Hemochromatosis, iron overload, and porphyria cutanea tarda

Bonkovsky, Herbert L.; Lambrecht, Richard W.

doi:10.1017/cbo9780511666476.045

Cited by 12 publications

(22 citation statements)

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“…Iron is a co-factor that influences the severity and progression of non-hemochromatotic liver diseases, especially steatohepatitis and viral hepatitis1–7. The most common known genetic factor that leads to iron loading is genetic variation in the HFE gene.…”

Section: Introductionmentioning

confidence: 99%

Iron Levels in Hepatocytes and Portal Tract Cells Predict Progression and Outcomes of Patients With Advanced Chronic Hepatitis C

et al. 2011

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Background & Aims Iron might influence severity and progression of non-hemochromatotic liver diseases. We assessed the relationships between iron, variants in HFE, and progression and outcomes using data from the HALT-C Trial. We determined whether therapy with pegylated interferon (PegIFN) affects iron variables. Methods Participants were randomly assigned to groups given long-term therapy with PegIFN (n=400) or no therapy (n=413) for 3.5 y and followed for up to 8.7 y (median 6.0 y). Associations between patient characteristics and iron variables, at baseline and over time, were made using Kaplan-Meier analyses, Cox regression models, and repeated measures analysis of covariance. Iron was detected by Prussian blue staining. Results Patients with poor outcomes (increase in Child-Turcotte-Pugh score to ≥ 7, development of ascites, encephalopathy, variceal bleeding, spontaneous bacterial peritonitis, hepatocellular carcinoma, death) had significantly higher baseline scores for stainable iron in hepatocytes and cells in portal tracts than those without outcomes. Staining for iron in portal triads correlated with lobular and total Ishak inflammatory and fibrosis scores (P<0.0001). High baseline levels of iron in triads increased the risk for poor outcome (hazard ratio=1.35, P=0.02). Iron staining decreased in hepatocytes but increased in portal stromal cells over time (P<0.0001). Serum levels of iron and total iron binding capacity decreased significantly over time (P <0.0001), as did serum ferritin (P=0.0003). Long-term therapy with PegIFN did not affect levels of iron staining. Common variants in HFE did not correlate with outcomes, including development of hepatocellular carcinoma. Conclusions Degree of stainable iron in hepatocytes and portal tract cells predicts progression and clinical and histological outcomes of patients with advanced chronic hepatitis C. Long-term therapy with low-dose PegIFN did not improve outcomes or iron variables.

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Section: Introductionmentioning

confidence: 99%

Iron Levels in Hepatocytes and Portal Tract Cells Predict Progression and Outcomes of Patients With Advanced Chronic Hepatitis C

et al. 2011

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“…Mutations in the HFE gene cause increased intestinal absorption, and 50-100% of patients with hereditary hemochromatosis (HH) are homozygous for the C282Y mutation [4][5][6]. Furthermore, compound or sole heterozygosity for the mutations C282Y and H63D provide increased capability of iron absorption and in particular circumstances lead to an iron-replete state [7][8][9].…”

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Prevalence of Hemochromatosis Gene <i>(HFE)</i> Mutations in Greek Patients with Myelodysplastic Syndromes

et al. 2003

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“…Although serum ferritin is well known to be a positive acute phase reactant, the increased frequency and degree of increase of serum ferritin level in chronic hepatitis C has been greater than for other types of chronic liver disease. 25 Therefore, we found higher ferritin levels in patients with CHC compared to the patients with CHB.…”

Section: Discussionmentioning

confidence: 56%

Relationship Between Hemochromatosis Gene Mutations and Degree of Fibrosis in Liver Disease Associated with Chronic Hepatitis B and C

Bağır¹,

Öksüz²,

Doğan³

et al. 2012

Turkiye Klinikleri J Med Sci

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Hemochromatosis, iron overload, and porphyria cutanea tarda

Cited by 12 publications

References 0 publications

Iron Levels in Hepatocytes and Portal Tract Cells Predict Progression and Outcomes of Patients With Advanced Chronic Hepatitis C

Iron Levels in Hepatocytes and Portal Tract Cells Predict Progression and Outcomes of Patients With Advanced Chronic Hepatitis C

Prevalence of Hemochromatosis Gene <i>(HFE)</i> Mutations in Greek Patients with Myelodysplastic Syndromes

Relationship Between Hemochromatosis Gene Mutations and Degree of Fibrosis in Liver Disease Associated with Chronic Hepatitis B and C

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