title: Clinical Correlates of Mass Effect in Autosomal Dominant Polycystic Kidney Disease.
abstract: Mass effect from polycystic kidney and liver enlargement can result in significant clinical complications and symptoms in autosomal dominant polycystic kidney disease (ADPKD). In this single-center study, we examined the correlation of height-adjusted total liver volume (htTLV) and total kidney volume (htTKV) by CT imaging with hepatic complications (n = 461) and abdominal symptoms (n = 253) in patients with ADPKD. "Mass-effect" complications were assessed by review of medical records and abdominal symptoms, by a standardized research questionnaire. Overall, 91.8% of patients had 4 or more liver cysts on CT scans. Polycystic liver disease (PLD) was classified as none or mild (htTLV < 1,600 mL/m); moderate (1,600 ≤ htTLV <3,200 mL/m); and severe (htTLV ≥ 3,200 mL/m). The prevalence of moderate and severe PLD in our patient cohort was 11.7% (n = 54/461) and 4.8% (n = 22/461), respectively, with a female predominance in both the moderate (61.1%) and severe (95.5%) PLD groups. Pressure-related complications such as leg edema (20.4%), ascites (16.6%), and hernia (3.6%) were common, and patients with moderate to severe PLD exhibited a 6-fold increased risk (compared to no or mild PLD) for these complications in multivariate analysis. Similarly, abdominal symptoms including back pain (58.8%), flank pain (53.1%), abdominal fullness (46.5%), and dyspnea/chest-discomfort (44.3%) were very common, and patients with moderate to severe PLD exhibited a 5-fold increased risk for these symptoms. Moderate to severe PLD is a common and clinically important problem in ~16% of patients with ADPKD who may benefit from referral to specialized centers for further management.
authors.affiliation: Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Hyunsuk
authors.initials: H
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Armed Forces Capital Hospital, Seongnam-si, Gyeonggi-do, Korea.
authors.forename: Hayne Cho
authors.initials: HC
authors.lastname: Park
authors.affiliation: Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Hyunjin
authors.initials: H
authors.lastname: Ryu
authors.affiliation: Nephrology Clinic, National Cancer Center, Goyang-si, Gyeonggi-do, Korea.
authors.forename: Kiwon
authors.initials: K
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Asan Medical Center, University of Ulsan, Seoul, Korea.
authors.forename: Hyo Sang
authors.initials: HS
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
authors.forename: Kook-Hwan
authors.initials: KH
authors.lastname: Oh
authors.affiliation: Division of Hepatology, Seoul National University Hospital, Seoul, Korea.
authors.forename: Su Jong
authors.initials: SJ
authors.lastname: Yu
authors.affiliation: Department of Radiology, Seoul National University Hospital, Seoul, Korea.
authors.forename: Jin Wook
authors.initials: JW
authors.lastname: Chung
authors.affiliation: Department of Radiology, Seoul National University Hospital, Seoul, Korea.
authors.forename: Jeong Yeon
authors.initials: JY
authors.lastname: Cho
authors.affiliation: Department of Radiology, Seoul National University Hospital, Seoul, Korea.
authors.forename: Seung Hyup
authors.initials: SH
authors.lastname: Kim
authors.affiliation: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.|Research Coordination Center for Rare Diseases, Seoul National University Hospital, Seoul, Korea.
authors.forename: Hae Il
authors.initials: HI
authors.lastname: Cheong
authors.affiliation: Department of Internal Medicine, Kangbuk Samsung Medical Center, Seoul, Korea.
authors.forename: Kyubeck
authors.initials: K
authors.lastname: Lee
authors.affiliation: Department of Biological Science, Sookmyoung Women's University, Seoul, Korea.
authors.forename: Jong Hoon
authors.initials: JH
authors.lastname: Park
authors.affiliation: Division of Nephrology, Department of Internal Medicine, University Health Network and University of Toronto, Ontario, Canada.
authors.forename: York
authors.initials: Y
authors.lastname: Pei
authors.affiliation: Research Coordination Center for Rare Diseases, Seoul National University Hospital, Seoul, Korea.|Department of Internal Medicine, Eulji General Hospital, Seoul, Korea.
authors.forename: Young-Hwan
authors.initials: YH
authors.lastname: Hwang
authors.affiliation: Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.|Research Coordination Center for Rare Diseases, Seoul National University Hospital, Seoul, Korea.
authors.forename: Curie
authors.initials: C
authors.lastname: Ahn
mesh_terms: D003560:Cysts / Q000150:complications* / Q000453:epidemiology / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008107:Liver Diseases / Q000150:complications* / Q000453:epidemiology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009929:Organ Size; D016891:Polycystic Kidney, Autosomal Dominant / Q000453:epidemiology / Q000209:etiology* / Q000473:pathology*; D015995:Prevalence; D011379:Prognosis; D056910:Republic of Korea / Q000453:epidemiology; D012189:Retrospective Studies