Abstract

In recent years, several studies have been published on the prognosis of children with congenital solitary kidney (CSK), but a worldwide consensus on management and follow-up is still lacking. Recently, the Italian Society of Pediatric Nephrology has published a consensus statement with recommendations for the diagnosis and management of the CSK. This article presents a compendium of the recommendations, including diagnostic approach, nutritional and lifestyle habits - with special attention to sports participation - and follow-up. Any antenatal suspicion/diagnosis of CSK should be confirmed by neonatal ultrasound (US), however the routine use of further imaging is not recommended. A normal CSK is expected to undergo compensatory enlargement, which should be assessed by US. In infants and children that show compensatory enlargement of the CSK at diagnosis, urinalysis is recommended but blood tests or genetic analysis are not. Extra-renal malformations should be searched for, in particular genital tract malformations in females. Protein and salt intake should not be restricted, but excess should be avoided. Sport participation should not be limited. A lifelong follow-up is recommended, tailored on risk stratification as follows: low risk - CSK with compensatory enlargement -, - medium risk - CSK without compensatory enlargement and/or additional malformations of the kidney or urinary tract - and high risk - decreased glomerular filtration rate and/or proteinuria, and/or hypertension -.

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