Decomposition experiments of 14C-labelled sickle alfalfa in chao soils of different texture and these soils after removal of CaCO3 were carried out under field and laboratory conditions respectively. The amount of res...Decomposition experiments of 14C-labelled sickle alfalfa in chao soils of different texture and these soils after removal of CaCO3 were carried out under field and laboratory conditions respectively. The amount of residual 14C in, or 14CO2 evolved from, the soils at intervals after the beginning of decomposition were measured and the distribution of native and labelled C between particle size fractions isolated from these soils was edtermined. Results showed that contents of both labelled (14C) and non-labelled (12C) carbon decreased with increasing particle size. The enrichment factor for 14C was higher than that for 12C in the clay fraction, the reverse being true for the silt enrichment factors. The effect of soil texture on the decomposition of plant material could not be observed in chao soils when the clay content was lower than 270g kg-1, while it became obvious once CaCO3 was removed from these soils. The decomposition rate of plant material in the soil from which the native CaCO3 was removed was correlated significantly to both the clay content of the soil and the application rate of CaCO3. A preliminary correction equation describing the effect of clay and CaCO3 on the decomposition of organic material in chao soil was derived from the results obtained.展开更多
Priapism is defined as a prolonged and persistent erection of the penis without sexual stimulation. This is a poorly understood disease process with little information on the pathophysiology of this erectile disorder....Priapism is defined as a prolonged and persistent erection of the penis without sexual stimulation. This is a poorly understood disease process with little information on the pathophysiology of this erectile disorder. Complications from this disorder are devastating due to the irreversible erectile damage and resultant erectile dysfunction （ED）. Stuttering priapism, though relatively rare, affects a high prevalence of men with sickle-cell disease （SCD） and presents a challenging problem with guidelines for treatment lacking or resulting in permanent ED. The mechanisms involved in the development of priapism in this cohort are poorly characterized; therefore, medical management of priapism represents a therapeutic challenge to urologists. Additional research is warranted, so we can effectively target treatments for these patients with prevention as the goal. This review gives an introduction to stuttering priapism and its clinical significance, specifically with regards to the patient with SCD. Additionally, the proposed mechanisms behind its pathophysiology and a summary of the current and future targets for medical management are discussed.展开更多
BACKGROUND Neonatal screening(NS)is a public health policy to identify genetic pathologies such as cystic fibrosis(CF),sickle cell disease,and other diseases.Sickle cell disea-se is the comprehensive term for a group ...BACKGROUND Neonatal screening(NS)is a public health policy to identify genetic pathologies such as cystic fibrosis(CF),sickle cell disease,and other diseases.Sickle cell disea-se is the comprehensive term for a group of hemoglobinopathies characterized by the presence of hemoglobin S.CF is an autosomal recessive multisystemic disease with pathophysiology involving deleterious mutations in the transmembrane re-gulatory gene that encodes a protein that regulates the activity of chloride and sodium channels in the cell surface epithelium.NS is crucial for early diagnosis and management,which ensures a better quality of life.AIM To report a case of the coexistence of sickle cell anemia(SCA)and CF and perform an integrative literature review.METHODS This is an observational study and a review of the literature focusing on two rare genetic pathologies identified simultaneously in NS from the perspective of a clinical case.The authors identified only 5 cases of SCA associated with CF.No clinical trials or review articles were identified considering the rarity of the coexistence of these two pathologies.RESULTS Herein,the authors reported the case of a girl who after undergoing NS on day 8 of life was diagnosed with SCA with an alteration in the dosage of immunoreactive trypsin.The diagnosis of CF was confirmed by the Coulometry Sweat Test.The rarity of the co-occurrence of these two severe genetic pathologies(CF and SCA)is a challenge for medical science.CONCLUSION This study adds to the few case reports present in the literature that highlight the identification of two severe diseases via NS.展开更多
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 hydroxyur展开更多
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 ...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.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
Research Background: Sickle cell trait has no treatment or cure and predominantly affects people who are Black, but can affect anyone of any race or ethnicity. While commonly incorrectly considered benign by providers...Research Background: Sickle cell trait has no treatment or cure and predominantly affects people who are Black, but can affect anyone of any race or ethnicity. While commonly incorrectly considered benign by providers and the public, people with a sickle cell trait experience life-threatening outcomes that are exacerbated by extreme conditions. There is a severe lack of awareness and understanding of sickle cell trait and the associated health complications among sickle cell trait carriers and healthcare providers. Purpose/Aim: Interventions that aim to improve awareness of sickle cell trait differ in approaches and are not well documented in the literature. This typology aims to highlight current efforts to inform targeted interventions that raise awareness through consistent messaging, educate people and providers on sickle