Sickle Cell Disease - Europe PMC

Does glucose-6-phosphate dehydrogenase deficiency worsen the clinical features of sickle cell disease? A multi-hospital-based cross-sectional study.

"Background The effect of glucose-6-phosphate deficiency on the clinical course of sickle cell disease is also variable. " The aim of this study was to determine the prevalence of G-6-PD deficiency in patients with SCD and its effects on their clinical course. Methods A cross-sectional analysis of 122 SCD patients and 211 healthy blood donors was carried out in Kisangani city. 36. 2% vs. 33. 6% respectively. No significant difference was observed when comparing the hemoglobin levels between SCD patients with and without G-6-PD deficiencies. However, in the six months leading up to the study, SCD patients with G-6-PD deficiency had on average more transfusions than non-deficient SCD patients, 0. 64 vs. 0. 04; 0. 96 vs. 0. 04 u00b1 0. 36. Conclusion The prevalence of G-6-PD deficiency in SCD patients was high, but did not differ from that observed in controls. ".

Source link: https://europepmc.org/article/MED/35617172

Attitudes and practices of unmarried adults towards sickle cell disease: emergent factors from a cross sectional study in Nigeria's capital.

"Introduction Sickle cell disease is a genetic autosomal blood disorder that causes red blood cells to turn hard and crescent shaped. • The overall mean score for attitude towards sickle disease was 6. 60 u00b1 2. 583. Female participants exhibited a positive attitude toward sickle cell disease, while older participants reported a positive outlook toward the disease, and education may have influenced perception of the disease. More than a tenth of the sample revealed that they would marry someone with sickle cell disease irrespective of their own genotype. Although two-thirds of the study participants stated that they would prioritize sickle cell screening in their families, a third of them expressed a contrary viewpoint. Conclusions from this report will help government and other interested stakeholders in implementing contextual policies and practices that would reduce the burden of sickle cell disease.

Source link: https://europepmc.org/article/MED/35430953

Higher hydroxyurea adherence among young adults with sickle cell disease compared to children and adolescents.

"Objective": "Objective To determine the connection between HU adherence to health care use and patients' characteristics. " Patients with chronic pain had reduced HU adherence, which was lower. In a subgroup analysis of only HbSS patients, similar trends in HU adherence and health care utilization were observed. Conclusions Young adults with SCD had significantly higher HU adherence than children and adolescents. Patients with lower HU adherence and/or chronic pain had an elevated health care use, according to increased health care utilization. Patients with high adherence to HU adherence and/or those without chronic pain had significantly higher HU adherence in SCD, as shown by their lab results, but not to children and adolescents. There were no studies that showed a clear connection between HU adherence and patients with elevated HU adherence and/or those without persistent pain who had higher HU adherence and/or those without persistent pain in SCD.

Source link: https://europepmc.org/article/MED/35234095

Overview of current progress and challenges in diagnosis, and management of pediatric sickle cell disease in Democratic Republic of the Congo.

"Objectives Sickle cell disease largely affects health conditions in childhood, mainly due to the hematologic system and contributing to high mortality rates in childhood. " SCD prevention, diagnosis, and treatment must be multidimensional, as a rule. Conclusions The laboratory diagnosis of SCD has progressed from traditional electrophoresis to rapid point-of-care tests that allow early neonate screening. HemoTypeSC TM is a cheap way to perform neonatal screening in the United Kingdom. According to the method used and the ethnic group in question, pediatric SCD prevalence in Sub-Saharan Africa was within 1-7 percent of homozygous and 15-40% of the heterozygous SCD. Conclusion "Despite some strides, the disease is still difficult in Sub-Saharan Africa due to limited early diagnostic testing and a shortage of specific medications. ".

Source link: https://europepmc.org/article/MED/35068390

Prevalence of venous thromboembolism and its associations in a large racially homogenous population of sickle cell disease patients.

