Sickling Test Research Articles

Assessing and Evaluating HbS Concentration in Asymptomatic Sickle Cell Patients and Patients With Sickle Cell Crisis Through High-Performance Liquid Chromatography (HPLC) in a Tertiary Hospital in Central India.

Background Sickle cell disease (SCD) is a significant health concern, particularly due to the variability in disease severity and frequency of crisis episodes among patients. Accurate assessment of HbS concentrations is crucial for understanding the disease's progression and severity. This study aimed to assess and evaluate HbS concentrations in sickle cell patients and those experiencing sickle cell crisis using high-performance liquid chromatography (HPLC). The objectives included screening individuals for SCD, diagnosing the disease using Hb electrophoresis, estimating HbS concentration via HPLC, and comparing HbS concentration values between sickle cell patients and those in crisis. Methods An analytical study design was employed at Jawaharlal Nehru Medical College, Sawangi, Wardha, Maharashtra, involving 80 participants diagnosed with SCD. Data collection included clinical assessments, routine sickling tests, Hb electrophoresis, and HPLC for HbS concentration measurement. Descriptive and inferential statistics were utilized for data analysis, including chi-square tests, Mann-Whitney U tests, and regression analyses. Results Significant differences in HbS concentrations were observed between different patient groups. Individuals with the SS pattern exhibited higher HbS levels than those with the AS pattern (p = 0.001). Non-crisis patients had significantly higher mean HbS concentrations than crisis patients (p = 0.001). A moderate positive correlation (0.476, p = 0.001) was found between HbS concentrations and clinical outcomes. No significant differences in HbS concentrations were noted based on sex or age group. Longitudinal analysis revealed a significant increase in HbS levels over time (p = 0.001). Conclusion The study underscores the importance of HbS concentration measurement in understanding the severity and progression of SCD. HPLC proves to be a valuable tool in accurately estimating HbS levels, aiding in better clinical management of the disease.

Sickle Cell Screening in Adults: A Current Review of Point-of-Care Testing.

In adults, the sickle cell solubility test (SCST) is the most common screening test to determine the presence of hemoglobin S (HbS) within a blood sample. The assay is inexpensive, rapid, highly sensitive and specific. However, the SCST cannot accurately quantify the level of HbS in a test sample and requires confirmatory testing to distinguish between sickle trait and sickle cell disease. Despite these limitations, it remains the standard screening tool for HbS in a variety of settings such as screening in the US military or by the National Collegiate Athletic Association. With an increased awareness of the importance of screening for sickle cell in adults, we herein describe the current sensitivity, specificity, positive predictive value, and negative predictive value of this test. We also review overall clinical utility of this laboratory measure and briefly discuss new point-of-care techniques designed to overcome the SCST's shortcomings.

Elevated foetal haemoglobin in tribal sickle cell anaemic patients: Blessing in disgust?

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. To assess the role of naturally compensated haemoglobin variants in tribal Sickle cell anaemic patients of North East Gujarat. 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. 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 < 0.05 considered statistically significant. 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. 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.

Prevalence of sickle cell trait and needs assessment for uptake of sickle cell screening among secondary school students in Kampala City, Uganda.

Sickle cell disease (SCD) is one of the most frequent and traumatizing genetic disease in Uganda, with the prevalence of the sickle cell trait (SCT) estimated at 13.3% leading to serious psycho-social and economic impact on the patients and their families. This study aimed to determine the burden of SCT and factors influencing the uptake of screening services among secondary school students in Uganda. We used an analytical cross-sectional design with a multi-stage sampling approach. A total of 399 students from four secondary schools in Kampala City were enrolled in this study. Data were gathered using semi-structured questionnaires and blood screening. We used the sickling test to determine the presence of sickle cell alleles among the participants and hemoglobin electrophoresis as a confirmatory test. Data gathered using the questionnaire were analyzed using descriptive and inferential statistics. In total, 5.8% of participants who were tested during this study had SCT. Most (80.2%) participants were not in an intimate relationship at the time of data collection. The majority (60.4%) had moderate knowledge about SCT screening and obtained information about screening from the school. Only 29 (7.3%) participants knew of a family member with sickle cell. Overall, participants had a negative attitude toward SCT screening (67%), although 41.6% believed that most people who were sickle cell carriers did not live long and were often sick. Statistically significant associations were found between testing for SCT and knowing a partner's sickle cell status (odds ratio [OR] 2.112, p = 0.043) and Anglican religion (OR 2.075, p = 0.047). Despite the moderate level of knowledge and negative attitudes, a relatively large number of participants had SCT. This highlights the need for a comprehensive health education package targeting adolescents to promote SCD/SCT screening.

