Precision Medicine in Neurodegeneration with Brain Iron Accumulation (NBIA) Disorders: An Update on Emerging Treatments. [PDF]
Abstract Background Neurodegeneration with Brain Iron Accumulation (NBIA) is a heterogeneous group of heritable, mostly recessive, progressive neurodegenerative diseases characterized by iron deposition in the basal ganglia and brainstem. There are no solid global epidemiological data on prevalence and incidence of NBIA subtypes, but registry data and ...
Schneider SA +3 more
europepmc +2 more sources
Sappanwood extract modulates hepatic structure-function in hepatomegaly and hepcidin related iron regulatory pathways in a phenylhydrazine induced hemolytic anemia rat model relevant to thalassemia. [PDF]
Phenylhydrazine (PHZ) induces hemolytic anemia characterized by increased erythrocyte destruction and ineffective erythropoiesis, leading to hepcidin suppression through disruption of the BMP/SMAD signaling pathway. Reduced hepcidin levels enhance intestinal iron absorption and hepatic iron accumulation, leading to hepatic iron overload.
Pratama MI +5 more
europepmc +2 more sources
Contemporary Management of Thalassemia: A Perspective on Current Standards and the Emergence of FDA-Approved Oral Therapy. [PDF]
ABSTRACT Background and Aims Thalassemia is an inherited hemoglobin disorder characterized by ineffective erythropoiesis, chronic anemia, and progressive multisystem complications that require lifelong management. Current treatment relies on regular red blood cell transfusions, iron chelation therapy, and supportive multidisciplinary care, while ...
Zainab F, Hasan AH, Abid MH, Abid MA.
europepmc +2 more sources
Therapeutic Silencing of Tmprss6 Reduces Iron-Induced Inflammation and Prolongs Survival in MDS Mice. [PDF]
ABSTRACT Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematopoietic disorders characterized by ineffective hematopoiesis, cytopenias, and an increased risk of progression to acute myeloid leukemia (AML). Despite advances in supportive and targeted therapies, disease‐modifying interventions remain limited.
Vilcassim S +13 more
europepmc +2 more sources
Novel Antidepressant-Like Properties of the Iron Chelator Deferiprone in a Mouse Model of Depression
Depressed individuals who carry the short allele for the serotonin-transporter-linked promotor region of the gene are more vulnerable to stress and have reduced response to first-line antidepressants such as selective serotonin reuptake inhibitors. Since
Hale, MW +11 more
core +2 more sources
Solubility and thermodynamic study of deferiprone in propylene glycol and ethanol mixture
This work aims to obtain the solubility, density and thermodynamic parameters of deferiprone in propylene glycol and ethanol. For this purpose, a shake-flask technique was applied for solid–liquid equilibration and the spectrophotometry method was ...
Samira Radmand +4 more
doaj +1 more source
This investigation dealt with the thermodynamic properties, saturated solubility values, and solvation behavior of deferiprone as an oral iron chelator agent in non-aqueous mixtures of propylene glycol and 2-propanol using experimental measurements and ...
Homa Rezaei +3 more
doaj +1 more source
Background Oral deferiprone was suggested to be more effective than subcutaneous desferrioxamine for removing heart iron. Oral once-daily chelator deferasirox has recently been made commercially available but its long-term efficacy on cardiac iron and ...
Alessia Pepe +19 more
doaj +1 more source
Latar belakang. Talasemia β Mayor merupakan kelainan herediter yang disebabkan gangguan produksi rantai globin. Transfusi rutin menyebabkan kelebihan besi yang tertimbun dalam jaringan sehingga menyebabkan kardiomiopati, gangguan liver, dan komplikasi ...
Aries - Krisbiyantoro +2 more
doaj +1 more source
Hematological Parameters in Beta Thalassemia Major Children [PDF]
Background: Thalassemia is a hematological disorder that is caused by mutations in the genes that encode hemoglobin chains. Mutations in the β-globin gene are the most common cause of genetic disorders in humans, with 350 β-thalassemia variants ...
Zamzam Mohamed +3 more
doaj +1 more source