"To determine the incidence of venous thromboembolism among adult sickle cell disease patients in N. . . . ia, he needs to determine the presence of venous thromboembolism. " Methods This was a multicentre retrospective review in which adult SCD patients' medical records were reviewed. 10 of the 509 SCD patients had VTE for a median duration of 2 years, with a median duration of 2 years. VTE was strongly linked to the following events: acute chest disease [p=0. 002, odds ratio 8, 95% CI, 1. 45-18. 91], and a vaino-occlusive crisis [p=0. 035]. Conclusion VTE among SCD patients in N. . . . ia is low. ".

Source link: https://europepmc.org/article/MED/35687045

Kimura Disease: An Unusual Presentation of Parotid Mass in a Sickle Cell Disease Patient

"Kimura disease is a rare benign chronic inflammation disease of unknown origin," the Asian race's young men are the first victims. " In this essay, we feature unusual occurrence of KD in a 40-year-old Saudi man with sickle cell disease who had swelling on the right side of his face. ".

Source link: https://europepmc.org/article/MED/PMC9167805

Sex as an Independent Risk Factor for Venous Thromboembolism in Sickle Cell Disease: A Cross-Sectional Study.

"Venous thromboembolism affects up to 25% of people with sickle cell disease, but risk factors are not well defined. " We wanted to determine the prevalence of VTE in SCD patients in our health care system and to identify the relationship between medical history, biological sex, and VTE. Between June 2014 and June 2019, we conducted a retrospective chart review of SCD patients who attended an outpatient hematology clinic within Penn Medicine between June 2014 and June 2019. 147 patients with SCD who were identified had a history of VTE; 100 were female and 47 were male. About one-quarter of the SCD patients in our health system had a history of VTE, with some patients reporting significantly higher rates than in the general population. Females had twice the chance of VTE compared to males, highlighting an important sex difference in SCD disease outcomes and raising concerns about optimal pregnancy and condomisation for females with SCD. ".

Source link: https://europepmc.org/article/MED/35675680

Suboptimal vancomycin levels in critically ill children with sickle cell disease and acute chest syndrome.

"Objectives" are a term used to determine the effects of glomerular hyper-filtration relating to sickle cell disease on vancomycin clearance and later on therapeutic drug levels in children admitted to the pediatric intensive care unit with acute chest syndrome. Results Twelve SCD patients with ACS and vancomycin were compared to 36 non-SCD patients. The ACS patients had significantly lower initial serum VTL compared to the control group, and their average VTL was still lower post-vancomycin dose adjustment despite being lower. The ACS patients' survival time was significantly longer than those in the control group's. The AUC was also significantly lower in the ACS patients than in the control group. Hence, the common dosing regimen for vancomycin in ACS patients may be ineffective. ".

Source link: https://europepmc.org/article/MED/35688759

Voxelotor for the treatment of sickle cell disease in pediatric patients.

"Introduction Sickle cell disease is the result of a series of hereditary blood disorders related to sickle hemoglobin polymerization. " In December 2021, Voxelotor was approved for the treatment of SCD in patients aged u22654 years. SCD-related clinical evidence is reviewed. Ongoing research into the pharmacokinetics and safety profile of voxelotor in pediatric patients is compared to similar clinical results in adult SCD. Expert opinion: Voxelotor provides a unique therapeutic option to address EOD's root causes and can potentially be used alongside other SCD drugs.

Source link: https://europepmc.org/article/MED/35671094

Retention of functional mitochondria in mature red blood cells from patients with sickle cell disease.

"Sickle cell disease is an inherited blood disorder that is more susceptible to haemolysis and can contribute to disease pathophysiology. " Patients with SCD can receive RBC transfusion, while SCD patients with SCD have heightened risks of alloimmunization. We speculated that RBCs from patients with SCD have functionally active mitochondria and can elicit a type 1 interferon reaction. In comparison to healthy blood donors, we examined blood samples from more than 100 patients with SCD and discovered elevated frequencies of mitochondria in reticulocytes and mature RBCs. The coculture of mitochondria-positive RBCs with neutrophils stimulated the production of type 1 interferons, which have been shown to raise RBC alloimmunization rates. These results show that mitochondria stored in mature RBCs are safe and can elicit immune responses, implying that inadequate retention of mitochondria in RBCs may play underappreciated role in SCD challenges and may be a RBC alloimmunization risk factor. ".

Source link: https://europepmc.org/article/MED/35670632

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