Comparative study of sickling test, solubility test, and hemoglobin electrophoresis in sickle cell anemia

Abstract Background: Madhya Pradesh, a state in India, bears the highest number of sickle cell anemia (SCA) cases, particularly among its tribal communities. The diagnosis of suspected SCA cases involves utilizing several methods. The sickling test operates on the principle of hemoglobin S (HbS) polymerization upon deoxygenation. By contrast, the solubility test involves the reduction of HbS using sodium hydrosulfite, followed by precipitation of lysed red blood cells. Hemoglobin (Hb) electrophoresis identifies Hb variants based on their migration in an electric field. Our objective was to assess the efficacy of sickling and solubility tests as screening tools for detecting SCA, comparing them against capillary Hb electrophoresis, which is considered the gold standard. Materials and Methods: All individuals suspected of having SCA between 2022 and 2023 were enrolled in the study. Clinical information, peripheral blood smear analysis, sickling test, solubility test, and capillary Hb electrophoresis were conducted, and the results were compared. Results: Of 250 cases assessed, Hb electrophoresis identified 225 cases of SCA, resulting in a sickle cell disease to sickle cell trait (SCT) ratio of 1:1.36 and a male-to-female ratio of 1:1.33. The highest number of cases were observed in the age group from 21 to 30 years. Generalized weakness (77%) and abdominal pain (52%) were the most frequently reported symptoms. The sickling test demonstrated a sensitivity, specificity, and diagnostic accuracy of 91.5%, 72%, and 89%, respectively, compared with Hb electrophoresis. The solubility test yielded values of 76.5%, 68%, and 75% for sensitivity, specificity, and diagnostic accuracy, while peripheral smear analysis showed 36%, 25%, and 42%, respectively. Conclusion: The sickling test displayed superior sensitivity and diagnostic accuracy compared with the solubility test, with no significant disparity in specificity between these two methods. Notably, the solubility test exhibited a higher incidence of false-negative results for SCT. Therefore, the sickling test is the preferred screening method for SCA.

PREVALENCE OF SICKLE CELL ANEMIA IN YOUTH BY COST EFFECTIVE STRATEGY ALONG WITH HPLC

This study aimed to determine the prevalence of sickle cell disease among students of Anand People’s Medicare Society (APMS) using cost-effective screening methods and confirming the results with high-performance liquid chromatography (HPLC). A total of 395 students from Anand, Gujarat, were screened using the sickling test and solubility test as primary screening methods. Haemoglobinopathies carriers were diagnosed and confirmed using HPLC at the Indian Red Cross Society, State Branch in Ahmedabad. Complete hemogram analysis was performed, and the results were tabulated and analyzed. The results indicated that HPLC exhibited 100% sensitivity, specificity, and accuracy, while the sickling test demonstrated a sensitivity of 66.66%, specificity of 97.90%, and accuracy of 97.21%. The solubility test showed a sensitivity of 53.84%, specificity of 96.85%, and accuracy of 95.44%. HPLC had a 100% positive predictive value (PPV) and negative predictive value (NPV), whereas the sickling test had a PPV of 55.55% and NPV of 99.20%. The solubility test yielded a PPV of 36.84% and NPV of 98.40%. In conclusion, the sickling test and solubility test are cost-effective and easily conducted, with the sickling test providing more reliable results with higher sensitivity and specificity compared to the solubility test. HPLC is considered the gold standard test for screening.

