Foetal haemoglobin and disease severity in sickle cell anaemia patients in Kampala, Uganda (original) (raw)
Related papers
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 ...
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.
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).
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...
Indian Journal of Case Reports, 2021
Sickle cell disease (SCD) is the most common inherited disorder of hemoglobin worldwide. In Nigeria, the prevalence of SCD is 20–30/1000 live births. The burden of the disease has reached a level where it contributes 9–16% of the under-five mortality in many West African countries. This case series evaluated the chromatographic patterns and red blood cell indices of sickle cell homozygous patients. Red cell indices, blood film, sickle solubility test, and chromatographic patterns using Bio-Rad HPLC D10 were evaluated for both patients. Both the patients were Nigerian and HPLC showed HbS window 81.7 and 81.6% and increased HbF, that is, 7.5 and 8.8%. HbA2 was normal in both the cases, that is, 2.2 and 2.6%. Our data suggest that homozygous sickle cell disease is very common among the Nigerian population with an increase in HbF along with HbS and HbA2 is normal.
Haemoglobin, and Hematological Features in Congolese Patients with Sickle Cell Anaemia
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 percentage of reticulocytes. Conclusion. Congolese SCA patients displayed low levels of HbF and F-cells that contribute to the severity of SCD.
Association of HbA2, HbF and HbS Values in Sickle Cell Disease Patients in Kano, Northwest Nigeria
2017
Background Sickle cell disease accounts for over 60% of the world’s major haemoglobinopathies with an estimated 2-3% of Nigerians having HbSS. The aim of this study was to determine the association of hemoglobin levels of A 2 , F and S in sickle cell disease patients. Methodology A total of 140 sickle cell anaemia (SCA) patients, aged 1-33 years and 68, age- matched apparently healthy subjects offbeat and HbAS were recruited for the study between January, 2014 and February, 2016. Hemoglobin levels of S, A 2 and F were determined by ion-exchange HPLC. Results: The mean values of HbS, HbA 2 and HbF of 81.14.98%, 2.9 0.81% and 7.2 4.1% in steady state showed no statistically significant differences when compared to 81.88 4.0%, 2.47 0.9% and 7.25 3.66% in vaso-occlusive crisis, respectively (P>0.05).HbF level was significantly higher in HbSS patients (7.2 4.1%) than HbAS (1.9 1.84%) and HbAA (0.56 0.56%) subjects (P˂0.05). HbS and HbF levels showed significant and strong negativ...
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...
Hemato
Hematological and biochemical reference values in sickle cell disease (SCD) are crucial for patient management and the evaluation of interventions. This study was conducted at Muhimbili National Hospital (MNH) in Dar es Salaam, Tanzania, to establish laboratory reference ranges among children and adults with SCD at steady state. Patients were grouped into five age groups and according to their sex. Aggregate functions were used to handle repeated measurements within the individual level in each age group. A nonparametric approach was used to smooth the curves, and a parametric approach was used to determine SCD normal ranges. Comparison between males and females and against the general population was documented. Data from 4422 patients collected from 2004–2015 were analyzed. The majority of the patients (35.41%) were children aged between 5–11 years. There were no significant differences (p ≥ 0.05) in mean corpuscular hemoglobin concentration (MCHC), lymphocytes, basophils, and dire...