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      FOETAL HAEMOGLOBIN (HbF) STATUS IN ADULT SICKLE CELL ANAEMIA PATIENTS IN IBADAN, NIGERIA

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          Abstract

          Aim:

          Elevated HbF, among other biological and environmental factors, is responsible for decrease in mortality in sickle cell anaemia (SCA). This study determined the levels of HbF in adult SCA patients in steady state compared with HbAA controls. HbF was discussed in line with the clinical course of the disease so as to emphasize the relevance of hydroxyurea in the management of adult SCA patients.

          Materials and methods:

          The HbF levels of 66 confirmed SCA patients and 31 HbAA controls were estimated using Betke method and HbF percentage was calculated using formula: %HbF percentage = A 413 filtrate x 100A 413 standard x 20.

          Result:

          A statistical significant difference in the mean of the levels of HbF in patients (5.16±4.04) compared to controls (1.04±0.44) (p = 0.000) was observed. The mean levels of HbF for males (4.71±3.49) compared to that of females (4.99) were statistically similar (p =0.773). It was also observed that the mean HbF level appears to be declining as age advances. SCA patients were classified to three categories viz: HbF <2% (21.2% SCA patients); HbF of 2.1% -10%, (68.2% SCA patients); and HbF of 10.1% -16%, (10.6% SCA patients).

          Conclusion:

          Substantial proportion of our patients actually will require treatment with hydroxylurea to stimulate HbF production especially those with HbF percentage of <2 and some with HbF percentage of 2.1 – 10%. HPFH may be considered rare since only 10. 6% had HbF at the range of 10.1-16%. This study showed that in treating our SCA patients in Nigeria we need to adopt and encourage the use of HbF activating agents like hydroxyurea or any other safe agent that will be found to stimulate HbF production in SCD patients.

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          Most cited references20

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          Pain in sickle cell disease. Rates and risk factors.

          Acute episodes of pain are the principal symptom of sickle cell disease, but little is known about the epidemiologic features of these episodes or risk factors for them, nor is it known whether patients with high rates of such episodes die prematurely. We prospectively studied the natural history of sickle cell disease in 3578 patients ranging from newborns to persons up to 66 years old who were followed at clinical centers across the United States. There were 12,290 episodes of pain in 18,356 patient-years. The average rate was 0.8 episode per patient-year in sickle cell anemia, 1.0 episode per patient-year in sickle beta 0-thalassemia, and 0.4 episode per patient-year in hemoglobin SC disease and sickle beta(+)-thalassemia. The rate varied widely within each of these four groups--e.g., 39 percent of patients with sickle cell anemia had no episodes of pain, and 1 percent had more than six episodes per year. The 5.2 percent of patients with 3 to 10 episodes per year had 32.9 percent of all episodes. Among patients with sickle cell anemia who were more than 20 years old, those with high rates of pain episodes tended to die earlier than those with low rates. High rates were associated with a high hematocrit and low fetal hemoglobin levels. alpha-Thalassemia had no effect on pain apart from its association with an increased hematocrit. The "pain rate" (episodes per year) is a measure of clinical severity and correlates with early death in patients with sickle cell anemia over the age of 20. Even when the fetal hemoglobin level is low, one can predict that small increments in the level may have an ameliorating effect on the pain rate and may ultimately improve survival. This outcome is particularly encouraging to investigators studying hydroxyurea and other treatments designed to increase the fetal hemoglobin level.
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            A QTL influencing F cell production maps to a gene encoding a zinc-finger protein on chromosome 2p15.

            F cells measure the presence of fetal hemoglobin, a heritable quantitative trait in adults that accounts for substantial phenotypic diversity of sickle cell disease and beta thalassemia. We applied a genome-wide association mapping strategy to individuals with contrasting extreme trait values and mapped a new F cell quantitative trait locus to BCL11A, which encodes a zinc-finger protein, on chromosome 2p15. The 2p15 BCL11A quantitative trait locus accounts for 15.1% of the trait variance.
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              • Article: not found

              Sickle cell anemia a molecular disease.

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                Author and article information

                Journal
                Ann Ib Postgrad Med
                Ann Ib Postgrad Med
                AIPM
                Annals of Ibadan Postgraduate Medicine
                Association of Resident Doctors (ARD), University College Hospital, Ibadan
                1597-1627
                June 2010
                : 8
                : 1
                : 30-33
                Affiliations
                [* ]Department of Haematology, University College Hospital, Ibadan, Nigeria
                [+ ]Department of Chemical Pathology; University College Hospital, Ibadan, Nigeria
                [++ ]IMRAT, College of Medicine, University of Ibadan.
                Author notes
                Correspondence: Olaniyi J.A. Department of Haematology University College Hospital, Ibadan, Nigeria
                Article
                AIPM-8-30
                10.4314/aipm.v8i1.63955
                4138770
                25161472
                fc48de42-a3b0-424b-a49b-7654e07e9cd7
                © Association of Resident Doctors, UCH, Ibadan

                This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

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                Categories
                Original Article

                sickle cell anaemia,hbf levels,hydroxyurea use
                sickle cell anaemia, hbf levels, hydroxyurea use

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