eMedicine Specialties > Pediatrics: General Medicine > Hematology
Sickle Cell Anemia: Multimedia
Updated: Jul 9, 2009
Multimedia
![]() | Media file 1: Molecular and cellular changes of hemoglobin S. |
![]() | Media file 2: Effects of therapy with hydroxyurea. |
![]() | Media file 3: Peripheral blood with sickled cells at 400X magnification. Photograph by Dr Ulrich Woermann. |
![]() | Media file 4: Peripheral blood smear with sickled cells at 1000X magnification. Photograph by Dr Ulrich Woermann. |
![]() | Media file 5: Peripheral blood smear with Howell-Jolly body, indicating functional asplenism. Photograph by Dr Ulrich Woermann. |
More on Sickle Cell Anemia |
| Overview: Sickle Cell Anemia |
| Differential Diagnoses & Workup: Sickle Cell Anemia |
| Treatment & Medication: Sickle Cell Anemia |
| Follow-up: Sickle Cell Anemia |
Multimedia: Sickle Cell Anemia |
| References |
| « Previous Page |
References
Heeney MM, Ware RE. Hydroxyurea for children with sickle cell disease. Pediatr Clin North Am. Apr 2008;55(2):483-501, x. [Medline].
[Best Evidence] Strouse JJ, Lanzkron S, Beach MC, et al. Hydroxyurea for sickle cell disease: a systematic review for efficacy and toxicity in children. Pediatrics. Dec 2008;122(6):1332-42. [Medline].
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. Jul 2 1998;339(1):5-11. [Medline].
[Guideline] US Preventive Services Task Force. Screening for sickle cell disease in newborns: U.S. Preventive Services Task Force recommendation statement. Agency for Healthcare Research and Quality (AHRQ). Sep 2007;[Full Text].
Cappellini MD, Piga A. Current status in iron chelation in hemoglobinopathies. Curr Mol Med. Nov 2008;8(7):663-74. [Medline].
Das BB, Sobczyk W, Bertolone S, Raj A. Cardiopulmonary stress testing in children with sickle cell disease who are on long-term erythrocytapheresis. J Pediatr Hematol Oncol. May 2008;30(5):373-7. [Medline].
Adamkiewicz TV, Sarnaik S, Buchanan GR, et al. Invasive pneumococcal infections in children with sickle cell disease in the era of penicillin prophylaxis, antibiotic resistance, and 23-valent pneumococcal polysaccharide vaccination. J Pediatr. Oct 2003;143(4):438-44. [Medline].
Adams RJ, Brambilla D. Discontinuing prophylactic transfusions used to prevent stroke in sickle cell disease. N Engl J Med. Dec 29 2005;353(26):2769-78. [Medline].
Boyd JH, Macklin EA, Strunk RC, DeBaun MR. Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood. Nov 1 2006;108(9):2923-7. [Medline].
Brown AK, Sleeper LA, Miller ST, Pegelow CH, Gill FM, Waclawiw MA. Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease. Arch Pediatr Adolesc Med. Aug 1994;148(8):796-804. [Medline].
Buchanan GR, DeBaun MR, Quinn CT, Steinberg MH. Sickle cell disease. Hematology Am Soc Hematol Educ Program. 2004;35-47. [Medline].
Castro O, Brambilla DJ, Thorington B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. Jul 15 1994;84(2):643-9. [Medline].
Charache S, Terrin ML, Moore RD, et al. 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. May 18 1995;332(20):1317-22. [Medline].
Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial. N Engl J Med. Jun 19 1986;314(25):1593-9. [Medline].
Gill FM, Sleeper LA, Weiner SJ, et al. Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease. Blood. Jul 15 1995;86(2):776-83. [Medline].
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. Feb 26 2004;350(9):886-95. [Medline].
Hankins JS, Fortner GL, McCarville MB, Smeltzer MP, Wang WC, Li CS. The natural history of conditional transcranial Doppler flow velocities in children with sickle cell anaemia. Br J Haematol. Jul 2008;142(1):94-9. [Medline].
Hankins JS, Ware RE, Rogers ZR, et al. Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study. Blood. Oct 1 2005;106(7):2269-75. [Medline].
Hulbert ML, Scothorn DJ, Panepinto JA, Scott JP, Buchanan GR, Sarnaik S. Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: a retrospective cohort study of 137 children with sickle cell anemia. J Pediatr. Nov 2006;149(5):710-2. [Medline].
Kato GJ, Onyekwere OC, Gladwin MT. Pulmonary hypertension in sickle cell disease: relevance to children. Pediatr Hematol Oncol. Apr-May 2007;24(3):159-70. [Medline].
Kaul DK, Liu XD, Zhang X, et al. Peptides based on alphaV-binding domains of erythrocyte ICAM-4 inhibit sickle red cell-endothelial interactions and vaso-occlusion in the microcirculation. Am J Physiol Cell Physiol. Nov 2006;291(5):C922-30. [Medline].
