google Scholar · View at Scopus
  107. J. E. Knight-Perry, J. J. Field, M. R. DeBaun, J. Stocks, J. Kirkby, and R. C. Strunk, “Hospital admission for acute painful episode following methacholine challenge in an adolescent with sickle cell disease,” Pediatric Pulmonology, vol. 44, no. 7, pp. 728–730, 2009. View at Publisher · View at Google Scholar · View at Scopus
  108. National Heart, Lung and Blood Institute, “New NHLBI guidelines for the diagnosis and management of asthma,” Lippincott Health Promotion Letter, vol. 2, no. 1, pp. 8–9, 1997.
  109. D. Rastogi, A. Shetty, R. Neugebauer, and A. Harijith, “National heart, lung, and blood institute guidelines and asthma management practices among inner-city pediatric primary care providers,” Chest, vol. 129, no. 3, pp. 619–623, 2006. View at Publisher · View at Google Scholar · View at Scopus
  110. L. C. Denlinger, C. A. Sorkness, V. M. Chinchilli, and R. F. Lemanske Jr., “Guideline-defining asthma clinical trials of the national heart, lung, and blood Institute's asthma clinical research network and childhood asthma research and education network,” Journal of Allergy and Clinical Immunology, vol. 119, no. 1, pp. 3–11, 2007. View at Publisher · View at Google Scholar · View at Scopus
  111. 3 NAEaPPEPR, Guidelines For the Diagnosis and Management of Asthma, National Heart, Lung and Blood Institutes, National Institutes of Health, Bethesda, Md, USA, 2007.
  112. W. C. Moore, “Update in asthma 2007,” The American Journal of Respiratory and Critical Care Medicine, vol. 177, no. 10, pp. 1068–1073, 2008. View at Publisher · View at Google Scholar · View at Scopus
  113. J. Corrarino, “New guidelines for diagnosis and management of asthma,” MCN: The American Journal of Maternal/Child Nursing, vol. 33, no. 2, p. 136, 2008. View at Publisher · View at Google Scholar · View at Scopus
  114. J. J. Strouse, C. M. Takemoto, J. R. Keefer, G. J. Kato, and J. F. Casella, “Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease,” Pediatric Blood and Cancer, vol. 50, no. 5, pp. 1006–1012, 2008. View at Publisher · View at Google Scholar · View at Scopus
  115. T. C. Griffin, D. Mcintire, and G. R. Buchanan, “High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease,” The New England Journal of Medicine, vol. 330, no. 11, pp. 733–737, 1994. View at Publisher · View at Google Scholar · View at Scopus
  116. A. J. Bennett and F. Rosner, “Glucocorticoid therapy of sickle-cell disease,” The Lancet, vol. 2, no. 8140, p. 474, 1979. View at Scopus
  117. D. S. Darbari, O. Castro, J. G. Taylor VI et al., “Severe vaso-occlusive episodes associated with use of systemic corticosteroids in patients with sickle cell disease,” Journal of the National Medical Association, vol. 100, no. 8, pp. 948–951, 2008. View at Scopus
  118. D. D. Gladman and C. Bombardier, “Sickle cell crisis following intraarticular steroid therapy for rheumatoid arthritis,” Arthritis and Rheumatism, vol. 30, no. 9, pp. 1065–1068, 1987. View at Scopus
  119. S. H. Embury, J. F. Garcia, N. Mohandas, R. Pennathur-Das, and M. R. Clark, “Effects of oxygen inhalation on endogenous erythropoietin kinetics, erythropoiesis, and properties of blood cells in sickle-cell anemia,” The New England Journal of Medicine, vol. 311, no. 5, pp. 291–295, 1984. View at Scopus
  120. R. Zennadi, B. J. Moeller, E. J. Whalen et al., “Epinephrine-induced activation of LW-mediated sickle cell adhesion and vaso-occlusion in vivo,” Blood, vol. 110, no. 7, pp. 2708–2717, 2007. View at Publisher · View at Google Scholar · View at Scopus
