|4.||End Stage Liver Disease
|1.||Barton, James C: 51 articles (12/2015 - 10/2002)|
|2.||Adams, Paul C: 42 articles (08/2015 - 07/2002)|
|3.||Acton, Ronald T: 36 articles (12/2015 - 10/2002)|
|4.||Ganz, Tomas: 32 articles (02/2015 - 10/2002)|
|5.||McLaren, Christine E: 31 articles (08/2015 - 02/2003)|
|6.||Nemeth, Elizabeta: 27 articles (02/2015 - 02/2005)|
|7.||Olynyk, John K: 26 articles (04/2015 - 04/2002)|
|8.||McLaren, Gordon D: 25 articles (08/2015 - 02/2003)|
|9.||Pietrangelo, Antonello: 23 articles (10/2015 - 03/2002)|
|10.||Powell, Lawrie W: 22 articles (08/2015 - 02/2002)|
03/01/2003 - "Our study shows that the HFE C282Y and H63D are determinants of iron parameters in the elderly and will be effective in detecting individuals at high risk of hemochromatosis. "
05/01/1991 - "In patients suffering from hemochromatosis MRI supplied additional information compared to CT and ultrasound revealing significant reduction of signal intensity due to reinforced enhancement of iron. "
10/01/1982 - "Mean liver iron concentrations (microgram/100 mg dry weight) were significantly greater in the alcoholics (156.4 +/- 7.8 (SEM); P less than 0.05) and in patients with idiopathic hemochromatosis (2094.5 +/- 230.7; P less than 0.01) than in control subjects (53.0 +/- 7.0). "
07/01/2007 - "The discovery of HFE gene has established the foundation for a better understanding of iron homeostasis and has changed hemochromatosis management: liver biopsy, gold standard diagnostic, was replaced by genetic test and it was suggested that population screening using genetic testing might be ideal for HFE related hemochromatosis. "
04/01/2004 - "Overall, of 8 patients with hemochromatosis, 3 without cardiac iron had improved cardiac function after phlebotomy, and 1 with cardiac iron had no cardiac dysfunction. "
07/01/2013 - "To determine the prevalence of HFE mutations in the hemochromatosis gene for 11 serum ferritin concentration intervals from 200 µg⁄L to 1000 µg⁄L in Caucasian participants in a primary care, population-based study. "
04/01/2008 - "Among 29,699 white subjects participating in the Scripps/Kaiser hemochromatosis study, only 59 had serum ferritin levels more than 1000 microg/L; 24 had homozygous mutant or compound heterozygous mutant HFE genotypes. "
10/01/2007 - "In the present study, we tested the association between common variants in candidate genes and hemochromatosis penetrance, in a large sample of C282Y homozygotes, using pretherapeutic serum ferritin level as marker of hemochromatosis penetrance. "
05/01/2006 - "This study was designed to evaluate the relationship between hemochromatosis genotypes, serum ferritin levels and presymptomatic vascular abnormalities in a cohort of healthy subjects. "
05/10/2004 - "In the same period, among those with diagnosed hemochromatosis, the mean prestudy ferritin level changed from 1848 ng/mL to 606 ng/mL after the study (P =.03). "
|3.||Transferrin (beta 2 Transferrin)IBA
05/26/1988 - "This study was designed to determine the efficacy of transferrin saturation as a screening tool for hemochromatosis and to assess the frequency of homozygosity for the HLA-linked hemochromatosis gene in a healthy population. "
02/01/2014 - "Total and cause-specific mortality by elevated transferrin saturation and hemochromatosis genotype in individuals with diabetes: two general population studies."
01/01/2014 - "The objective of this study was to determine whether maternal and/or infant transferrin (TF) and hemochromatosis (HFE) gene missense variants modify the association between maternal blood lead (MBL) and umbilical cord blood lead (UCBL). "
11/01/2002 - "Time-resolved measurements of cell membrane capacitance provide a new methodological approach to study the endocytic uptake of transferrin and its regulation by HFE, the hereditary hemochromatosis protein."
03/01/1998 - "The aims of this study were to estimate the mean transferrin saturation in hemochromatosis heterozygotes and normal individuals and to evaluate potential transferrin saturation screening levels. "
11/01/2010 - "Recent studies suggest suppressed hepcidin synthesis in the liver to be the molecular basis of hemochromatosis. "
01/01/2006 - "Recent studies indicate that hepcidin deficiency underlies most known forms of hereditary hemochromatosis. "
06/01/2005 - "At the opposite extreme, early studies indicate that hepcidin deficiency--due to the dysregulation of its synthesis or mutations in the hepcidin gene itself--is the immediate cause of most forms of hemochromatosis."
