Congenital ureteropelvic junction obstruction: human disease and animal models

Abstract

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.

Urinary Proteomics Yield Pathological Insights for Ureteropelvic Junction Obstruction

“…4) and are regulated by NFE2L2, which is activated upon exposure to ROS to effect transcriptional changes in response to oxidative stress (21). Oxidative stress has been implicated in the pathogenesis of UPJO and animal models previously (22)(23)(24). A total of 24 statistically significant proteins were associated with oxidative stress, of which 13 are represented in the putative biomarker panel (Table III).…”

The early and late period alterations on the contralateral renal parenchyma secondary to unilateral ureteropelvic junction obstruction: experimental study

“…Ancak, Klein ve ark. 'nın (19) yapmış oldukları literatür taramasında tanımlanan modellerin insanlarda görülen bu patolojiyi iyi taklit ettiği sonucuna varılmıştır. Tabi ki bu modellerin hiçbirisi mükemmel olmadığı için farklı tür ve farklı tekniklerle elde edilen verilerin birlikte değerlendiril-mesi bu patolojinin daha iyi anlaşılmasına yardımcı olacaktır.…”

Neonatal Imaging Evaluation of Common Prenatally Diagnosed Genitourinary Abnormalities

“…60 Ureteropelvic junction (UPJ) obstruction is the most frequently found cause of antenatal hydronephrosis, with a reported prevalence of 1 in 1500 newborns and a male and left-sided predominance. 14,60-62 Most neonates with UPJ obstruction are typically asymptomatic, and the condition is predominantly detected after postnatal evaluation for antenatal hydronephrosis 63 (Fig.…”