Sickle Cell Patients Research Articles

Prevalence of Drug Abuse and its Risk Factors among Attendees of Healthcare Centers in Saudi Arabia: A Systematic Review

Objectives: To investigate the prevalence and associated factors of drug abuse among the Saudi population. Methods: To locate relevant material, we searched PubMed, SCOPUS, Web of Science, and Science Direct thoroughly. The Rayyan QRCI was used during the procedure. Results: We included six studies with a total of 33222 patients, and 20,803 (62.6%) were males. Drug abuse prevalence ranged from 2.4% to 58.8%. The most common drugs used were amphetamine-cannabis benzodiazepine, Amphetamines & cannabis, Stimulants & cannabis, Heroin & alcohol, Khat chewing, and Marijuna & narcotic drugs. Patients' age, smoking habits, past medical history, and age at the beginning of drug use, male gender unmarried people, and Saudi nationals, used drugs at substantially greater rates. Interestingly, participants with higher education levels were more likely to use drugs such as amphetamines and cannabis while lower educational attainment were more likely to use Khat. Conclusion: When it came to substance misuse, the general Saudi population that visited health facilities had a pretty high rate. In light of these findings, we recommend carrying out additional research to obtain more precise and pertinent information about the incidence of substance usage in Saudi Arabia. To determine the incidence of morphine abuse across various populations, including healthcare professionals and people with chronic pain—such as sickle cell patients—we advised conducting additional research.

Homozygous Sickle Cell Disease after Age of 40: Follow-Up of a Cohort of 209 Patients in Senegal, West Africa.

The aim of this study was to describe the morbidity and mortality of homozygous sickle cell disease after the age of 40. This was a cohort study of 209 patients followed from 1994 to 2022. All hemoglobin electrophoresis-confirmed SS sickle cell patients over 40 years were included. A descriptive study of epidemiological, diagnostic, therapeutic, and evolutionary data was used to assess morbidity and mortality. Sex ratio (M/F) was 0.6. Median age was 47 (41-75). According to morbidity, 95.1% had less than 3 vaso-occlusive crises/year. Acute anemia was the most frequent complication (52.63%). Chronic complications were noted in 32.5%. At diagnosis, mean hemoglobin was 8.1 g/dl ± 1.9, HbS was 86.5 ± 10, and HbF was 9.4 ± 7.6. Number of patients transfused was 66%. We noted that 8.1% of patients died, 29.2% were lost to follow-up, and 62.7% were still being followed up. The risk factors identified for death were geographical origin, comorbidity, high HbS, low HbF, and thrombocytosis. This study shows that homozygous SCD is increasingly becoming an adult disease and that it can be carried into old age in Africa. Advanced age over 40 is marked by an upsurge in chronic complications, making it essential to set up a screening program and to organize multidisciplinary follow-up.

(325) A Descriptive Analysis of Digital Resources for Educating Sickle Cell Patients About Priapism

Abstract Introduction Men with sickle cell disease (SCD, HbSS or HbSC hemoglobinopathy) are at increased risk of priapism via increased cell surface adhesion molecule expression and blunted vasodilatory responses to nitric oxide. Previous studies have suggested that priapism knowledge is poor among men with SCD; however, studies have yet to characterize public-facing priapism educational resources. Objective To describe key features of online educational resources targeted to priapism patients with SCD in the US. Methods In March 2023, we searched the following keywords and terms using the Google search engine and search features within Facebook, YouTube, and TikTok: “painful erection”, “priapism”, “priapism patient education”, “priapism for patients”, and “priapism sickle cell patient”. Searches were performed via a computer located in Manhattan, New York and in the English language only. Results Google search results differed considerably when searching “painful erection” versus “priapism” and are depicted in Table 1: the former directed users to telehealth commercial websites while the latter directed users to websites hosted by healthcare and professional organizations and medical reference sites which primarily discussed etiology and need for prompt diagnosis of the condition. YouTube search results overwhelmingly focus on discussion of diagnosis, pathophysiology, and management of priapism, and most of these appear designed for a medical or medically-literate audience; however, when querying for “priapism sickle cell patient”, users are directed to patient testimonial or documentary style videos. The results of a Facebook pages search seemed intended for student education and associated with low quality media material. An equivalent search performed on Tik Tok yields videos with noticeably different characteristics, namely: starring and created by “younger” urologists and hematologists, depicting a diverse cohort of providers and patients, written/spoken in Spanish and Portuguese, informal dialogue, increased use of Q&A format. Compared to YouTube videos, TikTok posts were more recent, received more views, and were associated with more comments. Conclusions Our exploratory search for priapism educational resources revealed differences in content, tone, and format between online sites and mobile platforms. Based on view and comment statistics, it appears as though mobile platforms such as TikTok have an increasing role in disseminating knowledge about priapism. However, it remains unclear as to whether individual modalities are well-suited to deliver information in a specific disease phase (e.g. preventative, acute, or chronic). Importantly, the impact of word-of-mouth and private provider-to-patient interactions cannot be estimated by this methodology. Disclosure No.

