Sickle cell retinopathy and systemic disease

Sickle cell disease(SCD)is a widespread hemoglobinopathy that results in significant patient morbidity and mortality.Vascular occlusion can cause acute pain,acute chest syndrome,and avascular necrosis,while hemolysis and endothelial disruption can cause ischemic stroke,leg ulcers,pulmonary hypertension,and priapism.All ocular and orbital structures can be affected by SCD ischemic events,including orbital bone infarction,ischemic optic neuropathy,retinal artery occlusion,hyphema,secondary glaucoma,sickle cell maculopathy,and sickle cell retinopathy.Proliferative sickle cell retinopathy(PSR)is the most common cause of vision loss.Untreated PSR can lead to macular ischemia,vitreous hemorrhage,and tractional retinal detachment.Ophthalmic screening exams and multimodal imaging can lead to earlier detection of sickle cell retinopathy and improved patient outcomes.SCD patients undergoing vitreoretinal surgery may require coordination of care with hematologists to avoid ischemic complications.While hydroxyurea was the only United States Food and Drug Administration approved treatment for several decades,patients with SCD now have several more treatment options.Despite the United States screening all infants for SCD,there can be delays in diagnosis and treatment.This review article aims to provide an overview of sickle disease for the ophthalmologist,and to discuss emerging treatment options and current management of SCD ocular complications.

Review on Hydroxyurea Usage in Young Children with Sickle Cell Disease: Examining Hemoglobin Induction, Potential Benefits, Responses, Safety, and Effectiveness

Sickle cell disease (SCD) is a prevalent condition, particularly in the countries of sub-Saharan Africa, where the presence of specific genes associated with Malaria contributes to its high prevalence. Patients with sickle cell disease frequently experience painful episodes necessitating hospitalization, and their hemoglobin levels are typically lower than those of the general population. There are different treatment options available to manage complications, such as transfusing blood, hydroxyurea, and strong anti-pains. However, with all these treatments, patients still commonly experience pain crises and suffer from organ damage. Hydroxyurea, the sole approved medication for sickle cell anemia in developed and developing countries, is widely used in children despite being primarily indicated for adults. Multiple studies have demonstrated the efficacy of hydroxyurea in inducing HbF production in young children with SCD. Elevated HbF levels have been associated with improved clinical outcomes, including a reduction in vaso-occlusive crises, acute chest syndrome, and the need for blood transfusions. Furthermore, increased HbF levels have been shown to ameliorate disease-related organ damage, such as pulmonary hypertension and sickle cell retinopathy. The response to hydroxyurea treatment in young children with SCD is variable. Some patients achieve substantial increases in HbF levels and experience significant clinical benefits, while others may have a more modest response. Factors influencing the response include baseline HbF levels, genetic modifiers, treatment adherence, and dose optimization. Safety is a crucial consideration when using hydroxyurea in young children. Studies have shown that hydroxyurea is generally well-tolerated, with the most common adverse effects being myelosuppression, gastrointestinal symptoms, and dermatological manifestations. However,long-term effects and potential risks, such as renal dysfunction and reproductive impacts, require further investigation. The effectiveness of hydroxyurea in young children with SCD has been demonstrated in various clinical trials and observational studies. These studies have shown a significant reduction in disease-related complications and improved quality of life. However, optimal dosing, treatment duration, and long-term outcomes are still areas of ongoing research. This review focuses on recent studies investigating the benefits, effectiveness, responses, and safety of hydroxyurea in pediatric individuals diagnosed with sickle cell disease.

