成人常染色体隐性遗传性多囊肾病1例报告
常染色体隐性遗传性多囊肾病（autosomal recessive polycystic kidney disease,ARPKD）,也称多囊肾肝病（polycystic kidney and hepatic disease,PKHD）,是一种多发于儿童肾脏及肝脏的严重单基因遗传病,发病率为1∶20 000。该病主要表现为双肾形成多个进行性增大的囊肿,后期出现肾功能不全,严重者需透析治疗;肝脏表现为不同程度的肝囊肿和肝硬化、胆管扩张以及先天性的肝门静脉纤维化。患者多数早年夭折,极少存活至成年,成年病例国内未见报道,...
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| Published in | 临床肝胆病杂志 Vol. 33; no. 3; pp. 521 - 522 |
|---|---|
| Main Author | |
| Format | Journal Article |
| Language | Chinese |
| Published |
吉林大学白求恩第一医院肝胆胰外科,长春,130021
2017
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| Subjects | |
| Online Access | Get full text |
| ISSN | 1001-5256 |
| DOI | 10.3969/j.issn.1001-5256.2017.03.026 |
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| Abstract | 常染色体隐性遗传性多囊肾病（autosomal recessive polycystic kidney disease,ARPKD）,也称多囊肾肝病（polycystic kidney and hepatic disease,PKHD）,是一种多发于儿童肾脏及肝脏的严重单基因遗传病,发病率为1∶20 000。该病主要表现为双肾形成多个进行性增大的囊肿,后期出现肾功能不全,严重者需透析治疗;肝脏表现为不同程度的肝囊肿和肝硬化、胆管扩张以及先天性的肝门静脉纤维化。患者多数早年夭折,极少存活至成年,成年病例国内未见报道, |
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| AbstractList | R575; @@ 常染色体隐性遗传性多囊肾病（autosomal recessive polycystic kidney disease,ARPKD）,也称多囊肾肝病（polycystic kidney and hepatic disease,PKHD）,是一种多发于儿童肾脏及肝脏的严重单基因遗传病,发病率为1∶20 000。该病主要表现为双肾形成多个进行性增大的囊肿,后期出现肾功能不全,严重者需透析治疗;肝脏表现为不同程度的肝囊肿和肝硬化、胆管扩张以及先天性的肝门静脉纤维化。患者多数早年夭折,极少存活至成年,成年病例国内未见报道, |
| Author | 刘丰 玄志鲁 刘凯 韩铠泽 杨帆 刘亚辉 |
| AuthorAffiliation | 吉林大学白求恩第一医院肝胆胰外科,长春130021 |
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| DocumentTitleAlternate | A case of adult autosomal recessive polycystic kidney disease |
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| Keywords | 肝肿瘤 polycystic kidney,autosomal recessive 病例报告 case reoports 肝硬化 liver neoplasms 多囊肾,常染色体隐性 adult 成年人 liver cirrhosis |
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| Notes | polyeystic kidney, autosomal recessive; liver neoplasms; liver cirrhosis; adult; case reoports LIU Feng, XUAN Zhilu, LIU Kai, et al. （ Departrrtent of Hepatn - Biliary - Pancreatic Surgery, The First ttospital of Jilin University, Changehun 130021, China） 22-1108/R |
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| PublicationTitleAlternate | Chinese Journal of Clinical Hepatology |
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| Publisher | 吉林大学白求恩第一医院肝胆胰外科,长春,130021 |
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| Snippet | 常染色体隐性遗传性多囊肾病（autosomal recessive polycystic kidney disease,ARPKD）,也称多囊肾肝病（polycystic kidney and hepatic disease,PKHD）,是一种多发于儿童肾脏及肝脏的严重单基因遗传病,发病率为1∶20... R575; @@ |
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| SubjectTerms | 多囊肾,常染色体隐性 成年人 病例报告 肝硬化 肝肿瘤 |
| Title | 成人常染色体隐性遗传性多囊肾病1例报告 |
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