title: Liver involvement in early autosomal-dominant polycystic kidney disease.
abstract: OBJECTIVE
  Polycystic liver disease (PLD), the most common extrarenal manifestation of autosomal-dominant polycystic kidney disease (ADPKD), has become more prevalent as a result of increased life expectancy, improved renal survival, reduced cardiovascular mortality, and renal replacement therapy. No studies have fully characterized PLD in large cohorts. We investigated whether liver and cyst volumes are associated with volume of the hepatic parenchyma, results from liver laboratory tests, and patient-reported outcomes.
  
  
  METHODS
  We performed a cross-sectional analysis of baseline liver volumes, measured by magnetic resonance imaging, and their association with demographics, results from liver laboratory and other tests, and quality of life. The data were collected from a randomized, placebo-controlled trial underway at 7 tertiary-care medical centers to determine whether the combination of an angiotensin I-converting enzyme inhibitor and angiotensin II-receptor blocker was superior to the inhibitor alone, and whether low blood pressure (<110/75 mm Hg) was superior to standard blood pressure (120-130/70-80 mm Hg), in delaying renal cystic progression in 558 patients with ADPKD, stages 1 and 2 chronic kidney disease, and hypertension (age, 15-49 y).
  
  
  RESULTS
  We found hepatomegaly to be common among patients with ADPKD. Cysts and parenchyma contributed to hepatomegaly. Cysts were more common and liver and cyst volumes were greater in women, increasing with age. Patients with advanced disease had a relative loss of liver parenchyma. We observed small abnormalities in results from liver laboratory tests, and that splenomegaly and hypersplenism were associated with PLD severity. Higher liver volumes were associated with a lower quality of life.
  
