Word Key

Autosomal • Referring to chromosome, a non-sex-linked chromosome

Embolism • Occlusion of a blood vessel

Infarction • Area of tissue that has been deprived of blood and therefore has lost some of its function

Hypovolemic • Low blood pressure

Placebo • Substance having no medical effect when given to an individual as if a medicine

Viscosity • Thickness

References

1. Ingram VM. Gene mutations in human hemoglobins: The chemical differences between normal and sickle hemoglobins. Nature (Lond) 180:326, 1957.

2. Huisman TH, et al. A Syllabus of Human Hemoglobin Variants, 2nd ed. August, GA: The Sickle Cell Anemia Foundation, 1998.

3. McGhee DB. Structural defects in hemoglobin (hemoglo-binopathies). In: Rodak B, ed. Hematology: Clinical Principles and Applications, 2nd ed. Philadelphia: WB Saunders, 2002; 321.

4. Smith-Whitley K. Sickle Cell Disease: Diagnosis and Current Management. Workshop material from the American Society for Clinical Laboratory Sciences National Meeting, Philadelphia, July 2003.

5. Smith JA, Kinney TR (co-chairs). Sickle Cell Disease Guideline Panel: Sickle cell disease guideline and overview. Am J Hematol 47:152-154, 1994.

6. Pawars DR, Chan L, Schroeder WB. Bs- gene cluster hap-lotypes in sickle cell anemia: Clinical implications. Am J Pediatr Hematol Oncol 12:367-374, 1990.

7. Pawars DR, Weiss JN, Chan LS, et al. Is there a threshold level of fetal hemoglobin that ameliorates morbidity in sickle cell anemia? Blood 63:921-926, 1984.

8. Barnhart MI, Henry RL, Lusher JM. Sickle Cell. A Scope Publication. Kalamazoo, MI: The Upjohn Co., 1976; 12-14. Monograph.

9. Armbruster DA. Neonatal hemoglobinopathy screening. Lab Med 21:816, 1990.

10. Singer K, Motulsky AG, et al. Aplastic crisis in sickle cell anemia. J Lab Clin Med 35:721, 1950.

11. Pearson HA, Cornelius EA, et al. Transfusion reversible functional asplenia in young children with sickle cell anemia. N Engl J Med 283:334, 1970.

12. Gaston MH, Vwerter JL, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia: A randomized trial. N Engl J Med 314:1593-1599, 1986.

13. Gladwin MT, Sachdev V, Jison ML, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 350:886-895, 2004.

14. Barnhart MI, Henry RL, Lusher JM. Sickle Cell. A Scope Publication. Kalamazoo, MI: The Upjohn Co., 1974; 45. Monograph.

15. Vichinsky EP, Neumaur LD, Earles AN, et al. Causes and outcomes of acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group, 2000.

16. Konotey-Ahulu FI. Sickle cell disease. Arch Intern Med 133:616, 1974.

17. Dampier C, Ely E, Brodecki D, et al. Home management of pain in sickle cell disease: A daily diary study in children and adolescents. J Pediatr Hematol Oncol 24:643-647, 2002.

18. Adeyoju AB, Olujohungbe AB, Morris J, et al. Priapism in sickle cell disease: Incidence, risk factors and complications—An international multicenter study. BJU Int 90:898-902, 2002.

19. Babalola OE, Wambebe CO. When should children and young adults with sickle cell disease be referred for eye assessments? Afr J Med Sci 30:261-263, 2001.

20. Embury SH, et al. Sickle Cell Disease: Basic Principles and Clinical Practice. New York: Raven Press, 1994.

126 Part II • Red Cell Disorders

21. Adams RJ, et al. Prevention of a first stroke by transfusion in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography

N Engl J Med 317:781, 1987.

22. Pelehach L. Understanding sickle cell anemia. Lab Med 126:727, 1995.

23. Gaston M, Rosse WF; The Cooperative Group. The Cooperative Study of Sickle Cell Disease: Review of study designs and objectives. Am J Pediatr Hematol Oncol 4:197-201, 1982.

24. Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crisis in sickle cell anemia. N Engl J Med 332:1317-1322, 1995.

25. Pearson HA. Neonatal testing for sickle cell disease: A historical and personal view. Pediatrics 83(Suppl): 815-818, 1989.

26. Galacteros F, Kleman K, Caburi-Martin J, et al. Cord blood screening for hemoglobin abnormalities by thin layer isoelectric focusing. Blood 56:1068-1071, 1980.

27. Geist A. Hemoglobinopathies: Diagnosis and Care of Patients with Sickle Cell Disease. Indiana Univeristy Medical Center, personal correspondence.

Part III

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