TY - JOUR
T1 - Hepatic and renal manifestations in autosomal dominant polycystic kidney disease
T2 - A dichotomy of two ends of a spectrum
AU - van Gulick, J. J. M.
AU - Gevers, T. J. G.
AU - van Keimpema, L.
AU - Drenth, J. P. H.
PY - 2011/9
Y1 - 2011/9
N2 - Autosomal dominant polycystic kidney disease (AD PKD) is a multisystem disorder. It is the most common genetic cause of end-stage renal disease. One frequent extra-renal manifestation is hepatic cyst formation. The majority of AD PKD patients develop complications as a result of renal cyst formation; however, a small proportion develop extensive hepatic disease with minor renal features. Both phenotypes seem to represent the spectrum of AD PKD. This review discusses the current understanding of the pathogenesis of the disease, its manifestations and the mechanisms of cyst formation. Furthermore, it focuses on monitoring the disease and the treatment options currently available. © Van Zuiden Communications B.V.
AB - Autosomal dominant polycystic kidney disease (AD PKD) is a multisystem disorder. It is the most common genetic cause of end-stage renal disease. One frequent extra-renal manifestation is hepatic cyst formation. The majority of AD PKD patients develop complications as a result of renal cyst formation; however, a small proportion develop extensive hepatic disease with minor renal features. Both phenotypes seem to represent the spectrum of AD PKD. This review discusses the current understanding of the pathogenesis of the disease, its manifestations and the mechanisms of cyst formation. Furthermore, it focuses on monitoring the disease and the treatment options currently available. © Van Zuiden Communications B.V.
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=80054714430&origin=inward
UR - https://www.ncbi.nlm.nih.gov/pubmed/21978978
M3 - Review article
C2 - 21978978
SN - 0300-2977
VL - 69
SP - 367
EP - 371
JO - Netherlands journal of medicine
JF - Netherlands journal of medicine
IS - 9
ER -