Promoting Sickle Cell Trait Awareness and Education: A Typology of Interventions in the United States to Inform Ongoing Efforts to Patients and 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 typology allows clarity and informs consistency in messaging, which should be at the forefront of future sickle cell trait efforts.

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

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

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

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

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

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

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

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

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

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

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

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

Sickle Cell Disease in the Zinder Region in 2023: Prevalence and Sociodemographic Factors

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.

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

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

“FISH VERTEBRA” about 3 Sickle Cell Patients Followed at Laquintinie Hospital, Douala

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.

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

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

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

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

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

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

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

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

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

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

Iron Metabolism Abnormalities in Children with Homozygous Sickle Cell Disease in Brazzaville

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. .

Factors Associated with Non-Adherence to Treatment in Sickle Cell Patients Monitored at the National Reference Center for Sickle Cell Disease in Niger

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.

Study of the Management of Children with Major Sickle Cell Disease in Pediatric Emergencies at the Gabriel Toure University Hospital

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.

Correlation between Pubertal Delay in Adolescents with Homozygous Sickle Cell Disease and Socio-Demographic, Clinical Factors

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.

Glomerular Filtration Rate of Children with Sickle Cell Disease Compared to Non-Sickle Cell Patients in Donka Pediatric Emergencies and SOS Drepano-Guinea Center

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.