Criteria

Modified Ravine criteria[42]

Ultrasound diagnostic criteria for the diagnosis of autosomal-dominant PKD (ADPKD) in at-risk people (those from families with ADPKD have been developed.[42] Note that these criteria, if applied to magnetic resonance imaging or computed tomography, will result in false-positive diagnoses. The presence of at least one affected family member who developed end-stage renal disease (ESRD) at age 55 years or younger was highly predictive of a PKD1 mutation. In contrast, the presence of at least one affected family member who continued to have sufficient renal function or developed ESRD at age >70 was highly predictive of a PKD2 mutation.

Revised unified criteria for diagnosis of ADPKD

  • Age 15-29, 3 or more cysts (unilateral or bilateral)

  • Age 30-39, 3 or more cysts (unilateral or bilateral)

  • Age 40-59, 2 or more cysts in each kidney.

Revised ultrasound criteria for exclusion of ADPKD

  • Age 15-59, 1 cyst or more.

Mayo Clinic imaging classification[44]

Patients with ADPKD are grouped by prespecified imaging findings into typical (class 1) and atypical (class 2) ADPKD.

Typical ADPKD (bilateral and diffuse distribution, with mild, moderate, or severe replacement of kidney tissue by cysts, where all cysts contribute similarly to total kidney volume [TKV]), is further stratified into five different classes (1A-1E) on the basis of height-adjusted TKV and age.

Modeling based on these stratified classes predicts the change in estimated glomerular filtration rate over time.

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