Blood Transfusions For Survival Research Articles

Progress and development of CRISPR-Cas tool in the treatment of thalassemia

-thalassemia is the worlds major hemoglobin disorders. It is a hereditary chronic hemolytic anemia caused by a break in the -globin peptide chain genes of hemoglobin, resulting in reduced or complete absence of synthesis of the -globin peptide chains. Patients with -thalassemia rely on blood transfusions for survival, which exacerbates the disease burden on national economies and the financial burden on patients in developing countries. Repeated blood transfusions can also lead to organ damage. The fetuss protein genes are usually silenced early and then replaced by adult protein genes. But mutations that cause gammaglobin to persist in the fetus can improve the electrocution of globin. Therefore, reactivating the protein genes of the fetus is the core of research. CRISPR/Cas9 is the common way to reduce the effects of disease by correcting disease-causing mutations, increasing or disrupting protein expression. CRISPR/Cas9 and considered effective new methods for generating hemoglobin. Hence, the main focus of this review is to explore the details of CRISPR/Cas9 in-thalassemia treatment in the context of Mediterranean -thalassemia.

Thalassemia Screening by Sentiment Analysis on Social Media Platform Twitter

Thalassemia syndrome is a genetic blood disorder induced by the reduction of normal hemoglobin production, resulting in a drop in the size of red blood cells. In severe forms, it can lead to death. This genetic disorder has posed a major burden on public health wherein patients with severe thalassemia need periodic therapy of iron chelation and blood transfusion for survival. Therefore, controlling thalassemia is extremely important and is made by promoting screening to the general population, particularly among thalassemia carriers. Today Twitter is one of the most influential social media platforms for sharing opinions and discussing different topics like people’s health conditions and major public health affairs. Exploring individuals’ sentiments in these tweets helps the research centers to formulate strategies to promote thalassemia screening to the public. An effective Lexiconbased approach has been introduced in this study by highlighting a classifier called valence aware dictionary for sentiment reasoning (VADER). In this study applied twitter intelligence tool (TWINT), Natural Language Toolkit (NLTK), and VADER constitute the three main tools. VADER represents a gold-standard sentiment lexicon, which is basically tailored to attitudes that are communicated by using social media. The contribution of this study is to introduce an effective Lexicon-based approach by highlighting a classifier called VADER to analyze the sentiment of the general population, particularly among thalassemia carriers on the social media platform Twitter. In this study, the results showed that the proposed approach achieved 0.829, 0.816, and 0.818 regarding precision, recall, together with F-score, respectively. The tweets were crawled using the search keywords, “thalassemia screening,” thalassemia test, “and thalassemia diagnosis”. Finally, results showed that India and Pakistan ranked the highest in mentions in tweets by the public’s conversations on thalassemia screening with 181 and 164 tweets, respectively.

A Systematic Review on the Management of Transfusion-Related Acute Lung Injury in Transfusion-Dependent Sickle Cell Disease.

The onset of respiratory distress and acute lung injury (ALI) following a blood transfusion is known as transfusion-related acute lung injury (TRALI), although its pathophysiology remains unknown. Even though sickle cell disease (SCD) has been studied for more than a century, few therapeutic and management strategies adequately address the emergence of TRALI. TRALI, an immune-mediated transfusion response that can result in life-threatening consequences, is diagnosed based on clinical signs and symptoms. Early detection and treatment increase the chances of survival and, in most cases, result in a complete recovery. Our objective is to provide a firm grasp of the present status of SCD-related TRALI care and therapy.After exploring multiple databases, this study offers evidence-based guidelines to aid clinicians and other healthcare professionals make decisions concerning transfusion assistance for SCD and the management of transfusion-related complications. Other risk factors for acute lung injury including sepsis aspiration should be ruled out throughout the diagnostic process. Several recent studies have shown that immunotherapy or immunological targets can effectively prevent these complications. Red cell transfusions, red cell antigen matching optimization, and iron chelation can also help reduce negative consequences. It is to be noted that poor clinical outcomes can be avoided by early detection and treatment of hemolytic transfusion reactions. Finally, preventing the onset of TRALI may be the most effective therapeutic strategy for SCD patients who rely on blood transfusions for survival.

