Sickle Cell Disease Patients Research Articles

Exercise and training in sickle cell disease: Safety, potential benefits, and recommendations.

Sickle cell disease (SCD) is a genetic disorder characterized by complex pathophysiological mechanisms leading to vaso-occlusive crisis, chronic pain, chronic hemolytic anemia, and vascular complications, which require considerations for exercise and physical activity. This review aims to elucidate the safety, potential benefits, and recommendations regarding exercise and training in individuals with SCD. SCD patients are characterized by decreased exercise capacity and tolerance. Acute intense exercise may be accompanied by biological changes (acidosis, increased oxidative stress, and dehydration) that could increase the risk of red blood cell sickling and acute clinical complications. However, recent findings suggest that controlled exercise training is safe and well tolerated by SCD patients and could confer benefits in disease management. Regular endurance exercises of submaximal intensity or exercise interventions incorporating resistance training have been shown to improve cardiorespiratory and muscle function in SCD, which may improve quality of life. Recommendations for exercise prescription in SCD should be based on accurate clinical and functional evaluations, taking into account disease phenotype and cardiorespiratory status at rest and in response to exercise. Exercise programs should include gradual progression, incorporating adequate warm-up, cool-down, and hydration strategies. Exercise training represents promising therapeutic strategy in the management of SCD. It is now time to move through the investigation of long-term biological, physiological, and clinical effects of regular physical activity in SCD patients.

Iron metabolism in sickle cell disease patients undergoing chronic red blood cell exchange: A delicate homeostasis in balance.

Sickle cell disease (SCD) is an inherited haemoglobinopathy associated with significant morbidity and mortality. Automated red blood cell exchange (aRCE) plays a key role in managing SCD, eliciting both therapeutic and prophylactic effects. The ideal post-apheresis Ht target for chronic aRCE treatment is not yet unanimously recognized, as well as iron homeostasis can be different among patients. Ross etal. reported their experience on the chronic management of SCD patients undergoing aRCE with a final post-exchange Ht higher than the value commonly adopted, analysing red blood cell transfusion requirements and iron-related outcomes in the study population. Commentary on: Ross etal. Automated red blood cell exchange with a post-procedure haematocrit targeted at 34% in the chronic management of sickle cell disease. Br J Haematol 2024; 205:1556-1564.

Molecular genotyping versus serological diagnosis for RH blood group typing in sickle cell patients

ABSTRACT Objectives High rate of alloimmunization in sickle cell disease (SCD) patients poses a significant challenge in finding compatible blood unit. Accurate determination of the blood group genotype of them can help reduce the alloimmunization risk. Tetra ARMS PCR is a novel method that has been utilized recently to investigate SNPs in diseases in a fast and reliable way. Methods Our study included 104 SCD and sickle thalassemia (Sβ) patients referred to Baghaei-2-Hospital of Ahvaz in 2019 using a nonrandom sampling method. Blood samples were collected for serological and molecular tests. Rh genotyping was performed using Tetra ARMS PCR and compared with the serological results. Results Based on the Tetra ARMS PCR method, out of 104 patients, 7 (6.7%) were d/d, 40 (38.5%) were D/d, 57 (54.8%) were D/D, 25 (24%) were C/C, 59 (56.7%) were C/c, 20 (19.3%) were c/c, 4 (3.8%) were E/E, 25 (24%) were E/e, and patients 75 (72.2%) were e/e. There were discrepancies in the serological and molecular results for 11 patients. Conclusion Use of Tetra ARMS PCR in combination with serological methods for determining the Rh blood group system in donors and transfusion-dependent patients represents a remarkable transformation in the field of immunohematology.

Neurovascular Manifestations of Sickle Cell Disease

Sickle cell disease (SCD) is a hereditary blood disorder characterized by abnormal hemoglobin, leading to the sickle shape of red blood cells. It has several vascular complications and the cerebrovascular ones are among the most frequent and severe both in children and in adults. This review summarizes the main neurovascular manifestations of SCD, including acute stroke, silent cerebral infarction, large-vessel diseases (moyamoya arteriopathy and aneurysms), and brain bleeding. Both epidemiology, pathophysiology, and treatment issues are addressed and prevention of cerebrovascular events, including silent cerebral infarctions, is particularly relevant in SCD patients, being associated to poor functional outcome and cognitive complaints. Transfusions and hydroxyurea are the main available therapy at the moment, but contraindications, availability, and complications might prevent their long term use, particularly in low-income countries. The role of transcranial Doppler in monitoring the patients (mainly children) is analyzed and a practical approach has been selected in order to give the main messages from the current literature for a better management of SCD patients.

