DIAGNOSIS, GUIDELINES FOR COMPREHENSIVE CARE, AND CARE PATHS AND PROTOCOLS FOR MANAGEMENT OF ACUTE AND CHRONIC COMPLICATIONS  the Sickle Cell Disease Care Consortium (Arizona, Colorado, Georgia, Missouri, New Mexico, Tennessee, Texas, and Utah)

REFERENCES

General

1. Management and Therapy of Sickle Cell Disease, 4th edition. National Heart, Lung and Blood Institute, National Institutes of Health. NIH Publication No 02-2117, June, 2002.
2. Sickle Cell Disease Guideline Panel: Sickle Cell Disease: Screening, Diagnosis, Management, and Counseling in Newborns and Infants (Clinical Practice Guidelines, no. 6). Agency for Health Care Policy and Research, US Department of Health and Human Services. AHCPR Publication No. 93-0562, 1993.
3. Dover GJ, Platt OS: Sickle cell disease. In Nathan DG, Orkin SH (eds). Nathan and Oski's Hematology of Infancy and Childhood, 5th edition, Philadelphia, WB Saunders, 1998, p 762-809
4. Serjeant GR. Sickle Cell Disease, 2nd edition, Oxford, Oxford University Press, 1992.
5. Embury SH, Hebbel RP, Mohandes N, Steinberg MH (eds). Sickle Cell Disease: Basic Principals and Clinical Practice. New York, Raven Press, 1994.
6. Charache S, Johnson CS, editors. Sickle Cell Disease. Hematol/Oncol Clin N Am. 10(6), 1996.
7. Steinberg MH: Drug therapy: Management of sickle cell disease. N Engl J Med 1999; 340:1021-30.
8. Lane PA. Sickle cell disease. Pediatr Clin North Am 1996; 43:639-64.
9. Gill FM, Sleeper LA, Weiner SJ, et al. Clinical events in the first decade in a cohort of infants with sickle cell disease. Blood 1995; 86:776-83.
10. Platt OS, Brambilla DJ, Rosse W, et al. Mortality in sickle cell disease: Life expectancy and risk factors for early death. N Engl J Med 1994; 330:1639-44.
11. Miller ST, Sleeper LA, Pegelow CH, et al. Prediction of adverse outcomes in children with sickle cell disease. N Eng J Med 2000; 342:83-9.
12. Yang Y-M, Shah AK, Watson M, Mankad VN. Comparison of costs to the health sector of comprehensive and episodic health care for sickle cell disease patients. Public Health Reports 1995; 110:80-6.
13. Newborn Screening Task Force. Serving the family from birth to the medical home: A report from the Newborn Screening Task Force. Pediatrics 2000;106:383-427.
14. Pass KA, Lane PA, Fernhoff PM, et al. US newborn screening system guidelines II: Follow up of children, diagnosis, management, and evaluation. J Pediatr. 2000;37(suppl):S1-S46.
15. Committee on Infectious Diseases, American Academy of Pediatrics. Recommended childhood immunization schedule - United States, 2002 Pediatrics 2002;109:162-164.
16. American Academy of Pediatrics. Health Supervision for Children with Sickle Cell Disease. Pediatrics 2002;109:526-35.

