Routine haemoglobin electrophoresis screening in day case herniotomy in Nigerian children: Is it evidence-based? (original) (raw)
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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.
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 ...
Newborn screening for sickle cell disease in a Nigerian hospital
Public Health, 2008
Sickle cell disease (SCD) is the most common genetic disorder to affect Blacks. The mortality rate associated with SCD has remained high despite the use of appropriate interventions to manage the various forms of crisis. In developed countries, newborn screening programmes are conducted routinely, which has resulted in a reduction in the SCD mortality rate from 16% to <1%. In developing countries where the disease is prevalent, newborn screening programmes are yet to be established, and the acceptability of such programmes by the parents of newly delivered infants is unknown. Objectives: This study was carried out to determine the acceptability of newborn screening for SCD on mothers of newly delivered infants, and to establish disease prevalence amongst a newborn population in Nigeria. Study design: This prospective cross-sectional study was conducted at St. Philomena's Hospital, Benin City with mothers of newly delivered infants and their newborn babies. Methods: Newly delivered mothers were recruited consecutively into the study. Knowledge of their own haemoglobin phenotype status was assessed, and their wishes regarding SCD screening of their babies were determined. Babies were screened using isofocusing electrophoresis. Results: Six hundred and thirty mothers, delivered of 649 babies, were recruited into this study. Nineteen sets of babies were twins. Two mothers refused screening for their babies and 628 mothers or caregivers accepted screening; hence the acceptance rate was 99.7%. Four hundred and fifty-seven (71%) mothers did not know their own haemoglobin phenotype. Six hundred and forty-seven babies were screened for SCD. Of these, two samples were lost to testing and one baby had an indeterminate result; these three cases were not included in the analysis. Of the
Hematology & Transfusion International Journal, 2017
Background: Chronic anemia in SCD during pregnancy could lead to a cascade of maternal and fetal complications. The aim of this study is to assess the various types of ABO blood groups of pregnant SCD women at booking and the severity of anemia. The outcome of this study will help in making recommendations of ABO blood types most suitable to improve the quality of life of SCD patients. This may contribute to guideline for safe blood transfusion in SCD. Methodology: This was a-ten-year retrospective study of all registered SCD pregnant women seen at the antenatal clinic of Braithwaite Memorial Specialist Hospital (BMSH) Port Harcourt (January 2004-December 2013). Data on gestational age, Hemoglobin (HB) electrophoretic pattern, HB concentration and blood group were obtained at booking clinic. Data were analysed using Epi-info version 7.02 by WHO,
Newborn Screening for Sickle Cell Disease (SCD) in Awka South East Nigeria
Journal of Blood Disorders & Transfusion, 2018
Background: Sickle cell Diseases (SCD) is a genetic disorder. It is the most common genetic disorder affecting black race worldwide. It carries a high mortality and morbidity when not promptly and properly managed. Early detection is key to improving the quality of life of people living with sickle cell disease especially in childhood. It has resulted in reduction in mortality rate of SCD to <1% in developed countries through newborn screening but this reduction is yet to be achieved in developing countries. Objective: To share the experience of newborn screening of SCD at COOUTH Awka. To determine the prevalence of Sickle cell disease among neonates in Anambra State. Methodology: A retrospective study of screening for SCD in new-borns carried out at Chukwuemeka Odumegwu Ojukwu University Teaching Hospital (COOUTH) Awka from 1 st September 2013 to 27 th October 2017. This was done using isoelectric focusing machine. Results: A total of 4961 children were screened, 2410 were males, while 2551 were females. 75% (3733) of the neonates had their hemoglobin genotypes as AA, while 0.3% (16) were Hb SS genotype. Prevalence of Hb SS genotype amongst newborns was 0.32%. Conclusion: This study found the prevalence of sickle cell disease in newborns to be 0.32%. Newborn screening using Isoelectric focusing machine is very valuable for early diagnosis and prompt management of SCD. There is therefore need for increased awareness of this in Anambra State.
Acta Paediatrica, 2015
Aim: Neonatal screening for sickle cell anaemia is not common practice in the Democratic Republic of Congo, and we determined the prevalence in children with unknown electrophoresis of haemoglobin and anaemia. Methods: A cross-sectional study was conducted in four hospitals in the country's capital Kinshasa. Results: We screened 807 patients with anaemia (Hb < 6 g/dL) for sickle cell disease. The overall mean age at presentation was 42.7 months AE 29.7 months, and most patients (76.3%) were less than five years of age, with a peak incidence at seven to 36 months of age (45%). The median age at the first transfusion was 29 months (range 4-159 months). Of these 807 children, 36 (4.5%) were homozygous for haemoglobin S disease and 45 (5.6%) were heterozygotes. The proportion of patients with homozygous sickle cell anaemia was slightly higher in children with a medical history of hand foot syndrome, in children who had received more than three transfusions and in children up to 36 months of age at their first transfusion. Conclusion: The high prevalence of sickle cell anaemia in children in Sub-Saharan Africa underlines the need for neonatal screening or, if that is not possible, screening of all children with severe anaemia to identify patients with the disease and provide early management.
Open journal of blood diseases, 2022
Hemoglobinopathies, mainly Sickle cell disease (SCD), are the most common monogenic disorders in Africa. In Burkina Faso, data on these diseases are scarce, mainly hospital-based in Ouagadougou and its surroundings. In order to assess the incidence and allelic frequencies of the main hemoglobinopathies in newborns in Burkina Faso, we conducted a cross-sectional study from 2015 to 2019 in four hospitals. The study included babies of both sexes, regardless of ethnic group and parents' hemoglobin status. It was a newborn screening and hemoglobin variants were detected using isoelectric focusing on cord blood samples and confirmed using hemoglobin electrophoresis by high-performance liquid chromatography. The proportions and cumulative incidences of the different hemoglobinopathies were computed. Hardy-Weinberg equilibrium law was applied to calculate genotypic and allelic frequencies. The significant level was p < 0.05. Out of 11,337 newborns included, 47.8% were males and 60.2% were from Bobo-Dioulasso. Abnormal hemoglobin was found in 27.1%, representing a cumulative incidence of 1:4 newborns. The incidence of SCD was 1.9% (1:53 newborns) with 27.9% of homozygous SS. Homozygous CC and compound heterozygous Cβ-Thalassemia accounted for 1.1%.
Journal of Clinical Laboratory Analysis, 2017
Background: In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated around 40 000 neonates per year. However, it is notoriously difficult to perform conventional electrophoresis in all hospitals and laboratories, especially at peripheral levels and rural area. A panel of multiple clinical and laboratory features that would enhance sickle cell disease were assessed for the detection of the disease in highly resource-scarce settings. Methods: A prospective study was conducted in Kinshasa. Venous blood samples were drawn from each study participant in order to determine the hematologic parameters, the peripheral smears, and the hemoglobin electrophoresis. We used Cohen's κ statistic to examine the agreement of each variable and diagnosis of sickle cell disease. Results: A total of 807 patients were screened for sickle cell disease. Among these 807 children, 36 (4.5%