10/01/2015 - "Genetics, Genetic Testing, and Management of Hemochromatosis: 15 Years Since Hepcidin."
06/05/2014 - "Mutations affecting hepcidin regulators or the hepcidin gene itself cause hemochromatosis, a common genetic disorder. "
|5.||Deferoxamine (Desferal)FDA LinkGeneric
04/01/1996 - "[Long-term efficacy of subcutaneous administration of deferoxamine in patients with secondary hemochromatosis]."
06/01/2008 - "Deferoxamine (DFO) is a chelating agent used widely for the treatment of transfusional hemochromatosis. "
03/01/2006 - "Intramuscular deferoxamine in hereditary hemochromatosis."
07/01/1996 - "We report a patient with development of severe ocular toxicity during treatment with deferoxamine for transfusional hemochromatosis. "
04/01/1996 - "This regimen, in which the patient self-administered deferoxamine subcutaneously one or twice a day is seems to be the most practical method to protect against the progression of hemochromatosis."
|6.||Messenger RNA (mRNA)IBA
02/01/2013 - "Similarly, this group also showed a 4-fold increase in the messenger RNA expression of the hepatic hemochromatosis gene. "
05/01/2008 - "Analysis of SLC40A1 gene at the mRNA level reveals rapidly the causative mutations in patients with hereditary hemochromatosis type IV."
11/01/2002 - "In contrast, DMT-1 mRNA levels were at least twofold greater in patients with hereditary hemochromatosis and iron deficiency anemia when compared to controls (P = 0.02, P = 0.01, respectively). "
06/07/2005 - "Whereas in hereditary hemochromatosis patients without CD, DMT1 expression was up-regulated leading to excessive uptake of iron, we identified a significant reduction in protein and mRNA expression of DMT1 as a compensatory mechanism in this patient with HH and CD. "
09/01/2003 - "The objective was to study the expression of ferroportin mRNA and protein in C282Y-linked hemochromatosis liver and in controls. "
|7.||Glucose (Dextrose)FDA LinkGeneric
01/01/2008 - "To clarify the pathogenesis of diabetes associated with mutations of the hemochromatosis (HFE) gene, 17 carriers, 9 normal glucose tolerant (NGT) and 8 diabetic, were evaluated in an interventional trial. "
01/01/2011 - "We therefore examined glucose and fatty acid metabolism and hepatic glucose production in vivo and in vitro in a mouse model of hemochromatosis in which the gene most often mutated in the human disease, HFE, has been deleted (Hfe⁻(/)⁻). "
12/01/2010 - "Hereditary hemochromatosis (HH) is associated with abnormal glucose metabolism (AGM). "
11/01/2004 - "We therefore examined glucose homeostasis and beta-cell function in mouse models of hemochromatosis. "
04/01/1997 - "All subjects with hereditary hemochromatosis had impaired glucose tolerance as measured by K(g) (rate of glucose disappearance). "
|8.||Ribavirin (Virazole)FDA LinkGeneric
09/01/2012 - "Hemochromatosis gene mutations: prevalence and effects on pegylated-interferon and ribavirin therapy response in chronic hepatitis C in sardinia."
01/01/2003 - "Successfull response to alpha 2b interferon plus ribavirin associated with venesectomies in a patient with chronic hepatitis C and genetic hemochromatosis."
01/01/1999 - "Similar combinations should be discussed in patients with inherited hemoglobin disorders but iron overload (secondary hemochromatosis) associated with multiple transfusions may decrease the potential efficacy of interferon-alpha and chronic anemia may limit the use of ribavirin. "
11/01/2014 - "The aim of this case-control study was to assess hepcidin mRNA expression in liver tissue of patients with CHC in terms of iron metabolism parameters, hemochromatosis (HFE) gene mutations, disease activity, and efficacy of antiviral treatment with pegylated interferon and ribavirin. "
|9.||metal transporting protein 1 (ferroportin)IBA
03/01/2010 - "Study of the molecular cell biology of ferroportin and its mutants is key to understanding the pathogenesis of this increasingly recognized form of hemochromatosis, which responds poorly to conventional therapy."
04/01/2015 - "Ferroportin disease is a rare type of autosomal dominantly inherited hemochromatosis caused with mutations in the ferroportin gene (SLC40A1). "
01/01/2012 - "D270V could explain 'classical' ferroportin hemochromatosis phenotypes in some African Americans."
