PDF(Pubmed)
Abstract：
UNASSIGNED: Hemostatic iron regulator-hemochromatosis can result in progressive iron-loading and advanced hepatic fibrosis in some individuals. We studied total body and hepatic iron loading to determine whether the distribution of iron-loading influences the risk of advanced fibrosis.
UNASSIGNED: One hundred thirty-eight men and 66 women with hemochromatosis who underwent liver biopsy for staging of hepatic fibrosis had evaluation of hepatic iron concentration (HIC), hepatic iron index (HIC/age), total body iron stores (mobilizable iron), and mobilizable iron/HIC ratio (a marker of total body iron relative to hepatic iron). The potential impact of liver volume on mobilizable iron stores was assessed using magnetic resonance imaging in a separate cohort of 19 newly diagnosed individuals with hemochromatosis.
UNASSIGNED: Of 204 biopsied subjects, 41 had advanced fibrosis and exhibited 60% greater accumulation of mobilizable iron relative to HIC (mean 0.070 ± 0.008 g Fe/[μmol Fe/g]) compared with 163 subjects with low-grade fibrosis (mean 0.044 ± 0.002 g Fe/[μmol Fe/g], P < .0001). Linear regression modeling confirmed a discrete advanced hepatic fibrosis phenotype associated with greater mobilizable iron stores relative to HIC. The ratios of the upper to lower 95% limits of the distributions of liver volumes and the mobilizable iron/HIC ratios were 2.7 (95% confidence interval 2.3-3.0) and 9.7 (95% confidence interval 8.0-11.7), respectively, indicating that the distribution of liver volumes is not sufficiently wide to explain the variability in mobilizable iron/HIC ratios, suggesting that significant extrahepatic iron loading is present in those with advanced hepatic fibrosis.
UNASSIGNED: Advanced hepatic fibrosis develops in hemostatic iron regulator-hemochromatosis individuals who also have excessive extrahepatic mobilizable iron stores.
UNASSIGNED: One hundred thirty-eight men and 66 women with hemochromatosis who underwent liver biopsy for staging of hepatic fibrosis had evaluation of hepatic iron concentration (HIC), hepatic iron index (HIC/age), total body iron stores (mobilizable iron), and mobilizable iron/HIC ratio (a marker of total body iron relative to hepatic iron). The potential impact of liver volume on mobilizable iron stores was assessed using magnetic resonance imaging in a separate cohort of 19 newly diagnosed individuals with hemochromatosis.
UNASSIGNED: Of 204 biopsied subjects, 41 had advanced fibrosis and exhibited 60% greater accumulation of mobilizable iron relative to HIC (mean 0.070 ± 0.008 g Fe/[μmol Fe/g]) compared with 163 subjects with low-grade fibrosis (mean 0.044 ± 0.002 g Fe/[μmol Fe/g], P < .0001). Linear regression modeling confirmed a discrete advanced hepatic fibrosis phenotype associated with greater mobilizable iron stores relative to HIC. The ratios of the upper to lower 95% limits of the distributions of liver volumes and the mobilizable iron/HIC ratios were 2.7 (95% confidence interval 2.3-3.0) and 9.7 (95% confidence interval 8.0-11.7), respectively, indicating that the distribution of liver volumes is not sufficiently wide to explain the variability in mobilizable iron/HIC ratios, suggesting that significant extrahepatic iron loading is present in those with advanced hepatic fibrosis.
UNASSIGNED: Advanced hepatic fibrosis develops in hemostatic iron regulator-hemochromatosis individuals who also have excessive extrahepatic mobilizable iron stores.
摘要：
■止血铁调节剂-血色素沉着症可导致某些个体进行性铁负荷和晚期肝纤维化。我们研究了全身和肝脏铁负荷，以确定铁负荷的分布是否影响晚期纤维化的风险。
■138名男性和66名女性血色素沉着症患者接受肝活检以进行肝纤维化分期，评估肝铁浓度（HIC）。肝铁指数（HIC/年龄），全身铁储存(可移动铁)，和可动员铁/HIC比率（相对于肝铁的全身铁的标志物）。在19名新诊断为血色素沉着症的单独队列中，使用磁共振成像评估了肝脏体积对可动员铁储备的潜在影响。
■在204名活检对象中，与163名患有低度纤维化的受试者（平均0.044±0.002gFe/[μmolFe/g]相比，41名患有晚期纤维化，相对于HIC（平均0.070±0.008gFe/[μmolFe/g]），可动员铁的积累增加了60％，P<.0001)。线性回归模型证实了相对于HIC，与更大的可动员铁存储相关的离散晚期肝纤维化表型。肝脏体积分布的上限到下限95％的比率和可动员的铁/HIC比率分别为2.7（95％置信区间2.3-3.0）和9.7（95％置信区间8.0-11.7），分别,表明肝脏体积的分布不足以解释可动员铁/HIC比率的变异性，提示在晚期肝纤维化患者中存在显著的肝外铁负荷。
■在止血铁调节剂-血色素沉着症个体中发展为晚期肝纤维化，这些个体也具有过多的肝外可动员铁储备。
■138名男性和66名女性血色素沉着症患者接受肝活检以进行肝纤维化分期，评估肝铁浓度（HIC）。肝铁指数（HIC/年龄），全身铁储存(可移动铁)，和可动员铁/HIC比率（相对于肝铁的全身铁的标志物）。在19名新诊断为血色素沉着症的单独队列中，使用磁共振成像评估了肝脏体积对可动员铁储备的潜在影响。
■在204名活检对象中，与163名患有低度纤维化的受试者（平均0.044±0.002gFe/[μmolFe/g]相比，41名患有晚期纤维化，相对于HIC（平均0.070±0.008gFe/[μmolFe/g]），可动员铁的积累增加了60％，P<.0001)。线性回归模型证实了相对于HIC，与更大的可动员铁存储相关的离散晚期肝纤维化表型。肝脏体积分布的上限到下限95％的比率和可动员的铁/HIC比率分别为2.7（95％置信区间2.3-3.0）和9.7（95％置信区间8.0-11.7），分别,表明肝脏体积的分布不足以解释可动员铁/HIC比率的变异性，提示在晚期肝纤维化患者中存在显著的肝外铁负荷。
■在止血铁调节剂-血色素沉着症个体中发展为晚期肝纤维化，这些个体也具有过多的肝外可动员铁储备。