The Correlation of Leg Muscle Power with the Frequency of Sickle Kicks of Pencak Silat Athletes

There are many factors that affect Pencak Silat skills, namely the elements of physical condition and kicks. Based on the background in the introduction, it was found that the frequency of sickle kicks is related to the power of the leg muscles. Purpose. Thus, the purpose of this study is to find out how much there is a correlation between leg muscle power and the frequency of sickle kicks in pencak silat athletes. Material and methods. This type of research is correlational research using a quantitative approach. The population in this study is all pencak silat athletes in the Muna Regency Pencak Sor La Ode Pandu Organization. There were 30 male athletes who were taken as samples through purposive sampling techniques. There are several instruments used to collect data: the vertical jump test to measure leg muscle power and the sickle kick test by counting the number of left and right foot kicks for 30 seconds each to measure the frequency of sickle kicks. Results. The results showed that there is a significant correlation between leg muscle power and sickle kick frequency; this can be seen from the acquisition of sig values. 0.019 < 0.05, and the calculated r value = 0.425 > r table ( 0.05 = 0.361), while the determinant coefficient (R2) = 0.18, which means that the power of the limb muscles contributes to the frequency of sickle kicks by 18%. While 82% is influenced by other elements of physical conditions. Conclusions. Based on the research that has been done, it can be concluded that there is a significant correlation between leg muscle power and the frequency of pencak silat sickle kicks. The sickle kick is a kick that is often used in matches. A good sickle kick certainly requires elements of good physical condition as well. Leg muscle power is one of the elements of physical condition that most determine the outcome of a sickle kick. Good leg muscle power requires a well-defined form of exercise. Exercises such as polymetrics and ballistics can increase leg muscle power. The authors emphasize the importance of further research to expand what they did in this study, including expanding the sample size, other influencing variables, and the role of teachers and trainers in Pencak Silat learning. This needs to be done in depth to validate the results of this study.

How Sensitive Is the Sickle Prep? Clinical Implications

Introduction The sickle cell solubility test, commonly known as “sickle prep” is a qualitative measure that relies on the property that deoxygenated hemoglobin S (HbS) is insoluble. Accordingly, blood samples drawn from individuals with circulating HbS appear cloudy when mixed with sodium hydrosulfite. 1 The sickle cell solubility test has been reported to be very sensitive (99%) and specific (99.9%) for detection of HbS. However, false negatives have been reported to occur in sickle cell disease (SCD) patients with severe anemia or high levels of hemoglobin F. 2,3 In those situations HbS levels may be very low and it remains unclear what level of HbS is required for the solubility test to be positive. To address this issue, we have examined the sickle cell solubility test in a cohort of SCD patients undergoing red cell exchange, acknowledging that in many of these patients, HbS levels fall below 15% when tested immediately after completing the exchange procedure. Methods 118 patients with sickle cell anemia were screened based on outpatient visits to the Inova Adult Sickle Center from January 1, 2023 - March 31 st, 2023. Of these, 6 patients were excluded from analysis due to lack of recent sickle cell solubility test during this period. Of the total number of patients included in the analysis (n=112), 25 (22%) underwent red cell exchange during this period. For each individual, the two most recent HbS percentages with the concurrently-determined sickle cell solubility result (positive or negative) were analyzed. We examined the data in the context of known genotype (HbSS, HbSC, HbSβ 0, HbSβ +positive). In patients undergoing red cell exchange, the most recent pre and post transfusion hemoglobin S percentage were collected. Results: 200 distinct HbS levels were collected and analyzed. Of those, 5/200 tested negative for the sickle cell solubility test, yielding an overall sensitivity rate of 98%. Of those 5 patients, 4/5 had received a red cell exchange during this period, and (1/5) had severe transfusion dependent anemia. Of those with a negative sickle cell solubility test, 4/5 patients had an SS genotype, and 1/5 had SC genotype. In patients who received red cell exchange, the sensitivity rate for the sickle cell solubility test is 84%. For all patient samples with HbS >15%, the sickle solubility test was 100% sensitive. However, among the 11 samples with HbS levels below 15%, 5 were sickle prep negative thereby indicating a reduced sensitivity (55%) below that threshold. Nine (9) out of the 11 samples were of patients who had undergone red cell exchange. Conclusion: The sickle cell solubility is a very sensitive measure to establish the presence of HbS, but must be interpreted with caution in those with HbS concentrations of <15%. Because it is readily available (typically resulted within an hour) it may be of benefit in emergency departments for patients for whom there is question about the validity of the sickle cell diagnosis. At our institution, this has been a valuable addition to the initial management of patients for whom medical records are not available and who present with apparent acute vaso-occlusive crisis. We do not propose withholding analgesia for patients with a reported history of sickle cell disease pending this test, but rather recommend its use as a confirmatory test for patients presenting with a reported history of this disease.

