Liver Involvement in Early Autosomal-Dominant Polycystic Kidney Disease

• Published in: Clinical gastroenterology and hepatology Vol. 13; no. 1; pp. 155 - 164.e6
• Main Authors: Hogan, Marie C, Abebe, Kaleab, Torres, Vicente E, 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
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.01.2015
• Subjects: Adolescent; Adult; CKD; Cross-Sectional Studies; Female; Gastroenterology and Hepatology; HALT-PKD-A; Hepatic Cyst; Hepatomegaly - epidemiology; Hepatomegaly - pathology; Humans; Liver; Liver Function Tests; Male; Middle Aged; MRI Analysis; Polycystic Kidney, Autosomal Dominant - complications; Polycystic Kidney, Autosomal Dominant - diagnosis; Randomized Controlled Trials as Topic; Young Adult; magnetic resonance imaging; height-adjusted total liver volume; estimated glomerular filtration rate; ALP; MRI; alanine aminotransferase; ALT; PLD; LV; polycystic liver disease; BP; body surface area (corrected for height); HALT-PKD-A; total liver volume; ADPKD; LPV; liver parenchymal volume; alkaline phosphatase; autosomal-dominant polycystic kidney disease; htLCV; htTKV; Hepatic Cyst; CKD; Halt Progression of Polycystic Kidney Disease studies; BMI; Short-Form 36; eGFR; MRI Analysis; blood pressure; BSA(m 2); body mass index; height-adjusted total kidney volume; htLV; liver cyst volume; htLPV; QOL; SF-36; HALT-PKD; height-adjusted liver cyst volume; height-adjusted liver parenchymal volume; Halt Progression of Polycystic Kidney Disease Study A; quality of life; LCV; BSA(m2)
• ISSN: 1542-3565; 1542-7714
• DOI: 10.1016/j.cgh.2014.07.051

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.