1. 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 1986; 314:1593-9.
2. Falletta JM, Woods GM, Verter JI, et al. Discontinuing penicillin prophylaxis in children with sickle cell anemia. J Pediatr 1995; 127:685-90.
3. Lane PA, Rogers ZR, Woods GM, et al. Fatal pneumococcal septicemia in hemoglobin SC disease. J Pediatr 1994; 124:859-62.
4. Lane PA, O'Connell JL, Lear JL, et al. Functional asplenia in hemoglobin SC disease. Blood 1995; 85:2238-44.
5. Rogers ZR, Buchanan GR. Bacteremia in children with sickle hemoglobin C disease and sickle beta ⁺-thalassemia: Is prophylactic penicillin necessary? J Pediatr 1995; 127:348-54.
6. Wilimas JA, Flynn PM, Harris S, et al. A randomized study of outpatient treatment with ceftriaxone for selected febrile children with sickle cell disease. N Engl J Med 1993; 329:472-9.
7. Chesney PJ, Wilimas JA, Presbury G, et al. Penicillin- and cephalosporin-resistant strains of Streptococcus pneumoniae causing sepsis and meningitis in children with sickle cell disease. J Pediatr 1995; 127:526-32.
8. Hongeng S, Wilimas JA, Harris S, Day SW, Wang WC. Recurrent streptococcus pneumoniae sepsis in children with sickle cell disease. J Pediatr 1997;130:814-6.
9. Wright J, Thomas P, Serjeant GR. Septicemia caused by salmonella infection: An overlooked complication of sickle cell disease. J Pediatr 1997;130:394-9.
10. Platt OS. The febrile child with sickle cell disease: A pediatrician's quandary. J Pediatr 1997;130:693-4.
11. American Academy of Pediatrics, Committee on Infectious Disease. Recommendations for the prevention of pneumococcal infections, including the use of pneumococcal conjugate vaccine (Prevnar), pneumococcal polysaccharide vaccine, and antibiotic prophylaxis. Pediatrics. 2000;106:362-366.
12. Overturf GD and American Academy of Pediatrics, Committee on Infectious Diseases. Technical report: Prevention of pneumococcal infections, including the use of pneumococcal conjugate and polysaccharide vaccines and antibiotic prophylaxis. Pediatrics. 2000;106:367-376.

1. Platt OS, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease: Rates and risk factors. N Engl J Med 1991; 325:11-6.
2. Dampier CD, Setty BNY, Logan J, Ioli JG, Dean R. Intravenous morphine pharmacokinetics in pediatric patients with sickle cell disease. J Pediatr 1995; 126:461-7.
3. Hassell KL, Eckman JR, Lane PA. Acute multiorgan failure syndrome: A potentially catastrophic complication of severe sickle cell pain episodes. Am J Med 1994; 96:155-62.
4. Bellet PS, Kalinyak KA, Shukla R, Gelfand MJ, Rucknagel DL. Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. N Engl J Med 1995; 333:699-703.
5. Guideline for the Management of Acute and Chronic Pain in Sickle Cell Disease. American Pain Society, Glenview, IL, August 1999.

1. Miller ST, Hammerschlag MR, Chirgwin K, et al. Role of Chlamydia pneumoniae in acute chest syndrome of sickle cell disease. J Pediatr 1991; 118:30-3.
2. Lowenthal EA, Wells A, Emanuel PD, Player R, Prchal JT. Sickle cell acute chest syndrome associated with parvovirus B19 infection. Am J Hematol 1996; 51:207-13.
3. Vichinsky E, Williams R, Das M, et al. Pulmonary fat embolism: A distinct cause of severe acute chest syndrome in sickle cell anemia. Blood 1994; 83:3107-12.
4. Emre U, Miller ST, Gutierez M, Steiner P, Rao SP, Rao M. Effect of transfusion in acute chest syndrome of sickle cell disease. J Pediatr 1995; 127:901-4.
5. Vichinsky E, Styles L. Pulmonary complications. Hematol/Oncol Clin N Am 1996;10:1275-88.
6. Vichinsky EP, Styles LA, Colangelo LH, et al. Acute chest syndrome in sickle cell disease: Clinical presentation and course. Blood 1997;89:1787-92.
7. Quinn CT, Buchanan GR. The acute chest syndrome of sickle cell disease. J Pediatr 1999; 135:416-22.
8. Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med. 2000;342:1855-1865.

1. Emond AM, Collis R, Darvill D, Higgs DR, Maude GH, Serjeant GR. Acute splenic sequestration in homozygous sickle cell disease : Natural history and management. J Pediatr 1985; 107:201-6.
2. Kinney TR, Ware RE, Schultz WH, Filston HC. Long-term management of splenic sequestration in children with sickle cell disease. J Pediatr 1990; 117:194-9.
3. Aquino VM, Norvell JM, Buchanan GR. Acute splenic complications in children with sickle cell - hemoglobin C disease. J Pediatr 1997;130:961-5.
4. Wright JG, Hambleton IR, Thomas PW, Duncan ND, Venugopal S, Serjeant GR. Postsplenectomy course in homozygous sickle cell disease. J Pediatr 1999:134:304-9.

