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Renal Dysplasia/Hypoplasia

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Pediatric Nephrology

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Abstract

Congenital renal hypoplasia/dysplasia refers to the end result of a variety of pathogenic mechanisms that disturb the primary induction of metanephric progenitor cells (renal agenesis), dysregulation of nephron number (disorders of progenitor pool size or ureteric bud branching), aberrant progenitor cell differentiation (dysplastic tissue elements), disturbance of the renal vasculature (causing tubular dysgenesis), organ structure (multicystic/dysplastic kidney), and tissue migration (horseshoe kidney, ectopic kidney). Many of these disturbances are linked to mutation of genes in molecular cascades involved in the development of other organs; thus, renal hypoplasia/dysplasia can be seen with a variety of non-renal malformations. It has been useful to assemble large heterogeneous pediatric cohorts under the umbrella term of congenital anomalies of the kidney and urinary tract (CAKUT) as a way to study classification and identification of underlying genes without bias [1]. In this chapter we consider forms of CAKUT associated with reduced functional renal mass at birth.

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Authors and Affiliations

  1. Division of Pediatric Nephrology, Montreal Children’s Hospital, McGill University, Montreal, QC, Canada

    Paul Goodyer

  2. Department of Pediatrics and Department of Human Genetics, Division of Pediatric Nephrology, Montreal Children’s Hospital, McGill University, Montréal, QC, Canada

    Indra R. Gupta

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  1. Paul Goodyer
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  2. Indra R. Gupta
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Correspondence to Paul Goodyer .

Editor information

Editors and Affiliations

  1. Department of Pediatrics, Medical College of Wisconsin, Children’s Research Institute, Children’s Hospital, Health System of Wisconsin, Milwaukee, Wisconsin, USA

    Ellis D. Avner

  2. Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts, USA

    William E. Harmon

  3. Service de Néphrologie Pédiatrique, Hôpital Necker-Enfants Malades, Université Paris-Descartes, Paris, France

    Patrick Niaudet

  4. Department of Pediatrics, Wakayama Medical University, Wakayama City, Japan

    Norishige Yoshikawa

  5. Division of Nephrology, Bambino Gesù Children’s Hospital – IRCCS, Rome, Italy

    Francesco Emma

  6. Division of Nephrology and Hypertension, The Heart Institute, Cincinnati Children’s Hospital Medical Center, College of Medicine, Cincinnati, Ohio, USA

    Stuart L. Goldstein

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Goodyer, P., Gupta, I.R. (2016). Renal Dysplasia/Hypoplasia. In: Avner, E., Harmon, W., Niaudet, P., Yoshikawa, N., Emma, F., Goldstein, S. (eds) Pediatric Nephrology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-43596-0_4

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  • DOI: https://doi.org/10.1007/978-3-662-43596-0_4

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