Influence of Hemoglobin S Haplotypes on the Responses to Hydroxyurea Treatment in Children with Sickle Cell Disease in Abidjan, Côte d’Ivoire

Background: In Côte d’Ivoire so far, the circulating haplotypes have been inferred on the phenotypic profiling of SCD patients. The impact of the circulating haplotypes on the use of Hydroxyurea has not been assessed yet. Therefore the objective of this study is to identify in Abidjan the HbS haplotypes that modulate HU treatment responses. Methods: In a cross-sectional descriptive and analytical study, children aged 5 to 15 years with SCD, and carrying the hemoglobin phenotypes SSFA2 and SFA2, were recruited into a HU treatment cohort. Various parameters on the haplotypes and the outcomes of the treatment were analyzed. Results: Thirty nine children with SCD were included. The phenotypic profile of the cohort was 86.6% of SSFA2 and 15.4% of SFA2. Three haplotypes were found, the Benin haplotype, the Senegal haplotype, and an atypical one. The participants belonged to three genotypes, Benin/atypical (64.1%), Benin/Senegal (33.3%) and Senegal/Senegal (2.6%). Overall, HU treatment was successful in all haplotypes with 12 out of 39 patients failing treatment after 12 months in the Benin haplotype group. The association between HU treatment success and the Benin haplotype was found in terms of the decrease in the number of white blood cells and the students missing class. Conclusion: The study revealed that inferring haplotype based on the phenotypic profile could be inaccurate. The proportion of atypical haplotype that were not previously described in Côte d’Ivoire was high. All the haplotypes seemed to be associated with HU treatment success but some patients with Benin haplotype did not respond well.

Children with Sickle Cell Disease in Northern Benin: Follow up of a Cohort at the Borgou/Alibori Branch of Integrated Medical Healthcare Center for Infants and Pregnant Women with Sickle Cell Disease from 2017 to 2022

Introduction: Sickle cell disease is a public health problem in sub-Saharan Africa. A national referral center for the management of infants and pregnant women with sickle cell disease (CPMI-NFED) was created three decades ago in Cotonou, in the south of Benin with two regional branches including that of Parakou in the North for better access of patients to specialized care. This work is a review of five years of activities in order to describe the epidemiological, clinical, hematological and evolutionary profiles of the children followed up in the said branch. Method: This was a descriptive and retrospective cross-sectional study on the medical records of children with sickle cell disease, followed up at the regional branch of CPMI-NFED in Borgou/Alibori from June 1, 2017 to May 31, 2022. The variables studied were epidemiological, clinical, biological and evolutionary. Results: A total of 101 children with sickle cell disease were included in the study, including 78 homozygous SS (77.2%) and 23 heterozygous SC (22.8%). Their mean age at inclusion was 51.2 ± 37.6 months [6 - 204]. The sex ratio was 1.4. Vaso-occlusive crises were the main diagnostic circumstances in 42.3% of homozygotes. More than half of the children (51.5%) had a regular follow-up. The average baseline level of hemoglobin (Hb) in homozygous children was 8.8 ± 1.4 g/dl [5.8 - 11.5];and the rate of Hb S varied between 61.9 and 94.7%. In heterozygous SC children, the mean baseline level of Hb was 10.7 ± 0.6 g/dl [9.7 - 11.5]. Acute complications observed during follow-up were dominated by pneumonia and vaso-occlusive crises in both phenotypes. The overall mortality was 3% and only affected homozygous patients. Conclusion: On average, three out of four children were homozygous in our cohort. The main acute complications were infectious and vaso-occlusive. The mortality only affected homozygous carriers. Specialized follow-up has contributed to improving the quality of life of children with sickle cell disease. This could be implemented on a large scale for better survival of children with sickle cell disease.

Consolidation of a Femoral Neck Fracture in Sickle Cell Disease Patient after Surgical Abstention: A Beneficial and Favorable Outcome

Femoral neck fracture is classically a traumatic pathology in the elderly but it can also occur in young people during a road traffic accident or occasionally when the femoral neck has been weakened by a pathological process. In sickle cell patients, the hip is often subjected to aseptic necrosis. The authors report the case of the child NL, aged 5 years and 4 months in whom a fracture of the femoral neck classified Delbet 3 was found as well as his sickle cell status discovered and in whom treatment by surgical abstention was carried out with a favorable evolution. This association of factors of the femoral head necrosis or nonunion is unusual and confirms the osteogenetic potential of a child. However, monitoring is necessary because other anomalies may reveal themselves.

