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1. Full Text Hemochromatosis
by Oppl, B and Zwerina, J
ZEITSCHRIFT FUR RHEUMATOLOGIE, ISSN 0340-1855, 09/2015, Volume 74, Issue 7, pp. 609 - 616
Hereditary hemochromatosis is a frequent autosomal recessive iron storage disease in northern and western Europe. The classical clinical triad of liver... 
Iron metabolism | CLINICAL EXPRESSION | FERROPORTIN | Phlebotomy | HEPCIDIN | PREVALENCE | RHEUMATOLOGY | HEREDITARY HEMOCHROMATOSIS | H63D MUTATIONS | IRON-METABOLISM | HFE gene | C282Y | Arthropathy | Liver cirrhosis | Hemochromatosis - therapy | Liver Cirrhosis - etiology | Liver Cirrhosis - diagnosis | Hemochromatosis - complications | Humans | Treatment Outcome | Liver Cirrhosis - therapy | Hemochromatosis - diagnosis | Phlebotomy - methods | Evidence-Based Medicine
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2. Full Text Higher age at diagnosis of hemochromatosis is the strongest predictor of the occurrence of hepatocellular carcinoma in the Swiss hemochromatosis cohort: A prospective longitudinal observational study
by Nowak, Albina and Giger, Rebekka S and Krayenbuehl, Pierre-Alexandre
Medicine, ISSN 0025-7974, 10/2018, Volume 97, Issue 42, p. e12886
Hereditary hemochromatosis (HH) is the most common genetic disease in Caucasians which is characterized by an increased intestinal iron absorption, resulting... 
CHRONIC HEPATITIS | FIBROSIS | PROGNOSTIC-FACTORS | LONG-TERM SURVIVAL | PREVALENCE | HEREDITARY HEMOCHROMATOSIS | LIVER-DISEASE | risk factor | IRON OVERLOAD | MEDICINE, GENERAL & INTERNAL | HFE C282Y | hemochromatosis | CANCER-RISK | hepatocellular carcinoma | Liver Cirrhosis - etiology | Prospective Studies | Age Factors | Humans | Middle Aged | Hemochromatosis - blood | Male | Liver Cirrhosis - blood | Switzerland | Liver Neoplasms - etiology | Young Adult | Hemochromatosis - complications | Ferritins - blood | Adolescent | Aged, 80 and over | Adult | Female | Aged | Carcinoma, Hepatocellular - etiology | Longitudinal Studies | Complications and side effects | Hemochromatosis | Prognosis | Age factors in disease | Patient outcomes | Hepatoma | Diagnosis | Risk factors
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3. Full Text How I treat hemochromatosis
by Adams, Paul C and Barton, James C
Blood, ISSN 0006-4971, 07/2010, Volume 116, Issue 3, pp. 317 - 325
Hemochromatosis is a common genetic disorder in which iron may progressively accumulate in the liver, heart, and other organs. The primary goal of therapy is... 
NONTRANSFERRIN-BOUND IRON | VOLUNTARY BLOOD-DONORS | HFE C282Y HOMOZYGOTES | LONG-TERM SURVIVAL | MIDDLE-AGED WOMEN | HEREDITARY HEMOCHROMATOSIS | NATURAL-HISTORY | HEMATOLOGY | IDIOPATHIC HEMOCHROMATOSIS | GENETIC HEMOCHROMATOSIS | INTESTINAL IRON-ABSORPTION | Hemochromatosis - therapy | Hemochromatosis Protein | Membrane Proteins - genetics | Humans | Cytapheresis | Phlebotomy | Deferoxamine - therapeutic use | Male | Chelation Therapy | Histocompatibility Antigens Class I - genetics | Iron, Dietary - administration & dosage | Hemochromatosis - metabolism | Proton Pump Inhibitors - therapeutic use | Iron - metabolism | Blood Donors | Homozygote | Siderophores - therapeutic use | Female | Hemochromatosis - genetics
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4. Full Text Hereditary Hemochromatosis: Pathogenesis, Diagnosis, and Treatment
by Pietrangelo, Antonello
Gastroenterology, ISSN 0016-5085, 2010, Volume 139, Issue 2, pp. 393 - 408.e2
In the late 1800s, hemochromatosis was considered an odd autoptic finding. More than a century later, it was finally recognized as a hereditary, multi-organ... 
