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Biliary Tract and Liver Complications in Polycystic Kidney Disease
SUPPLEMENTAL MATERIAL
Page
Supplemental Tables
Supplemental Table 1: Management of biliary disease tract and serious
liver complications in patients with polycystic kidney disease treated by
Oxford Kidney Unit
Supplemental Table 2: Diagnostic and procedural codes used to define
biliary tract disease, serious liver complications and extra-renal
complications
Supplemental Figures
Supplemental Figure 1: PubMed literature search for biliary tract disease
and serious liver complications in autosomal dominant polycystic kidney
disease
Supplemental Figure 2: Organisms identified during biliary tract and liver
infections in patients with polycystic kidney disease treated by Oxford
Kidney Unit by; admissions (panel A), and by patient (panel B)
Supplemental Figure 3: Association between polycystic kidney disease
and risk of hospitalisation for different diseases by diagnostic position in
all-England Hospital Episode Statistics 1998-2012 (sensitivity analysis)
Supplemental Figure 4: Association between polycystic kidney disease
and risk of hospitalisation for different diseases by diagnostic position in
all-England Hospital Episode Statistics 1998-2012 (sensitivity analysis)
Supplemental Figure 5: Underlying causes of death in all people with
polycystic kidney disease by; prior biliary tract or serious liver
complication (panel A), or without such complications (panel B)
Supplemental Figure 6: Underlying causes of death in all people with
polycystic kidney disease and biliary tract or serious liver complications
in; females (panel A), or males (panel B)
Supplemental Table 1: Management of biliary tract disease and serious liver
complications in patients with polycystic kidney disease treated by Oxford Kidney
Unit
Complication Total number of
admissions (number of
patients)
Management Number of
Admissions
(number of
patients)
Biliary tract disease complications
Biliary sepsis (including cholecystitis and cholangitis
90 (21) Antibiotics 90 (21)
Cholelithiasis ± Choledocholithiasis
26 (21) Ursodeoxycholic acid *ERCP with;
- Stone removal
- Sphincterotomy
- Stent
- Sludge trawled
Percutaneous cholecystostomy
Laparoscopic/open cholecystectomy
Biliary bypass
Serious liver complications
Infected liver cyst and/or abscess
22 (12) Antibiotics
Ultrasound-guided cyst aspiration
Liver cyst de-roofing
Extrinsic compression from liver cysts 1 (1)
*ERCP and stent 1 (1)
Abnormal liver architecture/focal cholangitis
1 (1) Liver biopsy 1 (1)
Refractory ascites 1 (1)
Liver hemorrhage 1 (1) Partial hepatectomy 1 (1)
Liver failure 2 (2) Liver transplant 2 (2)
Subphrenic abscess 1 (1) Drainage and antibiotics 1 (1) *ERCP = Endoscopic retrograde cholangiopancreatography
ICD-10 (ICD-9)
codes
ICD- 10
descriptions
OPCS- 4
codes
OPCS- 4
descriptions
Aortic or other aneurysms I71. 1 - I71.2 (441.1-441.2)
I71.0 (441.0)
I71. 3 - I71.6 (441.3-441.9)
I72 (442)
Thoracic aortic aneurysm
Aortic dissection
Abdominal aortic aneurysm
Other aneurysm
L18-L19, L27-L
L48- 49
Abdominal aneurysm repair
Iliac artery aneurysm repair
Hiatus hernia and gastroesophageal reflux disease K44, Q40.1, Q79.0 (551.3, 552.3, 553.3, 750.6, 756.6)
K20-K21 (530.1-530.2)
Diaphragmatic hernia
Gastro-oesophageal reflux disease or oesophagitis Renal stones N20-N23 (592, 594) Urolithiasis ICD = International Classification of Disease. OPCS= Office of Population Censuses and Surveys’ Classification of Interventions and Procedures.
Supplemental Figure 3: Association between polycystic kidney disease and risk of hospitalisation for different disease
by diagnostic position in all−England Hospital Episode Statistics 1998−2012 (sensitivity analysis)
CI = confidence intervals. Outcomes include admissions with relevant diagnostic codes in either any diagnostic position or primary diagnostic position only, or any relevant procedural codes. Adjusted for age at entry as a continuous variable (using both linear and quadratic terms), sex, ethnicity, quintile of patients' Index of Multiple Deprivation score, region of residence, calendar year of first recorded admission and comorbidities (grouped into vascular, cancer and diabetes).
