Abstract
The sickle cell diseases are a major health problem for Afro-Caribbean peoples. Neonatal detection and prophylactic management can reduce mortality and morbidity in childhood. A study was therefore conducted analysing the results of the first two years of cord blood screening in the Camberwell health area. Thirteen cases of sickle cell disease and two of haemoglobin (Hb)C disease were identified among 2202 non-white infants screened. The carrier state, sickle cell trait (HbAS), was present in 11.9% and HbC trait (HbAC) in 4.1% of Afro-Caribbean infants. The incidence of disease and of carrier states was much higher in West Africans than in Caribbeans. The wider implications of screening and the need for a comprehensive plan of care are emphasised.
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- Anderson M. J., Davis L. R., Hodgson J., Jones S. E., Murtaza L., Pattison J. R., Stroud C. E., White J. M. Occurrence of infection with a parvovirus-like agent in children with sickle cell anaemia during a two-year period. J Clin Pathol. 1982 Jul;35(7):744–749. doi: 10.1136/jcp.35.7.744. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Davis L. R., Huehns E. R., White J. M. Survey of sickle-cell disease in England and Wales. Br Med J (Clin Res Ed) 1981 Dec 5;283(6305):1519–1521. doi: 10.1136/bmj.283.6305.1519. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Dozy A. M., Kan Y. W., Embury S. H., Mentzer W. C., Wang W. C., Lubin B., Davis J. R., Jr, Koenig H. M. alpha-Globin gene organisation in blacks precludes the severe form of alpha-thalassaemia. Nature. 1979 Aug 16;280(5723):605–607. doi: 10.1038/280605a0. [DOI] [PubMed] [Google Scholar]
- Embury S. H., Dozy A. M., Miller J., Davis J. R., Jr, Kleman K. M., Preisler H., Vichinsky E., Lande W. N., Lubin B. H., Kan Y. W. Concurrent sickle-cell anemia and alpha-thalassemia: effect on severity of anemia. N Engl J Med. 1982 Feb 4;306(5):270–274. doi: 10.1056/NEJM198202043060504. [DOI] [PubMed] [Google Scholar]
- Grover R., Wethers D., Shahidi S., Grossi M., Goldberg D., Davidow B. Evaluation of the expanded newborn screening program in New York City. Pediatrics. 1978 May;61(5):740–749. [PubMed] [Google Scholar]
- Henthorn J., Anionwu E., Brozovic M. Screening cord blood for sickle haemoglobinopathies in Brent. Br Med J (Clin Res Ed) 1984 Aug 25;289(6443):479–480. doi: 10.1136/bmj.289.6443.479. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Higgs D. R., Aldridge B. E., Lamb J., Clegg J. B., Weatherall D. J., Hayes R. J., Grandison Y., Lowrie Y., Mason K. P., Serjeant B. E. The interaction of alpha-thalassemia and homozygous sickle-cell disease. N Engl J Med. 1982 Jun 17;306(24):1441–1446. doi: 10.1056/NEJM198206173062402. [DOI] [PubMed] [Google Scholar]
- Konotey-Ahulu F. I. The sickle cell diseases. Clinical manifestations including the "sickle crisis". Arch Intern Med. 1974 Apr;133(4):611–619. [PubMed] [Google Scholar]
- Murtaza L. N., Stroud C. E., Davis L. R., Cooper D. J. Admissions to hospital of children with sickle-cell anaemia: a study in south London. Br Med J (Clin Res Ed) 1981 Mar 28;282(6269):1048–1051. doi: 10.1136/bmj.282.6269.1048. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Nussbaum R. L., Powell C., Graham H. L., Caskey C. T., Fernbach D. J. Newborn screening for sickling hemoglobinopathies. Houston, 1976 to 1980. Am J Dis Child. 1984 Jan;138(1):44–48. doi: 10.1001/archpedi.1984.02140390036011. [DOI] [PubMed] [Google Scholar]
- Pearson H. A., McIntosh S., Ritchey A. K., Lobel J. S., Rooks Y., Johnston D. Developmental aspects of splenic function in sickle cell diseases. Blood. 1979 Mar;53(3):358–365. [PubMed] [Google Scholar]
- Pearson H. A., O'Brien R. T., McIntosh S., Aspnes G. T., Yang M. M. Routine screening of umbilical cord blood for sickle cell diseases. JAMA. 1974 Jan 28;227(4):420–421. [PubMed] [Google Scholar]
- Powars D. R. Natural history of sickle cell disease--the first ten years. Semin Hematol. 1975 Jul;12(3):267–285. [PubMed] [Google Scholar]
- ROBINSON A. R., ROBSON M., HARRISON A. P., ZUELZER W. W. A new technique for differentiation of hemoglobin. J Lab Clin Med. 1957 Nov;50(5):745–752. [PubMed] [Google Scholar]
- Rogers D. W., Clarke J. M., Cupidore L., Ramlal A. M., Sparke B. R., Serjeant G. R. Early deaths in Jamaican children with sickle cell disease. Br Med J. 1978 Jun 10;1(6126):1515–1516. doi: 10.1136/bmj.1.6126.1515. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Rogers D. W., Serjeant B. E., Serjeant G. R. Early rise in the "pitted" red cell count as a guide to susceptibility to infection in childhood sickle cell anaemia. Arch Dis Child. 1982 May;57(5):338–342. doi: 10.1136/adc.57.5.338. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Sarnaik S. A., Lusher J. M. Neurological complications of sickle cell anemia. Am J Pediatr Hematol Oncol. 1982 Winter;4(4):386–394. doi: 10.1097/00043426-198224000-00006. [DOI] [PubMed] [Google Scholar]
- Serjeant G. R. Observations on the epidemiology of sickle cell disease. Trans R Soc Trop Med Hyg. 1981;75(2):228–233. doi: 10.1016/0035-9203(81)90322-9. [DOI] [PubMed] [Google Scholar]
- Topley J. M., Rogers D. W., Stevens M. C., Serjeant G. R. Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease. Arch Dis Child. 1981 Oct;56(10):765–769. doi: 10.1136/adc.56.10.765. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Warren N. S., Carter T. P., Humbert J. R., Rowley P. T. Newborn screening for hemoglobinopathies in New York State: experience of physicians and parents of affected children. J Pediatr. 1982 Mar;100(3):373–377. doi: 10.1016/s0022-3476(82)80432-0. [DOI] [PubMed] [Google Scholar]