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References

Overview

Emond AM, Collis R, Darvill D, et al. Acute splenic sequestration in homozygous sickle cell disease: natural history and management. J Pediatr 1985 Aug;107(2): 201-6.

Gilman PA, McFarlane JM, Huisman THJ. Natural history of sickle cell anemia: re-evaluation of a 15-year cord blood testing program [Abstract 452]. Pediatr Res 1976;10:376.

National Sickle Cell Anemia Control Act of 1972, 42 U.S.C. § 201.

Pearson HA, Gallagher D, Chilcote R, et al. Developmental pattern of splenic
dysfunction in sickle cell disorders. Pediatrics 1985 Sep;76(3):392-7.
Schneider RG, Gustafson LP, Haggard ME. The incidence of genetically
determined abnormalities in 11,427 cord blood samples. Presented at the 13th
International Congress of Hematology, August 1970.

Scott RB. Health care priority and sickle cell anemia: special communication.
JAMA 1970;214:731-4.

Population To Be Screened

Anyane-Yeboa K. Hemoglobinopathy screening during early pregnancy. Pediatrics 1989 May;83(5 Pt 2):881-3.

Barnes MG, Komarmy L, Novack AH. A comprehensive screening program for hemoglobinopathies. JAMA 1972 Feb 7;219(6):701-5.

Broghamer WL Jr, Lockwood WB, Keeling MM. Clinical assessment of three hemoglobin screening programs on a selected population. South Med J 1981 Dec;74(12):1443-6.

Carr CM, Chapatwala KD. Sickle gene frequency in a Southcentral Alabama family medical program. Alabama Med 1988 Oct;58(4):17-8.

Castro O, Winter WP, Lee TCK, et al. Prevalence of a-chain variants at birth. Am J Clin Pathol 1981 Jan;75(1):56-9.

Diaz-Barrios V. New York's experience. Pediatrics 1989 May;83(5 Pt 2);872-5.

Eddy DM, Hasselblad V. FAST*PRO software for meta-analysis by the confidence profile method. San Diego: Academic Press; 1992.

Foster K, Forbes M, Hayes R, et al. Cord blood screening for sickle hemoglobin: evidence against a female preponderance of hemoglobin S. South J Pediatr 1981 Jan;98(1):79-81.

Frame PS. A critical review of adult health maintenance, part 1: prevention of atherosclerotic diseases. J Fam Pract 1986;22:341.

Gardner RV, Keitt A. University of Florida sickle cell screening program for neonates: design and results. J Natl Med Assoc 1988 Mar;80(3):273-9.

Gaston MH, Verter JL, 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.

Grover R, Wethers DL, Shahidi S, et al. Evaluation of the expanded newborn screening program in New York City. Pediatrics 1978;61:740-9.

Harris MS, Eckman JR. Georgia's experience with newborn screening: 1981 to 1985. Pediatrics 1989 May;83(5 Pt 2):858-60.

Huisman THJ, Harris HF, Stewart A, et al. The frequencies of Hbs S and C in Georgia and South Carolina. Hum Genet 1991;87:102-3.

Lane PA, Mauro RD, Houston ML, et al. Universal neonatal screening for hemoglobinopathies is more cost-effective than screening targeted to high-risk infants [Abstract]. Presented at the Ninth National Neonatal Screening Symposium, Raleigh, NC, April 7-11, 1992.

Lobel JS, Cameron BF, Johnson E, et al. Value of screening umbilical cord blood for hemoglobinopathy. Pediatrics 1989 May;83(5 Pt 2):823-6.

Mack A. Florida's experience with newborn screening. Pediatrics 1989 May;83 (5 Pt 2):861-3.

Meany FJ, Riggle SM. Newborn screening report, 1990. Austin (TX): Council of Regional Networks for Genetic Services; 1992.

Pass KA, Gauvreau AF, Schedlbauer LM, et al. Newborn screening for sickle cell disease in New York State. In Carter TP, Wiley AM, editors. Genetic disease: screening and management. New York: AR Liss; 1986. p. 359-72.

Pearson HA. The kidney, hepatobiliary system, and spleen in sickle cell anemia. Sickle cell disease. Ann NY Acad Sci 1989;565:120-5.

Powars DR. Diagnosis at birth improves survival of children with sickle cell anemia. Pediatrics 1989;83(Suppl):830-3.

Ralston KK, Kmetz DR, Keeling MM, et al. Screening for major hemoglobinopathies in newborn blacks. J Ky Med Assoc 1981 Oct;79(10):649-51. Schedlbauer LM, Pass KA. Cellulose acetate/citrate agar electrophoresis of filter paper hemolysates from heel stick. Pediatrics 1989 May;83(5 Pt 2):839-42.

