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Encyclopedia of Pathology pp 1–2Cite as

Multicystic Renal Dysplasia

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  • First Online:

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Part of the book series: Encyclopedia of Pathology ((EP))

Definition

A nonheritable developmental disease resulting of abnormal metanephric differentiation.

Clinical Features

  • Incidence

    It occurs in 1 in 4300 live births. Multicystic dysplastic kidneys are also a finding of genetic disorders (Sanna-Cherchi et al. 2007).

  • Age

    It is a congenital urinary tract anomaly (Bernstein 1971).

  • Sex

    Multicystic renal dysplasia affects predominantly males.

  • Site

    The condition is usually unilateral.

  • Treatment

    Although previously, nephrectomy was the treatment of choice to avoid potential complications such as infection, pain, hypertension, and malignancy, to date the management is primarily conservative.

  • Outcome

    Most patients have a favorable prognosis.

Multicystic renal dysplasia is usually sporadic but may rarely be associated with obstruction, multi-malformation syndromes, chromosomal anomalies, or hereditary malformation syndromes. Unilateral multicystic renal dysplasia is the most common cause of renal mass in childhood. Bilateral lesions may be present...

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References and Further Reading

  • Bernstein, J. (1971). The morphogenesis of renal parenchymal maldevelopment (renal dysplasia). Pediatric Clinics of North America, 18, 395–407.

    Article  CAS  Google Scholar 

  • Sanna-Cherchi, S., Caridi, G., Weng, P. L., et al. (2007). Genetic approaches to human renal agenesis/hypoplasia and dysplasia. Pediatric Nephrology, 22, 1675–1684.

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Diagnostic and Public Health, Section of Pathology, University of Verona, Verona, Italy

    Anna Caliò & Guido Martignoni

  2. Department of Pathology, Pederzoli Hospital, Peschiera del Garda, Italy

    Diego Segala & Guido Martignoni

Authors
  1. Anna Caliò
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  2. Diego Segala
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  3. Guido Martignoni
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Corresponding author

Correspondence to Guido Martignoni .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Cite this entry

Caliò, A., Segala, D., Martignoni, G. (2019). Multicystic Renal Dysplasia. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4854-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_4854-1

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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