A translational approach to congenital non-obstructive hydronephrosis

作者：Thom Robyn P; Rosenblum Norman D^*

来源：Pediatric Nephrology, 2013, 28(9): 1757-1761.

DOI：10.1007/s00467-012-2321-7

• Hydronephrosis
• Hedgehog signaling
• Gli3
• Ubiquitin

Congenital hydronephrosis, defined as congenital dilatation of one or more components of the renal collecting system, is detected in 1-2% of all pregnancies. The majority of antenatally detected hydronephrosis is non-obstructive and either resolves or stabilizes. Management of persistently severe cases may include surgical intervention, the only available treatment. Recent data demonstrate that hedgehog signaling plays a critical role in regulating the structure and function of the collecting system in a manner dependent on the formation of GLI3 repressor. Here, we review the pathobiology and clinical management of non-obstructive hydronephrosis and describe how inhibitors of GLI3 repressor formation may serve as novel therapies for this disorder.

• 出版日期2013-9