Hemochromatosis (Bronze Diabetes)

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2009 relevant articles (40 outcomes, 192 trials/studies) found for this Disease

Description: A disorder due to the deposition of hemosiderin in the parenchymal cells, causing tissue damage and dysfunction of the liver, pancreas, heart, and pituitary. Full development of the disease in women is restricted by menstruation, pregnancy, and lower dietary intake of iron. Acquired hemochromatosis may be the result of blood transfusions, excessive dietary iron, or secondary to other disease. Idiopathic or genetic hemochromatosis is an autosomal recessive disorder of metabolism associated with a gene tightly linked to the A locus of the HLA complex on chromosome 6. (From Dorland, 27th ed)

Also Known As:

Bronze Diabetes; Hemochromatoses; Diabetes, Bronze

Relationship Network

Disease Context: Research Results

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 421

Inborn Genetic Diseases: 549

Inborn Errors Metabolism: 402

Inborn Errors Metal Metabolism

Hemochromatosis: 2009

Nutritional and Metabolic Diseases

Metabolic Diseases: 1267

Inborn Errors Metabolism: 402

Inborn Errors Metal Metabolism

Hemochromatosis: 2009

Iron Metabolism Disorders: 9

Iron Overload: 2875

Hemochromatosis: 2009

Related Diseases

1.	Iron Overload
2.	Anemia
3.	Fibrosis (Cirrhosis)
4.	Hepatolenticular Degeneration (Wilson's Disease)
5.	Alcoholism (Alcohol Abuse)

Experts

1.	Barton, James C: 16 articles (08/2008 - 10/2002)
2.	Acton, Ronald T: 11 articles (08/2008 - 10/2002)
3.	Adams, Paul C: 10 articles (08/2008 - 09/2003)
4.	Reboussin, David M: 8 articles (08/2008 - 01/2005)
5.	Camaschella, Clara: 8 articles (05/2008 - 05/2002)
6.	McLaren, Christine E: 7 articles (02/2008 - 01/2005)
7.	Beutler, Ernest: 6 articles (04/2008 - 09/2002)
8.	Harris, Emily L: 6 articles (02/2008 - 01/2005)
9.	Kowdley, Kris V: 6 articles (12/2007 - 02/2004)
10.	Adams, P C: 5 articles (08/2008 - 05/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hemochromatosis:

1.	Iron (Serum Iron Level)IBA 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" 02/01/1998 - "The discovery of a candidate gene for hereditary hemochromatosis undoubtedly will result in improved diagnosis of hereditary hemochromatosis and to a better understanding of certain aspects of iron absorption, hepatic iron uptake and release, and whole body iron metabolism" Order ALL the reference details at left...
2.	Ferritins (Ferritin)IBA 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)" 09/01/1995 - "CONCLUSIONS: Our study shows that serum ferritin levels exhibit a tendency to be significantly correlated with the true status of hemochromatosis in thalassemic patients; however, the discrepancies recorded in several patients and the scarce or total lack of correlation with MRI suggest exploring other approaches to this problem in order to make proper decisions about therapy." Order ALL the reference details at left...
3.	Deferoxamine (Desferal)FDA LinkGeneric 04/01/1996 - "Long-term efficacy of subcutaneous administration of deferoxamine in patients with secondary hemochromatosis]" 11/01/1964 - "THE EFFECT OF DESFERRIOXAMINE-B (DESFERAL) ON THE PLASMA CLEARANCE OF FE 59 IN HEALTHY PERSONS AND PATIENTS WITH PRIMARY HEMOCHROMATOSIS.]" 06/01/2008 - "PURPOSE: 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" Order ALL the reference details at left...
4.	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" 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" 04/01/1994 - "In the first study, hereditary hemochromatosis was identified in 14 white men on the basis of a persistent elevation in transferrin saturation above 55%" 10/01/2008 - "Serum transferrin saturation increase is associated with decrease of antibacterial activity of serum in patients with HFE-related genetic hemochromatosis." Order ALL the reference details at left...
5.	Glucose (Dextrose)FDA LinkGeneric 01/01/2008 - "OBJECTIVE: 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" 11/01/2004 - "We therefore examined glucose homeostasis and beta-cell function in mouse models of hemochromatosis" 04/01/1997 - "RESULTS: All subjects with hereditary hemochromatosis had impaired glucose tolerance as measured by K(g) (rate of glucose disappearance)" 04/01/1997 - "Factors affecting glucose tolerance in hereditary hemochromatosis." 10/01/1998 - "The husband was diagnosed as having idiopathic hemochromatosis, abnormal glucose tolerance, and hypogonadism accompanied by impotence, retrograde ejaculation, and azoospermia" Order ALL the reference details at left...
6.	hepcidinIBA 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." 10/01/2008 - "Hepcidin insufficiency as a primary cause of genetic hemochromatosis seems unlikely based on the clinical profile of the present patient and the hepcidin measurements." 10/01/2008 - "It has been speculated that hepcidin insufficiency or dysregulation may be the primary defect in genetic hemochromatosis" 10/01/2008 - "Is serum hepcidin causative in hemochromatosis" Order ALL the reference details at left...
7.	Messenger RNA (mRNA)IBA 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 - "RESULTS: 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" 09/01/2003 - "METHODS: In the present study, we investigated the mRNA and protein expression of Dcytb and hephaestin in duodenal biopsies from patients with iron deficiency, HFE, and non-HFE-associated hemochromatosis and in control subjects by means of real-time polymerase chain reaction, Western blot, and immunofluorescence" Order ALL the reference details at left...
8.	CopperIBA 01/01/2004 - "Wilson's disease and secondary copper hemochromatoses in hematological practice]" 10/01/2007 - "The aim of the present review is to summarize the genetic backgrounds of Japanese patients with hemochromatosis of unknown etiology, Wilson disease of primary copper toxicosis, and the black liver of Dubin-Johnson syndrome" 12/01/2005 - "The non-tumorous area showed NASH with moderate bridging fibrosis, without interface hepatitis, hemochromatosis, or copper accumulation" 01/01/2004 - "CONCLUSION: Primary or secondary nature of copper overloading in patients with hepatic cirrhosis and critical cytopenia, pathogenesis of cytopenic syndrome, practical significance of copper hemochromatoses diagnosis are discussed." 01/01/1995 - "In alpha-1-antitrypsin-deficiency, hemochromatosis, Wilson's disease, and chronic active hepatitis B cellular deposites typically located in hepatocytes such as alpha-1-AT, siderin, copper, HBs-Ag, and HBc-Ag can also be found in neoductular cells close to hepatocytes" Order ALL the reference details at left...
9.	Hemoglobins (Hemoglobin)IBA 10/01/2004 - "A literature review reports increased erythrocyte indices [hemoglobin concentration, hematocrit, mean corpuscular volume, mean corpuscular hemoglobin (MCH), MCH concentration] in subjects with hereditary hemochromatosis (HH)" 01/01/2000 - "Peripheral blood erythrocyte parameters in hemochromatosis: evidence for increased erythrocyte hemoglobin content." 07/01/1992 - "During 12 months' follow-up, an acceptable hemoglobin level was maintained without any need for blood transfusions; in addition, there was evidence indicating regression of hemochromatosis" 10/01/2007 - "Determinants and characteristics of mean corpuscular volume and hemoglobin concentration in white HFE C282Y homozygotes in the hemochromatosis and iron overload screening study." 03/01/2008 - "This genotype normally does not confer the hemochromatosis phenotype but may contribute to iron overload when present in an individual with increased hemoglobin synthesis" Order ALL the reference details at left...
10.	ErythropoietinIBA 01/01/2000 - "BACKGROUND AND OBJECTIVES: The purposes of this study were to evaluate the tolerance, efficacy and safety of isovolemic erythrocytapheresis (EA) in nonanemic patients with hereditary hemochromatosis (HH), and to assess the usefulness of recombinant human erythropoietin (rHuEPO) associated with EA to reduce treatment duration" 05/01/2005 - "We report the efficacy, tolerability and cost of erythocytoapheresis plus recombinant human erythropoietin (rHuEPO) in three patients with severe hereditary hemochromatosis (HH)" 05/01/2005 - "Erythrocytapheresis plus erythropoietin: an alternative therapy for selected patients with hemochromatosis and severe organ damage." 05/01/2001 - "Recombinant human erythropoietin (rHuEPO) in the treatment of primary hemochromatosis]" 01/01/2000 - "Erythrocytapheresis with recombinant human erythropoietin in hereditary hemochromatosis therapy: a new alternative." Order ALL the reference details at left...

