Skip to main content
NCBI home page
Journal of the National Medical Association logoLink to Journal of the National Medical Association
letter
. 1982 Aug;74(8):751–757.

Sickle Cell Trait: An Update

Lenworth N Johnson
PMCID: PMC2552960  PMID: 6752430

Abstract

A review of the literature on sickle cell trait was completed by Sears in 1978. Since that time, several papers have been published concerning the possible health risks of sickle cell trait. Data presented from these studies show that there is no association with sickle cell trait and overall survival, overall mortality, overall morbidity, frequency and length of hospitalization, short-term survival of renal transplant recipient, and inheritance of glucose-6-phosphate dehydrogenase. Association with sickle cell trait is very likely in the following: splenic infarction at high altitudes (over 10,000 feet), in unpressurized airplane flight and mountain climbing, bacteriuria and pyelonephritis in pregnancy, hyposthenuria, hematuria, and delayed resolution of anterior chamber hyphema. Although these conditions have a statistical significant association with sickle cell trait, they occur quite infrequently. Thus, when they are observed, other causes should be sought before attributing them to sickle cell trait. Reduced mortality from Plasmodium falciparum infection also shows significant association with sickle cell trait.

Full text

PDF
751

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ahmad A. Retinopathy in ophthalmologically asymptomatic patients with abnormal hemoglobins. Ann Ophthalmol. 1979 Mar;11(3):365–369. [PubMed] [Google Scholar]
  2. Ashcroft M. T., Desai P. Mortality and morbidity in Jamaican adults with sickle-cell trait and with normal haemoglobin followed up for twelve years. Lancet. 1976 Oct 9;2(7989):784–786. doi: 10.1016/s0140-6736(76)90612-7. [DOI] [PubMed] [Google Scholar]
  3. Ashcroft M. T., Desai P., Richardson S. A., Serjeant Growth, behaviour, and educational achievement of Jamaican children with sickle-cell trait. Br Med J. 1976 Jun 5;1(6022):1371–1373. doi: 10.1136/bmj.1.6022.1371. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Ashcroft M. T., Miall W. E., Milner P. F. A comparison between the characteristics of Jamaican adults with normal hemoglobin and those with sickle cell trait. Am J Epidemiol. 1969 Sep;90(3):236–243. doi: 10.1093/oxfordjournals.aje.a121066. [DOI] [PubMed] [Google Scholar]
  5. BRAIN P. The sickle cell trait; its clinical significance. S Afr Med J. 1952 Nov 22;26(47):925–928. [PubMed] [Google Scholar]
  6. Bernstein S. C., Bowman J. E., Noche L. K. Interaction of sickle cell trait and glucose-6-phosphate dehydrogenase deficiency in Cameroon. Hum Hered. 1980;30(1):7–11. doi: 10.1159/000153080. [DOI] [PubMed] [Google Scholar]
  7. Birnbaum G. J. Retinal vascular diseases. N Engl J Med. 1978 Nov 2;299(18):1017–1018. [PubMed] [Google Scholar]
  8. Brody J. I., Levison S. P., Jung C. J. Sickle cell trait and hematuria associated with von Willebrand syndromes. Ann Intern Med. 1977 May;86(5):529–533. doi: 10.7326/0003-4819-86-5-529. [DOI] [PubMed] [Google Scholar]
  9. Chatterjee S. N. National study on natural history of renal allografts in sickle cell disease or trait. Nephron. 1980;25(4):199–201. doi: 10.1159/000181781. [DOI] [PubMed] [Google Scholar]
  10. Cornille-Brøgger R., Fleming A. F., Kagan I., Matsushima T., Molineaux L. Abnormal haemoglobins in the Sudan savanna of Nigeria. II. Immunological response to malaria in normals and subjects with sickle cell trait. Ann Trop Med Parasitol. 1979 Apr;73(2):173–183. doi: 10.1080/00034983.1979.11687244. [DOI] [PubMed] [Google Scholar]
