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Rene singolo congenito
Congenital solitary kidney: a compendium of the recommendations of the italian Society of Pediatric Nephrology
Gruppo di lavoro sulle malformazioni congenite dei reni e delle vie urinarie della SINePe
Gennaio 2023 - pagg. 21 -30 | DOI: 10.53126/MEB42021
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 -.
Riassunto
Negli ultimi anni sono stati pubblicati vari studi sulla prognosi dei bambini con rene singolo congenito (RSC), ma al momento non c’è consenso sulla gestione e il follow-up di questa condizione. Recentemente, la Società Italiana di Nefrologia Pediatrica ha pubblicato delle raccomandazioni per la diagnosi e la gestione di bambini con RSC. In questo articolo, presentiamo un compendio di queste raccomandazioni inerenti l’approccio diagnostico, nutrizionale, le abitudini di vita con particolare attenzione alla pratica sportiva, e il follow-up. Raccomandiamo che ogni sospetto/diagnosi prenatale di RSC sia confermato da un’ecografia eseguita nel periodo neonatale, mentre l’uso routinario di altre tecniche di imaging non è raccomandato. Ci si attende che un RSC normale vada incontro a crescita compensatoria, che può essere valutata mediante ecografia. Nei lattanti e bambini con RSC in crescita compensatoria alla diagnosi, raccomandiamo l’esecuzione di un esame urine, ma non di esami ematochimici di funzione renale né esami genetici di routine. Deve essere ricercata la presenza di malformazioni extrarenali, in particolare quelle genitali nei soggetti di sesso femminile. Non sono necessarie diete ipoproteiche e iposodiche, ma l’eccesso va comunque evitato. L’attività sportiva non deve essere limitata. I soggetti con RSC necessitano di follow-up per tutta la vita basandosi sulla seguente stratificazione del rischio: -basso rischio: RSC in crescita compensatoria senza anomalie omolaterali associate dei reni e delle vie urinarie (CAKUT); - medio rischio: RSC senza crescita compensatoria e/o una CAKUT omolaterale associata; -alto rischio: riduzione del filtrato glomerulare e/o proteinuria e/o ipertensione.
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