Predictive Factors of Renal Damage during Sickle Cell Disease at the Hematology-Oncology Department of Donka University Hospital

Introduction: Sickle cell disease, which is the most common hereditary hemoglobinopathy in the world, attacks all body systems, particularly the kidneys. The view of this study was to investigate the predictive factors of kidney damage during sickle cell disease. Materials and methods: It was a retrospective, descriptive and analytical study on files of sickle cell patients hospitalized in the Hematology-Oncology Department of Donka University Hospital during a period from January 1, 2016 to December 31, 2019. Records of sickle cell patients with one or more renal abnormalities were retained. Sickle cell patients without kidney damage were also selected for a comparative study. Only patients without sickle cell disease were excluded. Results: Seventy-five (75) medical records were collected during the study period. From these cases, thirteen (13) records with kidney disease were observed, a frequency of 17%. The mean age of patients was 24.2 years for extremes of 10 and 65 years. The sex ratio was 1.6 in favor of men. The SSFA2 form was the most represented with 92%. 24-hour proteinuria was measured in 13 patients between whom 6 patients (46.2%) had a proteinuria level ≤ 1 g. Eight (8) patients (61.5%) were in stage 1 of chronic kidney disease. The most common type of renal involvement was tubulo-interstitial nephropathy with 8 patients (61.5%). Bivariate analysis showed that elevated serum creatinine (P 2 form of the sickness (P Conclusion: After the observation of an increased serum creatinine and urea, a predominance observation of the SSFA2 form, it should be possible to target patients for whom screening for kidney damage should henceforth be systematic.

Pregnancy Preferences in Females of Reproductive Age with Sickle Cell Disease at Laquintinie Hospital: A Cross-Sectional Analysis

Introduction: Females with sickle cell disease (SCD), despite having a delayed pubertal development, are subject to many worries relating to their abilities to conceive, their capacity to maintain a maternofetal-risk-free pregnancy till term and give birth to healthy children without sickle cell disease. Knowing that unplanned pregnancies are more likely to increase maternofetal morbidity and mortality, we sought out to explore the pregnancy preferences in female patients with sickle cell disease to promote healthy conception and childbirth in this vulnerable population. Methodology: We conducted a cross-sectional study involving female patients of child-bearing age with sickle cell disease followed at Laquintinie Hospital Douala or who were members of a local sickle cell association. Pregnancy preferences were determined using the Desire to Avoid Pregnancy (DAP) scale. Factors associated with pregnancy preferences were determined using multivariable linear regression model. Threshold for significance was set at p Results: Seventy-seven patients were included with a mean age of 24.71 ± 5.53 years. Forty patients (51.95%) had one sexual partner and thirteen women (32.5%) used contraceptive methods. Most patients (46.0%) responded “Strongly Agree” or “Agree” to the statement “it would be a good thing for me if I became pregnant in the next 3 months”. The mean DAP score was 1.63 ± 0.91 [1.28 ± 0.82 in those who had a sexual partner and 1.99 ± 0.86 in those who had no sexual partner]. Factors that were positively associated with DAP score were students (b = 0.32, 95% CI [0.21, 0.95], p = 0.003), monthly income ≥ 100,000 FCFA (b = 0.24, 95% CI [0.07, 0.81], p = 0.022), and not having a sexual partner (b = 0.26, 95% CI [0.11, 0.85], p = 0.012). Conclusion: Most female patients with SCD have a low desire to avoid pregnancy. This is especially true for patients who are not students, have a partner and have a low monthly income.

Asthma Attack Revealing Sickle Cell Disease

Sickle cell disease is a genetic disorder characterized by the presence of hemoglobin S (HbS) and a significant reduction in normal hemoglobin A (HbA) in red blood cells. In deoxygenated conditions, HbS molecules polymerize, causing vascular occlusions and hemolysis. Pulmonary complications associated with this disease result from vascular occlusion, ischemia-reperfusion, and inflammation. Literature reports that asthma is observed in 30% to 70% of patients with sickle cell disease. We present the case of a boy whose sickle cell disease diagnosis was established following an asthma attack.