High level of apnea-hypopnea index is associated with an increase in vaso occlusive complications in sickle cell patients

High level of apnea-hypopnea index is associated with an increase in vaso occlusive complications in sickle cell patients

A comparative analysis of blood-borne infections among sickle cell anemia patients and first-time donors in Gabon

Managing sickle cell disease often requires transfusions, exposing multi-transfused sickle cell patients to a heightened risk of transfusion-transmitted infections. This study aimed to assess the seroprevalence of the human immunodeficiency virus (HIV), hepatitis B surface antigen (HBsAg), and hepatitis C virus (HCV) in multi-transfused sickle cell patients and first-time donors in Libreville. The serological testing for HBsAg, anti-HIV, and anti-HCV antibodies was conducted using commercial enzyme immunoassays and confirmed by the COBAS modular analyzer (Roche Diagnostics). The seroprevalence of HIV, HCV antibodies, and HBsAg was 4%, 10%, and 10%, respectively, in multi-transfused sickle cell patients and 5%, 0%, and 8% in first-time donors. Interestingly, HIV and HBsAg seroprevalence were similar in both groups, indicating that transfusion was not associated with these infections. However, HCV antibody seroprevalence was significantly higher in multi-transfused sickle cell patients than in first-time donors (10% vs. 0%, p<0.001). Furthermore, the presence of anti-HCV antibodies in multi-transfused sickle cell patients was significantly associated with the number of donations received (7.20±2.37 vs. 3.96±2.06, p=0.042). These findings suggest that while blood transfusion is not a significant risk factor for HIV and HBsAg transmission, it may increase the risk of HCV transmission, particularly in multi-transfused sickle cell patients.

Sickle Cell Disease Update: New Treatments and Challenging Nutritional Interventions.

Sickle cell disease (SCD), a distinctive and often overlooked illness in the 21st century, is a congenital blood disorder characterized by considerable phenotypic diversity. It comprises a group of disorders, with sickle cell anemia (SCA) being the most prevalent and serious genotype. Although there have been some systematic reviews of global data, worldwide statistics regarding SCD prevalence, morbidity, and mortality remain scarce. In developed countries with a lower number of sickle cell patients, cutting-edge technologies have led to the development of new treatments. However, in developing settings where sickle cell disease (SCD) is more prevalent, medical management, rather than a cure, still relies on the use of hydroxyurea, blood transfusions, and analgesics. This is a disease that affects red blood cells, consequently affecting most organs in diverse manners. We discuss its etiology and the advent of new technologies, but the aim of this study is to understand the various types of nutrition-related studies involving individuals suffering from SCD, particularly in Africa. The interplay of the environment, food, gut microbiota, along with their respective genomes collectively known as the gut microbiome, and host metabolism is responsible for mediating host metabolic phenotypes and modulating gut microbiota. In addition, it serves the purpose of providing essential nutrients. Moreover, it engages in direct interactions with host homeostasis and the immune system, as well as indirect interactions via metabolites. Nutrition interventions and nutritional care are mechanisms for addressing increased nutrient expenditures and are important aspects of supportive management for patients with SCD. Underprivileged areas in Sub-Saharan Africa should be accompanied by efforts to define and promote of the nutritional aspects of SCD. Their importance is key to maintaining well-being and quality of life, especially because new technologies and products remain limited, while the use of native medicinal plant resources is acknowledged.