1. Mallouh AA, Qudah A. Acute splenic sequestration together with aplastic crisis caused by human parvovirus B19 in patients with sickle cell disease. J Pediatr 1993; 122:593-5.
2. Mustafa MM, McClain KL. Diverse hematologic effects of parvovirus B19 infection. Pediatr Clin N Am 1996;43:809-21.
3. Wierenga KJJ, Serjeant BE, Serjeant GR. Cerebrovascular complications and parvovirus infection in homozygous sickle cell disease. J Pediatr 2001; 139:438-42.

1. Ohene-Frempong K. Stroke in sickle cell disease: Demographic, clinical, and therapeutic considerations. Semin Hematol 1991; 28:213-19.
2. Wang WC, Kovnar EH, Tonkin IL, et al. High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease. J Pediatr 1991; 118:377-82.
3. Pegelow CH, Adams RJ, McKie V, et al. Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. J Pediatr 1995; 126:869-9.
4. Armstrong FD, Thompson RJ, Wang W, et al. Cognitive functioning and brain magnetic resonance imaging in children with sickle cell disease. Pediatrics 1996; 97:864-70.
5. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: Rates and risk factors. Blood 1998;91:288-94.
6. Adams RJ, McKie VC, Carl EM, et al. Long-term stroke risk in children with sickle cell disease screened with transcranial doppler. Ann Neurol 1997; 42:699-704.
7. Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, et al. Prevention of first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J of Med 1998; 339:5-11.
8. Cohen AR. Sickle cell disease - New treatments, new questions. N Engl J of Med; 1998; 339:42-3.
9. Kinney TR, Sleeper LA, Wang WC, et al. Silent cerebral infarcts in sickle cell anemia: A risk factor analysis. Pediatrics 1999;103:640-5.
10. Wang W, Enos L, Gallagher D, et al. Neuropsychologic performance of school-aged children with sickle cell disease: A report from the Cooperative Study of Sickle Cell Disease. J Pediatr 2001; 139:391-7.
11. Miller ST, Macklin EA, Pegelow CH, et al. Silent infarction as a risk factor for overt stroke in children with sickle cell anemia: A report from the Cooperative Study of Sickle Cell Disease. J Pediatr 2001; 139:385-90.
12. Scothorn DJ, Price C, Schwartz D, et al. Risk of recurrent stroke in children with sickle cell disease receiving blood transfusion therapy for at least five years after initial stroke. J Pediatr. 2002;140:348-54.

1. Rackoff WR, Ohene-Frempong K, Month S, Scott JP, Neahring B, Cohen AR. Neurologic events after partial exchange transfusion for priapism in sickle cell disease. J Pediatr 1992; 120:882-5.
2. Powars DR, Johnson CS. Priapism. Hematol/Oncol Clin N Am. 1996;10:1363-72.
3. Mantadakis E, Cavender JD, Rogers ZR, Ewalt DH, Buchanan GR. Prevalence of priapism in children and adolescents with sickle cell anemia. J Pediatr Hematol/Oncol, 1999;21:518-22.
4. Mantadikis 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, 2000;95:78-82.

1. Koshy M, Weiner SJ, Miller ST, et al. Surgery and anesthesia in sickle cell disease. Blood 1995; 86:3676-84.
2. Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the preoperative management of sickle cell disease. N Engl J Med 1995; 333:206-13.
3. Haberkern CM, Neumayr LD, Orringer EP, et al. Cholecystectomy in sickle cell anemia patients: Perioperative outcome of 364 cases from the national preoperative transfusion study. Blood 1997;89:1533-42.
4. Neumayr L, Koshy M, Haberkern C, et al. Surgery in patients with hemoglobin SC disease. Am J Hematol 1998; 57:101-8.