Genetic Patterns of Oral Cavity Microbiome in Patients with Sickle Cell Disease.

The Middle Eastern prevalence of sickle cell anemia, a genetic disorder that affects red blood cells, necessitates additional research. On a molecular level, we sought to identify and sort the oral microbiota of healthy individuals and those with sickle cell anemia. Furthermore, it is crucial to comprehend how changes in the genetic makeup of the oral microbiota impact the state of sickle cell anemia. Using next-generation sequencing, the 16S rRNA amplicon was examined using saliva samples from 36 individuals with sickle cell anemia and healthy individuals. These samples were obtained from sickle cell anemia patients (18 samples) and healthy control participants (controls, 18 samples). Various analyses are conducted using bioinformatic techniques to identify distinct species and their relative abundance. Streptococcus, followed by Fusobacterium nucleatum, Prevotella, and Veillonella were the most prevalent genera of bacteria in the saliva of the SCA and non-SCA individuals according to our findings. Rothia mucilaginosa, Prevotella scoposa, and Veillonella dispar species were the dominant species in both sickle cell anemia and non-sickle cell anemia subjects. Streptococcus salivarius, Actinomyces graevenitzii, Actinomyces odontolyticus, and Actinomyces georgiae spp. were the most prevalent bacterial spp. in the studied SCA cases. The sequencing of the 16S rRNA gene yielded relative abundance values that were visualized through a heatmap analysis. Alterations in the oral microflora's constitution can significantly affect the susceptibility of sickle cell anemia patients to develop more severe health complications. Salivary diagnosis is a potential tool for predicting and preventing oral microbiome-related diseases in the future.

Prevalence of metabolic syndrome in sickle cell disease patients: A cross‐sectional study at a tertiary hospital in Nepal

AbstractBackgroundSickle cell disease (SCD) is the most common hemoglobinopathy caused by an autosomal recessive genetic disorder leading to increased morbidity and mortality rates. SCD is prevalent in the Tharu community in the lowland (Terai) region of Nepal. Prevalence of metabolic syndrome among adults with SCD is poorly studied.MethodologyThis prospective cross‐sectional study was conducted at Seti Provincial Hospital in Dhangadhi, Nepal, among 140 adolescents and adults with SCD, aged 15–60 years. Anthropometric and laboratory data were collected using an assisted questionnaire, and the SPSS software version 23 was used for data analysis. Descriptive and inferential statistics were used to summarize the presence of metabolic syndrome and were stratified in separate analyses by age and sex. National Cholesterol Education Program‐Adult Treatment Panel III criteria were used to define metabolic syndrome.AimThis cross‐sectional study aimed to assess the prevalence of metabolic syndrome among SCD patients with SCD registered at the Seti Provincial Hospital in Dhangadhi, Nepal.ResultsThe prevalence of metabolic syndrome in the study participants was 7.8%. Our study revealed 5% of the patients overweight, and 1.4% obese. In this study, the mean triglyceride level was 118.5 mg/dL, and the mean high‐density lipoprotein (HDL) level was 36.2 mg/dL (men) and 36.7 mg/dL (women). This study found that the mean fasting blood glucose level was 88.6 gm/dL. Similarly, 3.5% of patients had increased systolic blood pressure, and 7.8% had raised diastolic blood pressure. Study shows that changes in triglyceride level (p = 0.013), waist circumference, and HDL level (p = 0.0001 and 0.0048, respectively) are significantly associated with smoking or alcohol consumption; however, change in blood pressure (p = 0.013) and fasting blood sugar level (p = 0.086) are not associated with smoking or alcohol consumption.ConclusionStudy concluded that though a lower proportion of SCD patients met the criteria for metabolic syndrome than in studies conducted in developed countries, it is crucial to consider metabolic syndrome while managing patients with SCD. Nevertheless, the authors advocate a more comprehensive study to draw significant conclusions.

Investigation of Delayed Transfusion Reactions in Sickle Cell Disease Patients Polytransfused in the Brazilian Amazon.

