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. 1988 Oct;72(10):727–732. doi: 10.1136/bjo.72.10.727

Sickle cell retinopathy in Jamaican children: further observations from a cohort study.

J F Talbot 1, A C Bird 1, G H Maude 1, R W Acheson 1, B J Moriarty 1, G R Serjeant 1
PMCID: PMC1041571  PMID: 2461218

Abstract

Serial retinal examinations were performed in children aged 5 years and older and fluorescein angiography/angioscopy in children 6 years and older participating in a cohort study of sickle cell disease. There were 1229 patient years of observation among 389 children aged 5-13 years. Peripheral retinal vessel closure was present in approximately 50% of children with SS and SC genotypes at age 6 years and increased to affect 90% of children by age 12 years. A matched pair analysis, comparing groups with minimal and complete closure, indicated that complete closure was associated with significantly lower total haemoglobin and fetal haemoglobin levels and significantly lower weight in SS disease, whereas in SC disease the risk factors appeared to be high mean cell volume and low platelet count. Proliferative retinopathy was rare, occurring only once in an 8-year-old boy with SC disease, despite 592 patient years of observation in children over this age.

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Selected References

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  1. BETKE K., MARTI H. R., SCHLICHT I. Estimation of small percentages of foetal haemoglobin. Nature. 1959 Dec 12;184(Suppl 24):1877–1878. doi: 10.1038/1841877a0. [DOI] [PubMed] [Google Scholar]
  2. Baum K. F., Dunn D. T., Maude G. H., Serjeant G. R. The painful crisis of homozygous sickle cell disease. A study of the risk factors. Arch Intern Med. 1987 Jul;147(7):1231–1234. [PubMed] [Google Scholar]
  3. Condon P. I., Serjeant G. R. Ocular findings in homozygous sickle cell anemia in Jamaica. Am J Ophthalmol. 1972 Apr;73(4):533–543. doi: 10.1016/0002-9394(72)90005-0. [DOI] [PubMed] [Google Scholar]
  4. Galinos S. O., Asdourian G. K., Woolf M. B., Stevens T. S., Lee C. B., Goldberg M. F., Chow J. C., Busse B. J. Spontaneous remodeling of the peripheral retinal vasculature in sickling disorders. Am J Ophthalmol. 1975 May;79(5):853–870. doi: 10.1016/0002-9394(75)90747-3. [DOI] [PubMed] [Google Scholar]
  5. Hawker H., Neilson H., Hayes R. J., Serjeant G. R. Haematological factors associated with avascular necrosis of the femoral head in homozygous sickle cell disease. Br J Haematol. 1982 Jan;50(1):29–34. doi: 10.1111/j.1365-2141.1982.tb01887.x. [DOI] [PubMed] [Google Scholar]
  6. Hayes R. J., Beckford M., Grandison Y., Mason K., Serjeant B. E., Serjeant G. R. The haematology of steady state homozygous sickle cell disease: frequency distributions, variation with age and sex, longitudinal observations. Br J Haematol. 1985 Feb;59(2):369–382. doi: 10.1111/j.1365-2141.1985.tb03002.x. [DOI] [PubMed] [Google Scholar]
  7. Hayes R. J., Condon P. I., Serjeant G. R. Haematological factors associated with proliferative retinopathy in homozygous sickle cell disease. Br J Ophthalmol. 1981 Jan;65(1):29–35. doi: 10.1136/bjo.65.1.29. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. 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]
  9. Mason K. P., Grandison Y., Hayes R. J., Serjeant B. E., Serjeant G. R., Vaidya S., Wood W. G. Post-natal decline of fetal haemoglobin in homozygous sickle cell disease: relationship to parenteral Hb F levels. Br J Haematol. 1982 Nov;52(3):455–463. doi: 10.1111/j.1365-2141.1982.tb03915.x. [DOI] [PubMed] [Google Scholar]
  10. Maude G. H., Hayes R. J., Serjeant G. R. The haematology of steady state homozygous sickle cell disease: interrelationships between haematological indices. Br J Haematol. 1987 Aug;66(4):549–558. doi: 10.1111/j.1365-2141.1987.tb01343.x. [DOI] [PubMed] [Google Scholar]
  11. Nagpal K. C., Goldberg M. F., Asdourian G., Goldbaum M., Huamonte F. Dark-without-pressure fundus lesions. Br J Ophthalmol. 1975 Sep;59(9):476–479. doi: 10.1136/bjo.59.9.476. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Nagpal K. C., Huamonte F., Constantaras A., Asdourian G., Goldberg M. F., Busse B. Migratory white-without-pressure retinal lesions. Arch Ophthalmol. 1976 Apr;94(4):576–579. doi: 10.1001/archopht.1976.03910030270003. [DOI] [PubMed] [Google Scholar]
  13. Serjeant B. E., Forbes M., Williams L. L., Serjeant G. R. Screening cord bloods for detection of sickle cell disease in Jamaica. Clin Chem. 1974 Jun;20(6):666–669. [PubMed] [Google Scholar]
  14. Serjeant G. R., Grandison Y., Lowrie Y., Mason K., Phillips J., Serjeant B. E., Vaidya S. The development of haematological changes in homozygous sickle cell disease: a cohort study from birth to 6 years. Br J Haematol. 1981 Aug;48(4):533–543. doi: 10.1111/j.1365-2141.1981.tb02750.x. [DOI] [PubMed] [Google Scholar]
  15. Serjeant G. R. Irreversibly sickled cells and splenomegaly in sickle-cell anaemia. Br J Haematol. 1970 Nov;19(5):635–641. doi: 10.1111/j.1365-2141.1970.tb01647.x. [DOI] [PubMed] [Google Scholar]
  16. Talbot J. F., Bird A. C., Rabb L. M., Maude G. H., Serjeant G. R. Sickle cell retinopathy in Jamaican children: a search for prognostic factors. Br J Ophthalmol. 1983 Nov;67(11):782–785. doi: 10.1136/bjo.67.11.782. [DOI] [PMC free article] [PubMed] [Google Scholar]

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