Body Composition in Sickle Cell Disease Patients in the Steady State

Introduction: Previous studies in sickle cell disease (SCD) patients have demonstrated low muscle mass (MM) and high fat mass (FM) percentages compared to individuals with normal hemoglobin. The relationship between the evolutions of body composition (BC) parameters in the active SCD population is not well elucidated. The aim of the present study was to assess the body composition in active adult SCD patients in sub-Saharan Africa during the inter-critical period. Methodology: We conducted a descriptive and analytical cross-sectional study over an 8-month period. Eighteen SCD patients (sex ratio = 1) reported regular physical activity (RPA) corresponding to level 3 (RPA of moderate intensity) of the 4-level Saltin-Grimby physical activity scale were recruited. Anthropometric and BC parameters such as BMI, percentage FM and percentage MM were evaluated using an impedance meter. Results: The study population had a mean age of 26 ± 7 years and a mean BMI of 20.5 ± 1.9 kg/m2. We found a significantly higher percentage of FM in women compared to the men (25.6% ± 10.2% vs 11.1% ± 2.1%;p = 0.001). For the proportion of MM, all the women had a normal percentage. The men mostly had high levels of MM compared with reference norms. Men had significantly greater MM percentages than women (45.4% ± 1.8% vs. 26.5% ± 1.4%;p Conclusion: RPA appears to have positive effects on BC in active population with SCD, and a greater-than-expected effect was observed on MM in male patients. Promoting regular, adapted physical activity would be a promising way of improving BC parameters and quality of life in the SCD population. Further studies with larger cohorts will be needed to better substantiate these results.

Hematological Malignancies in Sickle Cell Disease Patients: Report of Four Cases in Togo and Literature Review

Background: Hemopathies were rarely observed in major sickle cell disease patients some thirty years ago, probably due to the high mortality rate among the latter as a result of progressive complications. Thanks to advances in the management of sickle cell disease, patients' life expectancy has increased considerably, exposing them more frequently to neoplasia, including hematological malignancies. The increased risk of leukemogenesis is multifactorial and linked to the pathophysiological mechanisms of the clinical manifestations of sickle cell disease. Study Setting: The clinical haematology department of campus teaching hospital and the paediatric onco-haematology unit of Sylvanus Olympio teaching hospital in Lomé were used as study settings. Observations: Four hematologic malignancies were collected in a cohort of 5847 major sickle cell syndromes. The median age of the patients was 31.25 years (extremes: 14 and 58 years) and they were predominantly female (sex ratio M/F = 0.25). Two were on background therapy with hydroxyurea. Among the four patients, there were two cases of acute lymphocytic leukemia, including ALL3 in a 58-year-old SS woman and T-ALL2 in a 12-year-old SC. Then, a case of lymphocytic lymphoma in a 20-year-old SS man was reported and finally a case of chronic myelocytic leukemia in a 33-year-old woman of Sβ+ thalassaemia phenotype. Conclusion: To further report this coexistence, it is therefore essential to systematically consider hematological malignancies during major sickle cell syndromes even if there are similarities in the symptomatology of these two serious pathological situations.

Psychological Experience of Mothers of Children with Sickle Cell Disease Followed at the Pediatric Department of Bouaké University Teaching Hospital

