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Sickle Cell Disease - DOAJ

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Last Updated: 12 June 2022

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Clonal Hematopoiesis and the Risk of Hematologic Malignancies after Curative Therapies for Sickle Cell Disease

"In individuals with SCD, two large population studies showed an elevated risk of leukemia. " However, the risk of leukemia in SCD has been elevated after graft rejection and gene therapy. After graft rejection and gene therapy for SCD, we wonder why patients with SCD are at an elevated risk of CH and explain why leukemia prevalence is unexpectedly high. Given our current findings, we recommend advising patients on leukemia risk and discussing the benefits of an individualized risk/risk assessment that factors leukemia risk in patients receiving curative therapies for SCD.

Source link: https://doi.org/10.3390/jcm11113160


Long-Term Health Effects of Curative Therapies on Heart, Lungs, and Kidneys for Individuals with Sickle Cell Disease Compared to Those with Hematologic Malignancies

"The aim of curing children and adults with sickle cell disease is to maximize benefits and minimize intermediate and long-term adverse outcomes so that people can live an average life span with a high quality of life. " While more than 2000 people with SCD have been treated with curative therapy, no systematic studies have been done to investigate the long-term health effects of hematopoietic stem cell transplant in this population. These studies have the most reliable information to extrapolate the potential late health risks after curative therapy for SCD. ".

Source link: https://doi.org/10.3390/jcm11113118


In-Depth Immunological Typization of Children with Sickle Cell Disease: A Preliminary Insight into Its Plausible Correlation with Clinical Course and Hydroxyurea Therapy

"Sickle cell disease is a disorder of functional hypo-/a-splenism in which predisposition to bacterial infections is just a symptom of a broad range of immune-dysregulation disorders that have resulted in the clinical manifestation of a peculiar immunophenotype. " Comparisons between SCD patients and controls were revealed by chi-squared tests, t-tests, and Pearson's correlation analysis of clinical and immunological parameters. With memory, B-cell proliferation, and IgG expansion, the SCD HU2212 subgroup from controls has significant differences between the SCD HU+ group and controls, with nau00efve T cells, switched-memory B cells, and IgG expansion, separating the SCD HU2112 subgroup from controls. "The mean CD4+ central-memory T-cell% ratio was the single independent variable in the SCD group with a positive correlation with vaso-occlusive crisis score in the SCD group, with a positive correlation. ".

Source link: https://doi.org/10.3390/jcm11113037


The Effects of Sickle Cell Disease on the Quality of Life: A Focus on the Untold Experiences of Parents in Tanzania

"Tanzania is one of the world's top five countries with a high incidence of sickle cell disease. " Despite the fact that SCD's effects on quality of life have been documented in other countries, including N. . . . ia and the United States of America, only a few are known from Tanzania. Hence, this report focused on determining the consequences of SCD on children living with SCD and their parents' quality of life. The study used a qualitative method to interview prospective parents of children with SCD and who have used hydroxyurea for more than three years. In Dar-es-salaam, Tanzania, in-depth interviews were conducted with 11 parents of children with SCD at the Muhimbili University of Health and Allied Sciences. SCD and their parents' children have psycho-social, financial, and physical consequences of the illness.

Source link: https://doi.org/10.3390/ijerph19116871


Hematopoietic Stem Cell Gene-Addition/Editing Therapy in Sickle Cell Disease

"Autologous hematopoietic stem cell-targeted gene therapy provides a one-time cure for several inherited disorders, including sickle cell disease and u03b2-thalassemia. " Since SCD is the most common single-gene disease, curing SCD is a primary aim in HSC gene therapy. Patient CD34+ HSCs are genetically modified through addition of a therapeutic b2-globin gene with lentiviral transduction and autologous transplantation. Gene therapy in SCD using gene mutation and gene editing are among the topics addressed in this report.

Source link: https://doi.org/10.3390/cells11111843


Preventive measures of vaso-occlusive crisis among sickle cell disease patients in South-Eastern Nigeria: How much do our patients know?