Low-Cost Automated Microscopy and Morphology-Based Machine Learning Classification of Sickle Cell Disease and Beta-Thalassemia in Nepal and Canada

INTRODUCTION Sickle cell disease (SCD) is the most common inherited blood disorder, and is associated with a high mortality rate for children in low-resource settings, where the disease burden is high. Early screening and treatment options reduce morbidity and mortality. In addition to screening for SCD, screening for asymptomatic, heterozygous conditions, such as sickle cell trait (SCT) and β-thalassemia trait, is required for effective SCD and thalassemia screening programs. However, rural and remote communities lack accurate low-cost screening techniques. The goal of this study is to augment a common low-cost screening test, the sickling test, using automated microscopy and machine learning to classify individuals with SCD (HbS/β-thalassemia and HbSS), trait conditions (HbAS and HbA/β-thalassemia), and those without common beta-globin disorders (HbAA) for implementation in Nepal. As HbC mutation is not encountered in Nepal, screening for HbSC or HbAC was not included. Additionally, we aim to evaluate the feasibility of 5 low-cost techniques to supplement/replace the local gold standard test (Hb HPLC), which is inaccessible in rural/remote settings. METHODS In this study (ClinicalTrials.gov Identifier: NCT05506358), blood samples were collected and tested at respective clinical sites in Nepal (Mount Sagarmatha Polyclinic and Diagnostic Center, Nepalgunj) and Canada (St. Paul's Hospital and BC Children's Hospital, Vancouver). Participants (138 total: 111 Nepal; 27 Canada) between ages 2 to 74 whose diagnoses were previously established by Hb HPLC were recruited. Five low-cost tests (our augmented sickling test, HbS solubility test, HemoTypeSC, Sickle SCAN, Gazelle Hb variant test) and Hb HPLC were performed on all participants (30 HbAA; 23 HbA/β-thalassemia; 45 HbAS; 11 HbS/β-thalassemia; 29 HbSS). Informed consent was provided by the participants or parents, according to protocols approved by institutional/national research ethics boards. For the sickling test, a sealed wet preparation of blood mixed with 2% sodium metabisulphite was imaged with a robotic microscope (Octopi) with automated scanning/focusing. The images of blood cells were segmented using Cellpose 2.0, allowing for morphological characterization of cells and morphology based classification, with an 80:20 participant-wise split of training and testing data. RESULTS Using high-throughput imaging, more than 300,000 de-identified images of blood films were collected, resulting in more than 1.5 trillion segmented cells. The de-identified images and processed data will be released in an open-access data repository. The frequency distribution of 11 different morphological parameters were used for machine learning based classification, resulting in testing sensitivity and specificity of 82.9% and 82.4%, respectively. The confusion matrix (Figure 1) shows that sensitivity of detecting SCD cases (HbS/β-thalassemia and HbSS) was 98.6%, and most misclassifications were between trait and normal conditions. Comparing the different low-cost techniques (Table 1), the Gazelle Hb variant test had the highest sensitivity and specificity (97.0% and 99.3%), but is more than twice as expensive than the augmented sickling test, while lateral flow assays (HemoTypeSC and SickleSCAN) had lower sensitivity (74-75%) than the augmented sickling test, due to their inability to detect β-thalassemia trait. CONCLUSIONS The sickling test, which is traditionally unable to distinguish between SCT and SCD, was augmented to detect SCD and trait conditions (including β-thalassemia) with an overall sensitivity of 82.4% (98.6% sensitivity for SCD detection). The preliminary results indicate that image-based classification can be a promising low-cost and automated screening tool for low-resource settings, reducing the need for highly-trained personnel for device operation and disease detection. Furthermore, the open-access image dataset can be used to improve classification in the future, as segmentation/classification algorithms improve over time.