Sickle cell disease (SCD) affects approximately 100,000 people in the United States and millions worldwide, with the highest prevalence of 70% of SCD being found in individuals of African ethnicity. Delayed hemolytic, alloimmunization, and anamnestic transfusion reactions in multiple transfusion patients need to be investigated and managed to avoid a worsening of the patient's clinical status. This paper aims to investigate delayed transfusion reactions in SCD patients who were polytransfused in the Brazilian Amazon. The clinical and laboratory indicators of SCD patients with more than four transfusions were investigated. The patients were treated at the Fundação Hospitalar de Hematologia e Hemoterapia do Estado do Amazonas, Brazil. A total of 44 polytransfused patients with SCD were followed. Regarding Rh phenotype, it was possible to observe a frequency of 26.6% (12) patients with the RZRZ (DCE/DCE) phenotype, in addition to 4.5% (two) patients with RH and RHCE variants. It was also possible to observe 20.5% (nine) patients with an alloimmunization reaction, who presented the following alloantibodies: anti-RhD, anti-E, anti-K, anti-Jkb, anti-N, anti-S, and anti-Dia, two of which are unidentified. Of these, four (44.4%) patients also presented autoantibodies, anti-e, and three unidentified antibodies, and four (44.4%) patients presented an anamnestic reaction, with anti-RhD, K, and Jkb antibodies. Of the 44 patients monitored, 54.4% (24) had clinical and laboratory indicators of a delayed hemolytic reaction. Delayed transfusion reactions, often neglected, occur frequently. Therefore, transfusions need to be monitored for at least 28 days, with medical investigation of clinical and laboratory indicators to make greater use of this therapeutic resource.

Prevalence and factors associated with opioid use disorder among adolescents with sickle cell disease in Mulago hospital, Uganda

BackgroundOpioid use disorder (OUD) among adolescents with sickle cell disease (SCD) patients increases their risk of complications from sickle cell disease, such as infections, stroke, acute chest syndrome, sudden death, and organ failure. This negatively impacts families, communities, the national health system, and the economy. This study aimed to determine the prevalence and factors associated with opioid use disorder among adolescents with SCD at Mulago Hospital Uganda.MethodsThis study was carried out at the Sickle Cell Clinic of Mulago Hospital, the national referral hospital in Uganda. The study participants were adolescents aged 10 to 19 years. Following informed consent/ assent, a sociodemographic questionnaire, the WHO Alcohol, Smoking and Substance Involvement Screening Test - Young (ASSIST-Y), the Beck Depression Inventory-II (BDI II), and Generalized Anxiety Disorder − 7 (GAD-7) questionnaires were used to collect data. Data was entered in EpiInfo and analyzed in STATA 15.ResultsThe prevalence of opioid use disorder was 5.3%. The significant risk factor was increasing depressive score AOR: 1.11(95% CI: 1.01–1.22, p = 0.035), while living with a family was protective against opioid use disorders AOR: 0.01; (95% CI: 0.0004, 0.27, p = 0.007).ConclusionThere was a significant problem of OUD among adolescents with SCD. There is, therefore, needed to integrate screening of OUD and mental illnesses like depression among adolescents with SCD and to emphasize the importance of family support in their care.

Revolutionary breakthrough: FDA approves CASGEVY, the first CRISPR/Cas9 gene therapy for sickle cell disease.

Sickle cell disease (SCD) is a hereditary hemoglobinopathy resulting from a β-globin chain mutation that causes abnormal hemoglobin (HbS) polymerization and leads to severe complications. Current treatment options primarily focus on symptom management, with limited curative potential. Recently, Casgevy, the first CRISPR/Cas9-based gene therapy for SCD, has received breakthrough FDA approval. Clinical trials have shown that Casgevy administered to patients aged older than or equal to 12 years enables precise modifications in hematopoietic stem cells, resulting in elevated fetal hemoglobin (HbF) levels and a significant reduction in vaso-occlusive events. Unlike conventional treatments, this therapy offers a curative approach and eliminates the need for recurrent transfusions and transplants, thereby improving the quality of life of patients with SCD. Casgevy has emerged as a beacon of hope for SCD patients and signifies a potential paradigm shift in SCD management due to its safety, curative potential, and transformative impact, positioning it as a groundbreaking intervention. Nevertheless, ethical considerations surrounding CRISPR technology and regulatory frameworks must be addressed to ensure responsible application and equitable access to this one-time gene editing therapy. As the authors celebrate this scientific advancement, sustained interdisciplinary collaboration and ethical scrutiny are essential to navigating the evolving landscape of CRISPR technology in medicine. This review aims to provide a detailed insight into the application of Casgevy, challenges associated with its application, future prospects of this therapy, and its comparison with existing treatment options for SCD.

