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 s...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.展开更多
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 ass...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.展开更多
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 ad...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.展开更多
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 diseas...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.展开更多
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...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.展开更多
Introduction: In view of the number of sickle cell patients and due to a low production of descriptive studies, we decided to determine the prevalence of genes S and C of the disease in the Zinder region. The objectiv...Introduction: In view of the number of sickle cell patients and due to a low production of descriptive studies, we decided to determine the prevalence of genes S and C of the disease in the Zinder region. The objective was to contribute to improving the management of sickle cell disease in Zinder. Methodology: This was a systematic screening by the “Sickle Scan” test of any blood donor admitted to the Zinder Regional Blood Transfusion Center during the 6-month study period, from January to June 2023. The Sickle Scan is a qualitative lateral flow chromatography immunoassay using whole blood samples that aid in the rapid diagnosis of sickle cell disease. Results: The study was carried out on 613 samples during the period concerned. The frequency of sickle cell genes was 26.9% (n = 165) in all samples collected, with 23.1% (n = 142) and 3.8% (n = 23) for the S gene and the C gene, respectively. The 18 - 30 age group was the most represented with 64.4% (n = 395) cases. The median age of blood donors was 26 years ± 10 years (min = 18 years/max = 60 years). The sex ratio was 2.5. Donors of Nigerien nationality accounted for 84.1% (n = 516). There is a predominance of blood donors with an average monthly income between 34,000 and 70,000 CFA francs in 44.3% (n = 272), lived in permanent housing with drinking water supply. Sickle cell trait (SMA) was found in 22.5% (n = 138). Conclusion: The analysis of these results highlights a high frequency of the S gene for sickle cell disease. The population with an average monthly income is the most affected, with a male predominance.展开更多
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 functio...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.展开更多
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...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.展开更多
Introduction: Sickle cell disease is the most common genetic disease in the world, particularly in sub-Saharan Africa. It is a protean condition with multiple complications including disturbed iron metabolism. Objecti...Introduction: Sickle cell disease is the most common genetic disease in the world, particularly in sub-Saharan Africa. It is a protean condition with multiple complications including disturbed iron metabolism. Objectives: To determine the prevalence of iron metabolism abnormalities in children with homozygous sickle cell disease, to describe the epidemiological, clinical and paraclinical characteristics of children with these abnormalities and to identify associated factors. Patients and Methods: This was a cross-sectional analytical study conducted over 9 months in the mother-child consultation unit of the Brazzaville University Hospital, the National Reference Centre for Sickle Cell Disease and the paediatric department of the Blanche Gomes mother-child hospital. It concerned children aged between 3 months and 15 years followed up for homozygous sickle cell disease. The study was based on a haemogram, iron metabolism test, LDH, transaminases and CRP. Results: The overall prevalence of iron metabolism abnomalities was 40.7%. Of the 145 children included, 35.9% had iron overload and 4.8% iron deficiency. Iron overload was associated with infections, undernutrition, iron supplementation and number of blood transfusions. Iron deficiency was not significantly associated with any factor but recurrent infections were relatively more frequent. Conclusion: Abnormalities of iron metabolism in sickle cell patients are relatively frequent, which justifies monitoring during follow-up for early detection and better management. .展开更多
Introduction: Sickle cell disease is a real public health problem in the world and particularly in Niger where the prevalence of the S gene is estimated at 25% and that of the homozygous forms at between 1% and 2%. Tr...Introduction: Sickle cell disease is a real public health problem in the world and particularly in Niger where the prevalence of the S gene is estimated at 25% and that of the homozygous forms at between 1% and 2%. Treatment combines quarterly follow-up of patients and management of complications. The objective of this study was to identify the potential explanatory factors of non-adherence to treatment in sickle cell patients followed at the national reference center for sickle cell disease in Niger. Methods: This is a cross-sectional study of sickle cell cases followed at the CNRD in Niger. The population consisted of all sickle cell patients followed in this center in 2021. The data collection techniques were individual interviews and documentary reviews. Non-adherence was assessed with the Girerd test. Descriptive statistical tests and simple and multiple logistic regression models were performed. Results: A total of 368 patients were enrolled. The median age is 7 years (4;10) and the sex ratio is 1.04. Ninety-eight (98) or 26.6% were compliant and 270 (73.4%) were non-compliant. In multivariate analysis, the factors independently and negatively associated with non-adherence to treatment were schooling (adjusted OR [95% CI], p-value), 0.17 [0.10 - 0.30];p Conclusion: The factors influencing treatment compliance identified in this study are all modifiable. To prevent the complications of sickle cell disease, we must fight against ignorance, make care services accessible and make care free.展开更多