  11. Dean J., Schechter A. N. Sickle-cell anemia: molecular and cellular bases of therapeutic approaches (first of three parts). N Engl J Med. 1978 Oct 5;299(14):752–763. doi: 10.1056/NEJM197810052991405. [DOI] [PubMed] [Google Scholar]
  12. Deisseroth A., Nienhuis A., Lawrence J., Giles R., Turner P., Ruddle F. H. Chromosomal localization of human beta globin gene on human chromosome 11 in somatic cell hybrids. Proc Natl Acad Sci U S A. 1978 Mar;75(3):1456–1460. doi: 10.1073/pnas.75.3.1456. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Fleming A. F., Storey J., Molineaux L., Iroko E. A., Attai E. D. Abnormal haemoglobins in the Sudan savanna of Nigeria. I. Prevalence of haemoglobins and relationships between sickle cell trait, malaria and survival. Ann Trop Med Parasitol. 1979 Apr;73(2):161–172. doi: 10.1080/00034983.1979.11687243. [DOI] [PubMed] [Google Scholar]
  14. Francis C. K., Bleakley D. W. The risk of sudden death in sickle cell trait: noninvasive assessmanet of cardiac response to exercise. Cathet Cardiovasc Diagn. 1980;6(1):73–80. doi: 10.1002/ccd.1810060109. [DOI] [PubMed] [Google Scholar]
  15. Friedman A. H., Halpern B. L., Friedberg D. N., Wang F. M., Podos S. M. Transient open-angle glaucoma associated with sickle cell trait: report of 4 cases. Br J Ophthalmol. 1979 Dec;63(12):832–836. doi: 10.1136/bjo.63.12.832. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Gelpi A. P. Glucose-6-phosphate dehydrogenase deficiency in Saudi Arabia. Bull World Health Organ. 1967;37(4):539–546. [PMC free article] [PubMed] [Google Scholar]
  17. George C. Acute renal failure due to rhabdomyolysis in sickle cell trait. Intensive Care Med. 1979 Nov;5(4):204–205. doi: 10.1007/BF01683941. [DOI] [PubMed] [Google Scholar]
  18. Gima A. S., Bemis E. L. Absence of major illnesses in sickle cell trait: Results from a controlled study. J Natl Med Assoc. 1975 May;67(3):216–219. [PMC free article] [PubMed] [Google Scholar]
  19. Goldbaum M. H., Fletcher R. C., Jampol L. M., Goldberg M. F. Cryotherapy of proliferative sickle retinopathy, II: triple freeze-thaw cycle. Br J Ophthalmol. 1979 Feb;63(2):97–101. doi: 10.1136/bjo.63.2.97. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Goldberg M. F., Dizon R., Moses V. K. Sickled erythrocytes, hyphema, and secondary glaucoma: VI. The relationship between intracameral blood cells and aqueous humor pH, PO2, and PCO2. Ophthalmic Surg. 1979 Apr;10(4):78–88. [PubMed] [Google Scholar]
  21. Goldberg M. F., Dizon R., Raichand M., Goldbaum M., Jampol L. M. Sickled erythrocytes, hyphema, and secondary glaucoma: III. Effects of sicle cell and normal human blood samples in rabbit anterior chambers. Ophthalmic Surg. 1979 Apr;10(4):52–61. [PubMed] [Google Scholar]
  22. Goldberg M. F., Dizon R., Raichand M. Sickled erythrocytes, hyphema, and secondary glaucoma: II. Injected sickle cell erythrocytes into human, monkey, and guinea pig anterior chambers: the induction of sickling and secondary glaucoma. Ophthalmic Surg. 1979 Apr;10(4):32–51. [PubMed] [Google Scholar]
  23. Goldberg M. F. Sickled erythrocytes, hyphema, and secondary glaucoma: IV. The rate and percentage of sickling of erythrocytes in rabbit aqueous humor, in vitro and in vivo. Ophthalmic Surg. 1979 Apr;10(4):62–69. [PubMed] [Google Scholar]
  24. Goldberg M. F. Sickled erythrocytes, hyphema, and secondary glaucoma: V. The effect of vitamin C on erythrocyte sickling in aqueous humor. Ophthalmic Surg. 1979 Apr;10(4):70–77. [PubMed] [Google Scholar]
  25. Goldberg M. F. The diagnosis and treatment of secondary glaucoma after hyphema in sickle cell patients. Am J Ophthalmol. 1979 Jan;87(1):43–49. doi: 10.1016/0002-9394(79)90190-9. [DOI] [PubMed] [Google Scholar]