cell trait and the related health complications, and support the design and implementation of comprehensive sickle cell trait awareness initiatives. Methods: We conducted a scoping review of United States-based sickle cell trait interventions and performed a content analysis to identify the categories and characteristics of these efforts. We then organized the results into a typology according to established protocols. Results: Among 164 interventions, twenty-five (15%) met the typology inclusion criteria described above and were grouped into categories: Seven of twenty-five interventions were Educational Interventions (28%), three of twenty-five interventions (12%) were Combined Screening and Educational-Based Interventions, eight of twenty-five interventions (32%) were Policy and Guideline-Based Intervention, and six of twenty-five interventions (24%) were Sickle Cell Trait Organization-Led Interventions. Conclusions: There is a lack of consistency in messaging across interventions whether delivered by credible healthcare institutions or national organizations, which can result in lack of education and awareness and confusion around sickle cell trait. Categorizing interventions through a t展开更多
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 ...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.展开更多
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 de...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 lar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
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 copp...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. .展开更多
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 t...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.展开更多
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 wi...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.展开更多
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 factor...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.展开更多
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 nutritio...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.展开更多
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...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. .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
Vertebral involvement in particular is common in sickle cell patients. We report 3 cases of “fish vertebra” fractures in sickle cell patients aged 16, 18, and 24 years old respectively at Laquintinie Hospital, Doual...Vertebral involvement in particular is common in sickle cell patients. We report 3 cases of “fish vertebra” fractures in sickle cell patients aged 16, 18, and 24 years old respectively at Laquintinie Hospital, Douala. When the vertebral fractures were diagnosed, the 3 patients had back pain and kyphosis deformities of the dorsal spine. Treatment with an infusion of biphosphonates (zoledronic acid at a dose of 0.5 mg·per·kg) was offered to all three patients. Two out of three patients received treatment with biphosphonates with a successful outcome. Profound vitamin D deficiency is associated with increased bone remodeling and a history of fractures. In sickle cell anemia, vertebral fractures may also result from bone fragility, which is often overlooked as aseptic osteonecrosis and osteomyelitis, which are very often suspected.展开更多
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 behav...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.展开更多
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-...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.展开更多
文摘Decomposition experiments of 14C-labelled sickle alfalfa in chao soils of different texture and these soils after removal of CaCO3 were carried out under field and laboratory conditions respectively. The amount of residual 14C in, or 14CO2 evolved from, the soils at intervals after the beginning of decomposition were measured and the distribution of native and labelled C between particle size fractions isolated from these soils was edtermined. Results showed that contents of both labelled (14C) and non-labelled (12C) carbon decreased with increasing particle size. The enrichment factor for 14C was higher than that for 12C in the clay fraction, the reverse being true for the silt enrichment factors. The effect of soil texture on the decomposition of plant material could not be observed in chao soils when the clay content was lower than 270g kg-1, while it became obvious once CaCO3 was removed from these soils. The decomposition rate of plant material in the soil from which the native CaCO3 was removed was correlated significantly to both the clay content of the soil and the application rate of CaCO3. A preliminary correction equation describing the effect of clay and CaCO3 on the decomposition of organic material in chao soil was derived from the results obtained.
文摘Priapism is defined as a prolonged and persistent erection of the penis without sexual stimulation. This is a poorly understood disease process with little information on the pathophysiology of this erectile disorder. Complications from this disorder are devastating due to the irreversible erectile damage and resultant erectile dysfunction （ED）. Stuttering priapism, though relatively rare, affects a high prevalence of men with sickle-cell disease （SCD） and presents a challenging problem with guidelines for treatment lacking or resulting in permanent ED. The mechanisms involved in the development of priapism in this cohort are poorly characterized; therefore, medical management of priapism represents a therapeutic challenge to urologists. Additional research is warranted, so we can effectively target treatments for these patients with prevention as the goal. This review gives an introduction to stuttering priapism and its clinical significance, specifically with regards to the patient with SCD. Additionally, the proposed mechanisms behind its pathophysiology and a summary of the current and future targets for medical management are discussed.