Neighborhood Disadvantage and Healthcare Utilization in Pediatric Sickle Cell Patients

Background: Sickle cell disease (SCD) is a complex heritable blood disorder associated with higher acute healthcare utilization further complicated by psychosocial and socioeconomic factors. Other studies have indicated a relationship between neighborhood disadvantage and increased healthcare utilization. We hypothesize that those with greater neighborhood disadvantage will have greater acute healthcare utilization.
 Methods: Data was collected retrospectively from the electronic medical record between 9/30/2021 and 7/11/2023 for patients followed at Riley Hospital for Children’s Pediatric SCD Clinic who completed a psychosocial needs assessment (PAT). Number of emergency department (ED) visits, hospitalizations, and missed clinic visits were collected from the time the PAT was completed up to 1 year after (or the end of the data collection period). Data regarding area deprivation index (ADI) and childhood opportunity index (COI) were collected utilizing patient home address. Descriptive statistics were performed on all data above, as well as t-tests and chi square tests for univariate analyses.
 Results: 142 patients completed a PAT during the study period. 88% had a primary care provider documented, and 73% had public insurance. The median state and national ADI (6, 76.5) and COI (2, 2) demonstrated greater neighborhood disadvantage and less childhood opportunity. There was no statistically significant difference between ADI or COI and number of ED encounters or hospitalizations. However, patients who missed 1 or more SCD visits had a higher median state ADI than those who did not miss a visit (7.5 vs 5, p=0.002). Median state COI was also lower in this group than those who did not miss a visit (1 vs 2, p=0.001).
 Conclusion: Those with higher ADI and lower COI could benefit from more directed support to improve access to preventative care visits. Further analysis accounting for comorbidities (seizures, asthma, depression) could disambiguate a relationship between ADI or COI and acute care utilization.

Cerebrovascular and cardiovascular autonomic regulation in sickle cell patients with white matter lesions.

White matter lesions (WMLs) are frequent in sickle cell disease (SCD), with a prevalence described to be as high as 53% by age 30. Cerebrovascular regulation and cardiovascular autonomic regulation, more specifically the sympatho-vagal balance, can be altered in SCD. In this study the association between WMLs, cerebrovascular regulation and sympatho-vagal balance was assessed in SCD patients. Sickle cell disease patients with no history of stroke were prospectively evaluated for cerebrovascular reactivity using the breath-holding test (BHT), the sympatho-vagal balance (ratio low frequency/high frequency [HF]) using heart rate variability parameters and cerebral autoregulation in the time domain using correlation index Mx, and arterial cerebral compliance based on continuous assessment of cerebral blood flow velocities using transcranial Doppler ultrasound and arterial blood pressure with photo-plethysmography. WMLs were assessed with magnetic resonance imaging using Fazekas score grading and the presence of lacunes. Forty-one patients (F/M 25/16) were included. Median age was 37.5 years (19-65). Twenty-nine (70.7%) patients had SS genotype. Eleven patients had WMLs (26.8%). Patients with WMLs were significantly older (p < 0.001), had a lower HF (p < 0.005) and an impaired cerebral arterial compliance (p < 0.014). The receiver operating curve for the regression model including age and HF showed a higher area under the curve compared to age alone (0.946 vs. 0.876). BHT and Mx did not significantly differ between the two groups. Lower parasympathetic activity and impaired cerebral arterial compliance were associated with WMLs in adults with SCD. This could potentially yield to a better understanding of pathophysiological parameters leading to premature cerebrovascular ageing in SCD.

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

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

Total Hip Arthroplasty for Aseptic Osteonecrosis in Sickle Cell Patients at the Hospital General De Reference De Niamey / Niamey

Introduction: Aseptic osteonecrosis of the femoral head is one of the frequent bone sequelae of sickle cell disease. It is difficult and costly to treat. The aim of this study was to determine the complications and evaluate the functional results. Material and methods: This was a retrospective monocentric study including total hip replacements in sickle cell subjects (March 2015 to May 2019), excluding patients with severe comorbidity. The mean follow-up was 12 months. Results: During the study period, 17 patients with sickle cell disease underwent total hip arthroplasty for aseptic osteonecrosis of the femoral head. There were 10 women (58.82%) and 07 men (41.18%). The mean age of the patients, all sexes combined, at the time of surgery was 31 years. The distribution of the hemoglobinopathy phenotype was dominated by the SS homozygous form, accounting for 52.94% of patients. The mean preoperative PMA score was 8.41/18. Radiological classification by Ficat and Arlet showed that 82.35% of hips were stage 4 and 17.65% stage 3. Arthroplasty was performed via a posterolateral Moore approach under spinal anesthesia. Postoperative clinical re-evaluation showed marked improvement, with a mean postoperative PMA score of 17.17/18. Conclusion: The functional and radiological results obtained over time make total hip arthroplasty the treatment of choice for osteonecrosis of the femoral head in sickle cell disease.