Current status of beta-thalassemia and its treatment strategies.

BackgroundThalassemia is an inherited hematological disorder categorized by a decrease or absence of one or more of the globin chains synthesis. Beta‐thalassemia is caused by one or more mutations in the beta‐globin gene. The absence or reduced amount of beta‐globin chains causes ineffective erythropoiesis which leads to anemia.MethodsBeta‐thalassemia has been further divided into three main forms: thalassemia major, intermedia, and minor/silent carrier. A more severe form among these is thalassemia major in which individuals depend upon blood transfusion for survival. The high level of iron deposition occurs due to regular blood transfusion therapy.ResultsOverloaded iron raises the synthesis of reactive oxygen species (ROS) that are noxious and prompting the injury to the hepatic, endocrine, and vascular system. Thalassemia can be analyzed and diagnosed via prenatal testing (genetic testing of amniotic fluid), blood smear, complete blood count, and DNA analysis (genetic testing). Treatment of thalassemia intermediate is symptomatic; however; it can also be accomplished by folic supplementation and splenectomy.ConclusionThalassemia major can be cured through regular transfusion of blood, transplantation of bone marrow, iron chelation management, hematopoietic stem cell transplantation, stimulation of fetal hemoglobin production, and gene therapy.

RENAL DYSFUNCTION AND ELECTROLYTE IMBALANCE IN CHILDREN WITH BETA-THALASSEMIA MAJOR.

Background: Beta-thalassemia major (β-TM), is a type of chronic, microcytic anaemia that is characterized by impaired biosynthesis of the β-globin chain leading to accumulation of unpaired α-globin chain. Due to the severe anaemia caused in Thallassemia, patient must undergo repeated blood transfusions for survival. Repeated blood transfusions lead to built-up of iron overload which is responsible for tissue deposition of excess iron, ultimately resulting in iron toxicity. There are evidences of aminoaciduria, hypercalciuria, phosphaturia, magnesiuria, hyperuricosuria, low urine osmolality, and excess urinary secretion of markers of tubular damage such as N-acetyl-D-glucosaminidase in patients with beta-thalassemia major. In the present study, authors tried to investigate the proportion of patients diagnosed with β-Thalassemia Major manifesting renal dysfunction and electrolyte imbalance.
 Material and Methods: It was a hospital based case-control study involving 100 patients coming to Department of Pediatric Medicine, and belonging to the age group 1 to 18 years. Equal number of healthy age and sex matched children were taken as controls.
 Results: When cases and controls were compared with respect to albuminuria, blood pH, serum bicarbonate, phosphorus and blood urea, higher number of cases had deranged values as compared to the healthy controls [p<0.05].
 Conclusion: Renal damage and tubular dysfunctions leading to eletrolyte imbalances exist in children with β-thalassemia major.
 Keywords: kidney, Thalassemia, Electrolytes, children.

Non-transfusion Dependent Thalassemias: A Developing Country Perspective.

Non-transfusion-dependent thalassemias (NTDT) encompass a group of hereditary chronic hemolytic anemia, which, as the name indicates, not require regular blood transfusion for survival. These include β-thalassemia intermedia, hemoglobin E/β-thalassemia, and Hemoglobin H disease (α- thalassemia intermedia). Individuals with structural variant of hemoglobin especially Hemoglobin S and Hemoglobin C associated with "α" or "β" thalassemia in heterozygous condition may also present with similar features of NTDT. NTDT patients are not immune to the development of transfusion unrelated complications in the long run. These hereditary chronic hemolytic anemias are still under-recognized in developing countries like India, where the disease burden might be high causing significant morbidity. The pathophysiologic hallmark that characterizes this group of disorders (ineffective erythropoiesis, hemolysis, chronic anemia) leads to a number of serious complications, similar to transfusion dependent thalassemia. So, timely diagnosis and institution of appropriate preventive/remedial measures as well as education of patient population can help decrease the morbidity to a significant extent. In the present review, focus will be on the pathophysiological mechanisms and available management options of NTDT from a developing country perspective like India.