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
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)
Review
Nutrition & Dietetics
Lauren Pickel, Ioan-Andrei Iliuta, James Scholey, York Pei, Hoon-Ki Sung
Summary: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive growth of renal cysts and loss of functional nephrons. Evidence suggests that dietary interventions such as caloric restriction, intermittent fasting, and ketogenic diets have the potential to slow disease progression and confer metabolic benefits.
ADVANCES IN NUTRITION
(2022)
Article
Urology & Nephrology
Mireille El Ters, Pengcheng Lu, Jonathan D. Mahnken, Jason R. Stubbs, Shiqin Zhang, Darren P. Wallace, Jared J. Grantham, Arlene B. Chapman, Vicente E. Torres, Peter C. Harris, Kyongtae Ty Bae, Douglas P. Landsittel, Frederic F. Rahbari-Oskoui, Michal Mrug, William M. Bennett, Alan S. L. Yu
Summary: In ADPKD patients, higher baseline FGF23 levels were associated with faster rates of total kidney volume increase and decline in glomerular filtration rate, as well as increased risk of disease progression and adverse outcomes. FGF23 serves as an early prognostic biomarker with additive value in guiding the treatment and management of ADPKD patients.
KIDNEY INTERNATIONAL REPORTS
(2021)
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)
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)
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
Pediatrics
Iva Soric Hosman, Andrea Cvitkovic Roic, Margareta Fistrek Prlic, Ivana Vukovic Brinar, Lovro Lamot
Summary: Autosomal dominant polycystic kidney disease (ADPKD) is a leading cause of end-stage renal disease. Identification of easily measurable biomarkers for predicting disease progression is crucial for the development of targeted therapies. This review summarizes the research on serum and urine biomarkers for predicting ADPKD progression and response to therapy.
FRONTIERS IN PEDIATRICS
(2023)
Article
Urology & Nephrology
Jessica T. T. Camargo, Camilo A. Gonzalez, Lina Herrera, Nancy Yomayusa-Gonzalez, Milciades Ibanez, Ana M. M. Valbuena-Garcia, Lizbeth Acuna-Merchan
Summary: This study investigated the prevalence, geographical location, and ethnic groups of ADPKD patients undergoing dialysis or kidney transplant in Colombia between 2015 and 2019. The prevalence of ADPKD was lower compared to Europe and the US, and further genetic prevalence studies may be needed in some states with higher prevalence.
Article
Medicine, General & Internal
Alexander R. Chang, Bryn S. Moore, Jonathan Z. Luo, Gino Sartori, Brian Fang, Steven Jacobs, Yoosif Abdalla, Mohammed Taher, David J. Carey, William J. Triffo, Gurmukteshwar Singh, Tooraj Mirshahi
Summary: This study reveals substantial genetic and phenotypic variability in autosomal dominant polycystic kidney disease (ADPKD) among patients within a regional health system in the US. In addition to PKD1 and PKD2, LOF variants in IFT140, GANAB, and HNF1B were associated with ADPKD diagnosis. Patients with a family history of ADPKD had a higher yield for genetic determinants of the disease.
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION
(2022)
Article
Multidisciplinary Sciences
Katharina Hopp, Victoria A. Catenacci, Nidhi Dwivedi, Timothy L. Kline, Wei Wang, Zhiying You, Dustin T. Nguyen, Kristen Bing, Bhavya Poudyal, Ginger C. Johnson, Matthew R. Jackman, Marsha Miller, Cortney N. Steele, Natalie J. Serkova, Paul S. MacLean, Raphael A. Nemenoff, Berenice Gitomer, Michel Chonchol, Kristen L. Nowak
Summary: This study demonstrates that both daily caloric restriction (DCR) and intermittent fasting (IMF) can lead to significant weight loss and slow cyst growth in ADPKD patients. DCR has better efficacy in weight loss compared to IMF, and patients showed higher adherence and tolerability to DCR.
Article
Biochemistry & Molecular Biology
Johannes Leierer, Paul Perco, Benedikt Hofer, Susanne Eder, Alexander Dzien, Julia Kerschbaum, Michael Rudnicki, Gert Mayer
Summary: Changes in protein concentrations in the body fluids of ADPKD patients mainly involve dysregulation of various molecular processes, including markers such as EGF, APLN, VEGFA, and AGT. These markers are significantly correlated with renal function and disease progression.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Pharmacology & Pharmacy
Shunichiro Tsukamoto, Shingo Urate, Takayuki Yamada, Kengo Azushima, Takahiro Yamaji, Sho Kinguchi, Kazushi Uneda, Tomohiko Kanaoka, Hiromichi Wakui, Kouichi Tamura
Summary: This study used a network meta-analysis to compare the effects of different treatments for ADPKD. The results showed that tolvaptan and somatostatin analogs had significant efficacy in preserving kidney function and inhibiting TKV growth.
FRONTIERS IN PHARMACOLOGY
(2022)
Review
Medicine, General & Internal
Jing Xu, Cheng Xue, Xiaodong Wang, Lei Zhang, Changlin Mei, Zhiguo Mao
Summary: ADPKD, the most common inherited kidney disease worldwide, is mainly influenced by genes PKD1 and PKD2. Epigenetic modifications, particularly chromatin methylation and histone modifications, play a significant role in the development and progression of ADPKD. More research is needed to better understand and potentially treat the methylation abnormalities in ADPKD.
FRONTIERS IN MEDICINE
(2022)
Article
Gastroenterology & Hepatology
Bernhard Schlevogt, Vincent Schlieper, Jana Krader, Rita Schroeter, Thomas Wagner, Matthias Weiand, Andree Zibert, Hartmut H. Schmidt, Carsten Bergmann, Pavel Nedvetsky, Michael P. Krahn
Summary: In this study, a variant in the SEC61A1 gene was identified to be associated with the development of polycystic liver disease. The mutant SEC61A1 protein was shown to undergo enhanced proteasomal degradation, resulting in impaired synthesis of polycystin-2.
LIVER INTERNATIONAL
(2023)