  121. R. Zennadi, P. C. Hines, L. M. de Castro, J. Cartron, L. V. Parise, and M. J. Telen, “Epinephrine acts through erythroid signaling pathways to activate sickle cell adhesion to endothelium via LW-αvβ3 interactions,” Blood, vol. 104, no. 12, pp. 3774–3781, 2004. View at Publisher · View at Google Scholar · View at Scopus
  122. R. Zennadi, A. Chien, K. Xu, M. Batchvarova, and M. J. Telen, “Sickle red cells induce adhesion of lymphocytes and monocytes to endothelium,” Blood, vol. 112, no. 8, pp. 3474–3483, 2008. View at Publisher · View at Google Scholar · View at Scopus
  123. M. J. Telen, “Role of adhesion molecules and vascular endothelium in the pathogenesis of sickle cell disease,” Hematology/the Education Program of the American Society of Hematology, pp. 84–90, 2007. View at Scopus
  124. P. C. Hines, Q. Zen, S. N. Burney et al., “Novel epinephrine and cyclic AMP-mediated activation of BCAM/Lu-dependent sickle (SS) RBC adhesion,” Blood, vol. 101, no. 8, pp. 3281–3287, 2003. View at Publisher · View at Google Scholar · View at Scopus
  125. C. E. Eyler, T. Jackson, L. E. Elliott et al., “β2-adrenergic receptor and adenylate cyclase gene polymorphisms affect sickle red cell adhesion,” The British Journal of Haematology, vol. 141, no. 1, pp. 105–108, 2008. View at Publisher · View at Google Scholar · View at Scopus
  126. H. S. Nelson, S. T. Weiss, E. K. Bleecker, S. W. Yancey, and P. M. Dorinsky, “The salmeterol multicenter asthma research trial: a comparison of usual pharmacotherapy for asthma or usual pharmacotherapy plus salmeterol,” Chest, vol. 129, no. 1, pp. 15–26, 2006. View at Publisher · View at Google Scholar · View at Scopus
  127. W. Bailey, M. Castro, J. Matz et al., “Asthma exacerbations in African Americans treated for 1 year with combination fluticasone propionate and salmeterol or fluticasone propionate alone,” Current Medical Research and Opinion, vol. 24, no. 6, pp. 1669–1682, 2008. View at Publisher · View at Google Scholar · View at Scopus
  128. R. I. Liem, L. T. Young, and A. A. Thompson, “Prolonged QTc interval in children and young adults with sickle cell disease at steady state,” Pediatric Blood and Cancer, vol. 52, no. 7, pp. 842–846, 2009. View at Publisher · View at Google Scholar · View at Scopus
  129. P. Thottathil, J. Acharya, A. J. Moss et al., “Risk of cardiac events in patients with asthma and long-QT syndrome treated with β2 agonists,” The American Journal of Cardiology, vol. 102, no. 7, pp. 871–874, 2008. View at Publisher · View at Google Scholar · View at Scopus
  130. S. Couillard, M. Benkerrou, R. Girot, V. Brousse, A. Ferster, and B. Bader-Meunier, “Steroid treatment in children with sickle-cell disease,” Haematologica, vol. 92, no. 3, pp. 425–426, 2007. View at Publisher · View at Google Scholar · View at Scopus
  131. S. T. Miller, L. A. Sleeper, C. H. Pegelow et al., “Prediction of adverse outcomes in children with sickle cell disease,” The New England Journal of Medicine, vol. 342, no. 2, pp. 83–89, 2000. View at Publisher · View at Google Scholar · View at Scopus
  132. A. Sobota, D. A. Graham, M. M. Heeney, and E. J. Neufeld, “Corticosteroids for acute chest syndrome in children with sickle cell disease: variation in use and association with length of stay and readmission,” The American Journal of Hematology, vol. 85, no. 1, pp. 24–28, 2010. View at Publisher · View at Google Scholar · View at Scopus
  133. B. Robinson, “Reversal of sickle-cell crisis by dexamethasone,” The Lancet, vol. 1, no. 8125, p. 1088, 1979. View at Scopus
  134. J. C. Bernini, Z. R. Rogers, E. S. Sandler, J. S. Reisch, C. T. Quinn, and G. R. Buchanan, “Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease,” Blood, vol. 92, no. 9, pp. 3082–3089, 1998. View at Scopus
