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Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events.
Eur J Haematol. 2006 Mar; 76(3):193-9.EJ

Abstract

We analyzed the records of 153 Guadeloupean children with sickle cell anemia (SCA), for whom clinical and laboratory data were prospectively collected (mean follow-up duration 8.4 +/- 4.6 yr). Prevalence and age-specific frequencies of acute clinical events were determined and correlations between complications, hematological parameters and potential modulating factors investigated. Painful crisis and acute chest syndrome (ACS) were the two most common complications, affecting 65.4% and 58.8% of the patients, respectively. The frequency of acute anemia was 49.7% (acute splenic sequestration 24.8%; acute aplastic anemia 15.0%). Prevalences of septicemia-meningitis and osteomyelitis were 15.7% and 16.3%, respectively. A higher incidence of infections, painful crises and acute anemia was detected in patients who developed ACS. The well-documented protective effect of HbF level on the overall disease expression was observed with higher HbF level in asymptomatic than in symptomatic patients (17.5% +/- 8% vs. 9.9% +/- 6.4%, P = 0.01) with similar ages and sex ratio. It was also confirmed on ACS and, for the first time, further extended to acute anemic events and septicemia. Besides its effect on hematological parameters, alpha-thalassemia seems to have little impact on the prevalence of complications, as do beta(S)-globin haplotypes. Comparison with other series suggests that the natural history of SCA in Guadeloupe is more similar to that in Jamaica with regard to those reported in Europe and the United States, suggesting a potential impact of environmental factors on the clinical course of the disease.

Authors+Show Affiliations

Inserm UMR 458 Université des Antilles et de la Guyane.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

16451394

Citation

Tarer, Vanessa, et al. "Sickle Cell Anemia in Guadeloupean Children: Pattern and Prevalence of Acute Clinical Events." European Journal of Haematology, vol. 76, no. 3, 2006, pp. 193-9.
Tarer V, Etienne-Julan M, Diara JP, et al. Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events. Eur J Haematol. 2006;76(3):193-9.
Tarer, V., Etienne-Julan, M., Diara, J. P., Belloy, M. S., Mukizi-Mukaza, M., Elion, J., & Romana, M. (2006). Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events. European Journal of Haematology, 76(3), 193-9.
Tarer V, et al. Sickle Cell Anemia in Guadeloupean Children: Pattern and Prevalence of Acute Clinical Events. Eur J Haematol. 2006;76(3):193-9. PubMed PMID: 16451394.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events. AU - Tarer,Vanessa, AU - Etienne-Julan,Maryse, AU - Diara,Jean-Pierre, AU - Belloy,Marie Sylvaine, AU - Mukizi-Mukaza,Martin, AU - Elion,Jacques, AU - Romana,Marc, PY - 2006/2/3/pubmed PY - 2006/5/25/medline PY - 2006/2/3/entrez SP - 193 EP - 9 JF - European journal of haematology JO - Eur J Haematol VL - 76 IS - 3 N2 - We analyzed the records of 153 Guadeloupean children with sickle cell anemia (SCA), for whom clinical and laboratory data were prospectively collected (mean follow-up duration 8.4 +/- 4.6 yr). Prevalence and age-specific frequencies of acute clinical events were determined and correlations between complications, hematological parameters and potential modulating factors investigated. Painful crisis and acute chest syndrome (ACS) were the two most common complications, affecting 65.4% and 58.8% of the patients, respectively. The frequency of acute anemia was 49.7% (acute splenic sequestration 24.8%; acute aplastic anemia 15.0%). Prevalences of septicemia-meningitis and osteomyelitis were 15.7% and 16.3%, respectively. A higher incidence of infections, painful crises and acute anemia was detected in patients who developed ACS. The well-documented protective effect of HbF level on the overall disease expression was observed with higher HbF level in asymptomatic than in symptomatic patients (17.5% +/- 8% vs. 9.9% +/- 6.4%, P = 0.01) with similar ages and sex ratio. It was also confirmed on ACS and, for the first time, further extended to acute anemic events and septicemia. Besides its effect on hematological parameters, alpha-thalassemia seems to have little impact on the prevalence of complications, as do beta(S)-globin haplotypes. Comparison with other series suggests that the natural history of SCA in Guadeloupe is more similar to that in Jamaica with regard to those reported in Europe and the United States, suggesting a potential impact of environmental factors on the clinical course of the disease. SN - 0902-4441 UR - https://www.unboundmedicine.com/medline/citation/16451394/Sickle_cell_anemia_in_Guadeloupean_children:_pattern_and_prevalence_of_acute_clinical_events_ L2 - https://doi.org/10.1111/j.1600-0609.2005.00590.x DB - PRIME DP - Unbound Medicine ER -