Objective: To study the epidemiological, clinical and therapeutic aspects of sickle cell disease in children admitted to the pediatric emergency department of the Gabriel Toure University Hospital. Materials and metho...Objective: To study the epidemiological, clinical and therapeutic aspects of sickle cell disease in children admitted to the pediatric emergency department of the Gabriel Toure University Hospital. Materials and methods: This was a prospective study over 12 months in sickle cell children aged 6 months to 15 years. Results: The frequency of sickle cell disease was 4.67% with 71.1% of SS form. The age range of 60 - 120 months predominated with 43.4% and the sex ratio 1.4. Vaso-occlusive crises (VOC) were the most frequent reason for consultation (50.6%) and osteoarticular pain was the main symptom followed by fever and pallor with respectively 67.5%, 60.2%, 39.8%. The pain subsided in less than 72 hours in 39.8% and hyper hydration was performed in 85.1% of patients. Analgesic treatment was given in 90.4%, antibiotic therapy in 51.8% and phenotyped red blood cells transfusion was performed in 36.1%. The average duration of treatment was 10 days. Conclusion: Vaso-occlusive crisis remains the main reason for consultation in sickle cell disease children at Gabriel Toure University Hospital. The management consisted, in addition to the correction of the triggering factors, of hyper hydration and the administration of analgesics and blood products.展开更多
Introduction: Pubertal development is a process leading to the acquisition of reproductive capacities. Among the factors that inhibit pubertal development are chronic diseases including sickle cell anemia, which is a ...Introduction: Pubertal development is a process leading to the acquisition of reproductive capacities. Among the factors that inhibit pubertal development are chronic diseases including sickle cell anemia, which is a public health problem. Objectives: Describe the sociodemographic and clinical characteristics of adolescents with sickle cell disease. Report the prevalence of abnormalities of pubertal development. Identify associated factors that delay pubertal development. Patients and Methods: This was a multicenter analytical cross-sectional study over 7 months at the National Reference Center for Sickle Cell Disease and, at the Brazzaville University Hospital. It concerned adolescents with sickle cell disease aged between 10 to 19 years. The study focused on the sociodemographic characteristics of adolescents, the natural history of sickle cell anemia and the evaluation of secondary sexual characteristics using the Tanner classification. Nutritional status was assessed by calculating body mass index (BMI) and height/age and weight/age ratios. Results: Of the 347 adolescents included, the average age of the adolescents was 15.1 ± 2.5 years, 56.5% had normal puberty, 42.6% had delayed puberty and 0.9% had impuberty. The associated factors were under-nutrition with less than 3 meals/day (p = 0.0000), social status with more marked pubertal delay in orphans (p = 0.00127), more than 5 hospitalizations per year (p = 0.0013), pubertal delay was statistically significant in adolescents who had more than 3 vaso-occlusive crises (p = 0.0000), and those who had more than 5 blood transfusions since the discovery of the disease (p = 0.0127). Conclusion: The factors that hinder pubertal development in sickle cell patients are intrinsic (sickle cell anemia with its complications) and extrinsic (environmental: diet, social status). The appearance of secondary sexual characteristics is delayed on average by two years compared to the general population.展开更多
Introduction: Our study focused on the evaluation of renal function in children with sickle cell disease compared to children without sickle cell disease at the pediatric emergency unit of the Donka National Hospital ...Introduction: Our study focused on the evaluation of renal function in children with sickle cell disease compared to children without sickle cell disease at the pediatric emergency unit of the Donka National Hospital and SOS Drepano-Guinea center. Patients and Methods: This was a cross-sectional descriptive and analytical study lasting 3 months (October 1 to December 31, 2020). Were included, all sickle cell and non-sickle cell children aged 0 to 15 received on an outpatient basis and had undergone an exploration of renal function (serum creatinine and urine dipstick). Results: We performed the urine dipstick and serum creatinine in 75 children, 45 of whom were sickle cell and 30 were not sickle cell. 27 of our patients or 36% had a reduction in GFR, among them 9 or 20% were sickle cell and 18 or 60% were not sickle cell. The most affected age group was 5 to 9 years in sickle cell (66.7%) and non-sickle cell (38.9%). In sickle cell patients, 9 cases (100%) had mild renal failure (IRL). Non-sickle cell patients, had 14 cases or 77.8% of IRL and 4 cases (22.2%) of moderate IR. Sickle cell disease and antibiotics which had the respective p-value (0.01);(0.02), were statistically significant with the onset of renal failure. Conclusion: Several factors including sickle cell anemia and antibiotics are believed to be involved in lowering GFR. It would be essential to detect early the children received in consultation.展开更多
Objectives: Sickle cell disease (SCD) has a varied clinical and biological expression depending on the hemoglobin phenotype: SSFA<sub>2</sub>, SFA<sub>2</sub>, SAFA<sub>2</sub> and ...Objectives: Sickle cell disease (SCD) has a varied clinical and biological expression depending on the hemoglobin phenotype: SSFA<sub>2</sub>, SFA<sub>2</sub>, SAFA<sub>2</sub> and SC. Considering the antioxidant properties of the different haptoglobin phenotypes (Hp 1-1, Hp 2-1, Hp 2-2), it seemed relevant to know their influence on the morbidity of the different hemoglobin phenotype of SCD. Thus, the objective of this study was to identify associations between haptoglobin phenotype and morbidity of different SCD phenotypes. Methods: In a retrospective cross-sectional descriptive and analytical study, with a cohort of 170 black African carriers of hemoglobin S, in Ivory Coast, West Africa, hemoglobin and haptoglobin phenotypes were determined by electrophoretic methods. Results: The three major phenotypes of haptoglobin polymorphism were found in the SCD cohort: Hp 1-1 (24.1%), Hp 2-1 (56.5%), Hp 2-2 (19.4%). Vaso-occlusions were associated with haptoglobin phenotype Hp 1-1, (OR = 2.03;CI<sub>95%</sub> = [1.06 - 3.9];p Conclusions: Haptoglobin phenotype was associated to morbidity-adjusted hemoglobin phenotype. The study revealed a greater probability of a worse morbidity when the hemoglobin phenotype is homozygous. Unexpectedly, the worse morbidity is associated to Hp 1-1 haptoglobin phenotype, the most powerful antioxidant within the different haptoglobin phenotypes. Associations found were not systematic and need further studies to enlighten the determinism of SCD morbidity.展开更多
