Table 1.

The diagnostic criteria of ADPKD (ADPKD Diagnostic Guidelines, Second Edition, published by a Grant-in-Aid for Progressive Renal Diseases Research, Research on intractable disease, from the Ministry of Health, Labour and Welfare of Japan)

(1) Confirmation of family history

(a) Three or more bilaterally-manifested cysts confirmed with ultrasonography

(b) Five or more bilaterally-manifested cysts confirmed with CT and MRI imaging

2. Non-confirmation of family history

(a) Patients 15-years old or younger: three or more bilaterally-manifested cysts confirmed with either CT and MRI imaging or ultrasonography

(b) Patients 16-years old or older: five or more bilaterally-manifested cysts confirmed with either CT and MRI imaging or ultrasonography

Diseases to be excluded

(1) Multiple simple renal cyst

(2) Renal tubular acidosis

(3) Multicystic kidney (multicystic dysplastic kidney)

(4) Multilocular cysts of the kidney

(5) Medullary cystic disease of the kidney (juvenile nephronophthisis)

(6) Acquired cystic disease of the kidney

(7) Autosomal recessive polycystic kidney disease