the influence of fetal hemoglobin on clinical and hematological variables of children and adolescents with sickle cell anemia in basra, southern iraq
نویسندگان
چکیده
background: there are many parameters that modulate the severity of sickle cell anemia. fetal hemoglobin (hb f) is one of these major variables. however, its effect is clinically inconsistent. we conducted a descriptive study to assess the influence of hb f on clinical events and hematological variables in patients with sickle cell anemia. methods: 151 patients with sickle cell anemia with a stable condition, aged 1-18 years, were recruited from march through november 2010. the results of complete blood count and hb f level and various clinical variables were recorded. results: of the 151 patients, the hb f was more than 20%, 10-20%, and less than 10% in 77 (51%), 60 (39.7%), and 14 (9.3%) patients. a significant negative association was reported between hb f level and frequency of painful crisis (95% ci=0.05-0.96, or=0.22), acute chest syndrome (95% ci=0.01-0.43, or=0.07) and frequency of hospitalizations (95% ci=0.03-0.85, or=0.11). there was a significant positive association between hemoglobin level (95% ci=2.14-27.17, or=7.63) and splenomegaly (95% ci=1.37-57.4, or=12.88) with hb f level. conclusion: in children and adolescents with sickle cell anemia, the higher the hb f levels, the lesser clinical complications of the disease would be. therefore, patients with low hb f need close follow-up and monitoring since early age to detect complications as early as possible and consider use of disease modifying agents.
منابع مشابه
The Influence of Fetal Hemoglobin on Clinical and Hematological Variables of Children and Adolescents with Sickle Cell Anemia in Basra, Southern Iraq
Background: There are many parameters that modulate the severity of sickle cell anemia. Fetal hemoglobin (Hb F) is one of these major variables. However, its effect is clinically inconsistent. We conducted a descriptive study to assess the influence of Hb F on clinical events and hematological variables in patients with sickle cell anemia. Methods: 151 patients with sickle cell anemia with a st...
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Fetal hemoglobin in sickle cell anemia
Fetal hemoglobin (HbF) is the major genetic modulator of the hematologic and clinical features of sickle cell disease, an effect mediated by its exclusion from the sickle hemoglobin polymer. Fetal hemoglobin genes are genetically regulated, and the level of HbF and its distribution among sickle erythrocytes is highly variable. Some patients with sickle cell disease have exceptionally high level...
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عنوان ژورنال:
iranian journal of blood and cancerجلد ۷، شماره ۴، صفحات ۱۷۹-۱۸۳
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