background: sickle-cell anemia is characterized by defective hemoglobin synthesis with production of sickle hemoglobin. sickle red blood cells become deformed and rigid with difficulty to pass through narrow capillaries and frequent clotting and thrombosis leading to repetitive vascular occlusions and progressive organ damage. we conducted this work to study some adrenal functions using acth st...

background: patients with transfusional iron overload have depended on iron chelation therapy and improving chelation regimens have been of the highest priority. the aim of this study was to compare effect of combined versus monotherapy with deferoxamine (dfo) and deferiprone (dfp) in iron overloaded beta thalassemia (bt) major patients materials and methods we studied 36 bt major patients (mea...

A 32-year-old man presented with increases in serum alanine aminotransferase activity, iron concentration, and transferrin saturation, suggestive of hepatic dysfunction and iron overload. In addition, he had unusually low plasma concentrations of LDL-cholesterol and apolipoprotein (apo) B. Hepatic ultrasonography was consistent with fatty liver. On liver biopsy, marked steatosis and moderate to...

The hepatic peptide hormone hepcidin regulates dietary iron absorption, plasma iron concentrations, and tissue iron distribution. Hepcidin acts by causing the degradation of its receptor, the cellular iron exporter ferroportin. The loss of ferroportin decreases iron flow into plasma from absorptive enterocytes, from macrophages that recycle the iron of senescent erythrocytes, and from hepatocyt...

Diabetic nephropathy (DN) remains incurable and is the main cause of end-stage renal disease. We approached the pathophysiology of DN with systems biology, and a comprehensive profile of renal transcripts was obtained with RNA-Seq in ZS (F1 hybrids of Zucker and spontaneously hypertensive heart failure) rats, a model of diabetic nephropathy. We included sham-operated lean control rats (LS), sha...

Osteoporosis may complicate iron overload diseases such as genetic hemochromatosis. However, molecular mechanisms involved in the iron-related osteoporosis remains poorly understood. Recent in vitro studies support a role of osteoblast impairment in iron-related osteoporosis. Our aim was to analyse the impact of excess iron in Hfe-/- mice on osteoblast activity and on bone microarchitecture. We...

background: iron chelators are an important part of management of patients with thalassemia. it is prudent to compare efficacy of different iron chelators in treatment of iron overload in these patients who receive regular blood transfusion. we aimed to compare the efficacy and safety of available oral iron chelator; deferasirox (exjade®) with deferasirox (osveral®) in reducing iron overload in...

Iron-overload cardiomyopathy is a prevalent cause of heart failure on a world-wide basis and is a major cause of mortality and morbidity in patients with secondary iron-overload and genetic hemochromatosis. We investigated the therapeutic effects of resveratrol in acquired and genetic models of iron-overload cardiomyopathy. Murine iron-overload models showed cardiac iron-overload, increased oxi...

BACKGROUND AND AIMS In hereditary hemochromatosis, iron deposition in the liver parenchyma may lead to fibrosis, cirrhosis and hepatocellular carcinoma. Most cases are ascribed to a common mutation in the HFE gene, but the extent of clinical expression is greatly influenced by the combined action of yet unidentified genetic and/or environmental modifying factors. In mice, transcription factor N...

BACKGROUND Cardiovascular disease is an important cause of morbidity and mortality in sickle cell disease (SCD). We sought to characterize sickle cell cardiomyopathy using multimodality noninvasive cardiovascular testing and identify potential causative mechanisms. METHODS AND RESULTS Stable adults with SCD (n=38) and healthy controls (n=13) prospectively underwent same day multiparametric ca...