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Published on: February 4, 2015
Perspectives on human perinatal renal tract disease
1Nephro-Urology Unit, University College London Institute of Child Health, 30 Guilford Street, London, UK. a.woolf@ich.ucl.ac.uk
Insights
Perinatal renal tract diseases are a leading cause of childhood end-stage renal failure and later-life morbidity. Further research is needed to assess the long-term effectiveness of current treatments for protecting kidney function.
Area of Science:
- Pediatric Nephrology
- Fetal Medicine
- Medical Genetics
Background:
- Perinatal renal tract disease is the primary cause of end-stage renal failure in children.
- These conditions can lead to significant long-term morbidity, including hypertension.
- Current understanding of fetal diagnosis accuracy and lifetime outcomes for affected children is limited.
Purpose of the Study:
- To highlight the need for prospective trials evaluating existing therapies for protecting long-term kidney function.
- To address uncertainties in prenatal diagnosis of renal tract disorders.
- To explore the role of genetics in congenital renal malformations.
Main Methods:
- Review of current understanding and uncertainties in perinatal renal tract disease.
- Identification of the need for prospective clinical trials.
- Discussion of emerging genetic research in renal malformations.
Main Results:
- Existing therapies like prenatal surgical decompression and postnatal drugs require efficacy trials for long-term kidney protection.
- Specific drugs targeting renal cyst growth are under human investigation.
- Genetic mutations are increasingly identified as causes for renal tract malformations.
Conclusions:
- Prospective trials are essential to determine the long-term benefits of current and experimental treatments for perinatal renal tract disease.
- Advances in genetics offer explanations for congenital kidney disorders.
- Improved understanding and management are crucial for improving outcomes in affected children.
Abstract:
Perinatal renal tract disease is the main cause of end-stage renal failure in children. The diagnosis also includes many disorders of lesser severity, which may cause morbidity, such as hypertension, in later life. There remain uncertainties in the accuracy of some fetal diagnoses, and the life-time outcomes of children born with renal tract disease, who survive the neonatal period, are not known. There is a need for prospective trials to establish the efficacy, if any, with regard to protection of long-term kidney function of existing 'therapies', such as prenatal surgical decompression and postnatal drugs, which might slow the progression of renal failure. Specific drugs, which may block the growth of renal cysts, are being tested in humans. Advances in genetics are uncovering specific mutations in some children with renal tract malformations, providing parent and carers with explanations for the occurrence of such diseases.
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