Foetal Haemoglobin and Disease Severity in Nigerian Children with Sickle Cell Anaemia (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 Levels in Sickle Cell Disease (SCD) Patients in Sokoto, Nigeria
ackground: Sickle-cell disease (SCD) is a global public health problem occurring more commonly among people in the tropical and subtropical sub-Saharan regions where malaria is endemic. In this present study, we have investigated the haemoglobin F level (HBF) of 69 sickle cell disease subjects and 30 age and gender-matched apparently healthy controls. Methods: This case-control study was conducted among homozygous sickle cell patients attending the sickle cell clinics of Specialist Hospital Sokoto. About 3 millilitres of venous blood sample was collected from each participant into EDTA anticoagulated tubes. Estimation of haemoglobin F levels was carried out using Betke's method. The Packed Cell Volume (haematocrit) was determined using the Hawksley Haematospin 1300 micro haematocrit centrifuge. Results: The foetal haemoglobin level of 69 sickle cell disease patients (subjects) and 30 apparently healthy individual with genotype AA (control) was determined. The mean HbF and packed cell volume was significantly higher among the 69 sickle cell disease subjects (2.99 ±5.16) compared to controls (0.733 ±0.700) (p = 0.01). The packed cell volume was significantly higher among control participants (29.267±6.175) compared to the sickle cell disease subjects (24.57±6.99). Haemoglobin F level was compared based on the gender of the sickle cell disease subjects. The Haemoglobin F level although higher among male subjects (3.0469 ± 5.06510) compared to females (2.8836 ± 5.52), the difference however was not statistically significant (p =0.626). The haemoglobin F level was compared based of the age groups of the sickle cell disease subjects. The Haemoglobin F level appear to declining as age advances from 0.6-5yrs (3.26± 4.92) through 6-10yrs (3.01± 5.58) and 11-16yrs (2.34± 4.70).We observed a significant negative correlation between age and haemoglobin F levels among sickle cell disease subjects (r=-7.52, 0.001). Conclusion: In conclusion, we have observed that the HbF level is higher in sickle cell disease subjects compared to control participants with haemoglobin AA, that the haemoglobin F level is higher among male subjects compared to females, that the haemoglobin F level appears to decline as age advances and that a significant negative correlation exist between age and haemoglobin F levels among sickle cell disease subjects. We recommend that estimation of HbF level be carried out in conjunction with haemoglobin electrophoresis in the diagnosis, clinical management and in the determination of the clinical course of sickle cell disease. There is need to build the capacity in resource poor countries to optimize the diagnosis of sickle cell disease and other haemoglobinopathies. A national neonatal screen programme should be set up by the Nigerian Government to facilitate the early diagnosis and effective management of children with sickle cell disease.
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...
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).
Journal of Advances in Medicine and Medical Research, 2019
Introduction: Heterogeneity in sickle cell anaemia manifestations ranges from near asymptomatic cases to severe illness. Objective: This study determined the relationship between foetal haemoglobin F level, other haematological parameters and acute painful episode score of sickle cell disease patients in FCT Abuja Nigeria. Methods: 60 Sickle cell patients were selected for the study. 20 severe crises, 20 non-severe crisis SS were enrolled in the study. Control group comprised 20 apparently healthy haemoglobin AA individuals. Data were analysed descriptively. Results: Hb F level increased significantly in non-severe crisis sickle cell anaemia (7.12%± 3.6) and severe crisis (5.30%±2.3) groups, compared to the control group (0.32±1.8). This trend was also observed in RDW, MCHC and MCV. The mean Hb concentration and haematocrit (Hct) were significantly lower for both non- severe crisis and severe crisis SCA groups. There was no significant correlation between HbF and any of the haemato...
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.
Elevated foetal haemoglobin in tribal sickle cell anaemic patients: Blessing in disgust
International Journal of Clinical Biochemistry and Research, 2023
Background: Sickle cell anaemia is a molecular disease. WHO recognises it as a global public health problem. In India, it is common among tribal communities. Increased HbS is a culprit. Therapeutic research is focused on maintaining high levels of HbF and decreasing 2,3, BPG to target disease. Aim: To assess the role of naturally compensated haemoglobin variants in tribal Sickle cell anaemic patients of North East Gujarat. Settings and Design: Prospective, analytical, case control study conducted on randomly selected fifty tribal Sickle cell anaemic patients having disease for more than 5 years. Fifty age and sex-matched, healthy control subjects. Materials and Methods: Each fifty Tribal sickle cell anaemic patients and healthy control were included in the study. Total Haemoglobin level, Sickling test by NESTROFT method and Haemoglobin variants were analysed by alkaline haemoglobin electrophoresis. Frequencies of clinical crises were recorded by oral questioning. The results were analysed using SPSS version 20. Student unpaired t- test was employed to assess the significance of the differences. P-values < 0> Results: We observed decreased levels of total haemoglobin, high levels of HbF and HbA2 along with reduced HbA0. Compensatory increase of HbF in tribal SCA patients have shown lower frequency of clinical crises. Conclusion: Reduced HbA0 in sickle cell anaemia is compensated by naturally elevated HbF and HbA2 in tribal patients which has a beneficial influence on their general health. Keywords: Sickle cell anaemia, Electrophoresis, Haemoglobin, HbF, Tribal, Haemoglobin S. General health.
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.
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...