Congenital ureteropelvic junction obstruction: human disease and animal models.
Ureteropelvic junction (UPJ) obstruction is the most frequently observed cause of obstructive nephropathy in children. Neonatal and foetal animal models have been developed that mimic closely what is observed in human disease. The purpose of this review is to discuss how obstructive nephropathy alters kidney histology and function and describe the molecular mechanisms involved in the progression of the lesions, including inflammation, proliferation/apoptosis, renin-angiotensin system activation and fibrosis, based on both human and animal data. Also we propose that during obstructive nephropathy, hydrodynamic modifications are early inducers of the tubular lesions, which are potentially at the origin of the pathology. Finally, an important observation in animal models is that relief of obstruction during kidney development has important effects on renal function later in adult life. A major short-coming is the absence of data on the impact of UPJ obstruction on long-term adult renal function to elucidate whether these animal data are also valid in humans.
Klein
Julie
J
Institut National de la Santé et de la Recherche Médicale (INSERM), Toulouse, France.
Gonzalez
Julien
J
Miravete
Mathieu
M
Caubet
Cécile
C
Chaaya
Rana
R
Decramer
Stéphane
S
Bandin
Flavio
F
Bascands
Jean-Loup
JL
Buffin-Meyer
Bénédicte
B
Schanstra
Joost P
JP
D000818:Animals; D017209:Apoptosis; D049109:Cell Proliferation; D004195:Disease Models, Animal; D018450:Disease Progression; D006801:Humans; D057446:Hydrodynamics; D007668:Kidney / Q000254:growth & development* / Q000473:pathology* / Q000503:physiopathology; D051379:Mice; D011817:Rabbits; D051381:Rats; D012084:Renin-Angiotensin System / Q000502:physiology; D013552:Swine; D014517:Ureteral Obstruction / Q000151:congenital* / Q000473:pathology* / Q000503:physiopathology