Foetal Haemoglobin Levels in Sickle Cell Disease (SCD) Patients in Sokoto, Nigeria (original) (raw)
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Foetal haemoglobin and disease severity in sickle cell anaemia patients in Kampala, Uganda
BMC Blood Disorders, 2012
Background: Sickle cell anaemia (SCA) is a major chronic health problem in Uganda. In patients with SCA, the level of foetal haemoglobin (HbF) has been found to be important in influencing the clinical course of the disease. Thus populations with high levels of HbF like those in Saudi Arabia have been described as having a milder clinical course with fewer complications as compared to populations with lower levels. Disease modifying drugs can increase the Hb F levels and modify the presentation of SCA.
Foetal Haemoglobin and Disease Severity in Nigerian Children with Sickle Cell Anaemia
Mediterranean journal of hematology and infectious diseases, 2017
Foetal haemoglobin (HbF) is a major modifying factor influencing sickle cell disease (SCD) severity. Despite this, HbF estimation is not routinely done in Nigeria. The relationship between HbF and SCD severity among affected children is also poorly studied. In this descriptive cross-sectional study, we determined the relationship between steady state HbF levels and disease severity of Nigerian children aged 1 - 15 years with homozygous SCD. For each child, the socio-demographic characteristics and SCD clinical severity were determined. The latter was assessed based on the frequency of significant painful episodes, blood transfusion, and hospitalisation in the preceding 12 months; lifetime cumulative incidence of SCD-related complications; the degree of splenic and hepatic enlargement; current haematocrit and leucocyte count. Foetal haemoglobin levels were quantified with high-performance liquid chromatography. The mean HbF level of the 105 children with SCA was 9.9 ± 6.0%. Male had ...
2019
The significantly lower Hb levels in sickle cell disease (SCD) (in children and adults) than that of controls was as expected, however the Hb concentration was found to be slightly higher in vaso-occlusive crises (VOC) than steady state. This research was to determine the distribution of haemoglobin phenotype and haemoglobin concentration among children and adults in sickle cell disease patients in Nigeria. This was a case control study carried out in Port Harcourt, Nigeria. There were three groups of 45 subjects each: SCD patients in steady state, SCD in VOC (which constituted the cases) and normal controls with HbAA. These participants had their blood samples analyzed for full blood count. Results were analyzed with the SPSS version 20. Haemoglobin SC (HbSC) patients were seen only in the VOC group, 2 in children and two in adults. This may be because most steady state SCD patients do not usually come for follow up visits, therefore those with relatively more stable disease may at...
BMC Research Notes, 2012
Background: Sickle cell disease is a genetic abnormality involving the haemoglobin. Although, it is primarily a red cell disorders, the white blood cells and platelets are also affected by the mutation. The consequent haemoglobin S causes polymerization of haemoglobin resulting in haemolysis and anaemia. This study aims to provide baseline haematological values in sickle cell disease patients in steady state and compare the deviation from haemoglobin phenotype AA control values. Methods: A case-control study was conducted amongst homozygous sickle cell patients attending the sickle cell clinics of Lagos State University Teaching Hospital Ikeja and haemoglobin phenotype AA controls. About 4.5mls of blood sample was collected from each participant for full blood count analysis. All blood samples were screened for HIV and haemoglobin phenotypes confirmed using cellulose acetate haemoglobin electrophoresis at pH 8.6. Results: A total of 103 cases and 98 controls were enrolled. The overall mean haemoglobin concentration for cases was 7.93 ± 1.47 g/dl, packed cell volume 24.44 ± 4.68%, mean cell volume 81.52 ± 7.89 fl, and mean cell haemoglobin 26.50 ± 3.20 pg. While for controls, mean haemoglobin concentration was 13.83 ± 1.32 g/dl, packed cell volume 43.07 ± 3.95%, mean cell volume 86.90 ± 4.69 fl, and mean cell haemoglobin 28.50 ± 1.34 pg. The overall mean white blood cell counts for the cases was 10.27 ± 3.94 *10 3 /μl and platelet counts of 412.71 ± 145.09*10 3 /μl. While white blood cell count for the controls was 5.67 ± 1.59*10 3 /μl and platelet counts of 222.82 ± 57.62*10 3 /μl. Conclusion: Homozygous sickle cell disease patients have lower values of red cell parameters, but higher values of white cell and platelets counts compared to haemoglobin phenotype AA controls.
Anemia, 2012
High HbF levels and F cells are correlated with reduced morbidity and mortality in sickle cell disease (SCD). This paper was designed to determine the HbF and F cells levels in Congolese sickle cell anemia (SCA) patients in order to determine their impact on the expression of SCD. Population and Method. HbF levels were measured in 89 SCA patients (mean age 11.4 yrs) using a standard HPLC method. F cell quantitation was done in a second group of SCA patients (n = 42, mean age 8.9 yrs) and compared with a control group (n = 47, mean age 5 yrs). F cells were quantified by a cytofluorometric system (MoAb-HbF-FITC; cut off at 0.5%). Results. The mean value of HbF was 7.2% ± 5.0 with heterogeneous distribution, most patients (76%) having HbF < 8%. Mean values of F-cells in SCA patients and control group were 5.4% ± 7.6 (median: 2.19%; range 0,0-30,3%) and 0.5% ± 1.6 (median 0.0, range 0-5.18), respectively. SCA patients with F cells >4.5% developed less painful crisis and had higher...