Sickle cell disease is the most common inherited hemoglobinopathy and is characterized by painful vaso-occlusive crisis.Pregnancy in women with sickle cell disease is associated with an increased incidence of maternal...Sickle cell disease is the most common inherited hemoglobinopathy and is characterized by painful vaso-occlusive crisis.Pregnancy in women with sickle cell disease is associated with an increased incidence of maternal and perinatal complications,but notwithstanding,adequate care through preconception,antenatal,intrapartum,and postnatal stages ensures a better outcome.Despite the management of pregnant women with sickle cell disease being best accomplished using a multidisciplinary approach,few therapeutic interventions have been explored.This paper focuses on the effective conventional and non-conventional management of pregnant women with sickle cell disease with the aim of suppress the alternating effect of sickle cell disease and pregnancy for better maternal and fetal outcomes.The conscious use of these therapies integratively or alternatively can improve self-care and quality of life,decrease the occurrence of discomforts and complications,and reduce frequency of hospitalization.展开更多
Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell disease are common genetic defects of red blood cells that lead to hemolytic anemia. The prevalence of G6PD deficiency in sickle cell pat...Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell disease are common genetic defects of red blood cells that lead to hemolytic anemia. The prevalence of G6PD deficiency in sickle cell patients is unknown in Benin. Objective: This study aimed to determine the prevalence of G6PD deficiency in sickle cell patients at the CNHU-HKM of Cotonou. Methods: This prospective study was conducted from April to November 2022 at the blood-related diseases teaching clinic and included sickle cell patients in the stationary phase. G6PD determination was performed using the enzymatic method on a Mindray BS 200 machine following the Herz method. Hematological parameters were determined using the XT 4000i analyzer and supplemented by a blood smear stained with May Grunwald Giemsa. Data were analyzed using Epi Info 3.5.4 software. Results: One hundred and sixty-four sickle cell patients (80 SS homozygotes and 84 SC heterozygotes) in the intercritical phase, with a mean age of 26.30 ± 10.76 years, were included. The prevalence of G6PD deficiency was 9.1% (15 cases found in 7 SS patients and 8 SC patients). In G6PD-deficient patients, the mean concentration of the enzyme was lower in Hb SC heterozygotes than in Hb SS homozygotes: 3.56 IU/g Hb versus 4.98 IU/g Hb. The mean reticulocyte count was 231.43 G/L in the deficient group, compared to 216.32 G/L in the non-deficient group. Conclusion: The preliminary results of our study reveal a high prevalence of G6PD deficiency in sickle cell patients. The impact of this association on hematologic and biological parameters should be evaluated for better management of sickle cell disease.展开更多
Haemoglobinopathies are very serious clinical conditions caused by genetic mutations. They belong to autosomal recessive disorders and the most frequent genetic inherited diseases seen, specifically and above all amon...Haemoglobinopathies are very serious clinical conditions caused by genetic mutations. They belong to autosomal recessive disorders and the most frequent genetic inherited diseases seen, specifically and above all among Mediterranean countries. Thalassaemia syndromes (included Beta Thalassaemia and Sickle Cell Disease) have been the first diagnosed diseases since in intrauterine life using reccombinant DNA techniques. So, the better understanding of their pathophysiology has given a spectacolar improvement and a considerable impact on these conditional managements. Every year there are nearly 300,000 children born with haemoglobinopathies globally, and there are 60,000 - 70,000 children with Beta Thalassemia among them. Nowadays in Albania like everywhere, there is a significant increase of survival in these patients. As a result of life longevity and improvement of patients life quality, we can see that these patients may suffer from other concomitant illnesses. In our country, there are registered approximately 500 patients with haemoglobinopathies. We studied 50 pediatric patients at random ranging from age 2 until 18 years old. We excluded other pathologies among them. We found high values of biochemical indicators in blood (Ca 15-3 was found elevated in 75% of our patients, Lactate Dehydrogenases was found elevated in 70% of cases, Indirect Bilirubin was found elevated in 66% of cases). All three parameters are indicators of hemolysis. We found a correlation between high values of Ca 15-3 marker and high levels of LDH, Indirect Bilirubin and low level of hemoglobin (p < 0.05). Ca 15-3 is much elevated among patients non regularly transfused and in those who take an unsufficient amount of blood. Continuous monitoring of these biochemical parameters is going to help in the more effective follow up of patients with haemoglobinopathies.展开更多