Evaluating the reliability of the sickling test and peripheral blood film method for screening of sickle cell disease: a study protocol

Background: Mutations in beta-globin are the cause of a widespread condition known as sickle cell disease (SCD). Sickle cells cause organ damage, hemolysis of blood cells, weakness, and sometimes even death. SCD can be effectively managed and mortality can be reduced through early detection. This protocol’s study aims to evaluate the sensitivity and specificity of the sickling test, peripheral blood smear, and hemoglobin electrophoresis for SCD screening. Methods: In this study,75 cases of SCD will be selected. 5ml blood will be drawn into a dipotassium ethylenediaminetetraacetic acid tube for testing of SCD. Testing will involve: the sickling test, where an equal volume of K2EDTA blood will be mixed with 2% sodium metabisulfite; the peripheral blood smear, where Leishman’s stain will be placed on a thin film of blood for 2 minutes; and Hb electrophoresis, where equal parts of distilled water and packed cells will be combined and then the mixture will be centrifuged. Results: We will be comparing between the sickling test and the peripheral blood film method, and we will conclude depending upon the results as to which test is better. Conclusions: Using peripheral blood smear images, SCD can be quickly diagnosed. A peripheral smear-based differential diagnosis may be possible but requires special tests such as hemoglobin electrophoresis to confirm the diagnosis. Therefore, early diagnosis can help initiate transfusion therapy and create a better prognosis.

Screening of subclinical functional hemoglobin and red blood cell abnormalities among blood donors of Fayoum University Hospital in Egypt: Are RET-He, and IRF useful screening tools?

BackgroundThe effectiveness of red cell transfusion in a given blood unit that relied on both quantity and quality of donated cells undoubtedly affects prognostic outcomes. ObjectiveWe aimed to determine the frequency of subclinical functional hemoglobin and red cell abnormalities in donated blood of Fayoum University Hospital in Egypt. Additionally, to assess the usefulness of reticulocyte mean hemoglobin content (RET-He) and immature reticulocyte fraction (IRF) as screening measures for such abnormalities. Material and methodsThis cross-sectional study enrolled 200 volunteer blood donors who met the national standard criterion of blood donation. Complete blood count with reticulocyte parameters, serum ferritin, sickling test, G6PD assay, Mentzer index, and naked-eye single tube red cell osmotic fragility test were carried out. ResultsFunctional red cell abnormalities represented 44 % of this cohort. Out of them, 4.5 % had iron deficiency, 11 % had a positive sickling test, 19 % had G6PD deficiency, and 9.5 % had suspicious thalassemia. The sensitivity and specificity test for RET-He in selective identification of functional hemoglobin abnormalities in donated blood were 83.3 % and 61.2 %, respectively at a cutoff value of 26.9. Though there was no statistically significant effect of RET-He on the selective detection of G6PD deficiency, IRF had a statistically significant high level with a p-value of 0.04. ConclusionSubclinical functional red cell abnormalities seem to be prevalent among blood donors. Reticulocyte/ erythrocyte indices could be useful screening tools for red cell abnormalities. Further studies are required for assessing the impact of transfusing such abnormalities to neonates and other critical recipients.