Gastroenterology and Hepatology | Hereditary Disorders | Micronutrients | Iron Metabolism | Hepcidin | HFE | JUVENILE HEMOCHROMATOSIS | CLINICAL EXPRESSION | FERRITIN MESSENGER-RNA | CONTROLS IRON-METABOLISM | NONINVASIVE PREDICTION | ANTIMICROBIAL PEPTIDE HEPCIDIN | TRANSFERRIN RECEPTOR 2 | AUTOSOMAL-DOMINANT HEMOCHROMATOSIS | GASTROENTEROLOGY & HEPATOLOGY | IDIOPATHIC HEMOCHROMATOSIS | GENOME-WIDE ASSOCIATION | Hemochromatosis - therapy | Predictive Value of Tests | Genetic Predisposition to Disease | Liver - pathology | Humans | Liver - metabolism | Risk Factors | Treatment Outcome | Heredity | Hemochromatosis - metabolism | Hemochromatosis - pathology | Polymorphism, Genetic | Phenotype | Animals | Pedigree | Hemochromatosis - diagnosis | Mutation | Hemochromatosis - genetics | Iron - blood | Proteins | Transferrin | Genetic disorders | Hemochromatosis | Physiological aspects | Development and progression | Diagnosis | Index Medicus | Abridged Index Medicus
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5. Full Text Autopsy relevance determining hemochromatosis Case report
by Chmieliauskas, S and Banionis, D and Laima, S and Andriuskeviciute, G and Mazeikiene, S and Stasiuniene, J and Jasulaitis, A and Jarmalaite, S
MEDICINE, ISSN 0025-7974, 12/2017, Volume 96, Issue 49, p. e8788
Rationale: Hemochromatosis is a disorder, associated with an abnormal accumulation of iron leading to toxic organ damage. Clinical symptoms develop during a... 
forensic medicine | iron storage disease | MEDICINE, GENERAL & INTERNAL | pathology | HEPATIC IRON OVERLOAD | hemochromatosis | UPDATE | HEREDITARY HEMOCHROMATOSIS | CLINICAL DIAGNOSES | Acidosis - etiology | Humans | Middle Aged | Male | Autopsy - methods | Hemochromatosis - pathology | Multiple Organ Failure - diagnosis | Shock - etiology | Shock - diagnosis | Hemochromatosis - complications | Multiple Organ Failure - etiology | Acidosis - diagnosis | Fatal Outcome | Hemochromatosis - genetics
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6. Full Text Hemochromatosis: An endocrine liver disease
by Pietrangelo, Antonello
Hepatology, ISSN 0270-9139, 10/2007, Volume 46, Issue 4, pp. 1291 - 1301
This review acknowledges the recent and dramatic advancement in the field of hemochromatosis and highlights the surprising analogies with a prototypic... 
JUVENILE HEMOCHROMATOSIS | MAGNETIC-RESONANCE | HEPCIDIN | PHENOTYPE | MUTATIONS | HEREDITARY HEMOCHROMATOSIS | GASTROENTEROLOGY & HEPATOLOGY | IRON OVERLOAD | EXPRESSION | NONINVASIVE PREDICTION | FERROPORTIN DISEASE | Hemochromatosis Protein | Membrane Proteins - genetics | Humans | Histocompatibility Antigens Class I - genetics | Antimicrobial Cationic Peptides - metabolism | Iron - metabolism | Histocompatibility Antigens Class I - metabolism | Hepcidins | Homeostasis - physiology | Membrane Proteins - metabolism | Mutation | Endocrine System Diseases - physiopathology | Hemochromatosis - physiopathology
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7. Full Text Cardiac Involvement in Hemochromatosis
by Gulati, Vinay and Harikrishnan, Prakash and Palaniswamy, Chandrasekar and Aronow, Wilbert S and Jain, Diwakar and Frishman, William H
Cardiology in Review, ISSN 1061-5377, 03/2014, Volume 22, Issue 2, pp. 56 - 68
Cardiac hemochromatosis or primary iron-overload cardiomyopathy is an important and potentially preventable cause of heart failure. This is initially... 
