Clinical Pediatric Emergency Medicine
Volume 6, Issue 3 , Pages 138-148 , September 2005

Emergencies in Patients With Inherited Hemoglobin Disorders—An Emergency Department Perspective

  • Rebecca Hampton, MD

      Affiliations

    • Department of Emergency Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229
    • Division of Emergency Medicine, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45229
    • Corresponding Author InformationReprint requests and correspondence: Rebecca R. Hampton, MD, Department of Emergency Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229.
    • ,
  • Vinod Balasa, MD

      Affiliations

    • Division of Hematology/Oncology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229
    • Division of Emergency Medicine, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45229
    • ,
  • Sheryl E. Allen Bracey, MD, MS

      Affiliations

    • Department of Emergency Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229
    • Division of Emergency Medicine, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH 45229

References 

  1. Weatherall DJ, Clegg JB. Inherited haemoglobin disorders: an increasing global health problem. Bull World Health Organ. 2001;79:704–712
  2. Ranney HM. Hemoglobin: a historical perspective. In:  Steinberg MH,  Forget BG,  Higgs DR, et al. editor. Disorders of hemoglobin: genetics, pathophysiology, and clinical management. New York: Cambridge University Press; 2001;p. 1–25
  3. Uthman E. Hemoglobinathies and thalassemias. In: http://we2.airmail.net/uthman/hemoglobinathy/hemoglobinpathy.html
  4. Bachman D, Barkin R, Breenan S. Hematologic and oncologic disorders. In:  Barkin RM,  Caputo G editor. Pediatric emergency medicine: concepts and clinical practice. 2nd ed.. St Louis: Mosby; 1997;p. 897–912
  5. Clarke G, Higgins T. Laboratory investigation of hemoglobinopathies and thalassemias: review and update. Clin Chem. 2000;46:1284–1290
  6. Quirolo K, Vinchinsky E. Hemoglobin disorders. In:  Behrman RE,  Kliegman RM,  Jenson HB editor. Nelson textbook of pediatrics. 17th ed.. Philadelphia: WB Saunders; 2004;p. 1624–1634
  7. AAP Section on Hematology/Oncology Committee on Genetics . Health supervision for children with sickle cell disease. Pediatrics. 2002;109:526–535
  8. Sadowitz PD, Amanullah S, Souid AK. Hematologic emergencies in the pediatric emergency room. Emerg Med Clin North Am. 2002;20:177–198
  9. Steinberg M. Management of sickle cell disease. N Engl J Med. 1999;340:1021–1030
  10. Cohen AR. Hematologic emergencies. In:  Fleisher GR,  Ludwig S editor. Synopsis of pediatric emergency medicine. 4th ed.. Philadelphia: Lippincott, Williams and Wilkins; 2002;p. 351–355
  11. New England Journal of Medicine electronic images: www.nejm.org
  12. Neumayr L, Lennette E, Kelly D, et al. Mycoplasma disease and acute chest syndrome in sickle cell disease. Pediatrics. 2003;112:87–95
  13. Radiology cases in pediatric emergency medicine: http:/www.hawaii.edu/medicine/pediatrics/pemxray/pemxray.html.
  14. Vinchinsky E, Neuymayr L, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med. 2000;342:1855–1865
  15. Wethers DL. Sickle cell disease in childhood: part II. Diagnosis and treatment of major complications and recent advances in treatment. Am Fam Physician. 2000;62:1309–1314
  16. Bellet PS, Kalinyak KA, Shukla R, et al. Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. N Engl J Med. 1995;333:699–703
  17. Wilimas J, Flynn P, Harris S, Day S, Smith R, Chesney PJ, et al. Randomized study of outpatient treatment with ceftriaxone for selected febrile children with sickle cell disease. N Engl J Med. 1993;329:472–476
  18. Cohen AR. Hematologic emergencies. In:  Fleisher GR,  Ludwig S editor. Textbook of pediatric emergency medicine. 4th ed.. Philadelphia: Lippincott, Williams and Wilkins; 2000;p. 865–873
  19. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91:288–294
  20. Ohene-Frempong K. Stroke in sickle cell disease: demographic, clinical and therapeutic considerations. Semin Hematol. 1991;28:213–219
  21. 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. N Engl J Med. 1998;339:5–11
  22. Platt O, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease—life expectancy and risk factors for early death. N Engl J Med. 1994;330:1639–1644
  23. Serjeant GR, Serjeant BE, Thomas PW, et al. Human parvovirus infection in homozygous sickle cell disease. Lancet. 1993;341:237–240
  24. Sackey K. Hemolytic anemia: part I. Pediatr Rev. 1999;20:152–158
  25. Olivieri NF. The β thalassemias. N Engl J Med. 1999;341:99–109
  26. Wethers DL. Sickle cell disease in childhood: part I. Laboratory diagnosis, pathophysiology and health maintenance. Am Fam Physician. 2000;62:1013–1020
  27. Higgs DR. Alpha thalassemia. In:  Steinberg MH,  Forget BG,  Higgs DR, et al. editor. Disorders of hemoglobin: genetics, pathophysiology, and clinical management. New York: Cambridge University Press; 2001;p. 389–390
  28. Kwiatkowski JL, Cohen AR. Iron chelation therapy in sickle cell disease and other transfusion dependent anemias. Hematol Oncol Clin North Am. 2004;18:1355–1377
  29. Rehman H. Methemoglobinemia. West J Med. 2001;75:193–198

PII: S1522-8401(05)00087-X

doi: 10.1016/j.cpem.2005.06.002

Clinical Pediatric Emergency Medicine
Volume 6, Issue 3 , Pages 138-148 , September 2005

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