Diagnosis and management of childhood polycystic kidney disease

WE Sweeney, ED Avner - Pediatric nephrology, 2011 - Springer
A number of syndromic disorders have renal cysts as a component of their phenotypes.
These disorders can generally be distinguished from autosomal dominant polycystic kidney …

[HTML][HTML] Current insights into renal ciliopathies: what can genetics teach us?

HH Arts, NVAM Knoers - Pediatric nephrology, 2013 - Springer
Ciliopathies are a group of clinically and genetically overlapping disorders whose etiologies
lie in defective cilia. These are antenna-like organelles on the apical surface of numerous …

[HTML][HTML] Sirolimus and kidney growth in autosomal dominant polycystic kidney disease

AL Serra, D Poster, AD Kistler, F Krauer… - … England Journal of …, 2010 - Mass Medical Soc
Background In autosomal dominant polycystic kidney disease (ADPKD), aberrant activation
of the mammalian target of rapamycin (mTOR) pathway is associated with progressive …

Sirolimus therapy to halt the progression of ADPKD

N Perico, L Antiga, A Caroli, P Ruggenenti… - Journal of the …, 2010 - journals.lww.com
Activation of mammalian target of rapamycin (mTOR) pathways may contribute to
uncontrolled cell proliferation and secondary cyst growth in patients with autosomal …

Prospects for mTOR inhibitor use in patients with polycystic kidney disease and hamartomatous diseases

VE Torres, A Boletta, A Chapman… - Clinical Journal of the …, 2010 - journals.lww.com
Mammalian target of rapamycin (mTOR) is the core component of two complexes, mTORC1
and mTORC2. mTORC1 is inhibited by rapamycin and analogues. mTORC2 is impeded …

Inhibition of mTOR with sirolimus does not attenuate progression of liver and kidney disease in PCK rats

C Renken, DC Fischer, G Kundt… - Nephrology Dialysis …, 2011 - academic.oup.com
Background. Activation of the mTOR pathway has been implicated in the mediation of the
progression of polycystic kidney disease (PKD). Whereas targeted inhibition of mTOR has …

Mammalian target of rapamycin (mTOR) inhibitors: potential uses and a review of haematological adverse effects

S Sofroniadou, D Goldsmith - Drug Safety, 2011 - Springer
Mammalian target of rapamycin (mTOR) inhibitors (mTORis) constitute a relatively new
category of immunosuppressive and antineoplastic drugs. These share a unique …

Interventions for preventing the progression of autosomal dominant polycystic kidney disease

D Bolignano, SC Palmer, M Ruospo… - Cochrane Database …, 2015 - cochranelibrary.com
Background Autosomal dominant polycystic kidney disease (ADPKD) is the most common
inherited disorder causing kidney disease. Current clinical management of ADPKD focuses …

Novel targets for the treatment of autosomal dominant polycystic kidney disease

FA Belibi, CL Edelstein - Expert opinion on investigational drugs, 2010 - Taylor & Francis
Importance of the field: Autosomal dominant (AD) polycystic kidney disease (PKD) is the
most common life-threatening hereditary disorder. There is currently no therapy that slows or …

[HTML][HTML] A systematic review of reported outcomes in ADPKD studies

SS Jdiaa, NM Husainat, R Mansour, MA Kalot… - Kidney International …, 2022 - Elsevier
Introduction Autosomal dominant polycystic kidney disease (ADPKD) is a progressive
genetic kidney disease. Studies of ADPKD presented results using different outcome …