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. 1972 Jan;25(1):49–55. doi: 10.1136/jcp.25.1.49

Sickle-cell haemoglobin C disease in London

A J Black 1,2,3,4, P I Condon 1,2,3,4, B M Gompels 1,2,3,4, R L Green 1,2,3,4, R G Huntsman 1,2,3,4, G C Jenkins 1,2,3,4
PMCID: PMC477220  PMID: 5015374

Abstract

The manifestations of the sickling disorders are becoming increasingly familiar to clinicians in Great Britain. One of these disorders, sickle-cell haemoglobin disease, has hitherto received little attention, being regarded as a relatively mild condition. This paper describes some of the distinctive clinical features of the disease as seen in a series of nine cases which have recently presented in London, two of which were fatal. The special hazards of the condition in relation to pregnancy, air travel, and general anaesthesia are discussed.

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Selected References

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

  1. BARTON C. J., COCKSHOTT W. P. Bone changes in hemoglobin SC disease. Am J Roentgenol Radium Ther Nucl Med. 1962 Sep;88:523–532. [PubMed] [Google Scholar]
  2. BECKER J. A. Hemoglobin S-C disease. Am J Roentgenol Radium Ther Nucl Med. 1962 Sep;88:503–511. [PubMed] [Google Scholar]
  3. CHAPMAN A. Z., REEDER P. S., FRIEDMAN I. A., BAKER L. A. Gross hematuria in sickle cell trait and sickle cell hemoglobin-C disease. Am J Med. 1955 Nov;19(5):773–782. doi: 10.1016/s0002-9343(55)80022-8. [DOI] [PubMed] [Google Scholar]
  4. CHERNOFF A. I. The human hemoglobins in health and disease. N Engl J Med. 1955 Sep 8;253(10):416–concl. doi: 10.1056/NEJM195509082531006. [DOI] [PubMed] [Google Scholar]
  5. COLEMAN W. A., FURTH F. W. Splenic infarction in a patient with sickle-cell-hemoglobin-c disease; report of a case occurring following air travel. AMA Arch Intern Med. 1956 Aug;98(2):247–249. doi: 10.1001/archinte.1956.00250260121012. [DOI] [PubMed] [Google Scholar]
  6. EDINGTON G. M. The pathology of sickle cell haemoglobin C disease and sickle cell anaemia. J Clin Pathol. 1957 May;10(2):182–186. doi: 10.1136/jcp.10.2.182. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. EISENSTEIN M. I., FRIEDMAN S., POSNER A. C. Sickle-cell anemia in pregnancy; a review of the literature with additional case histories. Am J Obstet Gynecol. 1956 Sep;72(3):622–634. [PubMed] [Google Scholar]
  8. GRIGGS R. C., HARRIS J. W. The biophysics of the variants of sickle-cell disease. AMA Arch Intern Med. 1956 Mar;97(3):315–326. doi: 10.1001/archinte.1956.00250210061005. [DOI] [PubMed] [Google Scholar]
  9. Gilbertson A. A. Anaesthesia in West African patients with sickle-cell anaemia, haemoglobin SC disease, and sickle-cell trait. Br J Anaesth. 1965 Aug;37(8):614–622. doi: 10.1093/bja/37.8.614. [DOI] [PubMed] [Google Scholar]
  10. Gilbertson A. A., Ball P. A., Watson-Williams E. J. The management of anaesthesia in sickle cell states. Proc R Soc Med. 1967 Jul 7;60(7):631–636. [PMC free article] [PubMed] [Google Scholar]
