Liver involvement in early autosomal-dominant polycystic kidney disease

Marie C Hogan, Kaleab Abebe, Vicente Torres, Arlene B. Chapman, Kyongtae T. Bae, Cheng Tao, Hongliang Sun, Ronald D. Perrone, Theodore I. Steinman, William Braun, Franz T. Winklhofer, Dana C. Miskulin, Frederic Rahbari-Oskoui, Godela Brosnahan, Amirali Masoumi, Irina O. Karpov, Susan Spillane, Michael Flessner, Charity G. Moore, Robert W. Schrier

Research output: Contribution to journalArticle

54 Citations (Scopus)

Abstract

Background & Aims: 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.

Original languageEnglish (US)
Pages (from-to)155-164
Number of pages10
JournalClinical Gastroenterology and Hepatology
Volume13
Issue number1
DOIs
StatePublished - Jan 1 2015

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Autosomal Dominant Polycystic Kidney
Liver
Cysts
Hepatomegaly
Quality of Life
Hypersplenism
Kidney
Renal Replacement Therapy
Angiotensin Receptor Antagonists
Splenomegaly
Life Expectancy
Chronic Renal Insufficiency
Angiotensin-Converting Enzyme Inhibitors
Tertiary Care Centers
Hypotension
Randomized Controlled Trials
Cross-Sectional Studies
Placebos
Magnetic Resonance Imaging
Demography

Keywords

  • CKD
  • HALT-PKD-A
  • Hepatic cyst
  • MRI analysis

ASJC Scopus subject areas

  • Gastroenterology
  • Hepatology

Cite this

Liver involvement in early autosomal-dominant polycystic kidney disease. / Hogan, Marie C; Abebe, Kaleab; Torres, Vicente; Chapman, Arlene B.; Bae, Kyongtae T.; Tao, Cheng; Sun, Hongliang; Perrone, Ronald D.; Steinman, Theodore I.; Braun, William; Winklhofer, Franz T.; Miskulin, Dana C.; Rahbari-Oskoui, Frederic; Brosnahan, Godela; Masoumi, Amirali; Karpov, Irina O.; Spillane, Susan; Flessner, Michael; Moore, Charity G.; Schrier, Robert W.

In: Clinical Gastroenterology and Hepatology, Vol. 13, No. 1, 01.01.2015, p. 155-164.

Research output: Contribution to journalArticle

Hogan, MC, Abebe, K, Torres, V, Chapman, AB, Bae, KT, Tao, C, Sun, H, Perrone, RD, Steinman, TI, Braun, W, Winklhofer, FT, Miskulin, DC, Rahbari-Oskoui, F, Brosnahan, G, Masoumi, A, Karpov, IO, Spillane, S, Flessner, M, Moore, CG & Schrier, RW 2015, 'Liver involvement in early autosomal-dominant polycystic kidney disease', Clinical Gastroenterology and Hepatology, vol. 13, no. 1, pp. 155-164. https://doi.org/10.1016/j.cgh.2014.07.051
Hogan, Marie C ; Abebe, Kaleab ; Torres, Vicente ; Chapman, Arlene B. ; Bae, Kyongtae T. ; Tao, Cheng ; Sun, Hongliang ; Perrone, Ronald D. ; Steinman, Theodore I. ; Braun, William ; Winklhofer, Franz T. ; Miskulin, Dana C. ; Rahbari-Oskoui, Frederic ; Brosnahan, Godela ; Masoumi, Amirali ; Karpov, Irina O. ; Spillane, Susan ; Flessner, Michael ; Moore, Charity G. ; Schrier, Robert W. / Liver involvement in early autosomal-dominant polycystic kidney disease. In: Clinical Gastroenterology and Hepatology. 2015 ; Vol. 13, No. 1. pp. 155-164.
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AU - Hogan, Marie C

AU - Abebe, Kaleab

AU - Torres, Vicente

AU - Chapman, Arlene B.

AU - Bae, Kyongtae T.

AU - Tao, Cheng

AU - Sun, Hongliang

AU - Perrone, Ronald D.

AU - Steinman, Theodore I.

AU - Braun, William

AU - Winklhofer, Franz T.

AU - Miskulin, Dana C.

AU - Rahbari-Oskoui, Frederic

AU - Brosnahan, Godela

AU - Masoumi, Amirali

AU - Karpov, Irina O.

AU - Spillane, Susan

AU - Flessner, Michael

AU - Moore, Charity G.

AU - Schrier, Robert W.

PY - 2015/1/1

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N2 - Background & Aims: 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.

AB - Background & Aims: 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.

KW - CKD

KW - HALT-PKD-A

KW - Hepatic cyst

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