Introduction: Sickle cell disease (SCD) causes chronic hemolysis which is a risk factor for cholelithiasis. Its development may lead to severe and life-threatening complications. Objective: Determine the prevalence of...Introduction: Sickle cell disease (SCD) causes chronic hemolysis which is a risk factor for cholelithiasis. Its development may lead to severe and life-threatening complications. Objective: Determine the prevalence of cholelithiasis, the conditions of diagnosis and related factors. Materials and Method: We retrospectively reviewed records of 110 patients with sickle cell disease followed up in Charles de Gaulle University Pediatric Hospital from January 2003 to December 2013, including 103 patients who had abdominal ultrasonography. Results: Cholelithiasis prevalence was 24.3%. The mean age of patients was 10.8 years, (range 3 to 15 years). Sex ratio was 2.1. In 88% cases, cholelithiasis was diagnosed based on the characteristic symptoms of right hypocondrial pain, fever and icterus. Most factors associated with cholelithiasis were as follows: age above 10 years (OR = 4), occurrence of at least three (03) vaso-occlusive crises per year (OR = 7.6), history of blood transfusion (OR = 8), right hypochondrial pain (OR = 4.5) and icterus (OR = 15). Only 20% of patients suffering from a symptomatic cholelithiasis underwent laparoscopic cholecystectomy and results were conclusive. Conclusion: Patients with sickle cell disease, especially those aged above 10, should be routinely tested for cholelithiasis using abdominal ultrasonography at least once a year. Because of the difficulties in managing evolutive complications in case of an emergency in our context, we advocate laparoscopic cholecystectomy of any cholelithiasisas soon as it is diagnosed in children with sickle cell disease.展开更多
<span style="line-height:1.5;">Natural plant products have been used by the population of the south-eastern part of C</span><span lang="EN-US" style="line-height:1.5;">&...<span style="line-height:1.5;">Natural plant products have been used by the population of the south-eastern part of C</span><span lang="EN-US" style="line-height:1.5;">ô</span><span style="line-height:1.5;">te d’Ivoire in the management of sickle cell anemia. </span><span style="line-height:1.5;">This study was aimed at investigating the antisickling activity of the hydro-ethanolic extract </span><span "="" style="line-height:1.5;"><span>of a combination of the leaves of </span><i><span>J</span></i><span>. </span><i><span>secunda</span></i><span>, </span><i><span>J</span></i><span>. </span><i><span>gossypiifolia</span></i></span><span style="line-height:1.5;"> a</span><span "="" style="line-height:1.5;"><span>nd </span><i><span>P</span></i><span>. </span><i><span>nigrescens</span></i><span>.</span></span><span style="line-height:1.5;"> These three plants species were used in the Ivorian traditional herbal medicine. Preliminary phytochemistry was carried out using standard methods. As for the sickling reversal test, the Hb SS blood sickling was induced with 2% sodium metabisulfite. After 120 minutes of incubation, the plant extract was added. Every 30 minutes for a period of 120 minutes, a drop of the prepared solution was observed at (40</span><span style="line-height:1.5;">×</span><span "="" style="line-height:1.5;"><span>) magnification and the percentage of reversion calculated. The phytochemical analysis revealed the presence of Alkaloids, Flavonoids, Polyphenols, Catechic Tannins, Sterols and Polyterpenes. The results of the reversal test showed that the percentage of sickling reversal effect of the combination of the plants (75.00 ± 4.33</span><sup><span>b,c</span></sup><span>) was highly superior than the negative control (10.17 ± 0.55</span><sup><span>d</span></sup><span>) but was significantly (p < 0.05) the same as the percentage of the individual plant (</span><i><span>J</span></i><span>. </span><i><span>secunda</span></i><span>: 83.50 ± 2.33</span><sup><span>a</span></sup><span>;</span><i><span>J</span></i><span>. </span><i><span>gossypiifolia</span></i><span>: 78.00 ± 3.67</span><sup><span>b,c</span></sup><span> and </span><i><span>P</span></i><span>. </span><i><span>nigrescens</span></i><span>: 77.83 ± 2.89</span><sup><span>b,c</span></sup><span>) and the positive control (80.66 ± 2.22</span><sup><span>a,b</span></sup><span>). From the results, the extracts </span><i><span>J. secunda</span></i><span>, </span><i><span>J</span></i><span>. </span><i><span>gossypiifolia</span></i><span> and </span><i><span>P</span></i><span>. </span><i><span>nigrescens</span></i><span> have shown to be therapeutically beneficial to the population. Their use is also justified in the management of sickle cell disease in the south-eastern part of C</span></span><span lang="EN-US" style="line-height:1.5;">ô</span><span style="line-height:1.5;">te d’Ivoire.展开更多
Introduction: Nigeria accounts for 150,000 infants born with the Sickle Cell Disease (SCD) every year. Children with SCD are affected by the deleterious effect of user fees which reduces affordability and utilization ...Introduction: Nigeria accounts for 150,000 infants born with the Sickle Cell Disease (SCD) every year. Children with SCD are affected by the deleterious effect of user fees which reduces affordability and utilization of health services. Evidences supported that institutionalized health insurance increases intensity of utilization. Methodology: The study was conducted at AKTH, Kano, and north-western, Nigeria. The study was retrospective comparative cross-sectional study. 100 patients were enrolled, 50 for each arm. Paediatric SCD clinic outpatients’ records and a specialty designed form containing the variables of interest were used. Data were analysed using Minitab 16. Proportions, percentages, tables, charts and chi squared test were used to compare the two groups. Result: The study found no association between the two groups in the likelihood of being insured based on age (χ<sup>2</sup> = 1.478), gender (χ<sup>2</sup> = 0.224) and dwellings (χ<sup>2</sup> = 0.062). On health services utilization and insurance status, the study revealed that follow up clinic visits, unscheduled clinic visits and emergency room visits were more likely among the insured group compared to the uninsured group. While the health status and insurance status of the two groups over 12 months period showed a significant association with the insured more likely to have improved health status compared to uninsured (χ<sup>2</sup> = 28.019, p = 0.0001). Similarly, health status and health services utilization were significantly associated with insurance status (χ<sup>2</sup> = 12.191, p = 0.002). Conclusion: The insurance status of children with SCD is associated with their health services utilization and health status. However, age, gender and dwellings were not associated with insurance status of children with SCD. Therefore, when expanding insurance coverage (NHIS) among children with SCD considerations have to be given to increase health services utilization and resultant improvement in health status as these have the potential of reducing morbidity and mortality among children with SCD.展开更多
文摘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.
