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Abstract: PO1572

Volume Progression and Imaging Classification of Polycystic Liver in ADPKD

Session Information

Category: Genetic Diseases of the Kidneys

  • 1001 Genetic Diseases of the Kidneys: Cystic

Authors

  • Bae, Kyongtae Ty, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States
  • Tao, Cheng, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States
  • Feldman, Robert, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States
  • Yu, Alan S.L., University of Kansas Medical Center, Kansas City, Kansas, United States
  • Torres, Vicente E., Mayo Clinic Department of Internal Medicine, Rochester, Minnesota, United States
  • Chapman, Arlene B., University of Chicago, Chicago, Illinois, United States
  • Perrone, Ronald D., Tufts Medical Center, Boston, Massachusetts, United States
  • Brosnahan, Godela M., University of Colorado, Denver, Colorado, United States
  • Steinman, Theodore I., Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Braun, William E., Cleveland Clinic, Cleveland, Ohio, United States
  • Mrug, Michal, The University of Alabama at Birmingham School of Medicine, Birmingham, Alabama, United States
  • Bennett, William M., Legacy Health System, Portland, Oregon, United States
  • Harris, Peter C., Mayo Clinic Department of Internal Medicine, Rochester, Minnesota, United States
  • Landsittel, Doug, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States
  • Abebe, Kaleab, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States
Background

While polycystic liver in ADPKD is common and affects significantly the quality of life, volume progression of liver cysts is not well understood. This is in part because previous longitudinal studies focused on the progression of liver volume not liver cyst volume. Thus, the purpose of the study is to evaluate and classify polycystic liver progression in patients with ADPKD based on patient's age, sex, height-adjusted liver cystic volume (htLCV) and height-adjusted liver volume (htLV) measurements.

Methods

Longitudinal MRI data (CRISP and HALT studies) from 695 patients with ADPKD over a maximum follow-up of 14.23 years were evaluated to measure LCV and LV. Among them, 258 patients with LCV > 50mL and at least 2 time-points were included in the analysis. Linear mixed models on log-transformed htLCV and htLV were fitted as a function of participant’s age and study as well as a random intercept and slope. The slope coefficient was used to approximate the mean annual rate of change (MAROC) for each outcome. Using the age of 15 years as the hypothetical initial age for LCV=0, differential growth trajectories were plotted to categorize the participants according to their LCV growth rate and age.

Results

Overall, the MAROC was 10.8% for htLCV and 1.8% for htLV (P<0.0001). 232 participants had net-increases (last measurement > first) on htLCV, while 26 participants had net-decreases (or values remained the same) on htLCV. For the net-increase group, MAROC was 12.7% for htLCV and 2.2% for htLV (P<0.0001). For the net-decrease group, MAROC was -7.3% for htLCV (P=0.0168) and -2.1% for htLV (P=0.1116). According to the annual growth rate, 5 classes (A <5%, B 5-<10%, C 10-<15%, D 15-<20%, E ≥20%) were defined. The numbers for female and male participants in each class were (F,M): A (31,7); B(48,15); C (49,51); D (27,16); E (9,5).

Conclusion

The mean volume growth rate of the liver cyst was 6 times greater than that of the liver. Similar to the kidney imaging classification, the severity of polycystic liver may be categorized on the basis of patient’s age and liver cyst volume.

Funding

  • NIDDK Support