Table 1.

Baseline characteristics of individuals with ADPKD and either a biliary tract or serious liver complication: Oxford Kidney Unit case series (1967–2015)

SexAge at ADPKD Diagnosis, yrTime from ADPKD Diagnosis to First Biliary Tract Disease/Serious Liver Complication, yrCKD Stage at First Biliary Tract Disease/Serious Liver ComplicationBiliary Tract Disease and Serious Liver Complications
No. of Admissions with Gallbladder or Bile Duct DiseaseGallstones Ever DiagnosedNo. of Admissions with Liver Cyst InfectionBiliary Tract and Liver Disease Diagnoses or Procedures
W22225D18Yes0Cholelithiasis with complications
Ma28325D14Yes1Cholelithiasis requiring 4× ERCPs and sphincterotomy. Nonobstructive intrahepatic biliary tract duct dilation and hepatic fibrosis. Liver cyst infection
M48105T9Yes0Cholelithiasis with complications requiring ERCP and sphincterotomy
Wa15465T9No1Cholelithiasis with complications and liver abscess
Ma34265T8Yes3Biliary obstruction and sepsis due to enlarged liver cysts and gallstones. Cholecystectomy. Cyst deroofing. Liver cyst infections
M18445T7Yes0Cholelithiasis with complications, 3× ERCPs, and sphincterotomy
W51115D7Yes0Cholelithiasis with complications, 2× ERCPs, and biliary tract stenting
M29395T6Yes0Cholecystitis, biliary obstruction, and cholecystectomy. Refractory ascites
W18465T3Yes0Cholelithiasis with complications
Ma24325T3Yes0Cholelithiasis with complications requiring ERCP. Liver cyst deroofing
M26355T3No0Nonobstructive intrahepatic biliary tract duct dilation and liver fibrosis. Recurrent biliary sepsis
W31435D2Yes0Cholelithiasis with complications. Cholecystectomy
Wa26304/52Yes2Cholelithiasis with complications. Liver cyst infections and hemorrhage
M5825T1Yes0Cholelithiasis with complications requiring 3× ERCPs, sphincterotomy, and biliary tract stent
W57451No0Biliary sepsis
Wa21325D1Yes0Cholelithiasis and biliary sepsis requiring cholecystectomy. Subphrenic abscess, liver cyst aspiration, cyst deroofing, and partial hepatectomy
M56165D1No0Biliary sepsis
W52255T1Yes0Cholelithiasis with complications. ERCP
Wa681231Yes0Cholelithiasis with complications. Autoimmune hepatitis
W44105T1Yes0Cholelithiasis with complications. ERCP and sphincterotomy. Cholecystectomy
M45125T1Yes0Obstructive jaundice, cholecystitis
W47451Yes0Cholelithiasis requiring ERCP complicated by pancreatitis
W313131Yes0Cholecystectomy
M28445D1Yes0Cholecystectomy
M: 11; W: 13Median 31 (IQR, 26–49)Median 28 (IQR, 12–36)Median 3 (IQR, 1–8)83% (20 of 24)Median 0 (IQR, 0–0)
W32135D0No3Liver cyst infections. Massive polycystic liver with cyst drainage
W41255T0No3Recurrent liver cyst infections and liver abscess
Wa242251Yes2Liver cyst infections. Cholelithiasis
Wa4864/51Yes2Liver cyst infections. Cholelithiasis requiring cholecystectomy
W44550No2Liver cyst infections. Cyst deroofing (×2)
W30335D0No1Liver cyst infection. Cholelithiasis with complications requiring 2× ERCPs and biliary tract stent insertion
M20325D0No1Liver cyst infection
W48205T0No1Liver cyst infection
W38010No0Massive polycystic liver (transplanted)
W3685T0No0Cyst aspiration and deroofing
W49950No0Massive polycystic liver (transplanted)
M: 1; W: 10Median 38 (IQR, 31–46)Median 13 (IQR, 7–24)Median 0 (IQR, 0–0)18% (2 of 11)Median 1 (IQR, 0.5–2)
  • Cholelithiasis with complications indicates gallstones with biliary sepsis (cholecystitis and/or cholangitis). W, woman; M, man; ERCP, endoscopic retrograde cholangiopancreatogram; IQR, interquartile range. Biliary complications were the predominate presentation in the first 24 patients in the table, and liver complications predominated in the remaining 11 patients.

  • a Patients with both biliary tract and serious liver presentations. Imaging was available for 46% (85 of 185) of patients with ADPKD on maintenance RRT: 89% (76 of 85) had evidence of multiple liver cysts, 26% (22 of 85) had common bile duct dilation, and 16% (14 of 85) had gallstones.