文摘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.
文摘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.
文摘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.
文摘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.
文摘Introduction: In view of the number of sickle cell patients and due to a low production of descriptive studies, we decided to determine the prevalence of genes S and C of the disease in the Zinder region. The objective was to contribute to improving the management of sickle cell disease in Zinder. Methodology: This was a systematic screening by the “Sickle Scan” test of any blood donor admitted to the Zinder Regional Blood Transfusion Center during the 6-month study period, from January to June 2023. The Sickle Scan is a qualitative lateral flow chromatography immunoassay using whole blood samples that aid in the rapid diagnosis of sickle cell disease. Results: The study was carried out on 613 samples during the period concerned. The frequency of sickle cell genes was 26.9% (n = 165) in all samples collected, with 23.1% (n = 142) and 3.8% (n = 23) for the S gene and the C gene, respectively. The 18 - 30 age group was the most represented with 64.4% (n = 395) cases. The median age of blood donors was 26 years ± 10 years (min = 18 years/max = 60 years). The sex ratio was 2.5. Donors of Nigerien nationality accounted for 84.1% (n = 516). There is a predominance of blood donors with an average monthly income between 34,000 and 70,000 CFA francs in 44.3% (n = 272), lived in permanent housing with drinking water supply. Sickle cell trait (SMA) was found in 22.5% (n = 138). Conclusion: The analysis of these results highlights a high frequency of the S gene for sickle cell disease. The population with an average monthly income is the most affected, with a male predominance.
文摘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.
文摘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.
文摘Introduction: Sickle cell disease is the most common genetic disease in the world, particularly in sub-Saharan Africa. It is a protean condition with multiple complications including disturbed iron metabolism. Objectives: To determine the prevalence of iron metabolism abnormalities in children with homozygous sickle cell disease, to describe the epidemiological, clinical and paraclinical characteristics of children with these abnormalities and to identify associated factors. Patients and Methods: This was a cross-sectional analytical study conducted over 9 months in the mother-child consultation unit of the Brazzaville University Hospital, the National Reference Centre for Sickle Cell Disease and the paediatric department of the Blanche Gomes mother-child hospital. It concerned children aged between 3 months and 15 years followed up for homozygous sickle cell disease. The study was based on a haemogram, iron metabolism test, LDH, transaminases and CRP. Results: The overall prevalence of iron metabolism abnomalities was 40.7%. Of the 145 children included, 35.9% had iron overload and 4.8% iron deficiency. Iron overload was associated with infections, undernutrition, iron supplementation and number of blood transfusions. Iron deficiency was not significantly associated with any factor but recurrent infections were relatively more frequent. Conclusion: Abnormalities of iron metabolism in sickle cell patients are relatively frequent, which justifies monitoring during follow-up for early detection and better management. .
文摘Introduction: Sickle cell disease is a real public health problem in the world and particularly in Niger where the prevalence of the S gene is estimated at 25% and that of the homozygous forms at between 1% and 2%. Treatment combines quarterly follow-up of patients and management of complications. The objective of this study was to identify the potential explanatory factors of non-adherence to treatment in sickle cell patients followed at the national reference center for sickle cell disease in Niger. Methods: This is a cross-sectional study of sickle cell cases followed at the CNRD in Niger. The population consisted of all sickle cell patients followed in this center in 2021. The data collection techniques were individual interviews and documentary reviews. Non-adherence was assessed with the Girerd test. Descriptive statistical tests and simple and multiple logistic regression models were performed. Results: A total of 368 patients were enrolled. The median age is 7 years (4;10) and the sex ratio is 1.04. Ninety-eight (98) or 26.6% were compliant and 270 (73.4%) were non-compliant. In multivariate analysis, the factors independently and negatively associated with non-adherence to treatment were schooling (adjusted OR [95% CI], p-value), 0.17 [0.10 - 0.30];p Conclusion: The factors influencing treatment compliance identified in this study are all modifiable. To prevent the complications of sickle cell disease, we must fight against ignorance, make care services accessible and make care free.