2021
Background: Sickle cell anemia (SCA) is a common genetic disorder that causes considerable morbidity and mortality throughout the world. The study was performed in 82 Sudanese participants with sickle cell disease as study population with both sexes, age (2-12 years). Materials and Methods: Venous blood was collected using sterile disposable plastic syringe after cleaning the vein puncture area with 70% ethanol, the blood was added to the EDTA and gently mixed, then CBC was performed using (Sysmex-3000 plus) and (Electrophoresis) was done. Results: The results of this study show that most patients had raised level of hemoglobin F according to gender found that there were 43 males and 28 females had raised level of HbF, 7 males and 2 females were normal, and 36% of patients had raised level of HbA2 in both gender (20 males) (10 females). Conclusion: HbF in sickle cell anemia patients was found to be 85.3% (raised), HbS in sickle cell anemia patients was found to be 100% (raised) and HbA2 in sickle cell anemia patients was found to be 30.5% (Normal).
International Journal of Research in Medical Sciences, 2019
Background: Sickle Cell Disorder (SCD) is a major health problem in India. After introduction of High-Performance Liquid Chromatography (HPLC) in MKCG Medical College, this study is first of its kind to describe haemoglobin variants of SCD. The aim of the study was to document haematological profile and pattern of haemoglobin variants in SCD patients. Methods: A Hospital based cross sectional study was conducted in Pathology department, MKCG medical college from October 2018 to May 2019. Sickle cell patients were included and patients in Sickle cell crisis or transfused with blood in last 3 months were excluded. Hematological indices were measured by Sysmex XT 2000i blood analyzer. Quantification of hemoglobin variants was done by HPLC. All data were analyzed using SPSS and Independent t-test was applied. Results: In this study 100 heterozygous and 116 homozygous cases were reported. In homozygous cases Hb were significantly low and MCV, MCH, RDW-CV were significantly high than heterozygous. Hb level was significantly lower in homozygous children. Hb F was significantly higher in children and homozygous cases. A significant positive correlation was seen between Hb and RBC in both cases. Conclusions: In homozygous cases moderate anaemia (microcytic hypochromic to normocytic hypochromic) with High Hb F and in heterozygous cases mild anaemia (microcytic hypochromic) dominated the haematological profile. Children were significantly more anaemic than adults in homozygous cases. Anisocytosis was significantly more in homozygous cases and pediatric age group. Average fetal Haemoglobin variant (Hb F) was significantly more in homozygous cases and lower in adult group in both homo and heterozygous cases.
Fetal haemoglobin: A novel prognostic determinant in sickle cell anaemia
European Journal of Molecular & Clinical Medicine, 2020
Background-Acknowledgement of instrumental action of fetal hemoglobin(HbF) in improving the clinical condition of sickle cell disease (SCD) patients, began around 7 decades back, by Janet Watson who found that infants with SCD had lesser symptoms and their deoxygenated RBCs did not sickle or deform as aggressively as their mothers having sickle cell trait. She proposed high HbF levels in infant blood as compared to mothers for the observations of her study. Reduced incidence of crisis like vaso-occlusive, haemolytic, aplastic, megaloblastic, infectious and others have been found with higher HbF levels. With this backdrop the present study is being undertaken to know the reciprocity between HbF levels and the state of crisis in sickle cell. The study is expected to fill the missing link between "Why a few patients with HbS with haemoglobin pattern of SS suffer less from crisis than that of others with a similar haemoglobin pattern of SS" Objectives: • To know HbF levels in pateints with SCD with Hb (SS) pattern • To draw the comparisons between HbF levels in SCD with Hb(SS) pattern in subjects 1. With sickle cell crisis and 2. Without sickle cell crisis • To find out cutoff level of Hb F at its protective effect against the crisis in SCD Methodology • Study design-Observational and analytical study • Sample size-Total of 30 Patients of sickle cell Disease (Hb SS) • Study duration-1 year Expected Results: Incidence of Crisis in HbSS patients are expected to have a inverse relationship with HbF levels. A cutoff level of HbF above which episodes of crisis are lower can be expected to be found. Conclusion: Conclusions will be drawn from results obtained and an attempt to establish a cutoff HbF value which renders a protective action against crisis in SCD will be made.
Annual Research & Review in Biology
Background: Sickle cell disease (SCD) is the commonest genetic disorder worldwide with a global prevalence of 20-25 million. About 12-15 million affected persons are in Sub-Sahara Africa with Nigeria bearing the highest burden of people living with sickle cell disease. SCD is a disease characterized as an autosomal, recessive, heterogeneous, and a monogenetic disorder caused by an A-to-T point mutation in the β-globin gene responsible for the production of abnormal hemoglobin S (HbS), which polymerizes in the deoxygenated state and results in the sickling of erythrocytes. Haemoglobin variants are mutant forms of haemoglobin in a population usually occurring as a result of genetic changes in specific genes, or globins that causes change on alterations in the amino acid. They could affect the structure, behavior, the production rate and the stability of the specific gene. Well-known haemoglobin variants such as sick-cell anaemia are responsible for diseases and are considered haemogl...