Hepatic and renal manifestations in autosomal dominant polycystic kidney disease: a dichotomy of two ends of a spectrum

被引:0
作者
van Gulick, J. J. M. [1 ]
Gevers, T. J. G. [1 ]
van Keimpema, L. [1 ]
Drenth, J. P. H. [1 ]
机构
[1] Radboud Univ Nijmegen, Med Ctr, Dept Gastroenterol & Hepatol, NL-6525 ED Nijmegen, Netherlands
关键词
ADPKD; hepatic phenotype; polycystic liver; diagnosis; treatment; LIVER-DISEASE; CLINICAL CHARACTERISTICS; CYSTS; VOLUME; SCLEROTHERAPY; ADPKD; TRANSPLANTATION; FENESTRATION; SOMATOSTATIN; OCTREOTIDE;
D O I
暂无
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Autosomal dominant polycystic kidney disease (ADPKD) is a multisystem disorder. It is the most common genetic cause of end-stage renal disease. One frequent extra-renal manifestation is hepatic cyst formation. The majority of ADPKD patients develop complications as a result of renal cyst formation; however, a small proportion develop extensive hepatic disease with minor renal features. Both phenotypes seem to represent the spectrum of ADPKD. This review discusses the current understanding of the pathogenesis of the disease, its manifestations and the mechanisms of cyst formation. Furthermore, it focuses on monitoring the disease and the treatment options currently available.
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页码:367 / 371
页数:5
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