1. Wayne AS, Kevy SV, Nathan DG. Transfusion management of sickle cell disease. Blood 1993; 81:1109-23.
2. Rosse WF, Telen MJ, Ware RE. Transfusion Support for Patients with Sickle Cell Disease. Bethesda, American Association of Blood Banks Press, 1998.
3. Ambruso DR, Githens JH, Alcorn R, et al. Experience with donors matched for minor blood group antigens inpatients with sickle cell anemia who are receiving chronic transfusion therapy. Transfusion 1987; 27:94-8.
4. Styles LA, Vichinsky E. Effects of a long-term transfusion regimen on sickle cell-related illness. J Pediatr 1994; 125:909-11.
5. Koshy M, Burd L, Wallace D, Moawad A, Baron B. Prophylactic red-cell transfusions in pregnant patients with sickle cell disease: A randomized cooperative study. N Engl J Med 1988; 319:1447-52.
6. Styles LA, Vichinsky EP. Ototoxicity in hemoglobinopathy patients chelated with desferrioxamine. J Pediatr Hematol/Oncol 1996;18:42-5.
7. Olivieri NF, Brittenham GM. Iron-chelating therapy and the treatment of thalassemia. Blood 1997;89:739-61.
8. Vichinsky EP, ed. Transfusion-related iron overload in sickle cell anemia. Semin Hematol. 2001;38(suppl 1):1-84.
9. Harmatz P, Butensky E, Quirolo K, et al. Severity of iron overload in patients with sickle cell disease receiving chronic blood transfusion therapy. Blood. 2000;96:76-79.
10. Kim HC, Dugan NP, Silber JH, et al. Erythrocytapheresis therapy to reduce iron overload in chronically transfused patients with sickle cell disease. Blood. 1994;83:1136-1142.
11. Reed W, Vichinsky EP. Transfusion therapy: A coming-of-age treatment for patients with sickle cell disease. Am J Pediatr Hematol/Oncol 2001; 23:197-202.
12. Miller ST, Wright E, Abboud M, et al. Impact of chronic transfusion on incidence of pain and acute chest syndrome during Stroke Prevention Trial (STOP) in sickle cell anemia. J Pediatr 2001;139:785-9.
13. Files B, Brambilla D, Kutlar A, et al. Longitudinal changes in ferritin during chronic transfusion: A report from the Stroke Prevention Trial in sickle cell anemia (STOP). J Pediatr Hematol/Oncol 2002;24:284-90.

1. 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; 332:1317-22.
2. Scott J, Hillery CA, Brown ER, Misiewicz V, Labotka RJ. Hydroxyurea therapy in children severely affected with sickle cell disease. J Pediatr 1996; 128:820-8.
3. Ferster A, Vermylen C, Cornu G, et al. Hydroxyurea for treatment of severe sickle cell anemia: A pediatric clinical trial. Blood 1996;88:1960-64.
4. Jayabose S, Tugal O, Sandoval C, et al. Clinical and hematologic effects of hydroxyurea in children with sickle cell anemia. J Pediatr 1996;129:559-65.
5. Rogers ZR. Hydroxyurea therapy for diverse pediatric populations with sickle cell disease. Semin Hematol 1997;34(suppl 3):42-7.
6. Kinney TR, Helms RW, O'Branski EE, et al. Safety of hydroxyurea in children with sickle cell anemia: Results of the HUG-KIDS study, a phase I/II trial. Blood 1999; 94:1550-54.
7. Wang WC, Wynn LW, Rogers ZR, Scott JP, Lane PA, Ware RE. A two-year pilot trial of hydroxyurea in very young children with sickle cell anemia. J Pediatr 2001;139:790-6.
8. 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. 2002;140:225-9.

1. Ferster A, Bujan W, Corazza F, et al. Bone marrow transplantation corrects the splenic reticuloendothelial dysfunction in sickle cell anemia. Blood 1993; 81:1102-5
2. Vermylen C, Cornu G. Bone marrow transplantation for sickle cell disease : The European experience. Am J Pediatr Hematol/Oncol 1994; 16:18-21.
3. Walters MC, Patience M. Leisenring W, et al. Bone marrow transplantation for sickle cell disease. N Engl J Med 1996;335:369-76.
4. Platt OS, Guinan EC. Bone marrow transplantation in sickle cell anemia - The dilemma of choice. N Engl J Med 1996;335:426-8.
5. Walters MC, Storb R, Patience M, et al. Impact of bone marrow transplantation for symptomatic sickle cell disease: An interim report. Blood 2000;95:1918-24.
6. Walters MC. Bone marrow transplantation for sickle cell disease: Where do we go from here? J Pediatr Hematol Oncol. 1999;21:467-474.

7. Walters MC, Patience M, Leisenring W, et al. Stable mixed hematopoietic chimerism after bone marrow transplantation for sickle cell anemia. Biol Blood Marrow Transplant 2001; 7:665-673.

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Last modified: November 01, 2002