Hypoparathyroidism is thought to be a rare consequence of iron overload seen in beta-thalassemic transfused patients. This study was conducted to determine the prevalence of hypoparathyroidism in a large number of beta-thalassemic patients, and its potential correlation with the presence of other endocrinopathies caused by iron overload. Serum and urine biochemical parameters were measured in 243 thalassemic patients (136 females and 107 males) in order to determine the prevalence of hypoparathyroidism and evaluate bone turnover. The patients were divided into two groups according to the presence of hypoparathyroidism. We compared the prevalence of other endocrinopathies and disease complications in the two groups. Hypoparathyroidism was detected in 13.5% of the patients (33 subjects; 17 males and 16 females). Serum-intact parathyroid hormone, and total and ionized calcium were significantly lower, while phosphorus was significantly higher in thalassemic patients with hypoparathyroidism. The reduction in BMD was more prominent in normal thalassemic patients (Z score = -2.246 +/- 0.97) compared with those with hypoparathyroidism (Z score = -1.975 +/- 0.89), although the difference was not statistically significant. Disturbed glucose metabolism was more common in patients with hypoparathyroidism (P < 0.05). In addition, heart dysfunction was statistically more frequent in this group (odds ratio = 2.51, P < 0.05). Hypoparathyroidism is a not infrequently observed complication in thalassemic patients. Since the concentration of ferritin is not a valuable tool in the prediction of the development of hypoparathyroidism, parathyroid function should be tested periodically, particularly when other iron overload-associated complications occur.
Accumulation of free α-globin is a critical factor in the pathogenesis of β-thalassemia. Autophagy plays a crucial role in clearing toxic free α-globin, thereby reducing disease severity. However, the impact of natural mutations in autophagy-related genes (ATGs) on the phenotypic variability of β-thalassemia remains unclear.
Introduction HbA1c values used for diagnosing and treating diabetes can be affected by factors such as red blood cell lifespan, hemolysis, red cell transfusion, and the presence of minor Hb species like HbA2 and HBF in hemoglobinopathies like sickle cell disease, homozygous HbC disease, HbSC disease, and β-thalassemia. This study aims to compare HbA1c levels in transfusion-dependent thalassemia (TDT) patients and healthy individuals. Materials and methods This is a cross-sectional comparative study.
Cyrus Tang Medical Institute, National Clinical Research Center for Hematologic Diseases, Collaborative Innovation Center of Hematology, State Key Laboratory of Radiation Medicine and Protection, Soochow University, Suzhou, Jiangsu 215123, China.
- Hemoglobin Constant Spring (Hb CS) is a significant mutation linked to α-thalassemia caused by an anti-termination mutation in the α2-globin gene stop codon.
- A prime editing strategy was developed, demonstrating a successful introduction of Hb CS mutations in human cell lines (averaging 32% efficiency) and primary hematopoietic stem and progenitor cells (HSPCs) from healthy donors (averaging 27% efficiency).
- The prime editing process also corrected the Hb CS mutation to its normal form in HSPCs from patients with hemoglobin H Constant Spring, suggesting its potential as a therapeutic approach for treating this condition.
* 60 thalassemia patients were divided into three groups based on their chelation treatments: oral deferiprone daily, subcutaneous deferoxamine four times weekly, and a combination of both therapies.
* Results show that the combination therapy was the most effective in reducing iron levels and improving urinary iron excretion, while patients on deferoxamine had the least hepatic iron deposition, though still above normal levels.
Objective: This systematic review aimed to verify if individuals with thalassemia present changes in radiomorphometric indices and fractal analysis that can be accessed using panoramic radiographs.
Material And Methods: Observational studies that evaluated cortical and trabecular bone patterns in dental routine radiographs were selected from six bibliographic databases. Risk of bias was analyzed using the Joanna Briggs Institute Critical Appraisal Checklists.