Development and IND-enabling studies of a novel Cas9 genome-edited autologous CD34+ cell therapy to induce fetal hemoglobin for sickle cell disease

Sickle cell disease (SCD) is a common, severe genetic blood disorder. Current pharmacotherapies are partially effective and allogeneic hematopoietic stem cell transplantation (HSCT) is associated with immune toxicities. Genome editing of patient hematopoietic stem cells (HSCs) to reactivate fetal hemoglobin (HbF) in erythroid progeny offers an alternative potentially curative approach to treat SCD. Although the FDA released guidelines for evaluating genome editing risks, it remains unclear how best to approach pre-clinical assessment of genome-edited cell products. Here we describe rigorous pre-clinical development of a therapeutic γ-globin gene promoter editing strategy that supported an investigational new drug (IND) application cleared by the FDA. We compared γ-globin promoter and BCL11A enhancer targets, identified a potent HbF-inducing lead candidate, and tested our approach in mobilized CD34+ HSPCs from SCD patients. We observed efficient editing, HbF induction to predicted therapeutic levels, and reduced sickling. With single-cell analyses, we defined the heterogeneity of HbF induction and HBG1/HBG2 transcription. With CHANGE-seq for sensitive and unbiased off-target discovery followed by targeted sequencing, we did not detect off-target activity in edited HSPCs. Our study provides a blueprint for translating new ex vivo HSC genome editing strategies towards clinical trials for treating SCD and other blood disorders.

Regulatory Assessment of Casgevy for the Treatment of Transfusion-Dependent β-Thalassemia and Sickle Cell Disease with Recurrent Vaso-Occlusive Crises.

Sickle cell disease (SCD) and transfusion-dependent β-thalassemia (TDT) are hereditary haemoglobinopathies characterized by a reduction in functional β-globin chains. Both conditions cause tiredness and increase susceptibility to infection, which can lead organ failure, significantly reducing life expectancy and typically requiring those affected to undergo regular erythrocyte transfusion. Recently, a novel therapeutic treatment for SCD and TDT was approved by the UK regulatory body (Medicines and Healthcare products Regulatory Agency; MHRA). Exagamglogene autotemcel (Casgevy) is the first licensed therapy globally to utilize CRIPSR/Cas9 technology and induces an increase in expression of γ-globin chains to compensate for the reduction in functional β-globin. Casgevy represents a first-in-class therapeutic, and numerous considerations were made by the MHRA throughout its assessment of the medicine. These include, but are not limited to, the risk of tumorigenicity and off-target editing, a limited cohort size, the validity of proposed dosing and the conduction of only single-arm studies. The MHRA's analyses of the data to support the proposed indications are presented and discussed throughout this manuscript. Overall, the sponsors claims were considered well supported by their data, and Casgevy was licensed for the treatment of TDT or SCD in patients 12 years of age and older for whom hematopoietic stem cell (HSC) transplantation is appropriate, but a human leukocyte antigen-matched related HSC donor is not available.

Gall stones in children with sickle cell disease, management and outcomes

Background: It is rare to encounter a gall bladder stones in the paediatric age group. In Saudi Arabia, sickle cell disease (SCD) is common with incidence reach to 19.7%in some provinces. Cholecystitis is responsible for significant morbidity in SCD patients. Laparoscopic cholecystectomy (LC) is now considered as the procedure of choice for treating such cases. Over a period of 3 years, a total of 21 children with sickle cell anemia (SCA) had cholecystectomy. We exclude 3 patients who had splenectomy and cholecystectomy in the same time, the remaining 18 patients (6 males and 12 females) had LC only. Their age ranged from 4 years to 13 years (mean of 7.45±1.94 years). Methods: LC was the standard procedure performed in all the children in our study group. The mean duration of operation was 92.17±27.31 minutes (range from 62-167 minutes). Results: The mean post-operative stay after operation was 2.167±0.5 days (range from 1-3 days). One patient required conversion to open cholecystectomy (OC) because of severe adhesions, there were no immediate postoperative complications in the other 17 patients of our study group. Conclusions: In conclusion, elective LC in well prepared patients, is feasible and safe in children with SCA and should be the treatment of choice for both symptomatic and asymptomatic cholelithiasis. ERCP is a valuable diagnostic and therapeutic investigation both preoperatively and postoperatively.