文摘Objective: To study the epidemiological, clinical and therapeutic aspects of sickle cell disease in children admitted to the pediatric emergency department of the Gabriel Toure University Hospital. Materials and methods: This was a prospective study over 12 months in sickle cell children aged 6 months to 15 years. Results: The frequency of sickle cell disease was 4.67% with 71.1% of SS form. The age range of 60 - 120 months predominated with 43.4% and the sex ratio 1.4. Vaso-occlusive crises (VOC) were the most frequent reason for consultation (50.6%) and osteoarticular pain was the main symptom followed by fever and pallor with respectively 67.5%, 60.2%, 39.8%. The pain subsided in less than 72 hours in 39.8% and hyper hydration was performed in 85.1% of patients. Analgesic treatment was given in 90.4%, antibiotic therapy in 51.8% and phenotyped red blood cells transfusion was performed in 36.1%. The average duration of treatment was 10 days. Conclusion: Vaso-occlusive crisis remains the main reason for consultation in sickle cell disease children at Gabriel Toure University Hospital. The management consisted, in addition to the correction of the triggering factors, of hyper hydration and the administration of analgesics and blood products.
文摘Introduction: Pubertal development is a process leading to the acquisition of reproductive capacities. Among the factors that inhibit pubertal development are chronic diseases including sickle cell anemia, which is a public health problem. Objectives: Describe the sociodemographic and clinical characteristics of adolescents with sickle cell disease. Report the prevalence of abnormalities of pubertal development. Identify associated factors that delay pubertal development. Patients and Methods: This was a multicenter analytical cross-sectional study over 7 months at the National Reference Center for Sickle Cell Disease and, at the Brazzaville University Hospital. It concerned adolescents with sickle cell disease aged between 10 to 19 years. The study focused on the sociodemographic characteristics of adolescents, the natural history of sickle cell anemia and the evaluation of secondary sexual characteristics using the Tanner classification. Nutritional status was assessed by calculating body mass index (BMI) and height/age and weight/age ratios. Results: Of the 347 adolescents included, the average age of the adolescents was 15.1 ± 2.5 years, 56.5% had normal puberty, 42.6% had delayed puberty and 0.9% had impuberty. The associated factors were under-nutrition with less than 3 meals/day (p = 0.0000), social status with more marked pubertal delay in orphans (p = 0.00127), more than 5 hospitalizations per year (p = 0.0013), pubertal delay was statistically significant in adolescents who had more than 3 vaso-occlusive crises (p = 0.0000), and those who had more than 5 blood transfusions since the discovery of the disease (p = 0.0127). Conclusion: The factors that hinder pubertal development in sickle cell patients are intrinsic (sickle cell anemia with its complications) and extrinsic (environmental: diet, social status). The appearance of secondary sexual characteristics is delayed on average by two years compared to the general population.
文摘Introduction: Our study focused on the evaluation of renal function in children with sickle cell disease compared to children without sickle cell disease at the pediatric emergency unit of the Donka National Hospital and SOS Drepano-Guinea center. Patients and Methods: This was a cross-sectional descriptive and analytical study lasting 3 months (October 1 to December 31, 2020). Were included, all sickle cell and non-sickle cell children aged 0 to 15 received on an outpatient basis and had undergone an exploration of renal function (serum creatinine and urine dipstick). Results: We performed the urine dipstick and serum creatinine in 75 children, 45 of whom were sickle cell and 30 were not sickle cell. 27 of our patients or 36% had a reduction in GFR, among them 9 or 20% were sickle cell and 18 or 60% were not sickle cell. The most affected age group was 5 to 9 years in sickle cell (66.7%) and non-sickle cell (38.9%). In sickle cell patients, 9 cases (100%) had mild renal failure (IRL). Non-sickle cell patients, had 14 cases or 77.8% of IRL and 4 cases (22.2%) of moderate IR. Sickle cell disease and antibiotics which had the respective p-value (0.01);(0.02), were statistically significant with the onset of renal failure. Conclusion: Several factors including sickle cell anemia and antibiotics are believed to be involved in lowering GFR. It would be essential to detect early the children received in consultation.
文摘Objectives: Sickle cell disease (SCD) has a varied clinical and biological expression depending on the hemoglobin phenotype: SSFA<sub>2</sub>, SFA<sub>2</sub>, SAFA<sub>2</sub> and SC. Considering the antioxidant properties of the different haptoglobin phenotypes (Hp 1-1, Hp 2-1, Hp 2-2), it seemed relevant to know their influence on the morbidity of the different hemoglobin phenotype of SCD. Thus, the objective of this study was to identify associations between haptoglobin phenotype and morbidity of different SCD phenotypes. Methods: In a retrospective cross-sectional descriptive and analytical study, with a cohort of 170 black African carriers of hemoglobin S, in Ivory Coast, West Africa, hemoglobin and haptoglobin phenotypes were determined by electrophoretic methods. Results: The three major phenotypes of haptoglobin polymorphism were found in the SCD cohort: Hp 1-1 (24.1%), Hp 2-1 (56.5%), Hp 2-2 (19.4%). Vaso-occlusions were associated with haptoglobin phenotype Hp 1-1, (OR = 2.03;CI<sub>95%</sub> = [1.06 - 3.9];p Conclusions: Haptoglobin phenotype was associated to morbidity-adjusted hemoglobin phenotype. The study revealed a greater probability of a worse morbidity when the hemoglobin phenotype is homozygous. Unexpectedly, the worse morbidity is associated to Hp 1-1 haptoglobin phenotype, the most powerful antioxidant within the different haptoglobin phenotypes. Associations found were not systematic and need further studies to enlighten the determinism of SCD morbidity.