文摘BACKGROUND Neonatal screening(NS)is a public health policy to identify genetic pathologies such as cystic fibrosis(CF),sickle cell disease,and other diseases.Sickle cell disea-se is the comprehensive term for a group of hemoglobinopathies characterized by the presence of hemoglobin S.CF is an autosomal recessive multisystemic disease with pathophysiology involving deleterious mutations in the transmembrane re-gulatory gene that encodes a protein that regulates the activity of chloride and sodium channels in the cell surface epithelium.NS is crucial for early diagnosis and management,which ensures a better quality of life.AIM To report a case of the coexistence of sickle cell anemia(SCA)and CF and perform an integrative literature review.METHODS This is an observational study and a review of the literature focusing on two rare genetic pathologies identified simultaneously in NS from the perspective of a clinical case.The authors identified only 5 cases of SCA associated with CF.No clinical trials or review articles were identified considering the rarity of the coexistence of these two pathologies.RESULTS Herein,the authors reported the case of a girl who after undergoing NS on day 8 of life was diagnosed with SCA with an alteration in the dosage of immunoreactive trypsin.The diagnosis of CF was confirmed by the Coulometry Sweat Test.The rarity of the co-occurrence of these two severe genetic pathologies(CF and SCA)is a challenge for medical science.CONCLUSION This study adds to the few case reports present in the literature that highlight the identification of two severe diseases via NS.
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 hydroxyur
文摘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.
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Research Background: Sickle cell trait has no treatment or cure and predominantly affects people who are Black, but can affect anyone of any race or ethnicity. While commonly incorrectly considered benign by providers and the public, people with a sickle cell trait experience life-threatening outcomes that are exacerbated by extreme conditions. There is a severe lack of awareness and understanding of sickle cell trait and the associated health complications among sickle cell trait carriers and healthcare providers. Purpose/Aim: Interventions that aim to improve awareness of sickle cell trait differ in approaches and are not well documented in the literature. This typology aims to highlight current efforts to inform targeted interventions that raise awareness through consistent messaging, educate people and providers on sickle cell trait and the related health complications, and support the design and implementation of comprehensive sickle cell trait awareness initiatives. Methods: We conducted a scoping review of United States-based sickle cell trait interventions and performed a content analysis to identify the categories and characteristics of these efforts. We then organized the results into a typology according to established protocols. Results: Among 164 interventions, twenty-five (15%) met the typology inclusion criteria described above and were grouped into categories: Seven of twenty-five interventions were Educational Interventions (28%), three of twenty-five interventions (12%) were Combined Screening and Educational-Based Interventions, eight of twenty-five interventions (32%) were Policy and Guideline-Based Intervention, and six of twenty-five interventions (24%) were Sickle Cell Trait Organization-Led Interventions. Conclusions: There is a lack of consistency in messaging across interventions whether delivered by credible healthcare institutions or national organizations, which can result in lack of education and awareness and confusion around sickle cell trait. Categorizing interventions through a t
文摘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.
文摘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 lar
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. .
文摘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.
文摘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.
文摘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.
文摘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.
文摘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. .
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Vertebral involvement in particular is common in sickle cell patients. We report 3 cases of “fish vertebra” fractures in sickle cell patients aged 16, 18, and 24 years old respectively at Laquintinie Hospital, Douala. When the vertebral fractures were diagnosed, the 3 patients had back pain and kyphosis deformities of the dorsal spine. Treatment with an infusion of biphosphonates (zoledronic acid at a dose of 0.5 mg·per·kg) was offered to all three patients. Two out of three patients received treatment with biphosphonates with a successful outcome. Profound vitamin D deficiency is associated with increased bone remodeling and a history of fractures. In sickle cell anemia, vertebral fractures may also result from bone fragility, which is often overlooked as aseptic osteonecrosis and osteomyelitis, which are very often suspected.
文摘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.
文摘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.