The Ugandan sickle Pan-African research consortium registry: design, development, and lessons

BackgroundSub-Saharan Africa bears the highest burden of sickle cell disease (SCD) globally with Nigeria, Democratic Republic of Congo, Tanzania, Uganda being the most affected countries. Uganda reports approximately 20,000 SCD births annually, constituting 6.67% of reported global SCD births. Despite this, there is a paucity of comprehensive data on SCD from the African continent. SCD registries offer a promising avenue for conducting prospective studies, elucidating disease severity patterns, and evaluating the intricate interplay of social, environmental, and genetic factors. This paper describes the establishment of the Sickle Pan Africa Research Consortium (SPARCo) Uganda registry, encompassing its design, development, data collection, and key insights learned, aligning with collaborative efforts in Nigeria, Tanzania, and Ghana SPARCo registries.MethodsThe registry was created using pre-existing case report forms harmonized from the SPARCo data dictionary and ontology to fit Uganda clinical needs. The case report forms were developed with SCD data elements of interest including demographics, consent, baseline, clinical, laboratory and others. That data was then parsed into a customized REDCap database, configured to suit the optimized ontologies and support retrieval aggregations and analyses. Patients were enrolled from one national referral and three regional referral hospitals in Uganda.ResultsA nationwide electronic patient-consented registry for SCD was established from four regional hospitals. A total of 5,655 patients were enrolled from Mulago National Referral Hospital (58%), Jinja Regional Referral (14.4%), Mbale Regional Referral (16.9%), and Lira Regional Referral (10.7%) hospitals between June 2022 and October 2023.ConclusionUganda has been able to develop a SCD registry consistent with data from Tanzania, Nigeria and Ghana. Our findings demonstrate that it’s feasible to develop longitudinal SCD registries in sub-Saharan Africa. These registries will be crucial for facilitating a range of studies, including the analysis of SCD clinical phenotypes and patient outcomes, newborn screening, and evaluation of hydroxyurea use, among others. This initiative underscores the potential for developing comprehensive disease registries in resource-limited settings, fostering collaborative, data-driven research efforts aimed at addressing the multifaceted challenges of SCD in Africa.

Genome editing in K562 cells suggests a functional role for the XmnI Gg polymorphism: a widely used genetic marker in β-thalassemia and sickle cell disease patients.

The XmnI Gg -158 C/T polymorphism has been widely associated with fetal hemoglobin (HbF) levels, the severity of disease, and the response to the drug hydroxyurea (HU) in both β-thalassemia (β-thal) and sickle cell disease (SCD) patients. However, the functional significance of this single nucleotide polymorphism (SNP) remains unclear. To gain insight, green fluorescence protein (GFP) cassettes harboring the XmnI C or T alleles in their left homology arms (i.e. Gg promoters) were knocked into the Gg gene(s) of K562 cells via CRISPR/Cas9. Subsequently, the GFP fluorescence levels were compared in the ensuing cell populations and isolated clones. In both instances, median fluorescence intensities (MFI) of the knockin cells having the inserted XmnI T allele were higher than those having the XmnI C allele. Our results suggest that the XmnI T allele can increase Gg expression in K562 cells. The possible functional significance of the XmnI Gg -158 C/T polymorphism provides a rationale for the aforementioned associations. Furthermore, the XmnI polymorphism as a functional SNP substantiates its importance as a prognostic marker.

Lumbar Fusions in Patients with Sickle Cell Disease: A Propensity-Matched Analysis of Postoperative Complications

