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Congenital ureteropelvic junction obstruction: human disease and animal models
Author(s) -
Klein Julie,
Gonzalez Julien,
Miravete Mathieu,
Caubet Cécile,
Chaaya Rana,
Decramer Stéphane,
Bandin Flavio,
Bascands JeanLoup,
BuffinMeyer Bénédicte,
Schanstra Joost P.
Publication year - 2011
Publication title -
international journal of experimental pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.671
H-Index - 72
eISSN - 1365-2613
pISSN - 0959-9673
DOI - 10.1111/j.1365-2613.2010.00727.x
Subject(s) - fibrosis , nephropathy , medicine , kidney disease , disease , renal function , pathology , animal studies , inflammation , kidney , animal model , endocrinology , diabetes mellitus
Summary 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.