Imaging of osteonecrosis of the femoral head in sickle cell patients: About 36 cases

Imaging of osteonecrosis of the femoral head in sickle cell patients: About 36 cases

Liver X receptor agonist upregulates LPCAT3 in human aortic endothelial cells.

Endothelial cells (ECs) play an important role in tissue homeostasis. Recently, EC lipid metabolism has emerged as a regulator of EC function. The liver X receptors (LXRs) are involved in the transcriptional regulation of genes involved in lipid metabolism and have been identified as a potential target in cardiovascular disease. We aimed to decipher the role of LXRs in the regulation of lipid metabolism in human aortic endothelial cells. Lipid composition analysis of endothelial cells treated with the LXR agonist T0901317 revealed that LXR activation increased the proportion of polyunsaturated fatty acids (PUFAs) and decreased the proportion of saturated fatty acids. The LXR agonist decreased the uptake of fatty acids (FAs) by ECs. This effect was abolished by LXRα silencing. LXR activation increased the activity and the expression of lysophosphatidylcholine acyltransferase, LPCAT3, which is involved in the turnover of FAs at the sn-2 position of phospholipids. Transcriptomic analysis also revealed that LXRs increased the expression of key genes involved in the synthesis of PUFAs, including FA desaturase one and 2, FA elongase 5 and fatty acid synthase. Subsequently, the LXR agonist increased PUFA synthesis and enhanced arachidonic acid, eicosapentaenoic acid, and docosahexaenoic acid content in the EC phospholipids. Modification of the FA composition of ECs by LXRs led to a decrease of arachidonate and linoleate derived prostaglandins synthesis and release. No change on markers of inflammation induced by plasma from sickle cell patient were observed in presence of LXR agonist. These results identify LXR as a key regulator of lipid metabolism in human aortic endothelial cells and a direct effect of LXR agonist on lysophosphatidylacyl transferase (LPCAT3).

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

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

Chronic intra-pelvic migration of an infected hip arthroplasty in a young male sickle cell patient

Chronic intra-pelvic migration of an infected hip arthroplasty in a young male sickle cell patient

Clinical Features and Outcome of Sickle Cell Patients in Pediatric Emergency Departments in Lubumbashi, Democratic Republic of Congo

Clinical Features and Outcome of Sickle Cell Patients in Pediatric Emergency Departments in Lubumbashi, Democratic Republic of Congo

Study of the Mineral Element Status in Sickle Cell Patients Attending the Mixed Medicine and Sickle Cell Anemia Center &amp;#8220;Yolo Mabanga&amp;#8221; in Kinshasa in Democratic Republic of the Congo

Study of the Mineral Element Status in Sickle Cell Patients Attending the Mixed Medicine and Sickle Cell Anemia Center &amp;#8220;Yolo Mabanga&amp;#8221; in Kinshasa in Democratic Republic of the Congo

SARS-CoV-2 infection associated with hemopathies: An experience of a clinical hematology center in sub-Saharan Africa, Senegal

Abstract: INTRODUCTION: Many studies have reported the association of SARS-CoV-2 with benign and malignant hemopathies. Data from African series are scarce. This work was conducted in sub-Saharan Africa and aimed to study the clinical, biological, and evolutionary features of hemopathies associated with this infection. MATERIALS AND METHODS: It was a retrospective, cross-sectional study carried out over 32 months including 86 patients with benign or malignant hemopathies who underwent coronavirus disease-2019 (COVID-19) confirmed by the real-time reverse transcriptase-polymerase chain reaction or presenting with atypical clinical signs associated with highly suggestive computed tomography (CT) scan signs. RESULTS: The mean age of patients was 48.3 ± 18.7 years with a sex ratio of 0.75. The main benign hemopathies were sickle cell trait (SCT) (n = 51), sickle cell disease SS (n = 8), and sickle cell disease SC (n = 1), while malignant hemopathies were represented by multiple myeloma (n = 5), non-Hodgkin lymphoma (n = 5), and chronic lymphocytic leukemia (n = 4). The clinical symptoms mainly featured anemic syndrome (16.3%) and a vaso-occlusive crisis was found in 9.3% of homozygous sickle-cell patients. The infection was moderate in 48% of cases and severe in 19.7%. The severe forms were commonly found in patients with malignant hemopathies (47.6%) and the benign forms were noted in benign hemopathies (38.4%). Full blood count outlined anemia in 32.5% and lymphopenia in 23.2% of cases. On imaging, the CT scan reported severe lesions in 41.3% of cases. The outcome resulted in full recovery in 76.7% of cases, and mortality occurred in 23.3%. In univariate analysis, death was mainly noted in patients with lymphoid hemopathies (15%). Comorbidities (P &lt; 0.0001), lymphoid hemopathies (P &lt; 0.0001), and the severity of COVID-19 (P &lt; 0.0001) had a positive impact on death occurrence in univariate analysis. CONCLUSION: The association between SARS-CoV-2 and hemopathy is not uncommon and is dominated by benign hemopathies. Malignant hemopathies are at-risk underlying conditions justifying a hospital follow-up of mild forms, allowing better survival. Particular attention must be paid to SCT with comorbidities and those with sickle cell disease of disease.

