Publications
-
Iron Metabolism Under Conditions of Ineffective Erythropoiesis in β-Thalassemia
β-Thalassemia (BT) is an inherited genetic disorder that is characterized by ineffective erythropoiesis (IE), leading to anemia and abnormal iron metabolism. IE is an abnormal expansion of the number of…
Read More » -
Hepatocellular Carcinoma in β-Thalassemia Patients: Review of the Literature with Molecular Insight into Liver Carcinogenesis
With the continuing progress in managing patients with thalassemia, especially in the setting of iron overload and iron chelation, the life span of these patients is increasing, while concomitantly increasing…
Read More » -
Evaluation of Endocrine Complications in Beta-Thalassemia Intermedia Patients: A Cross Sectional Multi-Center Study
The diagnosis of beta thalassemia intermedia (BTI) is mainly based on the severity of clinical phenotype. It is associated with a wide range of specific complications including extramedullary hematopoiesis, leg…
Read More » -
Nutritional Deficiencies Are Common in Patients with Transfusion-dependent Thalassemia and Associated with Iron Overload
Patients with thalassemia are frequently deficient in key micronutrients. Attempts to correct these inadequacies through nutritional supplementation have been met with some success, although disparities between intake and circulating levels…
Read More » -
Amustaline-glutathione Pathogen-reduced Red Blood Cell Concentrates for Transfusion-dependent Thalassaemia
Transfusion-dependent thalassaemia (TDT) requires red blood cell concentrates (RBCC) to prevent complications of anaemia, but carries risk of infection. Pathogen reduction of RBCC offers potential to reduce infectious risk. We…
Read More » -
An ICET-A Survey on Occult and Emerging Endocrine Complications in Patients with β-Thalassemia Major: Conclusions and Recommendations
In adult thalassemia major (TM) patients, a number of occult and emerging endocrine complications, such as: central hypothyroidism (CH), thyroid cancer, latent hypocortisolism, and growth hormone deficiency (GHD) have emerged…
Read More » -
Iron Chelators or Therapeutic Modulators of Iron Overload: Are We Anywhere Near Ideal One?
Iron overload is an important cause of mortality and morbidity in chronic transfusion-dependent states commonly exemplified by transfusion-dependent beta-thalassaemia syndromes [including non-transfusion-dependent (NTD) thalassaemia intermedia] as well as transfusion-dependent myelodysplastic…
Read More » -
Estimating the Birth Prevalence and Pregnancy Outcomes of Congenital Malformations Worldwide
Congenital anomaly registries have two main surveillance aims: firstly to define baseline epidemiology of important congenital anomalies to facilitate programme, policy and resource planning, and secondly to identify clusters of…
Read More » -
Transient Elastography (TE) is a Useful Tool for Assessing the Response of Liver Iron Chelation in Sickle Cell Disease Patients
Sickle cell disease patients often need regular blood transfusions to improve both the quality of life and survival from the veno-occlusive complications of the disease. Deferasirox, a convenient long acting…
Read More » -
Rare Single Gene Disorders: Estimating Baseline Prevalence and Outcomes Worldwide
As child mortality rates overall are decreasing, non-communicable conditions, such as genetic disorders, constitute an increasing proportion of child mortality, morbidity and disability. To date, policy and public health programmes…
Read More »