Clinical Correlates of Mass Effect in Autosomal Dominant Polycystic Kidney Disease

PLoS One. 2015 Dec 7;10(12):e0144526. doi: 10.1371/journal.pone.0144526. eCollection 2015.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cysts / complications*
  • Cysts / epidemiology
  • Cysts / pathology*
  • Female
  • Follow-Up Studies
  • Humans
  • Liver Diseases / complications*
  • Liver Diseases / epidemiology
  • Liver Diseases / pathology*
  • Male
  • Middle Aged
  • Organ Size
  • Polycystic Kidney, Autosomal Dominant / epidemiology
  • Polycystic Kidney, Autosomal Dominant / etiology*
  • Polycystic Kidney, Autosomal Dominant / pathology*
  • Prevalence
  • Prognosis
  • Republic of Korea / epidemiology
  • Retrospective Studies

Supplementary concepts

  • Polycystic liver disease

Grants and funding

This research was supported by a grant of the Korea Health Technology R&D Project through the Korea Health Industry Development Institute (KHIDI), funded by the Ministry of Health & Welfare, Republic of Korea (grant number: HI12C0014). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.