All patients
Outcome
Polycystic
kidney
disease
(n=23,454)
Hospital
controls
(n=6,412,754)
Polycystic
kidney
disease
(n=23,454)
Hospital
controls
(n=6,412,754)
Number of events (% per year) Number of events (% per year)
Rate ratio (95% CI) Rate ratio (95% CI)
Rate ratio
Primary and secondary diagnosis
Primary diagnosis only
Cerebral aneurysm 311 (0.14%) 12,867 (0.02%) 5.95 (5.31−6.66)
Serious liver complication 767 (0.31%) 37,232 (0.07%) 4.67 (4.35−5.02)
Other abdominal wall hernia 1,050 (0.46%)^ 60,963 (0.11%)^ 4.22 (3.97−4.49)
Urinary tract infections 5,897 (3.21%) 546,411 (1.01%) 3.19 (3.11−3.27)
Cardiac valve disease 1,305 (0.53%) 125,584 (0.23%) 2.34 (2.22−2.47)
Biliary tract disease 2,617 (1.31%) 319,621 (0.59%) 2.24 (2.16−2.33)
Diverticular disease 3,151 (1.38%)^ 354,432 (0.65%)^ 2.12 (2.05−2.20)
Inguinal hernia 1,225 (0.43%) 174,625 (0.32%) 1.35 (1.27−1.42)
Supplemental Figure 4: Association between polycystic kidney disease and risk of hospitalisation for different diseases
by diagnostic position in all−England Hospital Episode Statistics 1998−2012 (sensitivity analysis)
CI = confidence intervals. Outcomes include admissions with relevant diagnostic codes in either any diagnostic position or primary diagnostic position only, or any relevant procedural codes. Adjusted for age at entry as a continuous variable (using both linear and quadratic terms), sex, ethnicity, quintile of patients' Index of Multiple Deprivation score, region of residence, calendar year of renal replacement therapy start and comorbidities (grouped into vascular, cancer and diabetes).
Treated end−stage renal disease patients
Outcome
Polycystic
kidney
disease
(n=5,813)
Other primary
renal diagnosis
(n=62,519)
Polycystic
kidney
disease
(n=5,813)
Other primary
renal diagnosis
(n=62,519)
Number of events (% per year) Number of events (% per year)
Rate ratio (95% CI) Rate ratio (95% CI)
Rate ratio
Primary and secondary diagnosis
Primary diagnosis only
Cerebral aneurysm 37 (0.13%) 150 (0.06%) 2.23 (1.53−3.26)
Serious liver complication 197 (0.70%) 1,561 (0.62%) 1.15 (0.98−1.33)
Other abdominal wall hernia 365 (1.23%)^ 1,242 (0.50%)^ 2.47 (2.19−2.80)
Urinary tract infections 1,369 (6.29%) 11,268 (4.93%) 1.28 (1.20−1.35)
Cardiac valve disease 366 (1.47%) 4,128 (1.63%) 0.90 (0.81−1.00)
Biliary tract disease 494 (1.92%) 4,014 (1.61%) 1.19 (1.08−1.31)
Diverticular disease 738 (2.70%)^ 4,069 (1.64%)^ 1.65 (1.52−1.79)
Inguinal hernia 269 (1.00%) 1,490 (0.59%) 1.70 (1.49−1.95)
Supplemental Figure 6: Underlying causes of death in all people
with polycystic kidney disease and prior biliary tract or serious
liver complications in; females (panel A), or males (panel B)
Biliary or liver 9%
Cardiac 12%
Other vascular 9%
Infections 10%
Cancer 21%
Renal 20%
Other-unspecified 18%
Biliary or liver 6%
Cardiac 19%
Other vascular 6%
Infections 11%
Cancer 28%
Renal 25%
Other-unspecified 15%
A: Females (509 deaths in 1,580 patients)
B: Males (619 deaths in 1,556 patients)