Introduction: Sickle cell disease has physical and emotional repercussions on the child and his family. The aim of this study was to describe the psychosocial experiences of mothers of children with sickle cell disease in order to improve the overall care of the child. Methods: This was a descriptive cross-sectional study carried out in the pediatrics department of Bouaké University Teaching Hospital from June to September 2023. It focused on mothers of major sickle-cell-affected children followed up in the pediatrics department of the Bouaké University Teaching Hospital. The variables studied were sociodemographic, psychological, social and economic. Results: Of the 40 mothers surveyed, 15% were not in school and 32.5% were unemployed. For them, sickle cell disease was of natural (genetic) origin in 90% and supernatural in 10%. They stated that the child had an average age of 36 months (extremes 7 and 108 months) when the disease was discovered. And 52% of them were satisfied with the way the disease was clearly and completely announced. Following the announcement, the questioned mothers said they had felt shock (35%), sadness (31.7%), guilt (23.3%) and discouragement (10%). Anxiety and depression were experienced by 77.5% and 22.5% respectively. In 60% of cases, they stated that the disease was incurable, and the outcome was fatal in 2.5% of cases. The child’s illness was a source of problems in the home in 25% of cases, represented by arguments in 92% and divorce in 8%. In 97.5% of cases, the mother told her family and friends about the child’s illness. In 90% of cases, the mother and child benefited from psychological support from family and friends. Conclusion: Sickle cell disease is a serious illness with a psychological and social impact on mothers. We recommend psychological support for mothers from the moment of diagnosis and throughout follow-up.

Comparative Study of Neurosurgical Complications of Thalassemia and Sickle Cell Disease

Introduction: Sickle cell disease and thalassemia are the most frequent hemoglobinopathies. During their evolution, they present certain complications, among which are two neurosurgical emergencies, namely spontaneous cranial epidural hematoma and non traumatic radiculo-medullary compression, with some particularities for each. Method: In order to highlight these particularities, we compared the characteristics of these two complications, from a number of publications reported between 2000 and 2021. Results: Sickle cell disease was complicated by spontaneous cranial epidural hematoma. Forty-two cases were reported, the mean age was 14.7 years (2 - 21 years) and the sex ratio was 6.4. The clinical presentation combined, in a non-traumatic context, signs of intracranial hypertension with those of neurological focalization. Neuroimaging showed epidural-type collection, often frontal and parietal in location. The incriminating mechanisms were ischemia, hemorrhage and extra medullary hematopoiesis. The treatment was surgical. Non traumatic radiculo-medullary was the complication of thalassemia. Of the 77 cases reported, the mean age was 27.5 years (9 - 66 years) and the sex ratio was 4.1. The lesions were epidural with a clear thoracic predominance and resulted from extra marrow hematopoiesis. Treatment included: hypertransfusion, radiotherapy, hydroxyurea and surgery. Vital and functional prognosis were globally satisfactory when the management was rapid. Conclusion: Cranial and spinal epidural lesions, respective complications of sickle cell disease and thalassemia, result from similar mechanisms. Their prognosis depends on the rapidity of management. .

A Health Service Transition to Adult Patient Care for Sickle Cell Disease

This paper explores the challenges related to long-term conditions,focusing on sickle cell disease(SCD)as a case study.Long-term conditions,characterized by the need for ongoing management,present a substantial burden on healthcare systems globally.A careful transition from pediatric to adult healthcare is needed for SCD.The discussion extends to the broader health service transition to adult patient care in SCD,emphasizing the World Health Organization’s definition of care transitions and the necessity for an integrated healthcare service.The emphasis is on a multidisciplinary approach to medical,mental health,and educational problems.A person-centered model of care should be used more consistently to resolve these long-term condition-related challenges.To evaluate the effectiveness of new interventions in improving the transition of care from pediatrics to adult patients with SCD,continuous quality improvement strategies should be implemented and prospectively measured in younger patients.In conclusion,this study highlights the critical importance of an effective transition from pediatric to adult healthcare.The continued research of effective transition practices is essential for the future and there is still a requirement to develop pragmatic approaches to enhance research on the transition to improve the quality of healthcare for patients with long-term conditions.

