Renal cystic diseases in children
- Patrick Niaudet, MD
Patrick Niaudet, MD
- Section Editor — Pediatric Nephrology
- Professor of Pediatrics
- Hôpital Necker-Enfants Malades, Paris, France
Renal cysts occur in a variety of diseases in children (table 1). Cysts may be due to nonhereditary fetal malformations or genetic disorders, or, rarely, they may be acquired. Cysts may also occur as an isolated finding or as part of a syndrome.
An overview of the different forms of pediatric renal cystic disease will be reviewed here.
NONHEREDITARY RENAL MALFORMATION
Cystic renal dysplasia, defined by microscopic features, is due to renal parenchymal malformation of the fetal kidney. As a result, the kidney contains primitive ducts and cysts, and nonrenal tissues such as cartilage, fat, and hematopoietic tissue. (See "Overview of congenital anomalies of the kidney and urinary tract (CAKUT)", section on 'Renal parenchymal malformations'.)
Although the nonheritable mechanisms responsible for this disorder remain unclear, an alteration in renal mitogen expression may, in part, underlie the abnormalities in renal parenchymal differentiation. One study of multicystic renal dysplasia, for example, found significant temporal and spatial alterations in insulin-like growth factor and insulin-like growth factor binding protein expression during early fetal life and the postnatal period . The pathogenetic significance of these changes is uncertain.
Cystic dysplasia is also often associated with antenatal obstruction of the urinary tract, which may be associated with posterior urethral valves (PUV) or ureteropelvic junction (UPJ) obstruction. Cystic dysplasia is frequently observed in the atrophic pole of a kidney with duplicated ureters or an ectopic ureterocele.
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- NONHEREDITARY RENAL MALFORMATION
- Multicystic dysplastic kidney
- - Course
- - Management
- - Segmental multicystic dysplasia
- GENETIC DISORDERS
- Glomerular cortical cysts
- - Renal cysts and diabetes (RCAD) syndrome
- - Nephronophthisis
- - Oral-facial-digital syndromes
- Tubular cysts
- ISOLATED RENAL CYSTS
- ACQUIRED RENAL CYSTS
- End-stage renal disease
- Liver transplantation
- SUMMARY AND RECOMMENDATIONS