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Chapter 14.1 - Fetal urinary tract obstruction

Pathophysiology

from Section 2 - Fetal disease

Published online by Cambridge University Press:  05 February 2013

By
Robert L. Chevalier
Edited by
Mark D. Kilby ,
Anthony Johnson  and
Dick Oepkes
Mark D. Kilby
Affiliation:
Department of Fetal Medicine, University of Birmingham
Anthony Johnson
Affiliation:
Baylor College of Medicine, Texas
Dick Oepkes
Affiliation:
Department of Obstetrics, Leiden University Medical Center
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Summary

Introduction

Fetal urinary tract obstruction accounts for the largest identifiable cause of kidney failure in infants and children. As for other congenital malformations, the etiology of most cases of congenital obstructive nephropathy is unknown. The site of obstruction along the urinary tract varies from the ureteropelvic junction (UPJ), to the ureterovesical junction and urethra. The hallmark of obstructive nephropathy is hydronephrosis with calyceal dilatation and renal parenchymal thinning depending on the location, timing, and severity of obstruction.

The human metanephros forms by the 5th week of gestation, and nephrogenesis continues through the 34th week, with ongoing perinatal and postnatal maturation through the first two years of life. Congenital obstructive lesions may be associated with abnormal renal development, resulting in renal agenesis, hypoplasia, or dysplasia. There are now substantial experimental data to support a direct effect of urinary tract obstruction on renal growth and development. The molecular basis of these effects has been investigated, suggesting a role for central regulators of epithelial differentiation, such as Wilms tumor-1 (WT1) and paired box gene 2 (PAX2), regulators of stromal development and extracellular matrix formation, as well as mediators of cell growth and survival, such as angiotensin, transforming growth factor-β (TGFβ), and B-cell lymphoma-2 (Bcl-2) [1].

Keywords

renal functional responsekidney failurenephronobstructive uropathyfetal urinary tract obstructionangiotensin II inhibitorabnormal renal development
Type
Chapter
Information
Fetal Therapy
Scientific Basis and Critical Appraisal of Clinical Benefits
 , pp. 238 - 245
Publisher: Cambridge University Press
Print publication year: 2012

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