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The relationship between iron overload and clinical characteristics in a Spanish cohort of 100 C282Y homozygous hemochromatosis patients

机译:西班牙100例C282Y纯合血色素沉着症患者队列中铁超负荷与临床特征的关系

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摘要

We studied the relationship between iron removed by venesection, sex, age, and clinical characteristics in a group of 100 Spanish probands with hereditary hemochromatosis (HH), all C282Y homozygous in the HFE gene. Iron overload was higher in men than in women (P < 0.0001) and increased with age (P = 0.02). Forty-four patients presented with liver disease (28 had fibrosis–cirrhosis of the liver), 24 with diabetes, 18 with arthropathy, and 13/73 men with impotence. No clinical consequences of hemochromatosis were observed in 43 patients. The number of clinical complications was higher in men (P = 0.01) and increased with age (P = 0.006) and with the amount of iron removed (P < 0.0001). The amount of iron removed was significantly higher by univariate analysis in patients with liver disease (P < 0.0001), diabetes (P = 0.007), arthropathy (P = 0.006), and impotence (P = 0.003) than in patients without these complications. In the multivariant analysis, only liver disease maintained a significant relationship with the amount of iron removed (P < 0.0001). Diabetes and arthropathy were closely related with previous liver disease, and impotence appeared mainly in hemochromatosic men with diabetes and alcoholism.
机译:我们研究了一组100例遗传性血色素沉着病(HH),HFE基因均为C282Y纯合子的西班牙先证者的穿刺切除铁,性别,年龄和临床特征之间的关系。男性的铁超载高于女性(P <0.0001),并随年龄增加(P = 0.02)。患有肝病的患者有44例(28例患有肝纤维化-肝硬化),24例患有糖尿病,18例患有关节炎和13/73例阳imp男性。在43例患者中未观察到血色素沉着症的临床后果。男性的临床并发症发生率更高(P = 0.01),并且随着年龄的增长而增加(P = 0.006),并且铁的去除量也有所增加(P <0.0001)。通过单因素分析,肝病患者(P <0.0001),糖尿病(P = 0.007),关节炎(P = 0.006)和阳imp(P = 0.003)的铁清除量明显高于无这些并发症的患者。在多变量分析中,只有肝脏疾病与铁清除量之间存在显着相关性(P <0.0001)。糖尿病和关节炎与先前的肝病密切相关,阳imp主要出现在患有糖尿病和酒精中毒的血色素性男性中。

著录项

  • 来源
    《Annals of Hematology》 |2007年第11期|831-835|共5页
  • 作者单位

    Hematology Department Hospital de l’Esperit Sant de Santa Coloma de Gramenet Barcelona Spain;

    Hematology Department Hospital de l’Esperit Sant de Santa Coloma de Gramenet Barcelona Spain;

    Hematology Department Hospital de l’Esperit Sant de Santa Coloma de Gramenet Barcelona Spain;

    Hematology Department Hospital de l’Esperit Sant de Santa Coloma de Gramenet Barcelona Spain;

    Hematology Department Hospital de Blanes-Calella Barcelona Spain;

    Hematology and Genetics Service Hospital de Sant Pau Barcelona Spain;

    Hematology and Genetics Service Hospital de Sant Pau Barcelona Spain;

    Hematology and Genetics Service Hospital de Sant Pau Barcelona Spain;

    Hematology and Genetics Service Hospital de Sant Pau Barcelona Spain;

  • 收录信息
  • 原文格式 PDF
  • 正文语种 eng
  • 中图分类
  • 关键词

    Hemochromatosis; Liver cirrhosis; Diabetes; Arthropathy; Impotence;

    机译:血色素沉着症;肝硬化;糖尿病;关节炎;阳I;

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