Identification of Delta-beta Thalassemia in a Family with Elevated Hb F: A Case Report [PDF]
Delta-beta Thalassemia is a rare variant of thalassemia with elevated Hb F. Heterozygous and homozygous state of delta-beta thalassemia present with features similar to Hereditary Persistence of Fetal Haemoglobin (HPFH) and beta thalassemia ...
Varun Kumar Singh +3 more
doaj
Identification of erythroferrone as an erythroid regulator of iron metabolism. [PDF]
Recovery from blood loss requires a greatly enhanced supply of iron to support expanded erythropoiesis. After hemorrhage, suppression of the iron-regulatory hormone hepcidin allows increased iron absorption and mobilization from stores.
Ganz, Tomas +5 more
core +1 more source
Calibration of myocardial T2 and T1 against iron concentration. [PDF]
BACKGROUND: The assessment of myocardial iron using T2* cardiovascular magnetic resonance (CMR) has been validated and calibrated, and is in clinical use.
A John Baksi +69 more
core +1 more source
Efficacy of lactoferrin oral administration in the treatment of anemia and anemia of inflammation in pregnant and non-pregnant women: an interventional study [PDF]
The discovery of the ferroportin-hepcidin complex has led to a critical review on the treatment of anemia and anemia of inflammation (AI). Ferroportin, the only known mammalian iron exporter from cells to blood, is negatively regulated by hepcidin, a ...
Antimo Cutone +6 more
core +2 more sources
The human ankyrin 1 promoter insulator sustains gene expression in a β-globin lentiviral vector in hematopoietic stem cells. [PDF]
Lentiviral vectors designed for the treatment of the hemoglobinopathies require the inclusion of regulatory and strong enhancer elements to achieve sufficient expression of the β-globin transgene.
Baldwin, Kismet M +12 more
core +4 more sources
Objectives: To assess the sensitivity of the various discrimination indicesas screening test in beta-thalassemia trait patients in our population. Study design: Crosssectional descriptive study. Period: 1-Sep 2012 to 31-Jan 2013 (5 months). Settings: PathologyDepartment, District Head Quarters (DHQ) Hospital, Rawalpindi.
Muhammad Ihtesham Khan +2 more
openaire +4 more sources
Transcriptional regulation of the ABCC6 gene and the background of impaired function of missense disease-causing mutations. [PDF]
The human ATP-binding cassette family C member 6 (ABCC6) gene encodes an ABC transporter protein expressed primarily in the liver and to a lesser extent in the kidneys and the intestines.
Arányi, Tamás +5 more
core +1 more source
Effects of vitamin e and zinc supplementation on antioxidants in beta thalassemia major patients [PDF]
Objective: In beta thalassemic patients, tissue damage occurs due to oxidative stress and it happens because of the accumulation of iron in the body. This study was conducted to determine the effect of zinc and vitamin E supplementation on antioxidant ...
Aboomardani, M. +5 more
core +1 more source
Restoring Iron Homeostasis via Smoothened Inhibition: A Novel Strategy Against Hearing Loss
. ABSTRACT Sensorineural hearing loss (SNHL) induced by noise or aminoglycoside antibiotics is a significant public health concern without any FDA‐approved pharmaceutical therapies. Dysregulation of iron homeostasis and its subsequently induced ferroptosis has increasingly been identified as a key mechanism underlying cochlear hair cell (HC) damage ...
Huanyu Mao +9 more
wiley +1 more source
Prevalence and intensity of depression in mothers of children with beta-thalassemia major in Talghani Hospital of Gorgan, Iran [PDF]
Background: Thalassemia is a chronic disease that it leads to psychological and social problems for parents. Mothers are at markedly increased risk of suffering from psychological distress and depression because they usually take on a considerable part ...
Jahazi, A. +3 more
core