cardiomyopathy | iron overload | hemochromatosis | VENTRICULAR DIASTOLIC FUNCTION | NON-HFE HEMOCHROMATOSIS | CARDIAC & CARDIOVASCULAR SYSTEMS | MYOCARDIAL IRON | BETA-THALASSEMIA MAJOR | T2-ASTERISK-CARDIOVASCULAR MAGNETIC-RESONANCE | TRANSFUSION-DEPENDENT THALASSEMIA | IRON-OVERLOAD CARDIOMYOPATHY | STEM-CELL TRANSPLANTATION | NT-PROBNP LEVELS | ASYMPTOMATIC HEREDITARY HEMOCHROMATOSIS | Heart - physiopathology | Hemochromatosis - therapy | Iron Chelating Agents - therapeutic use | Liver - pathology | Humans | Heart Failure - physiopathology | Phlebotomy | Survival Rate | Treatment Outcome | Iron - metabolism | Hemochromatosis - complications | Biopsy | Heart Failure - etiology | Heart Failure - mortality | Hemochromatosis - physiopathology
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8. Full Text Current approach to hemochromatosis
by Brissot, Pierre and Troadec, Marie-Bérengère and Bardou-Jacquet, Edouard and Lan, Caroline Le and Jouanolle, Anne-Marie and Deugnier, Yves and Loréal, Olivier
Blood Reviews, ISSN 0268-960X, 2008, Volume 22, Issue 4, pp. 195 - 210
Summary Iron overload diseases of genetic origin are an ever changing world, due to major advances in genetics and molecular biology. Five major categories are... 
Hematology, Oncology and Palliative Medicine | Iron overload | Ferroportin | Transferrin | Hemochromatosis | Hemojuvelin | Venesection | HFE | Saturation ferritin | Hepcidin | Transferrin receptor 2 | iron overload | TRANSFERRIN RECEPTOR-2 | hepcidin | JUVENILE HEMOCHROMATOSIS | transferrin | hemojuvelin | ferroportin | HEREDITARY HEMOCHROMATOSIS | PHENOTYPIC-EXPRESSION | venesection | ANTIMICROBIAL PEPTIDE HEPCIDIN | transferrin receptor 2 | HEPATIC IRON OVERLOAD | hemochromatosis | MICROCYTIC ANEMIA | AUTOSOMAL-DOMINANT HEMOCHROMATOSIS | MIDDLE-AGED WOMEN | saturation ferritin | HEMATOLOGY | FERROPORTIN DISEASE | Antimicrobial Cationic Peptides - immunology | Benzoates - therapeutic use | Hemochromatosis Protein | Humans | Iron Overload - immunology | Hemochromatosis - etiology | Antimicrobial Cationic Peptides - metabolism | Hemochromatosis - pathology | Histocompatibility Antigens Class I - metabolism | Iron Overload - metabolism | Cation Transport Proteins - metabolism | Hepcidins | Membrane Proteins - metabolism | Antimicrobial Cationic Peptides - genetics | Triazoles - therapeutic use | Hemochromatosis - therapy | Iron Chelating Agents - therapeutic use | Iron Overload - genetics | Membrane Proteins - genetics | Histocompatibility Antigens Class I - immunology | Phlebotomy | Membrane Proteins - immunology | Histocompatibility Antigens Class I - genetics | Hemochromatosis - immunology | Antimicrobial Cationic Peptides - deficiency | Cation Transport Proteins - immunology
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9. Full Text Iron, hemochromatosis, and hepatocellular carcinoma
by Kowdley, Kris V
Gastroenterology, ISSN 0016-5085, 2004, Volume 127, Issue 5, pp. S79 - S86
Hereditary hemochromatosis (HH) is associated with an increased risk for hepatocellular carcinoma (HCC). The risk previously had been estimated to be as high... 
