BACKGROUND While typical ultrasound patterns of ciliopathy-related cystic kidney diseases have been described in children, ultrasound findings can overlap between different diseases and atypical patterns exist. In this study, we assessed the presence of the "salt and pepper" pattern in different renal ciliopathies and looked for additional ultrasound features. METHODS This single-center, retrospective study included all patients with a molecular-proven diagnosis of renal ciliopathy, referred to our center between 2007 and 2017. Images from the first and follow-up ultrasound exams were reviewed. Basic ultrasound features were grouped into patterns and compared to genetic diagnoses. The "salt and pepper" aspect was described as enlarged kidneys with heterogeneous, increased parenchymal echogenicity. RESULTS A total of 41 children with 5 different renal ciliopathies were included (61% male; median age, 6 years [range, 3 days to 17 years]). The "salt and pepper" pattern was present in 14/15 patients with an autosomal recessive polycystic kidney disease (ARPKD). A similar pattern was found in 1/4 patients with an autosomal dominant polycystic kidney disease and in 1/11 patients with HNF1B mutation. Additional signs found were areas of cortical sparing, comet-tail artifacts, and color comet-tail artifacts. CONCLUSION Although the "salt and pepper" ultrasound pattern is predominantly found in ARPKD, it may be detected in other ciliopathies. The color comet-tail artifact is an interesting sign when suspecting a renal ciliopathy in case of enlarged hyperechoic kidneys with no detectable microcysts on B-mode grayscale ultrasound.

中文翻译：

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一组经分子验证的，与睫状体病相关的肾脏疾病诊断的儿童，其肾脏超声检查的“盐和胡椒”模式。

背景技术虽然已经描述了儿童中与睫状体病相关的囊性肾脏疾病的典型超声模式，但是超声发现可能在不同疾病之间重叠并且存在非典型模式。在这项研究中，我们评估了不同的肾脏纤毛病中“盐和胡椒”模式的存在，并寻找其他超声特征。方法这项单中心，回顾性研究包括2007年至2017年间转诊至我们中心的所有经分子检验证实患有肾纤毛病的患者。我们回顾了首次超声检查和后续超声检查的图像。将基本的超声特征分组为模式，并与遗传诊断进行比较。“盐和胡椒”方面被描述为肾脏变大，实质性回声增加。结果共纳入41名儿童，其中有5种不同的肾脏纤毛病（男61％；中位年龄6岁[范围：3天至17岁]）。14/15患有常染色体隐性隐性多囊肾病（ARPKD）的患者存在“盐和胡椒”模式。在常染色体显性遗传性多囊肾疾病的1/4患者和HNF1B突变的1/11患者中发现了类似的模式。发现的其他迹象还包括皮质稀疏区域，彗尾伪影和彩色彗尾伪影。结论尽管在ARPKD中主要发现“盐和胡椒”超声图，但在其他纤毛病中也可能检测到。