Bone status and HCV infection in thalassemia major patients.

Background Peripheral neuropathy is a controversial but serious complication of beta-thalassemia (β-Th). Although few studies have reported no relationship between neuropathy and thalassemia, many have linked it with increasing age, iron overload, and iron chelator toxicity. This study aims to investigate the presence of neuropathy in β-Th using corneal nerve fibers. Methodology A cross-sectional study was conducted using corneal confocal microscopy on individuals with intermediate and major β-Th who were compared to healthy individuals. The main outcome variables were corneal main nerve and branch nerve densities which were calculated using Image J software. The comparison between groups was done using the independent-samples F-test and Bonferroni post-hoc analysis. Results There was reduced corneal main nerve and branch nerve density in β-Th intermediate and major patients compared to the control group, and the results were statistically significant (p-value <0.05). However, a significant correlation was not observed between serum ferritin levels and corneal nerve parameters. Conclusions The reduction in corneal nerve parameters in β-Th patients compared to healthy controls can be an indication of peripheral neuropathy in β-Th. Further work is needed to confirm these findings.

https://assets.cureus.com/uploads/original_article/pdf/123950/20230101-6217-rr902t.pdf

Peripheral Neuropathy in Beta-Thalassemia: Corneal Confocal Microscopy-Based Study

Management of Hepatitis C in Children — A New Paradigm

Thalassemia is a genetic blood disorder inherited from the parent with unusual production of hemoglobin, infectious complications, immune abnormalities and iron overload with related to  organ damage that consider a chief causes of morbidity and mortality.  So this study aimed to evaluate the important causative agents and the role of IL-10 gene polymorphism in progressive disease. A Case-control study was performed to 46 Beta-thalassemia patients attended to inherited blood center in AL-Zahraa teaching hospital with group of 30 healthy individual as control. Blood sample was collected from all patients and control. IL-10 level were measured by enzyme-linked immunosorbent assay (ELISA). Singule nucleotide polymorphism detected by ARMS-PCR technique. The result shown that  46(46% ) patients with thalassemia , the male higher than female  with frequency (65%), according to infection  32(70%) were TTI with HCV a most common pathogen 19(41 %).This study  explain that serum level of IL-10 is significantly higher among patients with thalassemia (98±27.53)pg/ml compared to healthy control (10.67±1.49) pg\ml and was higher in HCV infected patients  (121.56 ±32.60)pg/ml than  non-infected with HCV record ( 73.61± 18.29)  pg/ml. 

Evaluate IL-10 level and gene polymorphism in β-Thalassemia patients with related to microbial infection and disease outcome

Background: Hemolytic anemia is widespread in Pakistan (2-5% prevalence), primarily due to glucose-6-phosphate dehydrogenase deficiency. Caregiver knowledge is crucial for improving the quality of life of affected children. Educating and supporting caregivers is essential for optimizing care. Objective: This study aimed to evaluate the impact of an educational intervention on caregiver knowledge regarding child care with inherited hemolytic anemia. Methods: An experimental design was conducted at children hospital Lahore, involving caregivers of children diagnosed with inherited hemolytic anemia. The educational intervention comprised structured sessions focusing on understanding the disease, its management, and strategies to enhance the quality of life for affected children. A two group's base study was conducted using standardized measures to gauge caregiver knowledge regarding the disease. Results: The research revealed that a substantial proportion of mothers were between the ages of 29 and 38, had completed secondary education. Furthermore, a significant number of affected children were in the 1-3 age range, mostly boys. There was a notable difference in mean scores of maternal knowledge between the control group (8.78+2.254) and the intervention group (10.83+1.669). Conclusion: Educational interventions empower caregivers to support children with inherited hemolytic anemia, enhancing management and quality of life outcomes. These initiatives are vital in chronic disease management, necessitating further research to investigate their long-term impacts and sustainability across diverse healthcare settings. 

Impact of educational intervention on caregiver knowledge regarding child care with inherited hemolytic anemia

A Second Australian Family with Hemoglobin North Shore (β134 Val→glu)

Background Thalassemia is a hereditary hemolytic anemia marked by a defect in synthesizing one or more globin chains in hemoglobin. In Pakistan, approximately 10,000 patients with thalassemia are primarily dependent on blood transfusions. The β-thalassemia patients require blood transfusions and iron chelation therapy. Patients who need blood transfusions are at an increased risk of contracting transfusion-transmitted infections (TTIs) such as hepatitis B and C viruses (HBV and HCV, respectively), as well as the human immunodeficiency virus (HIV). Objective This systemic review aims to assess the prevalence of TTIs in transfusion-dependent β-thalassemia patients in Asia. Methods The data for the systematic review were gathered from PubMed, Google Scholar, the Directory of Open Access Journals (DOAJ), and ScienceDirect using the following keywords: “prevalence, HBV, HCV, HIV, thalassemia, and transfusion-transmitted infections (TTIs)," and so on. This review includes the research articles that address the prevalence of viral infections in thalassemic patients following blood transfusion. Results A preliminary search of various databases identified 231 potential studies. 157 duplicate studies were eliminated, and the eligibility of 59 full-length articles was determined. Only 43 studies met the inclusion criteria. Among the 43 studies analyzed, 11 reported a high prevalence of HCV alone in thalassemic patients, while 21 reported a high prevalence of HCV and HBV infection in thalassemic patients. Eight studies reported the prevalence of all three TTIs examined, namely, HCV, HBV, and HIV, in patients with transfusion-dependent thalassemia. Conclusion Preventable transfusion-transmitted infections occur frequently, and robust national policies and hemovigilance are required to detect and mitigate the infection risk.

Prevalence of transfusion-transmitted infections in multiple blood transfusion-dependent thalassemic patients in Asia: A systemic review

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A Second Australian Family with Hemoglobin North Shore (β134 Val→glu)

Prevalence of transfusion-transmitted infections in multiple blood transfusion-dependent thalassemic patients in Asia: A systemic review