Performance evaluation of the Hemotype SCTM rapid test for the diagnosis of sickle cell disease

Introduction: The use of Rapid Orientation Diagnostic Tests ("RODTs") could play an important role in the mass screening of certain diseases. It saves a great deal of time while managing the flow of samples and the difficulties associated with their storage. This study aimed to identify the impact of the delay between sampling and analysis on the analytical performance of the Hemotype RODT SCTM. Methods: This was a descriptive cross-sectional study conducted over one month. The Immunology-Hematology Department of the CHU de Cocody served as the study setting. The study population consisted of sickle cell patients and hemoglobin C carriers of all ages and sexes. We performed hemoglobin electrophoresis on all samples on the day of collection (D0) and the Hemotype SCTM rapid test on D0, D2, D4, and D6. Sensitivity and specificity were used to determine analytical performance, and the chi2 test was used to compare qualitative variables. Results: The study involved 102 patients with a median age of 16 years (IQR: 9-24). Half of the participants (56.96%) were SSFA2 homozygous sickle cell disease major. On average, 87.1% of RODT results were valid from D0 to D6. At D0, the RODT had a sensitivity of 75% and a specificity of 81.40% for Hb A; Hb S had a sensitivity of 79.80% and a specificity of 100%; Hb C had a sensitivity of 35.71% and a specificity of 98.65%. The sensitivity of Hb C and the specificity of Hb A had increased statistically significantly over time. Cross-referencing the RODT results from D0 to D6 with those of electrophoresis showed a concordance of 86.95% for the AA phenotype, 79.52% for AS; 88.52% for SS; and 95.45% for SC. Conclusion: The HemoTypeSC™ test, with a sensitivity of 75% and specificity of 98% is suitable in certain areas where access to electrophoresis is limited. This performance is not impaired on samples stored for 6 days

A social profile of deaths related to sickle cell disease in India: a case for an ethical policy response.

India accounts for 14.5 percent of the global SCD newborns, roughly over 42,000 a year, second to sub-Saharan Africa. Despite the availability of cheap diagnostic and treatment options, SCD remains a largely neglected disease within healthcare policy and practice. Epidemiological modeling based on small, often dated, regional studies (largely from sub-Saharan Africa) estimate that between 50 and 90 percent of affected children will/die before the age of 5 years. This premise, coupled with targets of reducing under 5 mortality (SDG 4), privileges public health interventions for screening and prevention of new births, undermining investments in long-term health and social care. This paper presents a retrospective, descriptive analysis of the socio-demographic profile of 447 patients diagnosed with sickle cell or sickle-beta thalassemia, who died following admission at a tertiary care entre in India. We used anonymized hospital records of 3,778 sickle cell patients, admitted in pediatric and adult/medical wards between January 2016 and February 2021. A majority of hospital deaths occurred in the second and third decades of life, following a hospital admission for a week. The overall mortality during 2016-2019 was 14% with little gender difference over time. Contrary to our expectations, the number of hospital deaths did not increase during the first year of the COVID-19 pandemic, between 2020 and 2021. The conclusion highlights the importance of longitudinal, socio-demo-graphic data on deaths as providing important insights for identifying ethical policy interventions focused on improving SCD outcomes over time, reducing inequities in access to care, and preventing what might be considered "excess" deaths.

Maternal-Fetal Prognosis of Pregnancy and Childbirth in Sickle-Cell Patients at the Bernard Kouchner Community Medical Centre of Coronthie in Conakry (Guinea)

Maternal-Fetal Prognosis of Pregnancy and Childbirth in Sickle-Cell Patients at the Bernard Kouchner Community Medical Centre of Coronthie in Conakry (Guinea)