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Curr Opin Pediatr
2016[Apr]; 28
(2
): 209-15
PMID26849006
show ga
PURPOSE OF REVIEW: Renal dysplasia is classically described as a developmental
disorder whereby the kidneys fail to undergo appropriate differentiation,
resulting in the presence of malformed renal tissue elements. It is the commonest
cause of chronic kidney disease and renal failure in the neonate. Although
several genes have been identified in association with renal dysplasia, the
underlying molecular mechanisms are often complex and heterogeneous in nature,
and remain poorly understood. RECENT FINDINGS: In this review, we describe new
insights into the fundamental process of normal kidney development, and how the
renal cortex and medulla are patterned appropriately during gestation. We review
the key genes that are indispensable for this process, and discuss how patterning
of the kidney is perturbed in the absence of these signaling pathways. The recent
use of whole exome sequencing has identified genetic mutations in patients with
renal dysplasia, and the results of these studies have increased our
understanding of the pathophysiology of renal dysplasia. SUMMARY: At present,
there are no specific treatments available for patients with renal dysplasia.
Understanding the molecular mechanisms of normal kidney development and the
pathogenesis of renal dysplasia may allow for improved therapeutic options for
these patients.
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