"Knowledge and use of preventive steps of VOC by patients can reduce the risk of morbidity and mortality. " Results: A total of 154 SCD patients participated in the study, with a male-to-female ratio of 1: 1. In SCD, the majority of the respondents had a keen awareness of preventive steps against VOC. While 95 had fair activity, this report also revealed that 59 participants had a strong record of preventive steps against VOC. Although not statistically significant, the majority of those who had good practice of preventive steps were less likely to experience more than three crises in a year than those who do not have good preventive steps, although not statistically significant. Health education for sickle cell patients is a vital tool that may reduce morbidity and mortality by reducing the frequency of crises.

Source link: https://doi.org/10.4103/NJM.NJM_17_22


Fulminant dengue hepatitis in sickle cell disease: Recovery against the odds

"Diagnostic liver enzyme derangement may also affect the liver, as well as severe hepatic dysfunction. " This case series discusses the first ever association of fulminant hepatit hepatic disease as a result of the synergistic effects of dengue fever with sickle cell disease, and thus emphasizes the importance of preventing potentially lethal hepatit dysfunction in sickle cell disease patients.

Source link: https://doi.org/10.4103/jfmpc.jfmpc_2058_21


The prevalence of cumulative alloimmunization in patients with sickle cell disease at King Fahad University Hospital

A deletion in the beta-globin gene causes sickle cell disease. " Management's Red blood cell transfusion is considered the keystone of management. AIMS AND OBJECTIVES: The aim of this research was to determine the incidence of alloimmunization among SCD patients and its association with other important risk factors in order to provide recommendations for the care of SCD patients. RESULTS: One hundred and seven out of 556 SCD patients developed alloantibodies with a prevalence of. In 33 of the alloimmunized SCD patients, Anti-E was the most recognized alloantibody in 37 patients, followed by anti-K. We found a statistically significant correlation between the alloimmunization and age of the disease and immediate antiglobulin test positivity. All SCD patients should perform extended RBC phenotyping at the earliest opportunity, including the following RBC antigens identified either serologically or by genotyping. ".

Source link: https://doi.org/10.4103/joah.joah_122_21


Relationship between genotype variants and the age of first acute splenic sequestration in patients with sickle cell disease in a tertiary center of Saudi Arabia: A retrospective study

"We wanted to find a connection between the age of the first splenic sequestration episode and SCD variants. " METHODS: We conducted a retrospective chart review of SCD patients ages 226412 years admitted to the Department of Pediatric Hematology King Saud Medical City, Riyadh with the first episode of ASSC and no other complications from January 2014 to December 2019. RESULTS: In this analysis, we included 47 patients with SCD and first ASSC. Patients in the first episode of splenic sequestration occurred for 3. 26 years, according to the mean age of the patients. ASSSC recurrence decreased by 66% of patients using hydroxyurea in 19. 1% of patients. CONCLUSION: The study found that the HBSS version of SCD had the earliest appearance with the first episode of ASSC, but there was no statistical difference," the report concluded.

Source link: https://doi.org/10.4103/joah.joah_244_20


Health-related quality of life of adolescents with sickle cell disease on hydroxyurea: A case-control study

"The aim of the investigation was to determine the health-related QoL of patients with SCD on hydroxyurea and the causes affecting HRQoL domains. " MATERIALS AND METHODS: This case-control research included 174 patients with SCD; 87 of them were on HU for at least 1 year; and 85 were not receiving HU; 87 were not receiving HU. P 0. 001 for patients with SCD who were not on HU were found to have significantly lower SF-36 v2 scores than those with HU and control group scores, with significantly lower SF-36 v2 scores. When patients on HU had significantly lower SF-36 v2 scores in physical fitness, role physical, general health, and physical health, according to the control group, P 0. 001. Significant correlations were found between the duration of HU therapy and RP and mental health component scores, as well as between hemoglobin levels with social function," according to a multivariate linear regression study.

Source link: https://doi.org/10.4103/joah.joah_7_21

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions