Abstract
All acute admissions of patients with sickle cell disease who lived in the London borough of Brent and attended this hospital were analysed for a period of one year. Sixty three of the 211 patients who were followed up by the haematology department required 161 acute admissions during the year. Most admissions (126) were for the 42 patients with homozygous sickle cell disease; 147 (91%) were for vaso-occlusive episodes, 142 of which were for painful crises, three for cerebrovascular accidents, and two for renal papillary necrosis. Preschool children with sickle cell disease were admitted predominantly with limb pain, whereas in schoolchildren and adults the incidence of trunk pain was higher. Twenty four of the 93 episodes of trunk pain culminated in an episode of severe visceral sequestration usually affecting the lungs, the liver, or the mesenteric circulation. Two patients died: an 18 month old baby with an acute splenic sequestration crisis and a 19 year old man with a severe girdle syndrome (sickling in the mesenteric circulation, liver, and lungs). Infective episodes were rare (11 episodes) but severe: one haemophilus meningitis, two salmonella infections, and three aplastic crises due to parvovirus infections. The average duration of the hospital stay was 7.4 days per admission. It is concluded that because sickle cell disease causes appreciable morbidity in older children, adolescents, and adults a systematic approach to management is needed to deal with acute episodes such as sequestration syndromes.
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Selected References
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- Anionwu E., Walford D., Brozović M., Kirkwood B. Sickle-cell disease in a British urban community. Br Med J (Clin Res Ed) 1981 Jan 24;282(6260):283–286. doi: 10.1136/bmj.282.6260.283. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Brozovic M., Davies S. C., Yardumian A., Bellingham A., Marsh G., Stephens A. D. Pain relief in sickle cell crisis. Lancet. 1986 Sep 13;2(8507):624–625. doi: 10.1016/s0140-6736(86)92442-6. [DOI] [PubMed] [Google Scholar]
- Brozović M., Anionwu E. Sickle cell disease in Britain. J Clin Pathol. 1984 Dec;37(12):1321–1326. doi: 10.1136/jcp.37.12.1321. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Davies S. C., Luce P. J., Win A. A., Riordan J. F., Brozovic M. Acute chest syndrome in sickle-cell disease. Lancet. 1984 Jan 7;1(8367):36–38. doi: 10.1016/s0140-6736(84)90193-4. [DOI] [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]
- 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]
- Hatton C. S., Bunch C., Weatherall D. J. Hepatic sequestration in sickle cell anaemia. Br Med J (Clin Res Ed) 1985 Mar 9;290(6470):744–745. doi: 10.1136/bmj.290.6470.744. [DOI] [PMC free article] [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]
- Horn M. E., Dick M. C., Frost B., Davis L. R., Bellingham A. J., Stroud C. E., Studd J. W. Neonatal screening for sickle cell diseases in Camberwell: results and recommendations of a two year pilot study. Br Med J (Clin Res Ed) 1986 Mar 15;292(6522):737–740. doi: 10.1136/bmj.292.6522.737. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Nagel R. L., Fabry M. E., Pagnier J., Zohoun I., Wajcman H., Baudin V., Labie D. Hematologically and genetically distinct forms of sickle cell anemia in Africa. The Senegal type and the Benin type. N Engl J Med. 1985 Apr 4;312(14):880–884. doi: 10.1056/NEJM198504043121403. [DOI] [PubMed] [Google Scholar]
- Serjeant G. R. Fetal haemoglobin in homozygous sickle cell disease. Clin Haematol. 1975 Feb;4(1):109–122. [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]