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Renal hypodysplasia

Patrick Niaudet, MD
Section Editor
Tej K Mattoo, MD, DCH, FRCP
Deputy Editor
Melanie S Kim, MD


Disruption of normal renal development can lead to congenital anomalies of the kidney and urinary tract (CAKUT), including renal hypodysplasia, which is characterized by congenitally small kidneys with a reduced number of nephrons and dysplastic features.

Renal hypodysplasia, including its pathogenesis, etiologies, presentation, diagnosis, and management will be discussed here. Other CAKUT are discussed separately. (See "Overview of congenital anomalies of the kidney and urinary tract (CAKUT)".)


Normal renal development is dependent upon the interaction between the ureteric bud and metanephric mesenchyme, which induces organogenesis. The average of number of nephrons is approximately 900,000 to 1 million per kidney, but there is a wide range, extending from 200,000 to >2.5 million nephrons per kidney (figure 1) [1]. Nephrogenesis, which starts at 10 weeks of human gestation, can be disturbed by mutations in genes that are involved in this process or by environmental factors, such as nutrition during pregnancy. (See "Overview of congenital anomalies of the kidney and urinary tract (CAKUT)", section on 'Embryology'.)

Renal parenchymal malformations include the following (see "Overview of congenital anomalies of the kidney and urinary tract (CAKUT)", section on 'Renal parenchymal malformations' and 'Pathogenesis' below):

Renal aplasia (agenesis) – Congenital absence of kidney(s). (See "Renal agenesis: Prenatal diagnosis".)

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Literature review current through: Nov 2017. | This topic last updated: Dec 04, 2017.
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