Clinical and Bacteriological Profile of Infections in Sickle Cell Children in Two Referral Hospitals in Niamey, Niger

Introduction: Infections are significant causes of mortality in sickle cell children in resource-limited countries. This study aimed to determine the clinical profile and bacterial ecology of infections in children with sickle-cell disease in two referral hospitals in Niamey. Patients and methods: A retrospective descriptive study was conducted from January 2018 to July 2020 in two referral hospitals in Niamey. All children aged one (1) to 15 years with sickle cell disease admitted for suspected infection, including at least one bacterial culture, were studied. Bacteriological analysis was performed using the appropriate culture media, using BactAlert (Reference 4700003 BTA3D60 BioMérieux). Results: Over 36-months, 350 children with a mean age of 10.9 months were admitted. The sex ratio was 1.2. The SS electrophoretic profile was the most common (93.4%). Immunization status was up to date in 66% of patients. Fever was the common reason for consultation (55.1%). Infection was confirmed in 62 patients (17.7%). The primary diagnoses were bacterial gastroenteritis (24.2%) and urinary tract infection (19.4%). Blood cultures were isolated from Salmonella typhi (13.0%) and Escherichia coli (8.7%). Klebsiella spp (7.1%) and Escherichia coli (5.0%) were detected in cytobacteriological examination of urine. Salmonella typhi (23.5%) and Escherichia coli (5.9%) were isolated on coproculture. Conclusion: Bacterial ecology appears not different from that usually observed in sickle-cell children. Salmonella and Escherichia coli were predominant.

Aqueous Extract of Ceiba pentandra Stimulates the Production of Fetal Hemoglobin in Sickle Cell Patients

Subsequent studies have demonstrated the reversed activity of the aqueous extract of Ceiba pentandra on the deformity of sickled red blood cells in hypoxia conditions. The observation which related to an in vitro study had given rise to hopes as to the management of sickle cell disease (SCD) by the use of this plant species. In this paper, the authors aimed to investigate the effect of the aqueous extract of C. pentandra on the production of fetal hemoglobin in SCD patients. The work carried out hemoglobin electrophoresis, for a period of six months, on blood samples from SCD patients who voluntarily undergone routine treatment, based on the medicinal recipe prepared from the bark of the trunk and branches of C. pentandra, in a hospital center of herbal medicines located in Kinshasa. The medicinal recipe called BEAT-SS is a patented product of the hospital center named Centre de Phytothérapie Moderne NIECA. Blood samples from patients under treatment were taken to evaluate the behavior of different forms of hemoglobin (hemoglobin S, hemoglobin F and hemoglobin A2). Agarose gel electrophoresis with integrated reading was used for the separation of the different forms of hemoglobin, as well as their dosage on each sample of sickle blood. A reduction in the proportion of hemoglobin S and an increase in the proportion of fetal hemoglobin were found in all sickle cell patients during the treatment period. This observation could affirm that the management of sickle cell patients using the recipe prepared from the aqueous extract of C. pentandra could increase the level of fetal hemoglobin in these patients.

Suicidal Behaviours in Sickle Cell Patients at the Douala Sickle Cell Care Centre in Cameroon

Introduction: Sickle cell anaemia is a hereditary disease that combines physical and psychological manifestations, including suicidal tendencies. So far, to our knowledge, no study has been conducted on suicidal behaviours among people with sickle cell disease in Cameroon. This is what justifies our study on the prevalence and factors related to suicidal behaviors in our study population. Methods: We conducted a cross-sectional, analytical study of 171 sickle cell patients aged from 12 years upwards who came to the sickle cell disease care service of the Laquintinie Hospital in Douala, over a period of 6 months, that is, from 1 January to 31 June 2022. Data were collected using a structured questionnaire with questions on suicidal behaviours based on the “MINI” (Mini International Neuropsychiatric Interview). The data were processed using the SPSS 26.0 software. The related factors were studied in both a bivariate and multivariate analysis. Results: Female sickle cell patients accounted for 60.2% of the sample. The mean age was 23.36 ± 8.42 years. Suicidal ideation was prevalent in 56% of cases and 13% attempted suicide. The factors most associated with suicidal ideation were: primary level of education (OR = 0.08 (0.09 - 0.79);p = 0.03), feeling unworthy of life (OR = 0.40 (0.08 - 1.96);p = 0.02), not often being considered by those around them (OR = 2.97 (1.26 - 6.99);p = 0.01), lack of family support (OR = 0.34 (0.15 - 0.77);p = 0.01). Meanwhile, the factors associated with suicide attempts were: the fact of being rarely exposed to suicide-related media (OR = 4.17 (1.40 - 71.80);p = 0.03), and a constant feeling of sadness when returning home (OR = 18.02 (1.59 - 20.55);p = 0.01). Conclusion: More than half of sickle cell patients had had suicidal thoughts and 1/6 had made at least one suicide attempt, women and young adults being the most concerned. It is therefore necessary to ensure optimum psychological care for patients with sickle cell disease.