Sickle Cell Disease Guideline Panel. Sickle cell disease. Guideline report. AHCPR Pub. No. 93-0504. Rockville (MD): Agency for Health Care Policy and Research; in press.

Sprinkle RH, Hynes DM, Konrad TR. A reconsideration of universal neonatal hemoglobinopathy screening in the United States; in press.

Therrell BL, Simmank JL, Wilborn M. Experiences with sickle hemoglobin testing in the Texas newborn screening program. Pediatrics 1989 May;83(5 Pt 2);864-7. Tsevat JW, John B, Pauker SG, et al. Neonatal screening for sickle cell disease: a cost-effectiveness analysis. J Pediatr 1991 April;118(4 Pt 1):546-54.

Vichinsky E, Hurst D, Earles A, et al. Newborn screening for sickle cell disease: effect on mortality. Pediatrics 1988 Jun;81(6):749-55.

Wethers DL, Grover R. Screening the newborn for sickle cell disease: is it worth the effort. In: Carter TP, Wiley AM, eds, Genetic disease: screening and management. Proceedings of the 1985 Albany Birth Defects Symposium. New York: Alan R. Liss; 1986. p. 123-136.

Laboratory Screening for Sickle Cell Disease

Adam B, Bell C. Performance surveillance: sickle cell disease and other hemoglobinopathies. Infant Screening Quality Assurance Program 1992;2:1-8. Broghamer WL Jr, Lockwood WB, Keeling MM. Clinical assessment of three hemoglobin screening programs on a selected population. South Med J 1981 Dec;74(12):1443-6.

Chan MS, Schneider NJ. Laboratory screening of hemoglobinopathies. Health Lab Sci 1975 Apr;12(2):91-9.

Clinical Laboratories Improvement Amendments of 1988, 42 U.S.C. § 201.

Descartes M, Huang Y, Zhang YH, et al. Direct genotypic analysis for sickle cell disease using DNA from newborn screening specimens: correlation with results from the state laboratory. Joint meeting of the American Pediatric Society for Pediatric Research, Anaheim CA, May 7-10, 1990. [Abstract] Pediatr Res 1990;27(4 part 2):130A.

Fleiss JL. Statistical methods for rates and proportions. New York: Wiley; 1981. Galacteros F, Kleman K, Caburi-Martin J, et al. Cord blood screening for hemoglobin abnormalities by thin layer isoelectric focusing. Blood 1980;56: 1068-71.

Gardner RV, Keitt A. University of Florida sickle cell screening program for neonates: design and results. J Natl Med Assoc 1988 Mar;80(3):273-9. Garrick M, Dembure P, Guthrie R. Sickle-cell anemia and other hemoglobinopathies: procedures and strategy for screening employing spots of blood on filter paper as specimens. N Engl J Med 1973 Jun;288(24):1265-8. Githens JH, Lane PA, McCurdy RS, et al. Newborn screening for hemoglobinopathies in Colorado. Am J Dis Child 1990 Apr;144(4):466-70. Griffiths PD, Mann JR, Darbyshire PJ, et al. Evaluation of eight and a half years of neonatal screening for hemoglobinopathies in Birmingham. BMJ [Clin Res Ed] 1988 Jun;296:1583-5.

Hicks EJ, Hughes BJ. Comparison of electrophoresis on citrate agar, cellulose acetate, or starch for hemoglobin identification. Clin Chem 1975;21(8):1072-6. Huisman THJ. Usefulness of cation exchange high performance liquid chromatography as a testing procedure. Pediatrics 1989;83(Suppl):849-51. Huntsman RG, Metters JS, Yawson GI. The diagnosis of sickle cell disease in the newborn infant. J Pediatr 1972;80:279-81.

Jacobs S, Peterson L, Thompson L, et al. Newborn screening for hemoglobin abnormalities. A comparison of methods. Am J Clin Pathol 1986;85:713-5.

Kinney TR, Sawtschenko M, Whorton M, et al. Techniques' comparison and report of the North Carolina experience. Pediatrics 1989;83(Suppl):843-8.

Kleman KM, Lorey FW, unpublished data, 1992.

Kleman KM, Vichinsky E, Lubin BH. Experience with newborn screening using isoelectric focusing. Pediatrics 1989;83(Suppl):852-4.

Kramer MS, Rooks Y, Johnston D, et al. Accuracy of cord blood screening for hemoglobinopathies. JAMA 1979;241:485-6.

Lobel JS, Cameron BF, Johnson E, et al. Value of screening umbilical cord blood for hemoglobinopathy. Pediatrics 1989 May;83(5 Pt 2):823-6.

McMahon L, unpublished data, 1992.

Meany FJ, Riggle SM. Newborn screening report, 1990. Austin (TX): Council of Regional Networks for Genetic Services; 1992.

