婴儿型多囊肾的超声诊断及鉴别诊断

Ultrasonic diagnosis and differential diagnosis of infantile polycystic kidney disease

目的探讨婴儿型多囊肾的超声表现及鉴别诊断。方法回顾性分析10例婴儿型多囊肾(IPCKD)患儿的肾脏超声表现,并与分泌性尿路造影及肝、肾功能相对照。结果IPCKD超声表现为肾脏弥漫性增大,皮、髓质界限显示不清,肾实质内可见广泛成簇的强回声,弥漫性分布,后伴彗星尾,并伴有不同程度的肝、脾肿大,肝脏纤维化或肝内胆管囊状扩张;且肝、脾肿大程度及肝脏纤维化程度与肾功能受损程度成反比。肝功能基本正常。分泌性尿路造影均显示对比剂在髓质的囊肿中滞留,呈不规则斑纹或条状影像;滞留在集合管内产生放射状影像,肾盂肾盏轻度受压变窄。结论超声结合分泌性尿路造影有助于确诊IPCKD。

Objective To discuss ultrasonic diagnosis and differential diagnosis of infantile polycystic kidney disease （IPCKD）. Methods Ultrasonographic features of 10 infants with IPCKD were retrospectively analyzed, and compared with excretory urography and liver and renal functions. Results The ultrasonographic manifestations of IPCKD were characterized as diffusive enlargement of kidney, poor kidney structure formation, high intensity clustered echo in the renal parenehyma, diffusive distribution plus cometal tail sign, different degrees enlargement of liver and spleen, liver fibrosis or bile duct cystic dilatation of liver. The degree of kidney lesions was inversely proportional to the degrees of enlargement of liver and spleen, as well as liver fibrosis. The liver function of patient with IPCKD was relatively normal. Excretory urography showed contrast agent remaining in medullary cysts with irregular markings or strip images, remaining in the collection tube produced radial imaging, and renal pelvis and cup mild pressed and narrowed down. Conclusion Combinedd with excretory urography, ultrasonography helps in diagnosing infantile polyeystic kidney disease.