Mantadakis E, Ewalt DH, Cavender JD, Rogers ZR, Buchanan GR. Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism. Blood. Jan 1 2000;95(1):78-82. [Medline].
Miller ST, Sleeper LA, Pegelow CH, et al. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med. Jan 13 2000;342(2):83-9. [Medline].
Morris CR, Kato GJ, Poljakovic M, Wang X, Blackwelder WC, Sachdev V. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease. JAMA. Jul 6 2005;294(1):81-90. [Medline].
Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. Jan 1 1998;91(1):288-94. [Medline].
Okpala I. Leukocyte adhesion and the pathophysiology of sickle cell disease. Curr Opin Hematol. Jan 2006;13(1):40-4. [Medline].
Pegelow CH, Adams RJ, McKie V, et al. Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. J Pediatr. Jun 1995;126(6):896-9. [Medline].
Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. Jun 9 1994;330(23):1639-44. [Medline].
Quinn CT, Buchanan GR. Sickle-cell anemia with unusual bone changes. J Pediatr. Sep 2003;143(3):350.
Quinn CT, Rogers ZR, Buchanan GR. Survival of children with sickle cell disease. Blood. Jun 1 2004;103(11):4023-7. [Medline].
Raj A, Bertolone S, Bond S, Burnett D, Denker A. Cathlink 20: a subcutaneous implanted central venous access device used in children with sickle cell disease on long-term erythrocytapheresis--a report of low complication rates. Pediatr Blood Cancer. Jun 15 2005;44(7):669-72. [Medline].
Raj A, Bertolone S, Klapheke P, Burnett D, Suarez C. Impact of long-term erythrocytapheresis on splenic function in patients with sickle cell disease. J Pediatr Hematol Oncol. Oct 2002;24(7):545-7. [Medline].
Raj A, Bertolone SJ, Mangold S, Edmonds HL Jr. Assessment of cerebral tissue oxygenation in patients with sickle cell disease: effect of transfusion therapy. J Pediatr Hematol Oncol. May 2004;26(5):279-83. [Medline].
Raj AB, Condurache T, Bertolone S, Williams D, Lorenz D, Sobczyk W. Quantitative assessment of ventricular function in sickle cell disease: effect of long-term erythrocytapheresis. Pediatr Blood Cancer. Dec 2005;45(7):976-81. [Medline].
Raj AB, O'brien LM, Bertolone SJ, Gozal D, Edmonds HL Jr. Cerebral oximetry improves detection of sickle cell patients at risk for nocturnal cerebral hypoxia. Pediatr Pulmonol. Nov 2006;41(11):1088-94. [Medline].
Steinberg MH. Sickle cell anemia, the first molecular disease: overview of molecular etiology, pathophysiology, and therapeutic approaches. ScientificWorldJournal. Dec 25 2008;8:1295-324. [Medline].
Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. Oct 9-15 2004;364(9442):1343-60. [Medline].
Uong EC, Boyd JH, DeBaun MR. Daytime pulse oximeter measurements do not predict incidence of pain and acute chest syndrome episodes in sickle cell anemia. J Pediatr. Nov 2006;149(5):707-9. [Medline].
Vichinsky EP, Earles A, Johnson RA, Hoag MS, Williams A, Lubin B. Alloimmunization in sickle cell anemia and transfusion of racially unmatched blood. N Engl J Med. Jun 7 1990;322(23):1617-21. [Medline].
Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group. N Engl J Med. Jul 27 1995;333(4):206-13. [Medline].
Walters MC, Patience M, Leisenring W, et al. Bone marrow transplantation for sickle cell disease. N Engl J Med. Aug 8 1996;335(6):369-76. [Medline].
Wang WC, Helms RW, Lynn HS, et al. Effect of hydroxyurea on growth in children with sickle cell anemia: results of the HUG-KIDS Study. J Pediatr. Feb 2002;140(2):225-9. [Medline].
Zilberman MV, Du W, Das S, Sarnaik SA. Evaluation of left ventricular diastolic function in pediatric sickle cell disease patients. Am J Hematol. Jun 2007;82(6):433-8. [Medline].
Further Reading
Keywords
sickle cell anemia, sickle cell disease, crescent cell anemia, sickle-shaped erythrocytes, crescent-shaped erythrocytes, sickle cell crisis, ACS, acute chest syndrome, SCD, hemoglobin S, HbS, homozygotic HbSS, aplastic crisis, fifth disease, hemolytic anemia, Streptococcus pneumoniae, osteomyelitis, meningitis, acute chest syndrome, pulmonary infarction, respiratory distress, bone marrow infection, hand-foot syndrome, hypoxemia, dehydration, stroke, convulsion, priapism, bone marrow transplantation, hemorrhagic stroke, moya moya, cholecystitis, cholelithiasis, bile duct obstruction, unwanted erection, treatment, diagnosis










Multimedia: Sickle Cell Anemia