文摘Sickle cell disease is the most common inherited hemoglobinopathy and is characterized by painful vaso-occlusive crisis.Pregnancy in women with sickle cell disease is associated with an increased incidence of maternal and perinatal complications,but notwithstanding,adequate care through preconception,antenatal,intrapartum,and postnatal stages ensures a better outcome.Despite the management of pregnant women with sickle cell disease being best accomplished using a multidisciplinary approach,few therapeutic interventions have been explored.This paper focuses on the effective conventional and non-conventional management of pregnant women with sickle cell disease with the aim of suppress the alternating effect of sickle cell disease and pregnancy for better maternal and fetal outcomes.The conscious use of these therapies integratively or alternatively can improve self-care and quality of life,decrease the occurrence of discomforts and complications,and reduce frequency of hospitalization.
文摘Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell disease are common genetic defects of red blood cells that lead to hemolytic anemia. The prevalence of G6PD deficiency in sickle cell patients is unknown in Benin. Objective: This study aimed to determine the prevalence of G6PD deficiency in sickle cell patients at the CNHU-HKM of Cotonou. Methods: This prospective study was conducted from April to November 2022 at the blood-related diseases teaching clinic and included sickle cell patients in the stationary phase. G6PD determination was performed using the enzymatic method on a Mindray BS 200 machine following the Herz method. Hematological parameters were determined using the XT 4000i analyzer and supplemented by a blood smear stained with May Grunwald Giemsa. Data were analyzed using Epi Info 3.5.4 software. Results: One hundred and sixty-four sickle cell patients (80 SS homozygotes and 84 SC heterozygotes) in the intercritical phase, with a mean age of 26.30 ± 10.76 years, were included. The prevalence of G6PD deficiency was 9.1% (15 cases found in 7 SS patients and 8 SC patients). In G6PD-deficient patients, the mean concentration of the enzyme was lower in Hb SC heterozygotes than in Hb SS homozygotes: 3.56 IU/g Hb versus 4.98 IU/g Hb. The mean reticulocyte count was 231.43 G/L in the deficient group, compared to 216.32 G/L in the non-deficient group. Conclusion: The preliminary results of our study reveal a high prevalence of G6PD deficiency in sickle cell patients. The impact of this association on hematologic and biological parameters should be evaluated for better management of sickle cell disease.
文摘Haemoglobinopathies are very serious clinical conditions caused by genetic mutations. They belong to autosomal recessive disorders and the most frequent genetic inherited diseases seen, specifically and above all among Mediterranean countries. Thalassaemia syndromes (included Beta Thalassaemia and Sickle Cell Disease) have been the first diagnosed diseases since in intrauterine life using reccombinant DNA techniques. So, the better understanding of their pathophysiology has given a spectacolar improvement and a considerable impact on these conditional managements. Every year there are nearly 300,000 children born with haemoglobinopathies globally, and there are 60,000 - 70,000 children with Beta Thalassemia among them. Nowadays in Albania like everywhere, there is a significant increase of survival in these patients. As a result of life longevity and improvement of patients life quality, we can see that these patients may suffer from other concomitant illnesses. In our country, there are registered approximately 500 patients with haemoglobinopathies. We studied 50 pediatric patients at random ranging from age 2 until 18 years old. We excluded other pathologies among them. We found high values of biochemical indicators in blood (Ca 15-3 was found elevated in 75% of our patients, Lactate Dehydrogenases was found elevated in 70% of cases, Indirect Bilirubin was found elevated in 66% of cases). All three parameters are indicators of hemolysis. We found a correlation between high values of Ca 15-3 marker and high levels of LDH, Indirect Bilirubin and low level of hemoglobin (p < 0.05). Ca 15-3 is much elevated among patients non regularly transfused and in those who take an unsufficient amount of blood. Continuous monitoring of these biochemical parameters is going to help in the more effective follow up of patients with haemoglobinopathies.
文摘Introduction: Sickle cell disease (SCD) causes chronic hemolysis which is a risk factor for cholelithiasis. Its development may lead to severe and life-threatening complications. Objective: Determine the prevalence of cholelithiasis, the conditions of diagnosis and related factors. Materials and Method: We retrospectively reviewed records of 110 patients with sickle cell disease followed up in Charles de Gaulle University Pediatric Hospital from January 2003 to December 2013, including 103 patients who had abdominal ultrasonography. Results: Cholelithiasis prevalence was 24.3%. The mean age of patients was 10.8 years, (range 3 to 15 years). Sex ratio was 2.1. In 88% cases, cholelithiasis was diagnosed based on the characteristic symptoms of right hypocondrial pain, fever and icterus. Most factors associated with cholelithiasis were as follows: age above 10 years (OR = 4), occurrence of at least three (03) vaso-occlusive crises per year (OR = 7.6), history of blood transfusion (OR = 8), right hypochondrial pain (OR = 4.5) and icterus (OR = 15). Only 20% of patients suffering from a symptomatic cholelithiasis underwent laparoscopic cholecystectomy and results were conclusive. Conclusion: Patients with sickle cell disease, especially those aged above 10, should be routinely tested for cholelithiasis using abdominal ultrasonography at least once a year. Because of the difficulties in managing evolutive complications in case of an emergency in our context, we advocate laparoscopic cholecystectomy of any cholelithiasisas soon as it is diagnosed in children with sickle cell disease.
