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Unbound Medicine.
(hemochromatosis)
10,788 results
  • Bone morphogenic proteins in iron homeostasis. [Review]
    Bone. 2020 Jun 22 [Online ahead of print]Xiao X, Alfaro-Magallanes VM, Babitt JL
  • The bone morphogenetic protein (BMP)-SMAD signaling pathway plays a central role in regulating hepcidin, which is the master hormone governing systemic iron homeostasis. Hepcidin is produced by the liver and acts on the iron exporter ferroportin to control iron absorption from the diet and iron release from body stores, thereby providing adequate iron for red blood cell production, while limiting…
  • Hepatocyte-specific deletion of peroxisomal protein PEX13 results in disrupted iron homeostasis. [Journal Article]
    Biochim Biophys Acta Mol Basis Dis. 2020 Jun 18; 1866(10):165882.Rishi G, Bhatia M, … Subramaniam VN
  • Peroxisomes are organelles, abundant in the liver, involved in a variety of cellular functions, including fatty acid metabolism, plasmalogen synthesis and metabolism of reactive oxygen species. Several inherited disorders are associated with peroxisomal dysfunction; increasingly many are associated with hepatic pathologies. The liver plays a principal role in regulation of iron metabolism. In thi…
  • Liver Transplantation in Patients with Sickle Cell Disease in the United States. [Journal Article]
    J Surg Res. 2020 Jun 12; 255:23-32.Hogen R, Kim M, … Emamaullee J
  • CONCLUSIONS: Data from the SRTR demonstrate that patients with SCD can expect equivalent graft and patient survival after LT despite exhibiting more comorbidities at the time of LT. The low number of patients with SCD who underwent LT in the SRTR in comparison with the rate of chronic liver disease in this population raises the question as to whether a disparity in access to LT exists for this complex population.
  • Osmium absorption after osmium tetroxide skin and eye exposure. [Journal Article]
    Basic Clin Pharmacol Toxicol. 2020 Jun 11 [Online ahead of print]Friedova N, Pelclova D, … Kohout P
  • Osmium tetroxide is a strong oxidizing agent used in electron microscopy. Eye exposure may cause severe burns, and after inhalation or ingestion damage to the respiratory or gastrointestinal tract occurs. Exposure to osmium and its compounds is extremely rare. We present a case of a 32-year-old female stained by 9 ml of 2% osmium tetroxide in acetone during an accident in the laboratory, with rar…
  • Systematic review of the clinical outcomes of iron reduction in Hereditary Hemochromatosis. [Review]
    Hepatology. 2020 Jun 04 [Online ahead of print]Prabhu A, Cargill T, … Ryan JD
  • Hereditary Hemochromatosis is a condition caused by defects in iron-sensing genes leading to parenchymal iron loading. If diagnosed early and treated appropriately, many of the complications, including liver fibrosis, cirrhosis and liver cancer, arthritis, cardiomyopathy and diabetes, were thought to be avoided. As iron reduction by venesection became the mainstay of HH treatment before the intro…
  • StatPearls: Deferoxamine [BOOK]
    . StatPearls Publishing: Treasure Island (FL) Velasquez James J Wyckoff Heights Medical Center Wray Anton A. AA Wyckoff Heights Medical Center BOOK
  • Deferoxamine (DFO) is FDA approved to treat iron overload, either acute or chronic. The definition of iron overload is serial ferritin levels above 800 to 3000 ng/mL [1]. The FDA has not approved DFO as first-line therapy for hereditary hemochromatosis unless there is a contraindication to phlebotomy. Clinicians can also use DFO is also used as an off-label treatment for aluminum toxicity in chro…
  • Regulation of Iron Homeostasis and Related Diseases. [Review]
    Mediators Inflamm. 2020; 2020:6062094.Li Y, Huang X, … Wan D
  • The liver is the organ for iron storage and regulation; it senses circulating iron concentrations in the body through the BMP-SMAD pathway and regulates the iron intake from food and erythrocyte recovery into the bloodstream by secreting hepcidin. Under iron deficiency, hypoxia, and hemorrhage, the liver reduces the expression of hepcidin to ensure the erythropoiesis but increases the excretion o…
  • MR imaging assessment and quantification of liver iron. [Review]
    Abdom Radiol (NY). 2020 May 20 [Online ahead of print]França M, Carvalho JG
  • Iron overload is a common clinical problem resulting from hereditary hemochromatosis or secondary hemosiderosis (mainly associated with transfusion therapy), being also associated with chronic liver diseases and metabolic disorders. Excess of iron accumulates in organs like the liver, pancreas and heart. Without treatment, patients with iron overload disorders will develop liver cirrhosis, diabet…
  • From Environment to Genome and Back: A Lesson from HFE Mutations. [Review]
    Int J Mol Sci. 2020 May 15; 21(10)Rametta R, Meroni M, Dongiovanni P
  • The environment and the human genome are closely entangled and many genetic variations that occur in human populations are the result of adaptive selection to ancestral environmental (mainly dietary) conditions. However, the selected mutations may become maladaptive when environmental conditions change, thus becoming candidates for diseases. Hereditary hemochromatosis (HH) is a potentially lethal…
  • Polycythemia and Anemia in Hereditary Hemochromatosis. [Journal Article]
    Cureus. 2020 Apr 09; 12(4):e7607.Khan AA, Hadi Y, … Kupec J
  • Introduction Hereditary hemochromatosis is a syndrome of dysregulated iron homeostasis resulting in the excessive deposition of iron. Hemochromatosis causes pulmonary, pancreatic, and hepatic dysfunction, all of which are risk factors for anemia in the general population. Conversely, iron overload states are thought to predispose to polycythemia. The effect of the homozygosity and heterozygosity …
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