A Case Control Study to Evaluate the Cognitive and Brain Function of β-thalassemia Patients.
ThalassemiaBrain Function (Measured by ERP Level)β-thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in the synthesis of beta-globin chains. In recent studies done in β-thalassemia major patients abnormal iron deposition was evident using MRI in brain structures, cortex, putamen, and caudate nucleus . In most of the cases the neurological involvement is subclinical. Cognitive functioning was evaluated in beta thalassemia major, compared with healthy controls, using a neuropsychological battery including tests of abstract reasoning, attention, executive functions, language, constructional/visuospatial skills, and memory. Patients with beta thalassemia major, in particular those showing signs of hemosiderosis, had significantly impaired function in all neuropsychological tests. There was no relationship between cognitive performances and signs of deferoxamine toxicity, deferoxamine dosage, and levels of hemoglobin and ferritin. Event-related potentials (ERPs) are one of the most informative and dynamic methods of monitoring the information stream in the living brain. ERPs are linked in time with a physical or mental event, and are typically extracted from the scalp-recorded electroencephalogram (EEG) by means of signal averaging. ERPs have been used in the assessment of cognitive function in several disorders, including anemia and iron deficiency anemia. However, literature regarding cognitive function and ERP activity in thalassemia patients is extremely limited, especially in adults. The purpose of this study is to evaluate the cognitive and brain function in a group of 60 thalassemia patients and compare the results to healthy controls.
GDF 15 in Sickle Cell Disease and Hereditary Spherocytosis
Patients With Thalassemia Intermedia,Congenital Dyserythropoietic Anemia Type IPatients with thalassemia intermedia, congenital dyserythropoietic anemia type I , and sideroblastic anemia were found to express very high levels of serum GDF15, and this contributed to the inappropriate suppression of hepcidin with subsequent secondary iron overload.The aim of our present study is to asses the levels of GDF15 and hepcidin in patients with Sickle cell disease and hereditary spherocytosis
Quality of Life and Treatment Satisfaction in β-Thalassemia Patients Receiving Deferasirox
β-thalassemiaRegular blood transfusions are essential for the management of haematological conditions such as β-thalassemia major. As a result, however, patients with these conditions are susceptible to the development of transfusion-dependent iron overload (hemosiderosis or secondary iron overload
Complications in Children With B- Thalassemia Major
Beta-thalassemiaWorldwide, there are more than 60,000 births annually of serious forms of thalassemia .The World Health Organization considers thalassemia to be a major health burden. Beta- thalassemia is a group of recessively inherited disorders of hemoglobin synthesis characterized by reduced synthesis of the ß-globin chain caused by a mutation. The homozygous state results in severe anemia which needs regular blood transfusion.
B-Thalasaemia Trait and Its Possible Cardioprotective Role
Beta-Thalassemia TraitCoronary Artery DiseaseOur purpose is to investigate whether thallasaemia trait offers protection against cardiovascular events and whether this can be applied to specific characteristics of these people ( better lipid profile , better blood pressure profile) as scientific literature suggests.
Prevalence Of Microalbuminuria Among Children Suffering From Sickle Cell Nephropathy and Sickle...
Sickle Cell AnemiaBeta-Thalassemia1 moreSickle cell nephropathy is a known complication of sickle cell anemia (SCA) manifested by increase in glomerular filtration rate (glomerular hyperfiltration) and results in proteinuria and chronic renal failure. Our goal is to examine the prevalence of proteinuria and microalbuminuria as an early predictive factor of glomerular injury, among young people who suffer from SCA as well as those who suffer from combined sickle cell/beta-thalassemia.
Red Cell Distribution Width Index Versus Red Cell Distribution Width as Discriminating Guide for...
Microcytic Hypochromic AnemiaRed Cell Distribution Width Index versus Red Cell Distribution Width as Discriminating Guide for Iron Deficiency Anaemia and Beta Thalassemia Trait .
Dermatological Abnormalities in Beta-thalassemia Major
Beta-Thalassemiato study the frequency and pattern of dermatological abnormalities in egyptian childern with beta thalassemia.relationof abnormalities to duration of disease and frequency of transfusion
Assessment of Cognitive Functions, Psychological Symptoms, and Quality of Life Among B Thalassemia...
B ThalassemiaMultiple studies showed the negative impact thalassemia disease and its therapy have on the cognition of these patients. This study aims to assess and compare the cognition, psychological impact and quality of life among patients with B thalassemia major, requiring regular blood transfusion, patients with B thalassemia intermedia, who don't, and healthy controls. Thus, estimating the role of blood transfusion and hemosiderosis in cognitive impairment, psychological symptoms, and low quality of life in these patients.