Table 1.

Diagnostic criteria for ADPLD.

ADPLD	Criteria
Definite	•Presence of family history of PLD or detection of an ADPLD gene mutation •Presence of ≥1 liver cyst (if <40 yr old) or ≥4 liver cysts (if ≥40 yr old) •Without or with kidney cysts less than required for diagnosis of ADPKD27,28
Likely	•Absence of family history •Presence of ≥20 liver cysts (at any age) •Without or with kidney cysts less than required for diagnosis of ADPKD27,28
Possible	•Absence of family history •Presence of 5–19 liver cysts (at any age) •Without or with kidney cysts less than required for diagnosis of ADPKD27,28

Cysts detected by ultrasound, computed tomography, or magnetic resonance imaging measuring ≥5 mm in diameter.

ADPKD, autosomal-dominant polycystic kidney disease; ADPLD, autosomal-dominant polycystic liver disease; PLD, polycystic liver disease.