Table 1.

Classification of ADPKD patients by prespecified imaging findings

Class, Subclass, and TermDescription
1: Typical ADPKDBilateral and diffuse distribution, with mild, moderate, or severe replacement of kidney tissue by cysts, where all cysts contribute similarly to TKV. (Supplemental Figure 1, A and B)
2: Atypical ADPKD
 A
  UnilateralDiffuse cystic involvement of one kidney causing marked renal enlargement with a normal contralateral kidney defined by a normal kidney volume (<275 ml in men; <244 ml in women) and having no or only 1–2 cysts (Supplemental Figure 1C)
  SegmentalCystic disease involving only one pole of one or both kidneys and sparing the remaining renal tissue (Supplemental Figure 1D)
  AsymmetricDiffuse cystic involvement of one kidney causing marked renal enlargement with mild segmental or minimal diffuse involvement of the contralateral kidney defined by a small number of cysts (>2 but <10) and volume accounting for <30% of TKV (Supplemental Figure 1E)
  LopsidedBilateral distribution of renal cysts with mild replacement of kidney tissue with atypical cysts where ≤5 cysts account for ≥50% TKV (the largest cyst diameter is used to estimate individual cyst volume) (Supplemental Figure 1F)
 B
  Bilateral presentation with acquired unilateral atrophyDiffuse cystic involvement of one kidney causing moderate to severe renal enlargement with contralateral acquired atrophy (Supplemental Figure 1G)
  Bilateral presentation with bilateral kidney atrophyImpaired renal function (serum creatinine≥1.5 mg/dl) without significant enlargement of the kidneys, defined by an average length <14.5 cm, and replacement of kidney tissue by cysts with atrophy of the parenchyma (Supplemental Figure 1H)