HFE GENE-MUTATIONS | P53 MUTATIONS | OXIDATIVE STRESS | NONCIRRHOTIC LIVER | LIVER IRON | CANCER-RISK | CIRRHOTIC-PATIENTS | AFRICAN-AMERICANS | HEREDITARY HEMOCHROMATOSIS | GASTROENTEROLOGY & HEPATOLOGY | IDIOPATHIC HEMOCHROMATOSIS | Carcinoma, Hepatocellular - epidemiology | Carcinoma, Hepatocellular - physiopathology | Cell Proliferation | Prevalence | Hemochromatosis Protein | Membrane Proteins - genetics | Humans | Risk Factors | Histocompatibility Antigens Class I - genetics | Iron - adverse effects | Incidence | Liver Neoplasms - epidemiology | Hemochromatosis - complications | Liver Neoplasms - physiopathology | Iron - pharmacokinetics | Kidney Failure, Chronic | Transplantation of organs, tissues, etc | Liver cancer | Iron | Hemochromatosis | Hepatoma
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10. Full Text Rare HFE variants are the most frequent cause of hemochromatosis in non‐c282y homozygous patients with hemochromatosis
by Hamdi‐Rozé, Houda and Beaumont‐Epinette, Marie‐Pascale and Ben Ali, Zeineb and Le Lan, Caroline and Loustaud‐Ratti, Véronique and Causse, Xavier and Loreal, Olivier and Deugnier, Yves and Brissot, Pierre and Jouanolle, Anne‐Marie and Bardou‐Jacquet, Edouard
American Journal of Hematology, ISSN 0361-8609, 12/2016, Volume 91, Issue 12, pp. 1202 - 1205
p.Cys282Tyr (C282Y) homozygosity explains most cases of HFE‐related hemochromatosis, but a significant number of patients presenting with typical type I... 
DIAGNOSIS | GENE | COMPOUND HETEROZYGOSITY | IRON-OVERLOAD | C282Y | MUTATIONS | HEMATOLOGY | ASSOCIATION | Genetic Variation | Homozygote | Iron Overload - genetics | Hemochromatosis Protein - genetics | Humans | Middle Aged | Adult | Female | Male | Aged | Sequence Analysis, DNA | Hemochromatosis - genetics | Hemochromatosis | Life Sciences | Human health and pathology | Hépatology and Gastroenterology | Cancer
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11. Full Text Variable expressivity of HJV related hemochromatosis: "Juvenile" hemochromatosis?
by Hamdi-Roze, H and Ben Ali, Z and Ropert, M and Detivaud, L and Aggoune, S and Simon, D and Pelletier, G and Deugnier, Y and David, V and Bardou-Jacquet, E
BLOOD CELLS MOLECULES AND DISEASES, ISSN 1079-9796, 02/2019, Volume 74, pp. 30 - 33
Juvenile hemochromatosis is a rare autosomal recessive disease due to variants in the Hemojuvelin (HJV) gene. Although biological features mimic HFE... 
GENETIC-VARIATION | NON-HFE | PROTEIN | VARIANTS | IRON-OVERLOAD | MEMBRANE | HEMOJUVELIN GENE | MUTATIONS | HEREDITARY HEMOCHROMATOSIS | GUIDANCE MOLECULE | HEMATOLOGY | Transferrin | Humans | GPI-Linked Proteins | Male | Genetic Variation | Young Adult | Hemochromatosis - congenital | Age of Onset | Adult | Female | Hepcidins - deficiency | Hemochromatosis - diagnosis | Child | Iron Overload | Hemochromatosis | Molecular genetics | Genetic screening
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12. Full Text Hemochromatosis: Genetics and pathophysiology
by Beutler, Ernest
Annual Review of Medicine, ISSN 0066-4219, 2006, Volume 57, Issue 1, pp. 331 - 347
A number of genetic disorders can result in the accumulation of excess iron in the body. These causes of hereditary hemochromatosis include defects in genes... 
Ferroportin | Penetrance | Iron | Hepcidin | HFE | TRANSFERRIN RECEPTOR-2 | hepcidin | VOLUNTARY BLOOD-DONORS | penetrance | HJV GENE | ferroportin | IRON REGULATORY PEPTIDE | HEREDITARY HEMOCHROMATOSIS | PHENOTYPIC-EXPRESSION | FERROPORTIN-1 GENE | 1Q-LINKED JUVENILE HEMOCHROMATOSIS | ANTIMICROBIAL PEPTIDE HEPCIDIN | MEDICINE, GENERAL & INTERNAL | HEPATOCELLULAR-CARCINOMA | iron | Hemochromatosis Protein | Hepcidins | Membrane Proteins - genetics | Humans | GPI-Linked Proteins | Cation Transport Proteins - genetics | Histocompatibility Antigens Class I - genetics | Receptors, Transferrin - genetics | Antimicrobial Cationic Peptides - genetics | Hemochromatosis - genetics | Hemochromatosis - physiopathology | Physiology, Pathological | Hemochromatosis | Genetic aspects | Research | History | Population genetics | Risk factors
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