Study of the Mineral Element Status in Sickle Cell Patients Attending the Mixed Medicine and Sickle Cell Anemia Center “Yolo Mabanga” in Kinshasa in Democratic Republic of the Congo

Introduction: Sickle cell disease, also called sickle cell anemia, is a genotypic disorder prevalent in the black population;it is characterized by a hemolytic type anemia and can worsen following a deficiency of copper, zinc and serum iron. Methods: It was a question of evaluating the plasma status of copper and zinc by the photometric method, serum iron was measured by spectrophotometry, and finally ferritin and transferrin were measured by the immunoenzymatic method;in subjects with sickle cell disease and healthy subjects of all ages followed at the mixed medicine and sickle cell anemia center in Kinshasa (CMMASS). Results: A total of 60 subjects participated in this study. The sex ratio was 1.30;the average age of sickle cell patients was 7.4 years ± 3.8 and 27.4 years ± 5.1;for the control group, the average age was 8.2 years ± 4.2 and 29 years ± 6.7. 13.3% of children with sickle cell disease presented hypocupremia and 13.3% hypercupremia. For adults with sickle cell disease, 26.7% had hypocupremia and 13% had hypercupremia. Regarding zincemia, 67% of children and adults with sickle cell disease presented hypozincemia;60% of child subjects with sickle cell desease demonstrated hyposideremia;in adults with sickle cell desease 20% have hyposideremia and 13% have hypersideremia. Conclusion: Our results demonstrate not only the effective presence of iron overload in adult sickle cell patients, but also an iron deficiency in controls and sickle cell patients, ignoring hemolysis. .

Nutritional Profile of Children with Major Sickle Cell Syndrome at the Centre of Medical and Health Advice of Kipé, Conakry, 2018

Introduction: Growth is a reflection of a child’s health and nutritional status. Children with sickle cell disease often have slower statural and weight development. The aim of this study was to evaluate the nutritional profile of children with sickle cell disease (SCD) registered in the CEMECO centre database. Methodology: This was a cross-sectional study with simple random sampling of children aged 1 to 16 years registered in the clinic database. Results: We collected information on 208 children, 121 of whom had sickle cell disease and 87 of whom were normal, with a sex ratio of 1.02. The mean age of the sickle cell patients was 8.7 ± 4.4 years, while that of the non-sickle cell patients was 9.5 ± 4 years. Haemoglobin electrophoresis revealed 103 homozygous (SS), 18 double heterozygous (SC, SBetaThal, SE) and 87 normal (AA) and/or sickle cell trait (AS) sickle cell cases. We observed a significant difference in the height/age ratio (P ¥). Conclusion: The results of our study revealed stunted growth in children with sickle cell disease.

Bacteremia in Subjects with Sickle Cell Disease: High Rate of Gram-Negative Isolates in the West African Context!

