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Published on: January 7, 2019
Chronic Kidney Disease in Boys with Posterior Urethral Valves-Pathogenesis, Prognosis and Management
Richard Klaus1, Bärbel Lange-Sperandio1
1Division of Pediatric Nephrology, Department of Pediatrics, Dr. v. Hauner Children's Hospital, Ludwig-Maximilians University, Lindwurmstrasse 4, 80337 Munich, Germany.
Insights
Posterior urethral valves (PUV) cause irreversible kidney and bladder damage, leading to chronic kidney disease (CKD) in many patients. Early prediction and management are crucial for improving outcomes and slowing disease progression.
Area of Science:
- Pediatric Urology
- Nephrology
- Developmental Biology
Background:
- Posterior urethral valves (PUV) represent the most frequent lower urinary tract obstruction (LUTO) in infants.
- While surgically correctable, PUV causes irreversible kidney and bladder developmental impairment with lifelong consequences.
- Chronic kidney disease (CKD) and bladder dysfunction are common sequelae, with approximately 20% of patients progressing to end-stage kidney disease (ESKD).
Purpose of the Study:
- To review the pathophysiology of PUV, focusing on its impact on renal and bladder development.
- To discuss prognostic parameters, including prenatal and postnatal indicators of kidney disease progression.
- To outline current management strategies aimed at preserving renal function and optimizing outcomes in PUV patients.
Main Methods:
- Review of existing literature on posterior urethral valves, lower urinary tract obstruction, and chronic kidney disease.
- Analysis of prenatal and postnatal parameters used for predicting prognosis in PUV.
- Examination of established and experimental markers for CKD progression.
- Discussion of current therapeutic approaches and their impact on renal and graft survival.
Main Results:
- Subvesical obstruction in PUV leads to bladder hypertrophy, fibrosis, and impaired function.
- Kidney development is compromised, resulting in dysplasia, hypoplasia, inflammation, and fibrosis, characteristic of CKD.
- Nadir serum creatinine post-ablation is a key postnatal predictor of ESKD risk.
- Experimental markers like MCP-1, TGF-β, and microalbuminuria may indicate CKD progression.
Conclusions:
- Prenatal interventions improve survival but not renal outcomes.
- Management focuses on controlling bladder dysfunction and CKD progression through hypertension, proteinuria, and infection management.
- Aggressive bladder management is vital for graft survival in kidney transplant recipients with a history of PUV.
Abstract:
Posterior urethral valves (PUV) are the most common form of lower urinary tract obstructions (LUTO). The valves can be surgically corrected postnatally; however, the impairment of kidney and bladder development is irreversible and has lifelong implications. Chronic kidney disease (CKD) and bladder dysfunction are frequent problems. Approximately 20% of PUV patients will reach end-stage kidney disease (ESKD). The subvesical obstruction in PUV leads to muscular hypertrophy and fibrotic remodelling in the bladder, which both impair its function. Kidney development is disturbed and results in dysplasia, hypoplasia, inflammation and renal fibrosis, which are hallmarks of CKD. The prognoses of PUV patients are based on prenatal and postnatal parameters. Prenatal parameters include signs of renal hypodysplasia in the analysis of fetal urine. Postnatally, the most robust predictor of PUV is the nadir serum creatinine after valve ablation. A value that is below 0.4 mg/dl implies a very low risk for ESKD, whereas a value above 0.85 mg/dl indicates a high risk for ESKD. In addition, bladder dysfunction and renal dysplasia point towards an unbeneficial kidney outcome. Experimental urinary markers such as MCP-1 and TGF-β, as well as microalbuminuria, indicate progression to CKD. Until now, prenatal intervention may improve survival but yields no renal benefit. The management of PUV patients includes control of bladder dysfunction and CKD treatment to slow down progression by controlling hypertension, proteinuria and infections. In kidney transplantation, aggressive bladder management is essential to ensure optimal graft survival.
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