Results 51 to 60 of about 2,428 (197)

Fetal renal cystic disease and post-natal follow up—a single center experience

Frontiers in Pediatrics, 2023
IntroductionPrenatal sonographic evidence of large, echogenic, or cystic kidneys may indicate any one of a diverse set of disorders including renal ciliopathies, congenital anomalies of the kidney and urinary tract (CAKUT), or multisystem syndromic ...
Lorena Botero-Calderon, Anne Lawrence, Natalie O’Toole, Lisa M. Guay-Woodford, Lisa M. Guay-Woodford   +4 more
doaj   +1 more source

Hepatopancreaticobiliary pathology in the PCK model of ARPKD

The FASEB Journal, 2010
Autosomal Recessive Polycystic Kidney Disease (ARPKD) results in renal and hepatic fibrocystic pathology. This study evaluated hepatobiliary pathology in PCK rats, an orthologous model of slowly progressive ARPKD. Male and female rats were evaluated from 4–35 weeks, quantifying organ growth, serology, bile flow
Stephen Bruce Mason, Tracy Eggleston‐Gulyas, Xianyin Lai, Frank A. Witzmann, Vincent H. Gattone   +4 more
openaire   +1 more source

Is There a Functional Role of Mitochondrial Dysfunction in the Pathogenesis of ARPKD? [PDF]

Frontiers in Medicine, 2021
Polycystic kidney diseases (PKD) form a group of severe genetic kidney diseases that are characterized by fibrocystic changes of the kidneys and the liver. The main forms of PKD are autosomal dominant and autosomal recessive polycystic kidney disease (ADPKD, ARPKD).
openaire   +3 more sources

3D Liver Fibrosis Models in Lab: A Novel Modality for Drug Screening

Pharmacology Research &Perspectives, Volume 14, Issue 4, August 2026.
ABSTRACT Liver fibrosis is the common consequence of liver injury caused by a variety of chronic liver disorders. This condition leads to the development of more severe complications, particularly cirrhosis and hepatocellular carcinoma. Despite abundant studies, the fundamental cell and molecular mechanisms of liver fibrosis are still unknown.
Hani Keshavarz Alikhani, Fatemeh Majidi, Mahsa Ghasemzad, Anastasia Shpichka, Anastasia Nestorova, Zahra Hendi, Elham Rismani, Daria Kuznetsova, Peter Timashev, Massoud Vosough, Abbas Piryaei   +10 more
wiley   +1 more source

Role of genetic modifiers in an orthologous rat model of ARPKD

Physiological Genomics, 2012
Human data and animal models of autosomal recessive polycystic kidney disease (ARPKD) suggest that genetic factors modulate the onset and severity of the disease. We report here for the first time that ARPKD susceptibility is attenuated by introgressing the mutated Pkhd1 disease allele from the polycystic kidney (PCK) rat onto the FHH (Fawn-Hooded ...
Caitlin C, O'Meara, Matthew, Hoffman, William E, Sweeney, Shirng-Wern, Tsaih, Bing, Xiao, Howard J, Jacob, Ellis D, Avner, Carol, Moreno   +7 more
openaire   +3 more sources

Genetic Abnormalities and Clinical Management of Fetal Genitourinary System Anomalies in Eastern China

American Journal of Medical Genetics Part A, Volume 200, Issue 6, Page 1362-1371, June 2026.
ABSTRACT To investigate the correlation between genetic abnormalities and fetal genitourinary (GU) anomalies in Eastern China and to provide assistance for the clinical management of fetuses with different types of GU anomalies. Five hundred forty‐five fetuses with GU anomalies were enrolled, undergoing karyotyping, copy number variation sequencing ...
Jie Liang, Jiebin Wu, Lin Zhang, Yunmeng Qi, Yi Wang, Xu Cao, Jingfang Zhai   +6 more
wiley   +1 more source

Clinical and molecular characterisation of autosomal recessive polycystic kidney disease (ARPKD) in Afrikaans families [PDF]

, 2010
MSc (Med)(Genetic Counselling), Faculty of Health Sciences, University of the WitwatersrandAutosomal recessive polycystic kidney disease (ARPKD; MIM263200) is a severe recessively inherited disease of the kidneys and biliary tract, with an incidence of ...
Lambie, Lindsay Ann
core  

Design and Implementation of the Hepatorenal Fibrocystic Disease Core Center Clinical Database: A Centralized Resource for Characterizing Autosomal Recessive Polycystic Kidney Disease and Other Hepatorenal Fibrocystic Diseases

Frontiers in Pediatrics, 2017
Autosomal recessive polycystic kidney disease (ARPKD) and other hepatorenal fibrocystic diseases (HRFD) are relatively rare recessive disorders that constitute an important set of childhood nephropathies.
Lisa M. Guay-Woodford, Lisa M. Guay-Woodford, Bakri Alzarka, Hiroki Morizono, John W. Bollman, Dongkyu Kim   +5 more
doaj   +1 more source

Telmisartan ameliorates fibrocystic liver disease in an orthologous rat model of human autosomal recessive polycystic kidney disease. [PDF]

PLoS ONE, 2013
Human autosomal recessive polycystic kidney disease (ARPKD) produces kidneys which are massively enlarged due to multiple cysts, hypertension, and congenital hepatic fibrosis characterized by dilated bile ducts and portal hypertension.
Daisuke Yoshihara, Masanori Kugita, Mai Sasaki, Shigeo Horie, Koichi Nakanishi, Takaaki Abe, Harold M Aukema, Tamio Yamaguchi, Shizuko Nagao   +8 more
doaj   +1 more source

Paediatric renal transplantation: Paediatric surgeons' perspective

Surgical Practice, Volume 30, Issue 2, Page 117-122, May 2026.
Abstract Renal transplantation is the most effective treatment for paediatric end‐stage renal disease (ESRD), offering advantages in survival, growth and neurocognitive development that surpass other renal replacement therapies (RRT). The paediatric setting, however, introduces distinct complexities that distinguish it from adult practice.
Adrian Chi‐heng Fung, Mary Kim Elizabeth Tan Cudia, Ivy Hau‐yee Chan, Kenneth Kak‐yuen Wong   +3 more
wiley   +1 more source