  135. B. U. Mueller, K. J. Martin, W. Dreyer, L. I. Bezold, and D. H. Mahoney, “Prolonged QT interval in pediatric sickle cell disease,” Pediatric Blood and Cancer, vol. 47, no. 6, pp. 831–833, 2006. View at Publisher · View at Google Scholar · View at Scopus
  136. C. Boga, I. Kozanoglu, M. Yeral, and C. Bakar, “Assessment of corrected QT interval in sickle cell disease patients who undergo erythroapheresis,” Transfusion Medicine, vol. 17, no. 6, pp. 466–472, 2007. View at Publisher · View at Google Scholar · View at Scopus
  137. F. Akgül, E. Seyfeli, I. Melek et al., “Increased QT dispersion in sickle cell disease: effect of pulmonary hypertension,” Acta Haematologica, vol. 118, no. 1, pp. 1–6, 2007. View at Publisher · View at Google Scholar · View at Scopus
  138. C. T. Quinn, M. J. Stuart, K. Kesler et al., “Tapered oral dexamethasone for the acute chest syndrome of sickle cell disease,” The British Journal of Haematology, vol. 155, no. 2, pp. 263–267, 2011. View at Publisher · View at Google Scholar · View at Scopus
  139. M. S. Isakoff, J. A. Lillo, and J. N. Hagstrom, “A single-institution experience with treatment of severe acute chest syndrome: lack of rebound pain with dexamethasone plus transfusion therapy,” Journal of Pediatric Hematology/Oncology, vol. 30, no. 4, pp. 322–325, 2008. View at Publisher · View at Google Scholar · View at Scopus
  140. R. Kumar, S. Qureshi, P. Mohanty, S. P. Rao, and S. T. Miller, “A short course of prednisone in the management of acute chest syndrome of sickle cell disease,” Journal of Pediatric Hematology/Oncology, vol. 32, no. 3, pp. e91–e94, 2010. View at Publisher · View at Google Scholar · View at Scopus
  141. S. T. Holgate, “The acetaminophen enigma in asthma,” The American Journal of Respiratory and Critical Care Medicine, vol. 183, no. 2, pp. 147–148, 2011. View at Publisher · View at Google Scholar · View at Scopus
  142. S. C. Dharmage and K. J. Allen, “Does regular paracetamol ingestion increase the risk of developing asthma?” Clinical and Experimental Allergy, vol. 41, no. 4, pp. 459–460, 2011. View at Publisher · View at Google Scholar · View at Scopus
  143. A. M. Fitzpatrick, W. G. Teague, L. Burwell, M. S. Brown, and L. A. S. Brown, “Glutathione oxidation is associated with airway macrophage functional impairment in children with severe asthma,” Pediatric Research, vol. 69, no. 2, pp. 154–159, 2011. View at Publisher · View at Google Scholar · View at Scopus
  144. A. M. Fitzpatrick, W. G. Teague, F. Holguin, M. Yeh, and L. A. S. Brown, “Airway glutathione homeostasis is altered in children with severe asthma: evidence for oxidant stress,” Journal of Allergy and Clinical Immunology, vol. 123, no. 1, pp. 146.e8–152.e8, 2009. View at Publisher · View at Google Scholar · View at Scopus
  145. D. C. Rees, T. N. Williams, and M. T. Gladwin, “Sickle-cell disease,” The Lancet, vol. 376, no. 9757, pp. 2018–2031, 2010. View at Publisher · View at Google Scholar · View at Scopus
  146. S. Charache, M. L. Terrin, R. D. Moore et al., “Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia,” The New England Journal of Medicine, vol. 332, no. 20, pp. 1317–1322, 1995. View at Publisher · View at Google Scholar · View at Scopus
  147. S. A. Singh, A. C. Koumbourlis, and B. Aygun, “Resolution of chronic hypoxemia in pediatric sickle cell patients after treatment with hydroxyurea,” Pediatric Blood and Cancer, vol. 50, no. 6, pp. 1258–1260, 2008. View at Publisher · View at Google Scholar · View at Scopus