Optimal feature selection and detection of sickle cell anemia detection using enhanced whale optimization with clustering based boosted C5.0 algorithm for tribes of Nilgiris

Degradation of red blood cells (RBC) causes many diseases, like sickle cell anemia. Diagnosing this disease takes more time because peripheral blood samples must be examined under the microscope. Since the isolated RBC observation is subjective and high error rate leads to reduce the accuracy, the technology is needed to perform this approach. To fulfil these requirements, optimal feature selection and detection of sickle cell anemia using enhanced whale optimization with clustering based boosted C5.0 algorithm in tribes of Nilgiris is proposed in this manuscript. The input dataset is taken from real data set via non-government organization named NAWA (situated in Kotagiri). The reason behind of Nilgiri region is considered in this work is a collection of sickle cell anemia test results of the tribe people who lives in different areas of Nilgiri region. These images are pre-processed using wavelet packet transform cochlear filter bank method to eradicate the noises and to improve the superiority of image. After that, the features are extracted using force-invariant improved feature extraction method. To select the optimal features, enhanced whale optimization (EWO) algorithm is used. These optimal features are classified utilizing clustering based boosted C5.0 algorithm. The proposed method is activated on PYTHON. The proposed method shows 52.32%, 43.78%, 32.78% and 45.90% higher accuracy compared with the existing methods, such as RGSA-MLP-SCA, CRFA-SVM-SCA, AO-LSTM-SCA and BOA-CNN-SCA.

Screening of antenatal patients for anaemia and haemoglobinopathies

Introduction: Anaemia is an extremely common condition in pregnancy worldwide, conferring a number of health risks to mother and child. Iron deficiency is most widespread micronutritional deficiency in the world and disproportionately affects female because of increased iron requirements during menstruation, pregnancy and lactation. Haemoglobinopathies are a group of inherited disorders because of abnormalities in haemoglobin synthesis or structure. Thalassemia and Sickle cell anaemias are the most prevalent haemoglobinopathies and a national health burden in India so identifying these disorders during antenatal period is necessary to take appropriate measures. Methods: A prospective study of screening for haemoglobin variants in Antenatal Patients due to low haemoglobin and evaluation of other causes was performed for 1 year with 570 samples. In Low Hb patients’ CBC, Reticulocyte staining and sickling test was performed. Haemoglobin analysis was done by HPLC BIO RAD VARIANT II. In Iron Deficiency Anaemia (IDA) Serum ferritin & Serum Iron level were done and in Megaloblastic Anaemia(MA) Vitamin B12 level were done. Result: Prevalence of anaemia in antenatal patients was 90.25%; in this IDA presented with 84.21%, MA with 4.73% and Dimorphic anaemia 1.27%. Prevalence of haemoglobinopathies in this study was 9.75%; in this Beta Thalassemia Minor present with 5.08%, Sickle Cell Trait 4.03%, Hb D Punjab 0.52% and Hb Q india 0.17%. Conclusion: Antenatal screening for genetic disorders, such as Beta thalassemia, Sickle Cell Anaemia, aims to reduce the burden of these diseases by offering information to individual with a high likelihood of giving birth to affected babies and giving parents more choices regarding their reproductive decisions. For this, premarital and antenatal screening should be mandatory to prevent the birth of affected offspring.

Hemoglobin Shimonoseki HBA1:c.164A > G Illustrating the Continuing Utility of the Sickling Test and the Challenges in Antenatal Genetic Counselling.

Hemoglobin Shimonoseki HBA1:c.164A > G Illustrating the Continuing Utility of the Sickling Test and the Challenges in Antenatal Genetic Counselling.

Significant haemoglobinopathies: A guideline for screening and diagnosis: A British Society for Haematology Guideline: A British Society for Haematology Guideline.

