Results 61 to 70 of about 43,386 (236)

International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people

Nature Reviews Nephrology, 2019
These recommendations were systematically developed on behalf of the Network for Early Onset Cystic Kidney Disease (NEOCYST) by an international group of experts in autosomal dominant polycystic kidney disease (ADPKD) from paediatric and adult nephrology,
C. Gimpel, C. Bergmann, D. Bockenhauer, L. Breysem, M. Cadnapaphornchai, M. Cetiner, J. Dudley, F. Emma, M. Konrad, T. Harris, P. Harris, J. König, M. Liebau, M. Marlais, D. Mekahli, A. M. Metcalfe, Jun Oh, R. Perrone, M. Sinha, A. Titieni, R. Torra, S. Weber, P. Winyard, F. Schaefer   +23 more
semanticscholar   +1 more source

EGR1 Nuclear Condensates Promote Renal Cyst Development in Polycystic Kidney Disease

Exploration, EarlyView.
ABSTRACT Autosomal dominant polycystic kidney disease (ADPKD) is marked by aberrant cell proliferation driven by cAMP‐PKA and MAPK signaling pathways. EGR1, a transcription factor directly activated by the above two pathways, is critical in the over‐proliferation of tumor cells, which share similarities with cystic epithelial cells in ADPKD. This study
Chaoqun Ren, Zhaoxu Wu, Min Li, Yaqin Du, Hong Zhou, Yazhu Quan, Mengyao Xiong, Yiming Wang, Zhiwei Qiu, Shuai Zhu, Xiaowei Li, Jinzhao He, Cai Gao, Hui Cai, Tingting Li, Baoxue Yang   +15 more
wiley   +1 more source

Reno-appendiceal fistula in autosomal dominant polycystic kidney disease

Urology Case Reports, 2021
We present a very rare Case of a 53-year-old female with autosomal dominant polycystic kidney disease (ADPKD) who was incidentally found to have a reno-appendiceal fistula while undergoing open bilateral nephrectomy.
Madison S. Hill, Natalia Arias Villela, John C. LaMattina, Yvonne M. Rasko, Michael W. Phelan   +4 more
doaj   +1 more source

Automatic Measurement of Kidney and Liver Volumes from MR Images of Patients Affected by Autosomal Dominant Polycystic Kidney Disease.

Journal of the American Society of Nephrology, 2019
BACKGROUND The formation and growth of cysts in kidneys, and often liver, in autosomal dominant polycystic kidney disease (ADPKD) cause progressive increases in total kidney volume (TKV) and liver volume (TLV). Laborious and time-consuming manual tracing
M. V. van Gastel, Marie E. Edwards, V. Torres, B. Erickson, R. Gansevoort, T. Kline   +5 more
semanticscholar   +1 more source

Compartmentalisation in cAMP signalling: A phase separation perspective

British Journal of Pharmacology, EarlyView.
Cells rely on precise spatiotemporal control of signalling pathways to ensure functional specificity. The compartmentalisation of cyclic AMP (cAMP) and protein kinase A (PKA) signalling enables distinct cellular responses within a crowded cytoplasmic space.
Milda Folkmanaite, Manuela Zaccolo
wiley   +1 more source

Renal cysts in children: a single centre study

Pediatria i Medycyna Rodzinna, 2018
Kidney cysts may be congenital (associated or not with genetic disorders) or rarely acquired. They may be an isolated abnormality or be part of an anomaly syndrome.
Anna Medyńska, Konstancja Fornalczyk, Katarzyna Kiliś-Pstrusińska, Irena Wikiera-Magott, Dominik Kubicki, Danuta Zwolińska   +5 more
doaj   +1 more source

Metabolism-based approaches for autosomal dominant polycystic kidney disease

Frontiers in Molecular Biosciences, 2023
Autosomal Dominant Polycystic Kidney Disease (ADPKD) leads to end stage kidney disease (ESKD) through the development and expansion of multiple cysts throughout the kidney parenchyma.
Ivona Bakaj, Alessandro Pocai
doaj   +1 more source

European ADPKD Forum multidisciplinary position statement on autosomal dominant polycystic kidney disease care [PDF]

, 2017
Autosomal dominant polycystic kidney disease (ADPKD) is a chronic, progressive condition characterised by the development and growth of cysts in the kidneys and other organs and by additional systemic manifestations.
Alastair Kent, Albert C M Ong, Brenda de Coninck, Denis Clément, Dorien Peters, Elena Levtchenko, Hendrik Van Poppel, Joost P H Drenth, José Luis Górriz, Klemens Budde, Lorenzo E Derchi, Marianna Eleftheroudi, null null, null null, null null, Olivier Devuyst, Raymond Vanholder, Richard Sandford, Ron T Gansevoort, Tess Harris, Tevfik Ecder, Vicente E Torres, Yves Pirson   +22 more
core   +6 more sources

Autosomal dominant polycystic kidney disease

BMJ, 2016
#### What you need to know Polycystic kidney disease (PKD) is an inherited condition defined by the pathological development of fluid-filled cysts throughout the kidneys leading to organ enlargement (fig 1⇓) and chronic kidney disease. Autosomal dominant polycystic kidney disease (ADPKD) is the commonest inherited kidney disease1 and is the fourth ...
  +5 more sources

A Retrospective Cross‐Sectional Study of 142 Patients in a Multidisciplinary Tuberous Sclerosis Clinic

Clinical Genetics, EarlyView.
We found key differences between tuberous sclerosis patients with TSC1 and TSC2 variants. Patients carrying TSC2 variants had more severe and earlier‐onset symptoms. We also identified two distinct clinical subgroups which follow different disease courses: one characterized by predominant renal involvement and the other by more pronounced neurological ...
Hila Weisblum Neuman, Sara Via Dorembus, Omer Shlomovitz, Shoshana Greenberger, Einat Lahav, Sharon Mini‐Goldberger, Michal Tzadok   +6 more
wiley   +1 more source