ObjectiveThe present study compares postoperative outcomes between patients with and without sickle cell disease (SCD) undergoing one- to three-level lumbar spinal fusion for degenerative pathologies. MethodsPatients who underwent one- to three-level lumbar spinal fusion for degenerative pathologies from 2010–2021 were identified using the PearlDiver database. Patients were separated into 1) SCD and 2) non-SCD groups and were propensity-matched 1:1 for age, sex, Elixhauser Comorbidity Index (ECI), surgical approach, and various comorbidities. Complications were separately analyzed by single- and multi-level procedures using chi-squared and Mann-Whitney U testing. ResultsPropensity-score matching identified 1,934 SCD and non-SCD patients who underwent single-level fusion and 2,094 SCD and non-SCD patients who underwent multi-level fusion. Across single-level fusions, those with SCD had a significantly higher risk of neurovascular compromise (p < 0.001), venous thromboembolism (p = 0.004), pneumonia (p = 0.032), urinary tract infections (UTI) (p = 0.001), and greater postoperative opioid usage out to twelve months (p = 0.018). Across multi-level fusions, SCD carried higher risk for neurovascular compromise (p < 0.001), pneumonia (p = 0.010), and UTI (p < 0.001). All SCD patients had significantly higher opioid use at one month (p = 0.001) and at six months (p = 0.009) postoperatively. ConclusionsPatients with SCD undergoing lumbar spinal fusion demonstrate higher risks for coagulopathic, ischemic, and infectious-related complications, as well as long-term postoperative opioid use. Awareness of the unique complication profile in SCD patients may help guide surgeons in refining perioperative management strategies to optimize outcomes in patients with SCD.

Prevalence, Risk Factors, and Complications of Sickle Cell Disease in Saudi Arabia: A Systematic Review.

This study examined sickle cell disease (SCD) in Saudi Arabia. A systematic search of relevant databases was conducted to identify studies investigating SCD in the Saudi population. Studies were then screened based on predefined criteria and critically appraised for methodological quality. Data was extracted and synthesized to provide an overall picture of the SCD burden in Saudi Arabia. The most commonly reported complications were vaso-occlusive crises (VOC), acute chest syndrome (ACS), acute painful crisis, splenic sequestration, osteomyelitis, aplastic crisis, hemolytic crisis, serious bacterial infections, chronic vascular occlusion (CVO), depression, sickle cell nephropathy (SCN), obstructive sleep apnea (OSA), and renal complications. Reduced blood levels of antioxidant trace elements (Cu, Zn, and Se) may encourage oxidative stress, which in turn may contribute to the pathophysiology of SCD. Infections and ACS were common among young children (<7 years) while pain attacks were common in older children (>7 years). The high rate of hospitalizations among SCD patients highlights the need for better management strategies. Future research should focus on understanding the underlying causes of SCD complications and developing new ways to control them.

Distribution of Malaria Infection Among Different Genotypes in Three Senatorial Zones of Taraba State Nigeria

Malaria infection is recognized as a severe public health problem linked to most cases of morbidity and mortality in malaria endemic areas. The study was used to determine the distribution of malaria infection among the different genotypes in three senatorial zones in Taraba state. The study employed a community and laboratory based cross sectional study. The electrophoresis machine was used to determine the genotypes of the research subjects while Rapid diagnostic technique and the gold standard microscopy was used to determine the prevalence of malaria infection. The haematology auto-analyzer (Sysmex XTI 2000) was used to determine the haematological parameters of the subjects. A total of 3084 blood samples was obtained by venepuncture. The distribution of the genotypes are AA 1721 (55.8%), AS 966 (31.3%) and SS 397 (12.9%). The overall prevalence of malaria was 620 (20.1%). Malaria infection was highest in the southern senatorial zone (29.1%) than in the north and central zones (p&lt;0.05). Infection was high with the males (21.2%), age-related malaria infection was significant (p&lt;0.05) with age 1-10yrs recording the highest infection (28.0%). No significant difference (p&gt;0.00) was recorded in the marital status of the patient with the widows/widower having 23.4% while degree of infection was significant for education-related infection (p&lt;0.05) with the non-educated subjects recording highest infection (34.4%). Occupational related prevalence was significant (p&lt;0.05) with high infection among traders (162 (28.7%). Significant difference was recorded in the marital status with the widow/widower recording a high prevalence of malaria 112 (23.4%). Our finding in the current study confirm that malaria remains a major challenge and there is need for periodic prophylactic administration of malaria drugs in the treatment regime of sickle cell anaemia patients. Keywords: Malaria infection, genotypes, distribution, senatorial zones, Taraba State, Nigeria

Safety and efficacy of L-Glutamine in reducing the frequency of acute complications among patients with sickle cell disease: A randomized controlled study