11/01/2010 - "One patient with HJV hemochromatosis, 2 with TFR2 hemochromatosis, and 3 with ferroportin disease were found among the 13 Japanese patients. "
03/01/2010 - "Mutations in the SLC40A1 gene, which encodes ferroportin, are associated with autosomal dominant hemochromatosis. "
03/01/2009 - "Heme carrier protein 1 (HCP1) genetic variants in the Hemochromatosis and Iron Overload Screening (HEIRS) Study participants."
11/01/2011 - "Polarized distribution of heme transporters in retinal pigment epithelium and their regulation in the iron-overload disease hemochromatosis."
07/01/2012 - "In the hemochromatosis model with the iron sink transgene in epidermis, there was a statistically significant reduction in non-heme iron in serum and in the liver and kidney. "
11/01/1989 - "These studies confirm the presence of excessive nonheme iron absorption even from unfortified meals in patients with idiopathic hemochromatosis and suggest in addition that they are particularly susceptible to iron loading from diets containing a high proportion of heme iron. "
11/01/1987 - "Hepatic heme synthesis in a new model of experimental hemochromatosis: studies in rats fed finely divided elemental iron."
|1.||Chelation Therapy (Therapy, Chelation)
01/01/1992 - "He developed hemochromatosis despite from parenteral chelation therapy. "
01/01/2009 - "Despite a major improvement in the chelation therapy and supportive care, the major cause of death in these patients is cardiac failure due to secondary hemochromatosis. "
04/01/1996 - "A number of studies have shown that regular chelation therapy with deferoxamine is effective in patients with secondary hemochromatosis. "
10/01/2002 - "Although phlebotomy therapy is effective in removing excess iron in hereditary hemochromatosis, chelation therapy is required in the treatment of many patients who have combined secondary and transfusional iron overload due to disorders in erythropoiesis. "
01/01/2011 - "Iron chelation therapy in hereditary hemochromatosis and thalassemia intermedia: regulatory and non regulatory mechanisms of increased iron absorption [Kontoghiorghes GJ, Spyrou A, Kolganou A. "
03/01/2014 - "Liver transplantation (LT) is a key treatment for potential complications from HFE-related hereditary hemochromatosis (HH). "
06/01/2011 - "In patients with congenital hemochromatosis and HCC, liver transplantation is a therapeutic option with a 5-year posttransplantation survival rate as high as 80%. "
07/01/2009 - "Outcome of liver transplantation in hereditary hemochromatosis."
04/27/2009 - "Patients with hereditary hemochromatosis are known to have an increased risk for morbidity and mortality after orthotopic liver transplantation. "
01/01/2006 - "A literature search was performed to estimate resectability rates as well as outcomes after liver transplantation for HCC arising in hemochromatosis. "
|3.||Renal Dialysis (Hemodialysis)
09/01/2005 - "Hemochromatosis (HFE) gene mutations in Brazilian chronic hemodialysis patients."
08/01/1992 - "A wide-spread endocrine involvement as seen in this patient is a rare clinical feature of hemochromatosis secondary to massive blood transfusion in hemodialysis patients. "
05/01/2013 - "Correlation of hemochromatosis gene mutations and cardiovascular disease in hemodialysis patients."
10/01/2009 - "Hemochromatosis gene mutations and treatment of anemia in patients on hemodialysis."
08/01/1992 - "Primary hypothyroidism and multiple endocrine failure in association with hemochromatosis in a long-term hemodialysis patient."
|4.||Blood Transfusion (Blood Transfusions)
11/01/2012 - "Is blood of uncomplicated hemochromatosis patients safe and effective for blood transfusion? "
01/01/2013 - "Survival depends on blood transfusions, which in consequence lead to hemochromatosis. "
05/27/2010 - "Due to blood transfusions, thalassemics are often infected with either hepatitis C virus (HCV) or hepatitis B virus and often have hemochromatosis. "
09/01/2003 - "Hemochromatosis due to repeated blood transfusions. "
05/01/2003 - "Hemochromatosis secondary to repeated blood transfusions in a dog."
|5.||Heart Transplantation (Grafting, Heart)
06/01/2001 - "We describe the case of a 36-year-old woman with hereditary hemochromatosis (HH) resulting in end-stage cardiomyopathy and treated successfully with orthotopic cardiac transplantation. "
12/01/2011 - "Indications for combined liver-heart transplantation include heart failure with associated cardiac cirrhosis, familial amyloidosis, familial hypercholesterolemia and hemochromatosis, and homozygous β-thalassemia. "
06/01/2001 - "Cardiac transplantation in a patient with hereditary hemochromatosis: role of adjunctive phlebotomy and erythropoietin."