  26. Goldberg M. F., Tso M. O. Sickled erythrocytes, hyphema, and secondary glaucoma: VII. The passage of sickled erythrocytes out of the anterior chamber of the human and monkey eye: light and electron microscopic studies. Ophthalmic Surg. 1979 Apr;10(4):89–123. [PubMed] [Google Scholar]
  27. Gö S., Cağlar M., Yilgör E., Altay C. Coexistence of sickle cell trait and cystic fibrosis. Turk J Pediatr. 1978 Jan-Apr;20(1-2):58–62. [PubMed] [Google Scholar]
  28. HUMPHREYS J. Observations on the sickle cell trait in Nigerian soldiers. J Trop Med Hyg. 1952 Aug;55(8):173–176. [PubMed] [Google Scholar]
  29. Hampton M. L., Anderson J., Lavizzo B. S., Bergmen A. B. Sickle cell "nondisease". A potentially serious public health problem. Am J Dis Child. 1974 Jul;128(1):58–61. [PubMed] [Google Scholar]
  30. Heiner M., Teasdale S. J., David T., Scott A. A., Glynn M. F. Aorta-coronary bypass in a patient with sickle cell trait. Can Anaesth Soc J. 1979 Sep;26(5):428–434. doi: 10.1007/BF03006460. [DOI] [PubMed] [Google Scholar]
  31. Heller P., Best W. R., Nelson R. B., Becktel J. Clinical implications of sickle-cell trait and glucose-6-phosphate dehydrogenase deficiency in hospitalized black male patients. N Engl J Med. 1979 May 3;300(18):1001–1005. doi: 10.1056/NEJM197905033001801. [DOI] [PubMed] [Google Scholar]
  32. Israel R. H., Salipante J. S. Pulmonary infarction in sickle cell trait. Am J Med. 1979 May;66(5):867–869. doi: 10.1016/0002-9343(79)91139-2. [DOI] [PubMed] [Google Scholar]
  33. Janerich D. T., Kelly J. H., Ziegler F. D., Selvin S., Porter I. H., Robinson J. B., Herdman R. C. Age trends in the prevalence of the sickle cell trait. Health Serv Rep. 1973 Nov;88(9):804–807. [PMC free article] [PubMed] [Google Scholar]
  34. Katz S. H., Lubin B., Armstrong D. Letter: Growth of adolescents with sickle-cell trait. Lancet. 1974 Apr 27;1(7861):814–815. doi: 10.1016/s0140-6736(74)92891-8. [DOI] [PubMed] [Google Scholar]
  35. Kramer M. S., Rooks Y., Pearson H. A. Growth and development in children with sickle-cell trait. A prospective study of matched pairs. N Engl J Med. 1978 Sep 28;299(13):686–689. doi: 10.1056/NEJM197809282991303. [DOI] [PubMed] [Google Scholar]
  36. LEWIS R. A., HATHORN M. CORRELATION OF S HEMOGLOBIN WITH GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY AND ITS SIGNIFICANCE. Blood. 1965 Aug;26:176–180. [PubMed] [Google Scholar]
  37. Luzzatto L., Allan N. C. Relationship between the genes for glucose-6-phosphate dehydrogenase and for haemoglobin in a Nigerian population. Nature. 1968 Sep 7;219(5158):1041–1042. doi: 10.1038/2191041a0. [DOI] [PubMed] [Google Scholar]
  38. MARTI H. R., SCHOEPF K., GSELL O. R. FREQUENCY OF HAEMOGLOBIN S AND GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY IN SOUTHERN TANZANIA. Br Med J. 1965 Jun 5;1(5448):1475–1476. [PMC free article] [PubMed] [Google Scholar]
  39. MCCORMICK W. F., KASHGARIAN M. ABNORMAL HEMOGLOBINS. V. AGE AT DEATH OF PATIENTS WITH SICKLE CELL TRAIT. Am J Hum Genet. 1965 Mar;17:101–103. [PMC free article] [PubMed] [Google Scholar]
  40. MEADOR C. K. THE ART AND SCIENCE OF NONDISEASE. N Engl J Med. 1965 Jan 14;272:92–95. doi: 10.1056/NEJM196501142720208. [DOI] [PubMed] [Google Scholar]
  41. Magnuson T. R., Hunter S. W., Bonnabeau R. C., Jr Multiple vascular infarction. A manifestation of sickle cell trait in the absence of hypoxia. Minn Med. 1980 Jun;63(6):381–383. [PubMed] [Google Scholar]
  42. Marchbanks V. H., Jr The sickle trait and the black airman. Aviat Space Environ Med. 1980 Mar;51(3):299–300. [PubMed] [Google Scholar]
  43. McCormack M. K., Katz S. H., Dicker L. Letter: Delayed skeletal maturation in sickle cell trait. J Pediatr. 1975 Sep;87(3):489–490. doi: 10.1016/s0022-3476(75)80668-8. [DOI] [PubMed] [Google Scholar]
