Article
Urology & Nephrology
Kyongtae T. Bae, Cheng Tao, Robert Feldman, Alan S. L. Yu, Vicente E. Torres, Ronald D. Perrone, Arlene B. Chapman, Godela Brosnahan, Theodore I. Steinman, William E. Braun, Michal Mrug, William M. Bennett, Peter C. Harris, Avantika Srivastava, Douglas P. Landsittel, Kaleab Z. Abebe
Summary: This study aimed to evaluate the associations of polycystic liver progression with other disease progression variables and classify liver progression on the basis of patient's age, height-adjusted liver cystic volume, and height-adjusted liver volume. The use of height-adjusted liver cystic volume showed greater separations in volumetric progression of polycystic liver disease.
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2022)
Article
Medicine, General & Internal
Ahsan Alam, Emilie Cornec-Le Gall, Ronald D. Perrone
Summary: This article describes autosomal dominant polycystic kidney disease, its signs and symptoms, diagnosis, and treatment options.
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION
(2023)
Article
Medicine, General & Internal
Jin Liu, Xiaorui Yin, Hreedi Dev, Xianfu Luo, Jon D. D. Blumenfeld, Hanna Rennert, Martin R. R. Prince
Summary: This study investigates the association between autosomal dominant polycystic kidney disease (ADPKD) and pleural effusion. Pleural effusions were observed in 21% of ADPKD subjects compared to 8% in controls. In a subpopulation controlling for renal function, 25% of ADPKD subjects had pleural effusions compared to 5% of controls.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Urology & Nephrology
Fouad T. Chebib, Kristen L. Nowak, Michel B. Chonchol, Kristen Bing, Ahmad Ghanem, Frederic F. Rahbari-Oskoui, Neera K. Dahl, Michal Mrug
Summary: This review discusses the efficacy and safety of dietary interventions, such as caloric restriction, intermittent fasting, and ketogenic diet, in managing ADPKD. These interventions show promise in improving metabolic health and reducing oxidative stress in ADPKD, but limited clinical evidence supports their effectiveness.
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2023)
Review
Biochemistry & Molecular Biology
Adrian Cordido, Marta Vizoso-Gonzalez, Miguel A. Garcia-Gonzalez
Summary: ARPKD is a rare disorder linked to the PKHD1 and DZIP1L genes, with research elucidating molecular pathways involved in disease progression. However, understanding of the function of ARPKD proteins and the disease's molecular mechanism remains incomplete.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Biochemistry & Molecular Biology
Sarah A. Bowden, Euan J. Rodger, Aniruddha Chatterjee, Michael R. Eccles, Cherie Stayner
Summary: ADPKD is a heritable renal disease caused by uncontrolled cyst growth in the kidneys, leading to end-stage kidney disease, with limited therapeutic options available. Due to similarities with neoplasia, research suggests that ADPKD kidneys may have alterations in their epigenetic landscape.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Radiology, Nuclear Medicine & Medical Imaging
Feng Wang, Seo Yeon Lee, Fatemeh Adelnia, Keiko Takahashi, Kevin D. Harkins, Lilly He, Zhongliang Zu, Philipp Ellinger, Manuel Grundmann, Raymond C. Harris, Takamune Takahashi, John C. Gore
Summary: The purpose of this study was to compare multiple MRI parameters for assessing and predicting the severity of polycystic kidney disease (PKD). The results showed that R2, R1, and PSR are sensitive indicators of the presence of PKD.
MAGNETIC RESONANCE IN MEDICINE
(2023)
Review
Pharmacology & Pharmacy
Guangying Shao, Shuai Zhu, Baoxue Yang
Summary: ADPKD is a common hereditary kidney disease characterized by progressively enlarged cysts that destroy renal function, potentially leading to ESRD. Herbal medicines have shown potential in inhibiting cyst development and ADPKD progression, providing new insights for clinical therapeutic strategies.
FRONTIERS IN PHARMACOLOGY
(2021)
Review
Veterinary Sciences
Lorie Schirrer, Pablo Jesus Marin-Garcia, Lola Llobat
Summary: Polycystic kidney disease (PKD) is a common genetic disease in felines and other mammals, particularly in Persian cats. It results in fluid-filled cysts in the kidneys and other organs, leading to kidney failure. Imaging tests are currently the most reliable method for diagnosis.
VETERINARY SCIENCES
(2021)
Article
Radiology, Nuclear Medicine & Medical Imaging
Alexandra Roudenko, Soran Mahmood, Linda Du, Drew Gunio, Irina Barash, Florence Doo, Alon Slutzky, Nina Kukar, Barak Friedman, Alexander Kagen
Summary: The study proposes a semi-automated method for calculating TKV in ADPKD patients, showing excellent intraclass correlation among raters. This method allows for reliable and consistent measurements by radiologists of varying training levels.
Article
Engineering, Biomedical
Roman Pichler, Ludovica Rizzo, Kevin Troendle, Michaela Buehler, Hanna Brucker, Anna-Lena Mueller, Kelli Grand, Silvia Fare, Amandine Viau, Michael M. Kaminski, E. Wolfgang Kuehn, Fritz Koch, Stefan Zimmermann, Peter Koltay, Soeren S. Lienkamp
Summary: Embedding renal tubular cells in 3D microenvironments or controlling their assembly using bioprinting can improve their physiological properties and aid in disease modeling. Induced renal tubular epithelial cells (iRECs) can be cultured in various biomaterials and bioprinted tubular structures, showing compatibility with the substrates and methods. Transcriptomic analysis reveals differentially expressed genes specific to each biomaterial, and the use of iRECs helps unmask disease phenotypes.
Review
Biochemistry & Molecular Biology
Claudio Ponticelli, Gabriella Moroni, Francesco Reggiani
Summary: Autosomal-Dominant Polycystic Kidney Disease (ADPKD) is a genetic disorder caused by mutations in PKD1 or PKD2 genes. The involvement of autophagy in ADPKD remains a subject of investigation, with potential implications on cyst formation and fibrosis. Autophagy inducers have shown promising results in preclinical studies and may provide a potential avenue for future investigations.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Physiology
Mary Claire Doss, Sean Mullen, Ronald Roye, Juling Zhou, Phillip Chumley, Elias Mrug, Darren P. Wallace, Feng Qian, Peter C. Harris, Bradley K. Yoder, Harrison Kim, Michal Mrug
Summary: Measurement of total kidney volume (TKV) using MRI is a valuable approach for monitoring disease progression in PKD. A template-based semiautomatic image segmentation method (SAM) was developed and validated in three commonly used PKD models, showing high accuracy and efficiency in TKV assessment.
AMERICAN JOURNAL OF PHYSIOLOGY-RENAL PHYSIOLOGY
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Chenglin Zhu, Hreedi Dev, Arman Sharbatdaran, Xinzi He, Daniil Shimonov, James M. Chevalier, Jon D. Blumenfeld, Yi Wang, Kurt Teichman, George Shih, Akshay Goel, Martin R. Prince
Summary: Total kidney volume measured on MRI is an important biomarker for assessing the progression of autosomal dominant polycystic kidney disease and response to treatment. In this study, we examined the variability of kidney volume measurements among five commonly acquired MRI pulse sequences in abdominal MRI exams in 105 patients with ADPKD.
Article
Medicine, General & Internal
Tatsuya Suwabe, Yoshifumi Ubara, Daisuke Ikuma, Hiroki Mizuno, Noriko Hayami, Masayuki Yamanouchi, Naoki Sawa
Summary: This case study suggests that rigorous blood pressure control may be beneficial for ameliorating polycystic liver disease.
