HEMOCHROMATOSIS相关论文
Iron is an essential element for most living organisms on earth,including human beings.An inability to maintain iron hom......
An unhappy triad: Hemochromatosis, porphyria cutanea tarda and hepatocellular carcinoma-A case repor
Liver fibrosis and cirrhosis are predisposing factors for the development of hepatocellular carcinoma (HCC). Hemosideros......
AIM: To evaluate the prevalence of HFE gene mutation and indices of disturbed iron homeostasis in alcoholics with and wi......
目的 评价安络化纤丸联合干扰素γ治疗鼠血吸虫肝纤维化的效应机制及其对肝色素沉积的影响.方法 将30只昆明小鼠分为健康对照组、......
遗传性血色病是一种遗传代谢性肝病,是由血色病相关基因突变导致过多的铁沉积在肝脏、胰腺、皮肤、垂体、性腺等全身多个器官组织,表......
By modulating hepcidin production, an organism controls intestinal iron absorption, iron uptake and mobilization from st......
There is a strong association between porphyria cutanea tarda (PCT) and chronic viral hepatitis C. Therapy for chronic v......
原发性血色病是因为铁吸收过多形成体内铁贮存过量而导致以肝实质损害为主要特征的遗传性疾病,欧美国家常见,通常在40 ~ 50岁发病,......
2007年,中南大学湘雅二医院建立肝铁浓度测定方法,至今已测定370例患者,发现6例肝铁浓度>5000μg/g(肝组织干重),其中2例>15 000 μg......
原发性血色病是由6号染色体短臂上的血色病遗传基因突变引起的常染色体隐性遗传病.其发病机制为铁质过度沉积于组织器官,并对其结......
1.患者资料:患者女性,40岁,因反复乏力、皮肤巩膜黄染30余年,发现肝硬化6年于2013年8月1日入院.入院时查体:慢性肝病面容,全身皮肤......
一、病例资料男性患者,47岁,因"肝功能异常50余天"入院.患者2月前无明显诱因出现四肢乏力,下肢明显,生物化学检查显示:ALT为69.3 U......
目的探讨不同原因造成的血色病肝脏铁沉积程度、分布特点及其与组织学改变的关系。方法对31例血色病患者肝活体组织标本进行HE、网......
为探究吕家坨井田地质构造格局,根据钻孔勘探资料,采用分形理论和趋势面分析方法,研究了井田7......
总结1例继发性血色病合并多器官损害患者的护理,采取去铁胺治疗,护理上给予卧床休息与活动指引、吸氧、监测生命体征等,低铁饮食、......
An unhappy triad: Hemochromatosis, porphyria cutanea tarda and hepatocellular carcinoma-A case repor
Liver fibrosis and cirrhosis are predisposing factors for the development of hepatocellular carcinoma (HCC). Hemosideros......
Genetic diagnosis history and osteoarticular phenotype of a nontransfusion secondary hemochromatosis
BACKGROUND It is not easy to identify the cause of various iron overload diseases because the phenotypes overlap.Therefo......
Genetic diagnosis history and osteoarticular phenotype of a nontransfusion secondary hemochromatosis
BACKGROUND It is not easy to identify the cause of various iron overload diseases because the phenotypes overlap.Therefo......
因血色病中的慢性铁负荷能引起器官损伤,肾脏缺血再灌注时的损也与铁有关,因此我们评价了轻度亚急性铁负荷增加鼠脂南过氧化物及缺血......
遗传性血色素沉着症(hereditary hemochromatosis,HH)是北欧人群最常见的遗传疾病之一。最近HH在诊断、管理及治疗方面有了新的进......
Liver steatosis correlates with iron overload but not with HFE gene mutations in chronic hepatitis C
BACKGROUND: Liver steatosis and iron overload, which are frequently observed in chronic hepatitis C (CHC), may contribut......
AIM: To report a patient with C282Y homozygocity, depleted body iron and intestinal atrophy caused by celiac disease (CD......
尽管在成像和遗传的进步允许基因铁超载的大多数盒子的非侵略的诊断,肝病理仍然保持经常有用( 1 )联系了损害由分级纤维变性并且寻......
An unhappy triad: Hemochromatosis, porphyria cutanea tarda and hepatocellular carcinoma-A case repor
肝纤维变性和肝硬化正在预先安排为肝细胞癌(HCC ) 的发展的因素。含铁血黄素沉积也被描述了触发致癌作用。重要的铁超载在世袭 he......
由 modulating hepcidin 生产,一个有机体从店控制肠的铁吸收,铁举起和动员到会身体铁需要。在最近的年里,在我们也为理解一些人的混......
目的探讨血色病的CT表现,以提高对本病的认识。方法回顾性分析经穿刺活检病理证实的6例血色病患者的CT表现,并结合文献,对此病的病......
1关于基因检测的建议1.1对普通人群不推荐进行HFE基因相关性血色病基因检测,因为本病的外显率低且只有少数C282Y纯合子会发展为铁超......
遗传性血色病(hereditary hemochromatosis,HH)是由铁代谢紊乱引起体内铁负荷过多所致的一种常染色体院性遗传疾病。临床表现为乏力......
目的:探讨1例新型的遗传性血色病(HH)患者家系 HFE 基因突变形式。方法对确诊的1例 HH 患者分析其与5位相关亲属的血色病基因,提取血液......
目的了解3个携带HJV E3D变异的遗传性血色病家系基因变异及临床表型特点。方法 3个遗传性血色病家系中的先证者均完成了病史采集、......
目的探讨血色素沉积症(HC)的临床病理特点,为临床诊治提供依据。方法选择行肝穿刺确诊HC患者20例,肝组织行HE和组织化学染色,进行临......
目的:探讨血色素沉着症的MRI表现,分析其MRI特征,对病变组织的铁质沉积作初步定量分析,同时评价MRI在血色素沉着症的诊断中的价值。......
The regulation of iron metabolism involves multiple organs including the duodenum,liver and bone marrow.The recent disco......
Neonatal cholestasis and hepatosplenomegaly caused by congenital dyserythropoietic anemia type 1:A c
BACKGROUND Congenital dyserythropoietic anemia type 1(CDA1)is an autosomal recessive disorder of ineffective erythropoie......
Comprehensive analysis of HFE gene in hereditary hemochromatosis and in diseases associated with acq
BACKGROUND Patients with hepatitis C virus(HCV)and hepatocellular carcinoma(HCC)may or not develop iron overload(IO),whi......
Efficacy of deferasirox for the treatment of iron overload in a child affected by Juvenile Hemochrom
We report the case of a 7 years old girl with Juvenile Hemochromatosis, due to homozygous mutation of HJV, which had inc......
Safe trafficking of iron across the cell membrane is a delicate process that requires specific protein carriers. While m......
Juvenile hemochromatosis:HAMP mutation and severe iron overload treated with phlebotomies and defera
Juvenile hemochromatosis(JH)is a rare condition classified as an autosomal recessive disorder that leads to severe iron ......
Advances in recent years in the understanding of, and the genetic diagnosis of hereditary hemochromatosis (HH) have chan......
Due to blood transfusions,thalassemics are often infected with either hepatitis C virus(HCV)or hepatitis B virus and oft......
AIM To investigat the influence of hemochromatosis gene(Hfe)mutation on 59Fe labelled duodenal heme absorption in mice.M......