Acute clinical events in 299 homozygous sickle cell patients living in France
| Autor: | P. Beauvais, P Bégué, M Belloy, Micheline Maier-Redelsperger, Robert Girot, B. Quinet, M Guilloud-Bataille, M. G. Neonato, M. de Montalembert, Malika Benkerrou, Rolande Ducrocq, Josué Feingold, Jacques Elion |
|---|---|
| Rok vydání: | 2000 |
| Předmět: |
medicine.medical_specialty
education.field_of_study Anemia business.industry Osteomyelitis Population Hematology General Medicine medicine.disease Asymptomatic Sickle cell anemia Acute chest syndrome Surgery Hemoglobinopathy Internal medicine medicine medicine.symptom education business Cohort study |
| Zdroj: | European Journal of Haematology. 65:155-164 |
| ISSN: | 0902-4441 |
| DOI: | 10.1034/j.1600-0609.2000.90210.x |
| Popis: | A subset of 299 patients with homozygous sickle cell anaemia, enrolled in the cohort of the French Study Group on sickle cell disease (SCD), was investigated in this study. The majority of patients were children (mean age 10.1 +/- 5.8 yr) of first generation immigrants from Western and Central Africa, the others originated from the French West Indies (20.2%). We report the frequency of the main clinical events (mean follow-up 4.2 +/- 2.2 yr). The prevalence of meningitis-septicaemia and osteomyelitis was, respectively, 11.4% and 12% acute chest syndrome was observed in 134 patients (44.8%). Twenty patients (6.7%) developed stroke with peak prevalence at 10-15 yr of age. One hundred and seventy-two patients (58%) suffered from one or more painful sickle cell crises, while the others (42.5%) never suffered from pain. The overall frequency of acute anaemic episodes was 50.5%, (acute aplastic anaemia 46%; acute splenic sequestration 26%). A group of 27 patients were asymptomatic (follow-up > 3 yr). Epistatic mechanisms influencing SCD were studied. Coinherited alpha-thalassemia strongly reduced the risk of stroke (p |
| Databáze: | OpenAIRE |
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