Antenatal screening/testing of pregnant women should be carried out according to the guidelines of the National Health Service (NHS) Sickle Cell and Thalassaemia Screening Programme. Newborn screening and, when necessary, follow-up testing and referral, should be carried out according to the guidelines of the NHS Sickle Cell and Thalassaemia Screening Programme. All babies under 1 year of age arriving in the United Kingdom should be offered screening for sickle cell disease (SCD). Preoperative screening for SCD should be carried out in patients from ethnic groups in which there is a significant prevalence of the condition. Emergency screening with a sickle solubility test must always be followed by definitive analysis. Laboratories performing antenatal screening should utilise methods that are capable of detecting significant variants and are capable of quantitating haemoglobins A2 and F at the cut-off points required by the national antenatal screening programme. The laboratory must ensure a provisional report is available for antenatal patients within three working days from sample receipt.

Elastic Power Bands Affect the Improvement of PencakSilat Athlete Sickle Kicks

This study aims to determine the effect of Elastic Power Bands training on increasing the sickle kick in the Pencak Silat Athletes of Porprov Kab. Luwu. This research method is quantitative research in the form of an experimental One-Group Pre-test Post-test Design carried out by giving X treatment to the subject. The population in this study was the PencakSilat Athletes of the Province of Luwu Regency, totaling 19 people. The sampling technique was used when all population members were sampled, namely the saturated sample technique. The data collection technique used a sickle kick ability test through pair training. The results of this study indicate that the Elastic Power Bands exercise positively affects the ability to perform crescent kicks in the Porprov Pencak Silat Athletes of Luwu Regency. The test results of the athlete's sickle kick using the Elastic Power Bands exercise, in the pre-test, the average value obtained was 66.4667, and in the post-test, the average value obtained was 80.2667. From the two tests performed, it can be seen that the average post-test has a higher score than the average pre-test, which means that the Elastic Power Bandster exercise has an effect on increasing crescent kick ability. So there is an average difference between the results of the pre-test and post-test, which means that there is a significant effect of Elastic Power Bands training on the pencaksilat sickle kick.

Introduction of a sickling test EQA program

The RCPAQAP introduced a Sickling Test External Quality Assurance (EQA) Program in 2022. The program is designed for screening and confirmatory methods for Haemoglobin S, and is suitable for solubility, reduced oxygen and ICT/kit-based methods. We sought to review the returned results from the first two surveys to determine any potential method differences.

Challenges of bone marrow transplant for sickle cell disease in resource limited setting

Sickle cell disorders (SCD) are a group of inherited disorders that results from both parent being carriers, haemoglobin AS resulting in haemoglobin (SS), causing anemia, infections, pain, stroke, priapism, sequenstration crisis, multiple organ dysfunctions etc. There are several ways of managing sickle cell disorder but the best way, which is considered a gold standard cure for the disease is a successful bone marrow transplant of haematopoietic stem cells (HSCT). Bone marrow (BM), Peripheral blood (PBSC) and Umbilical cord blood (UCB) are rich in stem cells. In order to have a good bone marrow transplant, without graft rejection, the laboratory plays a vital role especially in compatibility testing of donor and recipients at the various stages of the transplant, which includes initial stage, during the transplant and follow up testing to ensure tolerance to the new graft and testing for chimerism. There are various types of transplant which include Autologous, Allogeneic (Sibling/ Unrelated Donor), Parent or relative, etc. The various pre transplant tests includes: Sickling test, High Performance Liquid Chromatograph (HPLC), Genetic studies, RBC Indices Hb – 6-9 gm/dL, Peripheral Smear, Retic count Reticulocytosis, ESR, Bone marrow analysis. Post-transplant test Includes Engraftment Analysis, Complete Blood counts, kidney function, liver function, Cholesterol, HIV, Hepatitis B, Hepatitis C, additional infectious studies (Endemic testing), Urinalysis etc. Haemopoietic transplantation challenges in a resource limited settings like Nigeria are enormous and they includes: Danger of serious illness associated with donor-to-patient stem cell transplant, lack of appropriate donors, Graft-versus-host disease (GVHD), Inadequate Human resource, Inadequate equipped facility, cost of the service, Corruption, Lack of political will, Leadership incompétence, Lack of strategic planing, Policy inconsistency, Lack of qualified personnel, Poor healthcare administration, Conflict of interest among health workers. The cost of Bone Marrow Transplantation is highly exorbitant to Nigerians and government need to play vital role towards funding part of the cost and alleviating the pain of sickle cell. Effective implementation of National Health Insurance Scheme and address corruption are the most efficient ways the cost would be subsidized. Appropriate information campaigns largely championed by maternal and child health-care professionals would significantly contribute to raising the level of awareness and acceptance of Cord Blood donation. Training and re training of medical personnel cannot be over emphasized.

