Sickle Cell Disease and Other Hemoglobinopathies

Goldman's Cecil Medicine: Twenty Fourth Edition

Volumn 1, Issue , 2012, Pages 1066-1075

  Steinberg, Martin H ^a,b  

^a BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b BOSTON MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 84879346295     PISSN: None     EISSN: None     Source Type: Book    
DOI: 10.1016/B978-1-4377-1604-7.00166-4     Document Type: Chapter

References (18)

1. Gaston MH, Verter J, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia. N Engl J Med 1986, 314:1593-1599.
2. Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med 1995, 332:1317-1322.
3. Lanzkron S, Strouse JJ, Wilson R, et al. Systematic review: hydroxyurea for the treatment of adults with sickle cell disease. Ann Intern Med 2008, 148:939-955.
4. Steinberg MH, McCarthy WF, Castro O, et al. The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: a 17.5 year follow-up. Am J Hematol 2010, 85:403-408.
5. Adams RJ, McKie VC, Hsu L, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography [see comments]. N Engl J Med 1998, 339:5-11.
6. Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. N Engl J Med 1995, 333:206-213.
7. Meerpohl JJ, Antes G, Rücker G, et al. Deferasirox for managing transfusional iron overload in people with sickle cell disease. Cochrane Database Syst Rev. 2010;8:CD007477.
8. Bauer DE, Orkin SH Update on fetal hemoglobin gene regulation in hemoglobinopathies. Curr Opin Pediatr 2011, 23:1-8. Review.
9. Brousseau DC, Owens PL, Mosso AL, et al. Acute care utilization and rehospitalizations for sickle cell disease. JAMA 2010, 303:1288-1294. Acute care encounters and hospitalizations are very common.
10. Hsieh MM, Kang EM, Fitzhugh CD, et al. Allogeneic hematopoietic stem-cell transplantation for sickle cell disease. N Engl J Med 2009, 361:2309-2317. Stem cell transplantation with an HLA-identical donor can "cure" sickle cell anemia in adults.
11. Rees DC, Williams TN, Gladwin MT Sickle-cell disease. Lancet 2010, 376:2018-2031. Review.
12. Tsaras G, Owusu-Ansah A, Boateng FO, et al. Complications associated with sickle cell trait: a brief narrative review. Am J Med 2009, 122:507-512. Sickle cell trait is "benign" but can be associated with certain complications.
13. Wolfson JA, Schrager SM, Coates TD, et al. Sickle-cell disease in California: a population-based description of emergency department utilization. Pediatr Blood Cancer 2011, 56:413-419. Nearly two thirds visited an emergency department each year, and almost 50% were on Medicaid.
14. Brawley OW, Cornelius LJ, Edwards LR, et al. National Institutes of Health Consensus Development Conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med 2008, 148:932-938. Consensus guidelines.
15. Gladwin MT, Kato GJ, Weiner D, et al. Nitric oxide for inhalation in the acute treatment of sickle cell pain crisis: a randomized controlled trial. JAMA 2011, 305:893-902. Nitric oxide did not improve crisis resolution.
16. Kato GJ, Gladwin MT, Steinberg MH Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes. Blood Rev 2007, 21:37-47. A review of the effect of hemolysis on nitric oxide dysregulation.
17. Smith WR, Penberthy LT, Bovbjerg VE, et al. Daily assessment of pain in adults with sickle cell disease. Ann Intern Med 2008, 148:94-101. The acute pain episode may be only the tip of the sickle cell pain iceberg.
18. Steinberg MH Genetic etiologies for phenotypic diversity in sickle cell anemia. Sci World J 2009, 9:46. The phenotype of sickle cell anemia is likely to be affected by the interactions of many modifying genes.