Fig.2.

Artist’s rendering, ultrasound, and MRI findings showing the spectrum of kidney abnormalities in ARPKD. Percentages refer to the frequency of each pattern within our study population of 62 clinically and molecularly diagnosed ARPKD patients followed at the NIH Clinical Center [8]. (We had originally published this figure in the Clinical Journal of the American Society of Nephrology [8]; it is republished here with permission). A) Normal-sized kidneys with hyperechogenicity and ductal dilations involving parts of the medulla (white dots on artist’s rendering). B) Mildly enlarged kidneys with hyperechogenicity and ductal dilations involving most of the medulla but sparing the cortex. C) Enlarged kidneys with diffuse hyperechogenicity and ductal dilations sparing only parts of the cortex. Some macrocysts (black) are present. D) Massively enlarged kidneys with complete involvement of medulla and cortex and numerous macrocysts. MRI: magnetic resonance imaging; USG: ultrasonography; HR-USG: high-resolution ultrasonography.