To evaluate the safety and efficacy of L-glutamine in reducing vaso-occlusive crisis (VOC) and improving cerebral arterial blood flow in children with sickle cell disease (SCD). This is an interventional randomized controlled trial that recruited sixty SCD patients, aged 9.2 ± 3.7 years, who had at least two VOCs during the last 12 months and on a stable dose of hydroxyurea. They were randomly assigned in a 1:1 ratio to receive glutamine (0.3 gm/kg/dose/12h) orally for 24 weeks or the standard of care (SOC). All patients had VOCs in the last year > 3, those on glutamine had a higher number of VOCs and hospitalization for VOC in the last year. There was a decreasing trend in the number, severity, and hospitalization of VOC and a significantly lower cumulative number of VOCs and hospitalizations in the glutamine group than in SOC (p = 0.008, p < 0.001 respectively). Time-averaged mean maximum velocity for the glutamine group had a marginal increase in both middle cerebral arteries, all values remained normal within a normal range, and in both internal carotid arteries, values increased from abnormally low to normal ranges at week 24. Glutamine reduced the number of VOCs and severity and may have a potentially favorable impact on the cerebral arterial flow velocities.

Provider Implicit Racial Bias in Pediatric Sickle Cell Disease.

This study is to (1) assess implicit racial bias among pediatric providers and (2) use virtual patient (VP) vignettes to determine the impact of implicit racial bias on clinical decision-making in pediatric sickle cell disease (SCD) pain care. This cross-sectional study was conducted at a mid-sized, freestanding children's hospital in the northeast. Participants (N = 52) were pediatric SCD providers (87% cisgender female, 90% White, M age = 38.78). Providers completed a demographic questionnaire, the race Implicit Association Test (IAT) with adult and child faces, and a measure of SCD explicit bias (5-point Likert scale). Providers also made clinical decisions for four VP vignettes depicting Black and White youth in the emergency department (ED) with either SCD or cancer pain. Frequency tables were calculated. On the race IAT, providers demonstrated a pro-White implicit bias for both adult (81%) and child (89%) faces. Responses to the explicit bias measure reflected low levels of agreement with negative stereotypes about SCD patients. No significant differences emerged in providers' pain treatment decisions for Black vs. White, or SCD vs. cancer VPs. Findings indicate pediatric providers harbor implicit racial bias similar to the general population. Findings from VP vignettes did not demonstrate that pain treatment decision-making differed based on race or diagnosis. This may be due to standardized protocols and procedures in the pediatric emergency setting. Future research is needed to clarify the role of implicit bias in clinical decision-making and the potential efficacy of treatment protocols in preventing biases from interfering with pediatric SCD pain care.

Haematological Derangements Due to Plasmodium falciparum Among Sickle Cell Anaemia Patients in Taraba State Nigeria

Malaria infection is recognized as a severe public health problem linked to most cases of morbidity and mortality in malaria endemic areas. The study was used to determine the hematological derangement of plasmodium falciparum among sickle cell anaemia patients in Taraba state. The study employed a community and laboratory based cross sectional study. The findings shows that malaria infection was highest in the southern senatorial zone (29.1%) than in the north and central zones (p&lt;0.05). Infection was high with the males (21.2%), age-related malaria infection was significant (p&lt;0.05) with age 1-10yrs recording the highest infection (28.0%). No significant difference (p&gt;0.00) was recorded in the marital status of the patient with the widows/widower having 23.4% while degree of infection was significant for education-related infection (p&lt;0.05) with the non-educated subjects recording highest infection (34...4%). Occupational related prevalence was significant (p&lt;0.05) with high infection among traders (162 (28.7%). Significant difference was recorded in the marital status with the widow/widower recording a high prevalence of malaria 112 (23.4%). Haematological parameters show no significant association in malaria parasitized packed cell volume (t=0.284, p&gt;0.05) and monocytes (t=1.361, p&gt;0.05). Significant association was recorded in total white blood count (t=3.647, p&gt;0.05), neutrophil (t=20.794, p&lt;0.05), erythrocyte sedimentation rate (t=30.017, p&lt;0.05), eosinophil (t=4.847, p&lt;0.0.07), basophil (t=11.35, p&lt;0.05), platelet (t=30.378, p&lt;0.05) and lymphocytes (t=21.369, p&lt;0.03).Our finding for this current study confirm that malaria remains a major challenge and there is need for periodic prophylactic administration of malaria drugs in the treatment regime of sickle cell anaemia patients. Keywords: Malaria infection, hematological derangement, plasmodium falciparum, Taraba State, Nigeria.