Miller ST, Stilerman TV, Rao SP, et al. Newborn screening for sickle cell disease: when is an infant "lost to follow-up"? Am J Dis Child 1990 Dec;144(12):1343-5. National Committee for Clinical Laboratory Standards. Blood collection on filter paper for neonatal screening programs (NCCLS Doc. No. LA4-A). Villanova (PA): National Committee for Clinical Laboratory Standards; 1988.

Pass KA, Gauvreau AF, Schedlbauer LM, et al. Newborn screening for sickle cell disease in New York State. In Carter TP, Wiley AM, editors. Genetic disease: screening and management. New York: AR Liss; 1986. p. 359-72.

Rowley PT. Newborn screening for hemoglobinopathies. Semin Perinatol 1990 Dec;14:483-7.

Schedlbauer LM, Pass KA. Cellulose acetate/citrate agar electrophoresis of filter paper hemolysates from heel stick. Pediatrics 1989 May;83(5 Pt 2):839-42. Schmidt RM, Brosius EM, Holland S, et al. Use of blood specimens collected on filter paper in screening for abnormal hemoglobin. Clin Chem 1976;22:685-7. Schroeder WA, Jakway J, Powars D. Detection of hemoglobin S and C at birth: a rapid screening procedure by column chromatography. J Lab Clin Med 1975;82:304-8.

Steinberg MH, editor. Newborn screening for hemoglobinopathies: program development and laboratory methods. Bethesda (MD): National Heart, Lung, and Blood Institute; 1990.

Therrell BL, Panny SR, Davidson A, et al. Newborn screening system guidelines. Statement of the Council of Regional Networks for Genetic Services. Austin (TX): Council of Regional Networks for Genetic Services; 1990. p. 1-10.

Therrell BL, Pass KA, Hassemer D, et al. Laboratory techniques for neonatal hemoglobinopathy screening. Austin (TX): Texas Department of Health; 1990.

Medical Management of Newborns and Infants with Sickle Cell Disease

Adams T, McKie V, Nichols F, et al. The use of transcranial ultrasonography to predict stroke in sickle cell disease. N Engl J Med 1992 Feb;326:605-10.

Anglin DL, Siegel JD, Pacini DL. Effect of penicillin prophylaxis on nasopharyngeal colonization with Streptococcus pneumoniae in children with sickle cell anemia. J Pediatr 1984 Jan;104(1):18-22.

Bainbridge R, Higgs DR, Maude GH, et al. Clinical presentation of homozygous sickle cell disease. J Pediatr 1985 Jun;106(6):881-5.

Bakshi SS, Grover R, Cabezon E, et al. Febrile episodes in children with sickle cell disease treated on an ambulatory basis. J Assoc Acad Minor Phys 1991;2(2): 80-3.

Belgrave FZ, Molock SD. The role of depression in hospital admissions and emergency treatment of patients with sickle cell disease. J Natl Med Assoc 1991 Sep;83(9):777-81.

Brown AK, Miller ST, Agatisa P. Care of infants with sickle cell disease.
Pediatrics 1989 May;83(5 Pt 2):897-900.

Buchanan GR, Schiffman G. Antibody responses to polyvalent pneumococcal vaccine in infants with sickle cell anemia. J Pediatr 1980 Feb;96(2):264-6. Buchanan GR, Siegel JD, Smith SS, et al. Oral penicillin prophylaxis in children with impaired splenic function: a study of compliance. Pediatrics 1982 Dec;70(6): 926-30.

Buchanan GR, Smith SJ. Pneumococcal septicemia despite pneumococcal vaccine and prescription of penicillin prophylaxis in children with sickle cell anemia. Am J Dis Child 1986 May;140(5):428-32.

Centers for Disease Control. Risk associated with human parvovirus B19 infection. MMWR 1989;38:81-97.

Chudwin DS, Wara DW, Matthay KK. Increased serum opsonic activity and antibody concentration in patients with sickle cell disease after pneumococcal polysaccharide immunization. J Pediatr 1983 Jan;102(1):51-4.

Consensus Conference. Newborn screening for sickle cell disease and other hemoglobinopathies. JAMA 1987 Sep;258(9):1205-9.

Cummins D, Heuschkel R, Davies SC. Penicillin prophylaxis in children with sickle cell disease in Brent study of physicians knowledge, patient compliance. BMJ 1991 Apr;302(6783):989-90.

Davies SC, Brozovic M. The presentation, management, and prophylaxis of sickle cell disease. Blood Rev 1989;3:29.

Day S, Brunson G, Wang W. A successful education program for parents of infants with newly diagnosed sickle cell disease. J Pediatr Nurs 1992;7(1):52-7.

De Ceulaer K, McMullen KW, Maude GH, et al. Pneumonia in young children with homozygous sickle cell disease: risk and clinical features. Eur J Pediatr 1985 Sep;144(3):255-8.

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