| 文亮,韩丹.婴儿及少儿型常染色体隐性多囊性肾病的CT与MRI表现[J].中国医学影像技术,2017,33(2):271~275 |
| 婴儿及少儿型常染色体隐性多囊性肾病的CT与MRI表现 |
| CT and MRI manifestations of infantile and juvenile autosomal recessive polycystic kidney disease |
| 投稿时间：2016-07-23 修订日期：2016-12-05 |
| DOI：10.13929/j.1003-3289.201607099 |
| 中文关键词: 常染色体隐性多囊性肾病 磁共振成像 体层摄影术,X线计算机 |
| 英文关键词:Autosomal recessive polycystic kidney disease Magnetic resonance imaging Tomography,X-ray computed |
| 基金项目: |
|
| 摘要点击次数: 1895 |
| 全文下载次数: 914 |
| 中文摘要: |
| 目的 观察婴儿及少儿型常染色体隐性多囊性肾病（ARPKD）的CT、MRI表现。方法 回顾性分析11例婴儿及少儿型ARPKD患者腹部CT及MRI上肝叶比例、双肾体积改变以及胆管与肾脏囊样病灶的表现。结果 11例婴儿及少儿型ARPKD患者中，5例（5/11，45.45%）肝叶比例失调。5例（5/11，45.45%）中央区域肝内胆管以梭形扩张为主、3例（3/11，27.27%）肝边缘部胆管扩张明显、3例（3/11，27.27%）沿门静脉周围可见多发囊样灶。2例（2/11，18.18%）肾脏体积增大。8例（8/11，72.73%）双肾髓质可见多发小囊样灶、3例（3/11，27.27%）双肾皮髓质多发囊肿。8例（8/11，72.73%）门静脉增粗、脾大。2例（2/11，18.18%）腹部可见多发肿大淋巴结。5例（5/11，45.45%）MR平扫T1WI显示双肾皮髓质分界不清。结论 婴儿或少儿型ARPKD以肝胆改变为主，特征性CT及MRI表现为肝叶比例与肾脏体积的改变合并胆管扩张及双肾囊样灶。 |
| 英文摘要: |
| Objective To observe the CT and MRI appearances of infantile and juvenile autosomal recessive polycystic kidney disease (ARPKD).Methods The CT and MRI appearance of 11 patients with infantile or juvenile ARPKD were analyzed retrospectively, including changes of liver lobe proportion, kidney size and cystic lesion of biliary tree and kidney.Results Among 11 cases of infantile and juvenile ARPKD, five (5/11, 45.45%) cases showed changes of liver lobe proportion. Fusiform dilation of central ducts was found in 5 cases (5/11, 45.45%), prominent biliary dilation in peripherial liver was noted in 3 cases (3/11, 27.27%), cystic lesions along portal vein were shown in 3 cases (3/11, 27.27%). Two cases (2/11, 18.18%) showed renal enlargement. Multiple cystic lesions in the medullae and multifocal parenchymal cysts of bilateral kidneys were found in 8 (8/11, 72.73%) and 3 (3/11, 27.27%) cases respectively. Portal vein enlargement and splenomegaly were found in 8 cases (8/11, 72.73%). Two cases (2/11, 18.18%) were associated with abdominal lymphadenopathy. In unenhanced MR, 5 cases (5/11, 45.45%) showed obscuration of corticomedullary junction in kidneys.Conclusion The main findings are changes of liver and bile ducts in infantile or juvenile ARPKD. The characteristic CT and MRI manifestations are changes of liver lobe proportion and kidney size associated with hepatobiliary dilation and kidney cystic lesions. |
| 查看全文 查看/发表评论 下载PDF阅读器 |
|
|
|
