**Acute and Chronic Kidney Diseases**

**1** 

*Columbus, Ohio,* 

 *USA* 

**Congenital Obstructive Nephropathy:** 

*and The Research Institute at Nationwide Children's Hospital,* 

Susan E. Ingraham and Kirk M. McHugh

 **Clinical Perspectives and Animal Models** 

*Department of Pediatrics, Division of Pediatric Nephrology, The Ohio State University* 

Congenital obstructive nephropathy is the leading cause of pediatric chronic kidney disease (CKD). Consequently, it engenders a tremendous societal burden in terms of morbidity and mortality and in health care expenses over the lifespan of affected patients. The challenges clinicians face in the diagnosis, prognosis, and treatment of congenital obstructive nephropathy illustrate the utility of developing effective experimental models for the study of this complex disease process. In this review, we characterize congenital obstructive nephropathy with its myriad causes and manifestations, outline current standards of diagnosis and care, and discuss experimental animal models with relevance in unraveling

Congenital obstructive nephropathy is a complex process of pathologic changes in kidney development and function that arise when antegrade urine flow is impaired beginning *in utero*. The term *congenital obstructive uropathy* is frequently used to describe this condition. However, every urologic obstruction – whether anatomic, mechanical, or functional – should be approached with the knowledge that obstruction can affect the kidneys. For this

Intrinsic anatomic obstructions may occur in isolation or accompanied by other pathology such as renal hypodysplasia. Functional obstructions also occur, which may be transient and self-resolving, or chronic with potentially profound consequences on renal function. Although the etiologies of congenital obstructive nephropathy are myriad, any restriction of urine flow has the potential to produce hydronephrosis, altered renal development, and progression of CKD. This direct link between obstructed urine flow and abnormal kidneys represents a central paradigm of urogenital pathogenesis that has far-reaching implications

Congenital obstructive nephropathy is the most common cause of CKD in children and is among the top three etiologies of pediatric end-stage renal disease (ESRD; NAPRTCS, 2009).

clinical conundrums and molecular mechanisms of this important renal disease.

reason, we prefer the term *congenital obstructive nephropathy.*

**2. Epidemiology of congenital obstructive nephropathy** 

(Woolf & Thiruchelvam, 2001).

**1. Introduction** 