文摘<span style="line-height:1.5;">Natural plant products have been used by the population of the south-eastern part of C</span><span lang="EN-US" style="line-height:1.5;">ô</span><span style="line-height:1.5;">te d’Ivoire in the management of sickle cell anemia. </span><span style="line-height:1.5;">This study was aimed at investigating the antisickling activity of the hydro-ethanolic extract </span><span "="" style="line-height:1.5;"><span>of a combination of the leaves of </span><i><span>J</span></i><span>. </span><i><span>secunda</span></i><span>, </span><i><span>J</span></i><span>. </span><i><span>gossypiifolia</span></i></span><span style="line-height:1.5;"> a</span><span "="" style="line-height:1.5;"><span>nd </span><i><span>P</span></i><span>. </span><i><span>nigrescens</span></i><span>.</span></span><span style="line-height:1.5;"> These three plants species were used in the Ivorian traditional herbal medicine. Preliminary phytochemistry was carried out using standard methods. As for the sickling reversal test, the Hb SS blood sickling was induced with 2% sodium metabisulfite. After 120 minutes of incubation, the plant extract was added. Every 30 minutes for a period of 120 minutes, a drop of the prepared solution was observed at (40</span><span style="line-height:1.5;">×</span><span "="" style="line-height:1.5;"><span>) magnification and the percentage of reversion calculated. The phytochemical analysis revealed the presence of Alkaloids, Flavonoids, Polyphenols, Catechic Tannins, Sterols and Polyterpenes. The results of the reversal test showed that the percentage of sickling reversal effect of the combination of the plants (75.00 ± 4.33</span><sup><span>b,c</span></sup><span>) was highly superior than the negative control (10.17 ± 0.55</span><sup><span>d</span></sup><span>) but was significantly (p < 0.05) the same as the percentage of the individual plant (</span><i><span>J</span></i><span>. </span><i><span>secunda</span></i><span>: 83.50 ± 2.33</span><sup><span>a</span></sup><span>;</span><i><span>J</span></i><span>. </span><i><span>gossypiifolia</span></i><span>: 78.00 ± 3.67</span><sup><span>b,c</span></sup><span> and </span><i><span>P</span></i><span>. </span><i><span>nigrescens</span></i><span>: 77.83 ± 2.89</span><sup><span>b,c</span></sup><span>) and the positive control (80.66 ± 2.22</span><sup><span>a,b</span></sup><span>). From the results, the extracts </span><i><span>J. secunda</span></i><span>, </span><i><span>J</span></i><span>. </span><i><span>gossypiifolia</span></i><span> and </span><i><span>P</span></i><span>. </span><i><span>nigrescens</span></i><span> have shown to be therapeutically beneficial to the population. Their use is also justified in the management of sickle cell disease in the south-eastern part of C</span></span><span lang="EN-US" style="line-height:1.5;">ô</span><span style="line-height:1.5;">te d’Ivoire.
文摘Introduction: Nigeria accounts for 150,000 infants born with the Sickle Cell Disease (SCD) every year. Children with SCD are affected by the deleterious effect of user fees which reduces affordability and utilization of health services. Evidences supported that institutionalized health insurance increases intensity of utilization. Methodology: The study was conducted at AKTH, Kano, and north-western, Nigeria. The study was retrospective comparative cross-sectional study. 100 patients were enrolled, 50 for each arm. Paediatric SCD clinic outpatients’ records and a specialty designed form containing the variables of interest were used. Data were analysed using Minitab 16. Proportions, percentages, tables, charts and chi squared test were used to compare the two groups. Result: The study found no association between the two groups in the likelihood of being insured based on age (χ<sup>2</sup> = 1.478), gender (χ<sup>2</sup> = 0.224) and dwellings (χ<sup>2</sup> = 0.062). On health services utilization and insurance status, the study revealed that follow up clinic visits, unscheduled clinic visits and emergency room visits were more likely among the insured group compared to the uninsured group. While the health status and insurance status of the two groups over 12 months period showed a significant association with the insured more likely to have improved health status compared to uninsured (χ<sup>2</sup> = 28.019, p = 0.0001). Similarly, health status and health services utilization were significantly associated with insurance status (χ<sup>2</sup> = 12.191, p = 0.002). Conclusion: The insurance status of children with SCD is associated with their health services utilization and health status. However, age, gender and dwellings were not associated with insurance status of children with SCD. Therefore, when expanding insurance coverage (NHIS) among children with SCD considerations have to be given to increase health services utilization and resultant improvement in health status as these have the potential of reducing morbidity and mortality among children with SCD.