  44. McCormack M. K., Scarr-Salapatek S., Polesky H., Thompson W., Katz S. H., Barker W. B. A comparison of the physical and intellectual development of black children with and without sickle-cell trait. Pediatrics. 1975 Dec;56(6):1021–1025. [PubMed] [Google Scholar]
  45. Molineaux L., Fleming A. F., Cornille-Brøgger R., Kagan I., Storey J. Abnormal haemoglobins in the Sudan savanna of Nigeria. III. Malaria, immunoglobulins and antimalarial antibodies in sickle cell disease. Ann Trop Med Parasitol. 1979 Aug;73(4):301–310. doi: 10.1080/00034983.1979.11687263. [DOI] [PubMed] [Google Scholar]
  46. Nhonoli A. M., Kujwalile J. M., Kigoni E. P., Masawe A. E. Correlation of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency and sickle cell trait (Hb-AS). Trop Geogr Med. 1978 Mar;30(1):99–101. [PubMed] [Google Scholar]
  47. Nicholson G. D., Amin U. F., Brooks S. E., Alleyne G. A. End-stage renal failure in sickle cell trait. West Indian Med J. 1979 Dec;28(4):235–239. [PubMed] [Google Scholar]
  48. Orbell S. F., Bell R. M. Sickle cell trait and intellectual ability. Cent Afr J Med. 1977 Aug;23(8):179–182. [PubMed] [Google Scholar]
  49. Oster J. R., Lanier D. C., Jr, Vaamonde C. A. Renal response to potassium loading in sickle cell trait. Arch Intern Med. 1980 Apr;140(4):534–536. [PubMed] [Google Scholar]
  50. Page M. M., MacKay J. M., Paterson G. Sickle cell trait and diabetic retinopathy. Br J Ophthalmol. 1979 Dec;63(12):837–838. doi: 10.1136/bjo.63.12.837. [DOI] [PMC free article] [PubMed] [Google Scholar]
  51. Patz A. Current concepts in ophthalmology. Retinal vascular diseases. N Engl J Med. 1978 Jun 29;298(26):1451–1454. doi: 10.1056/NEJM197806292982605. [DOI] [PubMed] [Google Scholar]
  52. Perumal J. R., Corbett M. Avascular necrosis of the femoral head in sickle cell trait. Rheumatol Rehabil. 1978 May;17(2):113–113. doi: 10.1093/rheumatology/17.2.113. [DOI] [PubMed] [Google Scholar]
  53. Polednak A. P., Janerich D. T. Birth characteristics of Blacks with sickle cell trait: a case-control study. Hum Biol. 1980 Feb;52(1):15–21. [PubMed] [Google Scholar]
  54. Rayman R. B. Sickle cell trait and the aviator. Aviat Space Environ Med. 1979 Nov;50(11):1170–1172. [PubMed] [Google Scholar]
  55. Sears D. A. The morbidity of sickle cell trait: a review of the literature. Am J Med. 1978 Jun;64(6):1021–1036. doi: 10.1016/0002-9343(78)90458-8. [DOI] [PubMed] [Google Scholar]
  56. Sethuraman M., Rao K. V. A survey of glucose-6-phosphate dehydrogenase deficiency & sickle-cell trait on a local population of Tirupati. Indian J Exp Biol. 1978 Oct;16(10):1098–1099. [PubMed] [Google Scholar]
  57. Ssebabi E. C., Bulwa F. M. Sickle cell trait anaemia in pregnancy. East Afr Med J. 1977 May;54(5):258–265. [PubMed] [Google Scholar]
  58. Weinger R. S., Benson G. S., Villarreal S. Gross hematuria associated with sickle cell trait and von Willebrand's disease. J Urol. 1979 Jul;122(1):136–137. doi: 10.1016/s0022-5347(17)56288-6. [DOI] [PubMed] [Google Scholar]
  59. Wheeler T. E., Nelsen V. M., Beidleman B., Dowling K. Alc hemoglobin in patients with diabetes mellitus and sickle cell trait. J Fla Med Assoc. 1977 Nov;64(11):757–761. [PubMed] [Google Scholar]
  60. Whitten C. F., Fischhoff J. Psychosocial effects of sickle cell disease. Arch Intern Med. 1974 Apr;133(4):681–689. [PubMed] [Google Scholar]
  61. Wolfe H. L., Rios M. A. Hypertension, proteinuria, and segmental renal parenchymal fibrosis with sickle cell trait. Tex Med. 1979 Mar;75(3):60–62. [PubMed] [Google Scholar]

Articles from Journal of the National Medical Association are provided here courtesy of National Medical Association

RESOURCES