Antenatal screenings and maternal diagnosis among pregnant women in Sao Tome & Principe-Missed opportunities to improve neonatal health: A hospital-based study.

Newborn mortality and adverse birth outcomes (ABOs) in Sao Tome & Príncipe (STP) are overwhelmingly high, and access to quality-antenatal care (ANC) is one of the strategies to tackle it. This study aimed to fill the gaps in ANC screenings with a focus on how to improve neonatal outcomes. We conducted a retrospective hospital-based study in which ANC pregnancy cards were reviewed. Screenings were described and compared according to the total number of ANC contacts: 1-3 (inadequate), 4-7 (adequate), and ≥8 (complete). The collected data were entered into QuickTapSurvey and exported to SPSS version 25 for analysis. Statistical significance was considered at a p-value ≤0.05. A total of 511 ANC pregnancy cards were reviewed. Mothers' mean age was 26.6 (SD = 7.1), 51.7% had a first trimester early booking, 14.9% (76) had 1-3 ANC contacts, 46.4% (237) had 4-7 and 38.7% (198) ≥8. Screening absence was found in 24%-41%, lack of money was registered in 36%. Pregnant women had no screening performed for HIV in 4.5%, syphilis in 8.8%, HBV 39.3%, malaria 25.8%, hemoglobin 24.5%, blood glucose 45.4%, urine 29.7%, stool exams 27.8% and 41.1% had no ultrasound. Screening completion for blood group, HIV, malaria, urine, hemoglobin, and coproparasitological exam were found to have a statistically significant difference (p<0.001) for the complete ANC group when compared to other groups. Antenatal problems identified were: 1) bacteriuria (43.2%); 2) maternal anemia (37%); 3) intestinal parasitic infections (59.2%); 4) sickle cell solubility test positive (13%); and 5) a RhD-negative phenotype (5.8%). Missed-ANC treatments were up to 50%. This study reveals a coverage-quality gap in STP since no pregnant woman is left without ANC contact, although most still miss evidence-based screenings with an impact on neonatal outcomes. Strategies such as implementing a total free ANC screening package in STP would enhance maternal diagnosis and prompt treatments.

Clinico-Hematological Profile in Hemoglobin SE Disease: A Case Series from a Tertiary Care Centre.

Hemoglobin SE (HbSE) disease are rare globally and there is paucity of literature regarding this condition. Cases reported in the Indian scenario so far have been limited to the tribal populations. This case series aims to highlight the rarity of this double heterozygous state and to raise awareness of its community prevalence beyond the tribal population. This is a case series over a 5-year observation period with six cases of double heterozygosity for HbS and HbE in our tertiary care centre. Four cases were in the 8-15years' age group and 2 cases of 24-25years' age group reported for initial evaluation due to easy fatigability and weakness: Two cases were siblings with history of consanguineous marriages in the family. Mild Pallor, variable icterus, spleen was just palpable in three of the cases and low MCV recorded in all cases. Sickling tests were positive and high performance liquid chromatography (HPLC) revealed both HbS > 50% and HbE fractions ≥ 25%. It's important to detect this rare condition, prevalent in consanguineous marriages as dreaded complications like sickling crisis may manifest during pregnancy and air travel. Detection and genetic counselling is important for prognosis, planning follow up and therapy for this uncommon double heterozygous state.