  
  CONCLUSIONS
  Hepatomegaly is common even in early stage ADPKD and is not accounted for by cysts alone. Parenchymal volumes were larger, compared with liver volumes of patients without ADPKD or with those predicted by standardized equations, even among patients without cysts. The severity of PLD was associated with altered biochemical and hematologic features, as well as quality of life. ClinicalTrials.gov identifier: NCT00283686.
authors.affiliation: Mayo Clinic College of Medicine, Rochester, Minnesota. Electronic address: hogan.marie@mayo.edu.
authors.forename: Marie C
authors.initials: MC
authors.lastname: Hogan
authors.affiliation: University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Kaleab
authors.initials: K
authors.lastname: Abebe
authors.affiliation: Mayo Clinic College of Medicine, Rochester, Minnesota.
authors.forename: Vicente E
authors.initials: VE
authors.lastname: Torres
authors.affiliation: Emory University School of Medicine, Atlanta, Georgia.
authors.forename: Arlene B
authors.initials: AB
authors.lastname: Chapman
authors.affiliation: University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Kyongtae T
authors.initials: KT
authors.lastname: Bae
authors.affiliation: University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Cheng
authors.initials: C
authors.lastname: Tao
authors.affiliation: University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Hongliang
authors.initials: H
authors.lastname: Sun
authors.affiliation: Tufts Medical Center, Boston, Massachusetts.
authors.forename: Ronald D
authors.initials: RD
authors.lastname: Perrone
authors.affiliation: Beth Israel Deaconess Medical Center, Boston, Massachusetts.
authors.forename: Theodore I
authors.initials: TI
authors.lastname: Steinman
authors.affiliation: Cleveland Clinic, Cleveland, Ohio.
authors.forename: William
authors.initials: W
authors.lastname: Braun
authors.affiliation: University of Kansas Medical Center, Kansas City, Kansas.
authors.forename: Franz T
authors.initials: FT
authors.lastname: Winklhofer
authors.affiliation: Tufts Medical Center, Boston, Massachusetts.
authors.forename: Dana C
authors.initials: DC
authors.lastname: Miskulin
authors.affiliation: Emory University School of Medicine, Atlanta, Georgia.
authors.forename: Frederic
authors.initials: F
authors.lastname: Rahbari-Oskoui
authors.affiliation: University of Colorado Anschutz Medical Campus, Aurora, Colorado.
authors.forename: Godela
authors.initials: G
authors.lastname: Brosnahan
authors.affiliation: University of Colorado Anschutz Medical Campus, Aurora, Colorado.
authors.forename: Amirali
authors.initials: A
authors.lastname: Masoumi
authors.affiliation: University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Irina O
authors.initials: IO
authors.lastname: Karpov
authors.affiliation: University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Susan
authors.initials: S
authors.lastname: Spillane
authors.affiliation: National Institute of Diabetes and Digestive Diseases, National Institutes of Health, Bethesda, Maryland.
authors.forename: Michael
authors.initials: M
authors.lastname: Flessner
authors.affiliation: University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Charity G
authors.initials: CG
authors.lastname: Moore
authors.affiliation: University of Colorado Anschutz Medical Campus, Aurora, Colorado.
authors.forename: Robert W
authors.initials: RW
authors.lastname: Schrier
mesh_terms: D000293:Adolescent; D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006529:Hepatomegaly / Q000453:epidemiology* / Q000473:pathology*; D006801:Humans; D008099:Liver; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000175:diagnosis*; D016032:Randomized Controlled Trials as Topic; D055815:Young Adult
keywords: CKD; HALT-PKD-A; Hepatic Cyst; MRI Analysis
references.citation: Clin J Am Soc Nephrol. 2010 Nov;5(11):2034-9
references.pmid: 20671227
references.citation: Am J Kidney Dis. 1996 Mar;27(3):321-6
references.pmid: 8604699
references.citation: AJR Am J Roentgenol. 1995 Feb;164(2):339-42
references.pmid: 7839965
references.citation: Hepatology. 2004 Oct;40(4):774-82
references.pmid: 15382167
references.citation: Hepatology. 2001 Feb;33(2):339-45
references.pmid: 11172335
references.citation: Acta Radiol. 1999 Jul;40(4):444-8
references.pmid: 10394876
references.citation: Liver Int. 2014 Nov;34(10):1578-83
references.pmid: 24313956
references.citation: Mayo Clin Proc. 1990 Jul;65(7):1020-5
references.pmid: 2198393
references.citation: Nephrol Dial Transplant. 1999 May;14(5):1113-6
references.pmid: 10344347
references.citation: Gastroenterology. 2009 Nov;137(5):1661-8.e1-2
references.pmid: 19646443
references.citation: Am J Kidney Dis. 2011 Jun;57(6):856-62
references.pmid: 21458899
references.citation: J Magn Reson Imaging. 2003 Aug;18(2):210-5
references.pmid: 12884334
references.citation: J Am Soc Nephrol. 2015 Jan;26(1):39-47
references.pmid: 24994926
references.citation: J Hepatol. 2013 Jul;59(1):153-9
references.pmid: 23499726
references.citation: Am J Hypertens. 2013 Apr;26(4):473-80
references.pmid: 23467203
references.citation: Clin J Am Soc Nephrol. 2010 May;5(5):783-9
references.pmid: 20185596
references.citation: Clin J Am Soc Nephrol. 2011 Oct;6(10):2508-15
references.pmid: 21903983
references.citation: N Engl J Med. 1993 Jul 29;329(5):332-42
references.pmid: 8321262
references.citation: Clin J Am Soc Nephrol. 2010 Jan;5(1):102-9
references.pmid: 20089507
references.citation: Abdom Imaging. 2004 Jul-Aug;29(4):482-90
references.pmid: 15024516
references.citation: Hepatology. 1988 Nov-Dec;8(6):1627-34
references.pmid: 3192176
references.citation: Gut. 1998 Jan;42(1):131-4
references.pmid: 9505899
references.citation: Gastroenterology. 2013 Aug;145(2):357-65.e1-2
references.pmid: 23665274
references.citation: AJR Am J Roentgenol. 2012 Nov;199(5):1060-9
references.pmid: 23096180
references.citation: Ann Surg. 2009 Jul;250(1):112-8
references.pmid: 19561475
references.citation: Kidney Int. 2012 Mar;81(6):577-85
references.pmid: 22205355
references.citation: Liver Transpl Surg. 1999 Sep;5(5):366-8
references.pmid: 10477836
references.citation: J Am Soc Nephrol. 2010 Jun;21(6):1052-61
references.pmid: 20431041
references.citation: J Am Soc Nephrol. 2000 Sep;11(9):1767-1775
references.pmid: 10966503
references.citation: Clin J Am Soc Nephrol. 2006 Jan;1(1):64-9
references.pmid: 17699192
references.citation: Hepatology. 1990 Jun;11(6):1033-7
references.pmid: 2365280
references.citation: Hypertension. 1983 May-Jun;5(3):353-62
references.pmid: 6341219
references.citation: Nephrol Dial Transplant. 2012 Apr;27(4):1607-13
references.pmid: 21873624
references.citation: Clin Nephrol. 1997 Jan;47(1):19-22
references.pmid: 9021236
references.citation: Liver Transpl. 2002 Mar;8(3):233-40
references.pmid: 11910568
references.citation: Am J Kidney Dis. 2001 Oct;38(4):777-84
references.pmid: 11576881
references.citation: Gastroenterology. 1952 Jan;20(1):60-78
references.pmid: 14897088
references.citation: Nephrol Dial Transplant. 2012 Sep;27(9):3532-9
references.pmid: 22773240
references.citation: Hepatology. 1997 Nov;26(5):1282-6
references.pmid: 9362373