常染色体隐性多囊肾病和先天性肝纤维化（ARPKD/CHF）
Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF).
作者信息
Turkbey Baris, Ocak Iclal, Daryanani Kailash, Font-Montgomery Esperanza, Lukose Linda, Bryant Joy, Tuchman Maya, Mohan Parvathi, Heller Theo, Gahl William A, Choyke Peter L, Gunay-Aygun Meral
机构信息
Molecular Imaging Program, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
出版信息
Pediatr Radiol. 2009 Feb;39(2):100-11. doi: 10.1007/s00247-008-1064-x. Epub 2008 Dec 17.
Abstract
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.
摘要
常染色体隐性多囊肾病/先天性肝纤维化是一种遗传性疾病，其特征为肾集合管非梗阻性梭形扩张，导致海绵肾增大，以及肝脏导管板畸形，从而引起先天性肝纤维化。常染色体隐性多囊肾病/先天性肝纤维化有涉及肾脏和肝脏的广泛临床表现。影像学在常染色体隐性多囊肾病/先天性肝纤维化的诊断和随访中起重要作用。在常染色体隐性多囊肾病/先天性肝纤维化患者中联合使用传统超声和高分辨率超声以及磁共振胰胆管造影，无需电离辐射和造影剂即可详细确定肾脏和肝胆表现的范围。
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