Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF)

被引：95
作者：
Turkbey, Baris [2 ]
Ocak, Iclal [2 ]
Daryanani, Kailash [3 ]
Font-Montgomery, Esperanza [1 ]
Lukose, Linda [1 ]
Bryant, Joy [1 ]
Tuchman, Maya [1 ]
Mohan, Parvathi [4 ]
Heller, Theo [5 ]
Gahl, William A. [1 ]
Choyke, Peter L. [2 ]
Gunay-Aygun, Meral [1 ,6 ]
机构：
[1] NHGRI, Med Genet Branch, NIH, Bethesda, MD 20892 USA
[2] NCI, Mol Imaging Program, NIH, Bethesda, MD 20892 USA
[3] NIH, Ctr Clin, Dept Radiol, Bethesda, MD 20892 USA
[4] George Washington Univ, Dept Pediat Gastroenterol, Washington, DC USA
[5] NIDDK, NIH, Bethesda, MD USA
[6] NIH, Intramural Program, Off Rare Dis, Off Directors, Bethesda, MD 20892 USA
来源：
PEDIATRIC RADIOLOGY | 2009年 / 39卷 / 02期
基金：
美国国家卫生研究院;
关键词：
Autosomal recessive polycystic kidney disease; Congenital hepatic fibrosis; US; MR cholangiography; Children; GENE;
D O I：
10.1007/s00247-008-1064-x
中图分类号：
R72 [儿科学];
学科分类号：
100202 ;
摘要：
ARPKD/CHF is an inherited disease characterized by non-obstructive fusiform dilatation of the renal collecting ducts leading to enlarged spongiform kidneys and ductal plate malformation of the liver resulting in congenital hepatic fibrosis. ARPKD/CHF has a broad spectrum of clinical presentations involving the kidney and liver. Imaging plays an important role in the diagnosis and follow-up of ARPKD/CHF. Combined use of conventional and high-resolution US with MR cholangiography in ARPKD/CHF patients allows detailed definition of the extent of kidney and hepatobiliary manifestations without requiring ionizing radiation and contrast agents.
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收藏
页码：100 / 111
页数：12
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