Myocardial injury in patients with sickle cell anaemia and myocardial ischaemia in Calabar, Nigeria.

Conclusions Most SCA children have an ischaemic ECG with elevated serum cTnT, especially during VOC, which suggests ischaemic-induced cardiac injury. However, elevated serum cTnT can occur without an ischaemic ECG and vice versa. Performing only electrocardiography or cTnT to detect ischaemia-induced cardiac injury may be misleading. Therefore, when there is a high index of suspicion, both tests should be undertaken, especially during VOC, to ensure prompt, effective treatment. Abbreviations AHC, apparently healthy children; AUC, area under the ROC curve; BMI, body mass index; cTnT, cardiac troponin T; ECG, electrocardiogram; EDTA, ethylene diamine tetra-acetic acid; GFR, glomerular filtration rate; HbS, haemoglobin S; HLA, human leucocyte antigen; LVH, left ventricular hypertrophy; ROC, receiver operating characteristic; SCA, sickle cell anaemia; UCTH, University of Calabar Teaching Hospital; URL, upper reference limit; USFDA, United States Food and Drug Administration; USNIH, United States National Institutes of Health; VOC, vaso-occlusive painful crisis; WHF, World Heart Federation. PMID: 32662752 [PubMed - as supplied by publisher]
Source: Paediatrics and international child health - Category: Pediatrics Tags: Paediatr Int Child Health Source Type: research

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CONCLUSIONS: There is no evidence for managing adults, or children who do not have HbSS sickle cell disease. In children who are at higher risk of stroke and have not had previous long-term transfusions, there is moderate quality evidence that long-term red cell transfusions reduce the risk of stroke, and low quality evidence they also reduce the risk of other sickle cell disease-related complications. In primary and secondary prevention of stroke there is low quality evidence that switching to hydroxyurea with phlebotomy has little or no effect on the liver iron concentration. In secondary prevention of stroke there is lo...
Source: Cochrane Database of Systematic Reviews - Category: General Medicine Authors: Tags: Cochrane Database Syst Rev Source Type: research
This study aims to investigate, retrospectively, the epidemiological and clinical characteristics, laboratory results, radiologic findings and outcomes of novel coronavirus disease-19 (COVID-19) in patients with transfusion dependent β thalassemia (β-thalassemia major-TM), non-transfusion dependent β thalassemia (β-thalassemia intermedia -TI) and sickle cell disease (SCD).Design, setting:A total of 17 Centers, from 10 countries, following 9,499 patients with hemoglobinopathies participated in the survey.Main outcome measures:Clinical, laboratory and radiologic findings and outcomes of patients with COVI...
Source: Mediterranean Journal of Hematology and Infectious Diseases - Category: Hematology Authors: Source Type: research
In conclusion, HbA reduces severity of HbS in HbS-β + thalassemia. HbS-HbD dis ease can manifest as a transfusion dependent illness. HbSS reduces severity of G6PD deficiency after first few years of life. HbSS and hemophilia coinheritance ameliorates symptoms of hemophilia.
Source: Indian Journal of Hematology and Blood Transfusion - Category: Hematology Source Type: research
The majority of sickle cell disease (SCD) patients suffer from numerous health problems like chronic pain and anemia. Oftentimes these patients require emergency health care, including unscheduled blood transfusions to treat or prevent severe complications accompanied with SCD. This poem explores a Black SCD patient’s experiences with implicit and explicit biases among health care providers as a person with this type of condition seeks emergency care. (PsycInfo Database Record (c) 2020 APA, all rights reserved)
Source: Families, Systems, and Health - Category: International Medicine & Public Health Source Type: research
This article reviews the evidence pertaining to crizanlizumab in SCD by searching records in Medline, Embase, and International Pharmaceutical Abstracts. Crizanlizumab, a P‐selectin inhibitor, mitigates the microvascular vaso‐occlusion in SCD. In the multi‐center, randomized, double‐blind SUSTAIN trial, a higher dose of crizanlizumab decreased the incidence of VOC by 45% and prolonged the median time to the first and second VOC. A post hoc subgroup analysis demonstrated that the proportion of patients who had no VOC incidence during the study period was greater in the crizanlizumab group, and this benefit was consi...
Source: Pharmacotherapy: The Journal of Human Pharmacology and Drug Therapy - Category: Drugs & Pharmacology Authors: Tags: REVIEW OF THERAPEUTICS Source Type: research
Authors: Cox SE, Hart E, Kirkham FJ, Stotesbury H Abstract L-Glutamine is a conditionally essential amino acid required for synthesis of the pyridines for nucleotides, including nicotinamide adenine dinucleotide (NAD) and glutathione, as well as glutamate, and becomes essential during oxidative stress exposure. The NADH:[NAD⁺ + NADH] (redox) ratio in sickle red blood cells (RBCs) is lower than in normal RBCs, consistent with oxidative stress, therefore glutamine availability is important in sickle cell disease (SCD). RBC glutamine levels vary between SCD studies but the ratio glutamine:glutamate was inversely rel...
Source: Drugs of Today - Category: Drugs & Pharmacology Tags: Drugs Today (Barc) Source Type: research
Sickle cell disease (SCD) is the most common monogenic disorder, affecting approximately 100,000 Americans, primarily those of African and Afro-Caribbean descent [1]. Patients with SCD are faced with decreased life expectancy (42 years for men, 48 years for women [2,3]) and severely impaired quality of life [1]. In addition to the symptoms of anemia and pain crises, patients with SCD struggle with various effects of their illness. Because of the high frequency of prepubertal mortality in the past, fertility had not been an area of focus in this population until fairly recently [4].
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Source Type: research
Sickle cell disease (SCD) is the most common monogenic disorder, affecting approximately 100,000 Americans, primarily those of African and Afrocaribbean descent [1]. SCD patients confront decreased life expectancy (42 for men, 48 for women [2,3]), and severely impaired quality of life [1]. In addition to the symptoms of anemia and pain crises, SCD patients struggle with a variety of effects of their illness. Because of the high frequency of pre-pubertal mortality in the past, fertility had not been an area of focus in this population until fairly recently [4].
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Source Type: research
CONCLUSION: This case highlights the utility of LT as a viable treatment option for acute sickle cell intrahepatic cholestasis. PMID: 32231764 [PubMed]
Source: World Journal of Hepatology - Category: Gastroenterology Tags: World J Hepatol Source Type: research
We present a new method to obtain erythrocyte shape classification using peripheral blood smear sample images. The aim of the method is to segment the cells, to separate clusters and classify cells (circulars, elongated and others). We compared our method with state-of the-art. Results showed that our method with is superior for the diagnosis support of sickle cell anemia.
Source: Medical and Biological Engineering and Computing - Category: Biomedical Engineering Source Type: research
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