Therapies and Procedures

1.	Chelation Therapy (Therapy, Chelation) 01/01/1992 - "He developed hemochromatosis despite from parenteral chelation therapy" 04/01/1996 - "A number of studies have shown that regular chelation therapy with deferoxamine is effective in patients with secondary hemochromatosis" 09/01/1997 - "One infant, with the original diagnosis of neonatal hemochromatosis, was treated with a variety of antioxidants and chelation therapy, as recently reported" 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" 04/01/2008 - "Complete hematopoietic recovery after continuous iron chelation therapy in a patient with severe aplastic anemia with secondary hemochromatosis." Order ALL the reference details at left...
2.	Liver Transplantation 11/01/2005 - "The aim of our study was to compare the outcome of medical treatment vs. liver transplantation in infants with neonatal hemochromatosis (NH) referred to King's College Hospital from 1990-2002" 04/01/2008 - "Neonatal hemochromatosis is the most common specific indication for liver transplantation in the first three months of life and appears to be the treatment of choice, and must as well be considered as soon as it becomes apparent that medical support, which should include chelation-antioxidant treatment, is ineffective, before irreversible neurological complications appear." 01/01/2006 - "A literature search was performed to estimate resectability rates as well as outcomes after liver transplantation for HCC arising in hemochromatosis" 06/01/2004 - "Patient and graft survival after liver transplantation for hereditary hemochromatosis: Implications for pathogenesis." 06/01/2004 - "Liver transplantation: an "in vivo" model for the pathophysiology of hemochromatosis?" Order ALL the reference details at left...
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" 08/01/1992 - "Primary hypothyroidism and multiple endocrine failure in association with hemochromatosis in a long-term hemodialysis patient." 07/01/1992 - "Correction of pancytopenia and hemochromatosis by r-huEPO in a hemodialysis patient." 01/01/1988 - "There have been some reports on the risk of developing hemosiderosis in hemodialysis patients when heavily transfused and simultaneously possessing hemochromatosis alleles (HA)" Order ALL the reference details at left...
4.	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" 06/01/2001 - "Cardiac transplantation in a patient with hereditary hemochromatosis: role of adjunctive phlebotomy and erythropoietin." Order ALL the reference details at left...
5.	Plasmapheresis 07/01/1998 - "The use of therapeutic plasmapheresis in the combined therapy of a female patient with idiopathic hemochromatosis]" 07/01/1998 - "Stable remission could be attained by maintenance desferal therapy after a course of therapeutic plasmapheresis, which can be regarded as a stage in basic therapy for idiopathic hemochromatosis." Order ALL the reference details at left...

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