  11. Konotey-Ahulu F. I. Anaesthetic deaths and the sickle-cell trait. Lancet. 1969 Feb 1;1(7588):267–268. doi: 10.1016/s0140-6736(69)91287-2. [DOI] [PubMed] [Google Scholar]
  12. Konotey-Ahulu F. I. Sickle-cell disease and monocular blindness in Africans. Lancet. 1968 Jul 27;2(7561):222–222. doi: 10.1016/s0140-6736(68)92660-3. [DOI] [PubMed] [Google Scholar]
  13. LEHMANN H., NWOKOLO C. The River Niger as a barrier in the spread eastwards of haemoglobin C: a survey of haemoglobins in the lbo. Nature. 1959 Jun 6;183(4675):1587–1588. doi: 10.1038/1831587a0. [DOI] [PubMed] [Google Scholar]
  14. LISKER S. A., FINKELSTEIN D., SCHWARTZ S., MARULYALI K., VALDES-DAPENA A. THE COEXISTENCE OF ACUTE RHEUMATIC FEVER AND SICKLE CELL-HEMOGLOBIN C DISEASE. Circulation. 1965 Jan;31:108–112. doi: 10.1161/01.cir.31.1.108. [DOI] [PubMed] [Google Scholar]
  15. MOSER K. M., SHEA J. G. The relationship between pulmonary infarction, cor pulmonale and the sickle states. Am J Med. 1957 Apr;22(4):561–579. doi: 10.1016/0002-9343(57)90110-9. [DOI] [PubMed] [Google Scholar]
  16. MOVITT E. R., MANGUM J. F., PORTER W. R. Sickle cell-hemoglobin C disease. Quantitative determination of iron kinetics and hemoglobin synthesis. Blood. 1963 May;21:535–545. [PubMed] [Google Scholar]
  17. OBER W. B., BRUNO M. S., SIMON R. M., WEINER L. Hemoglobin S-C disease with fat embolism: report of a patient dying in crisis: autopsy findings. Am J Med. 1959 Oct;27:647–658. doi: 10.1016/0002-9343(59)90048-8. [DOI] [PubMed] [Google Scholar]
  18. Onuaguluchi G., Akande E. O. Severe crises with jaundice in young non-pregnant adults with sickle-cell haemoglobin-C disease. Lancet. 1966 Apr 2;1(7440):737–739. doi: 10.1016/s0140-6736(66)90893-2. [DOI] [PubMed] [Google Scholar]
  19. PATON D. The conjunctival sign ox sickle-cell disease. Further observations. Arch Ophthalmol. 1962 Nov;68:627–632. doi: 10.1001/archopht.1962.00960030631010. [DOI] [PubMed] [Google Scholar]
  20. RIVER G. L., ROBBINS A. B., SCHWARTZ S. O. S-C hemoglobin: a clinical study. Blood. 1961 Oct;18:385–416. [PubMed] [Google Scholar]
  21. SHELLEY W. M., CURTIS E. M. Bone marrow and fat embolism in sickle cell anemia and sickle cell-hemoglobin C disease. Bull Johns Hopkins Hosp. 1958 Jul;103(1):8–25. [PubMed] [Google Scholar]
  22. SMITH E. W., CONLEY C. L. Clinical features of the genetic variants of sickle cell disease. Bull Johns Hopkins Hosp. 1954 Jun;94(6):289–318. [PubMed] [Google Scholar]
  23. SMITH E. W., CONLEY C. L. Sicklemia and infarction of the spleen during aerial flight; electrophoresis of the hemoglobin in 15 cases. Bull Johns Hopkins Hosp. 1955 Jan;96(1):35–41. [PubMed] [Google Scholar]
  24. Serjeant G. R., Richards R., Barbor P. R., Milner P. F. Relatively benign sickle-cell anaemia in 60 patients aged over 30 in the West Indies. Br Med J. 1968 Jul 13;3(5610):86–91. doi: 10.1136/bmj.3.5610.86. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. TUTTLE A. H., KOCH B. Clinical and hematological manifestations of hemoglobin CS disease in children. J Pediatr. 1960 Mar;56:331–342. doi: 10.1016/s0022-3476(60)80184-9. [DOI] [PubMed] [Google Scholar]

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