Background: Sickle cell disease is one of the most common monogenic diseases in the world, affecting approximately 70 million people, 80% in sub-Saharan Africa and 1 in 10 in Senegal. Sickle cell anemia causes functional asplenia (associated with repeated thrombosis of splenic vessels), resulting in increased susceptibility to infection. However, several studies have reported differences in the spectrum of bacterial infections in malaria-endemic areas. Therefore, we proposed to conduct a study to determine the rate of positive blood cultures and the bacteriological spectrum in sickle cell patients. Materials and Method: This is a descriptive cross-sectional study of blood culture samples from patients who received a request for hemoglobin electrophoresis as part of their treatment at the Principal’s hospital in Dakar. The study took place from January 2008 to December 2021. For each patient, we collect demographic information, including age, gender, and the service from which the analysis request originated. Data were collected in the laboratory’s computer system and entered into Microsoft Excel (2007). Statistical analyzes were performed using Epi-Info 7 software. Results: Our study included 1419 patients. The most common types of hemoglobin profiles were: normal profile (n = 1025), AS profile (n = 283), SS profile (n = 104), SC profile (n = 7). This corresponds to the proportions of 72%, 20%, 0.5% and 7.5% for the profiles Normal, AS, SC and SS. The male proportion was 61.1%, 61.5%, 57.1, respectively %, 55.8% for Normal, SA, SC, and SS profiles. A total of 19,090 individual blood culture bottles were collected from 1419 impatient patients as follows: Normal profile (n = 18,042 bottles), AS profile (n = 677 culture bottles), SS profile (n = 362 bottles). The majority of blood culture orders come from pediatric services, accounting for 70% of the total number of orders. Of 19,090 vials examined in this current study, 19.6% developed a positive blood culture. Overall, the most commonly isolated bacteria were Staphylococci (41.1%), Enterobacteriaceae (36.7%), Bacillaceae (10.2%), unfermented (6.30%), Streptococci (5.01%), and a small proportion of yeast (0.75%). There is no significant difference in bacterial spectrum between the SS profile and the normal profile of individuals (p = 0.104). Coagulase-negative staphylococci accounted for 32%, 24%, and 40% of the species isolated in the normal AS and SS profiles. respectively. Coagulase-negative staphylococci were the most commonly isolated organisms in SS. Group E and sptagged streptococci each account for less than 2% of the organisms isolated in SS. Pneumococci were not found. Bacillus accounts for 25% of isolates in SS subjects compared to 9% in normal and AS subjects, respectively. Pseudomonas aeruginosa and Burkholderia cepacia then make up 10% of the isolates in the subjects of the SS profile as non-fermenters. Conclusion: Our study shows that enterobacteria and staph are prevalent in people with sickle cell disease. There is no significant difference in bacterial spectrum between SS subjects compared to subjects with a normal profile. The rarity of Streptococcus pneumoniae in bacteremia isolates underlines the need for further studies with larger patient numbers to better understand the spectrum of bacterial infections in patients with sickle cell disease in West Africa.

Early Morbidity and Mortality in Neonates of Mothers with Sickle Cell Disease at the Borgou/Alibori Center Departmental Teaching Hospital in Benin

Introduction: Association of sickle cell disease and pregnancy is a risky situation for both the mother and neonate. Objective: To determine the early morbidity and mortality among neonates of mothers with sickle cell disease at Borgou/Alibori Center Departmental Teaching Hospital (CHUD B/A) in Benin. Patients and Methods: This was a descriptive and analytical observational study conducted at CHUD-B/A from January 1, 2015, to August 31, 2019. It included pregnant women with sickle cell disease who gave birth to a liveborn neonate at the term of at least 28 weeks of amenorrhea. Variables studied were sociodemographic, clinical, and evolutionary. Analysis of the factors associated with early death was also carried out with a significance threshold set at p Results: Out of a total of 119 pregnant women, 95 neonates were recorded. Main morbidities were: prematurity and intrauterine growth restriction (49.5%);respiratory distress (40%), bacterial infection (30.5%), and perinatal asphyxia (21.1%). Early mortality rate was 8.4%. In bivariate analysis, the factors associated with early death were: parity (p < 0.001), the severity of maternal anemia (p < 0.008), birth weight under 1500 g (p < 0.002), the birth term under 32 weeks of amenorrhea (p < 0.001), resuscitation for at least 5 minutes (p = 0.001). In multivariate analysis, resuscitation for at least 5 minutes (p = 0.007) was mainly associated with early death. Conclusion: One out of two neonates of mothers with sickle cell disease has a low birth weight. Early mortality is high due to perinatal asphyxia. Hence the multidisciplinary care of these mothers.