Management of the congenital solitary kidney: consensus recommendations of the Italian Society of Pediatric Nephrology

Abstract

Background In recent years, several studies have been published on the prognosis of children with congenital solitary kidney (CSK), with controversial results, and a worldwide consensus on management and follow-up is lacking. In this consensus statement, the Italian Society of Pediatric Nephrology summarizes the current knowledge on CSK and presents recommendations for its management, including diagnostic approach, nutritional and lifestyle habits, and follow-up. Summary of the recommendations We recommend that any antenatal suspicion/diagnosis of CSK be confirmed by neonatal ultrasound (US), avoiding the routine use of further imaging if no other anomalies of kidney/urinary tract are detected. A CSK without additional abnormalities is expected to undergo compensatory enlargement, which should be assessed by US. We recommend that urinalysis, but not blood tests or genetic analysis, be routinely performed at diagnosis in infants and children showing compensatory enlargement of the CSK. Extrarenal malformations should be searched for, particularly genital tract malformations in females. An excessive protein and salt intake should be avoided, while sport participation should not be restricted. We recommend a lifelong follow-up, which should be tailored on risk stratification, as follows: low risk: CSK with compensatory enlargement, medium risk: CSK without compensatory enlargement and/or additional CAKUT, and high risk: decreased GFR and/or proteinuria, and/or hypertension. We recommend that in children at low-risk periodic US, urinalysis and BP measurement be performed; in those at medium risk, we recommend that serum creatinine also be measured; in high-risk children, the schedule has to be tailored according to kidney function and clinical data.

Early Diagnosis of Solitary Functioning Kidney: Comparing the Prognosis of Renal Agenesis and Multicystic Dysplastic Kidney

“…We performed this examination only in selected children (SFK length below the 5th percentile, urinary tract dilatation, UTI) and thus may not have detected all SFK cases with VUR. However, our practice of performing MCUG is in line with recent recommendations [19].…”

“…Of the 22 hypertensive patients, ve had only nocturnal hypertension, which is impossible to detect without ABPM. Although according to the Italian Society of Pediatric Nephrology consensus recommendations, ABPM should not be used preventively in all children with a SFK [19], we intend to continue preventive ABPM for the time being based on our results.…”

Time to Change Our Viewpoints to Assess Renal Risks in Patients with Solitary Kidneys beyond Traditional Approaches?

“…Studies to develop preventive strategies for long-term complications in this patient group are important. Developing similar guidelines for adults as well as pediatric patients may be instructive [76]. The phosphatase and tensin homolog (PTEN) is a negative regulator of protein kinase B and the mammalian target of the rapamycin pathway.…”

‘Grumbling’ Midgut Volvulus in an Older Paediatric Patient With Congenital Solitary Kidney: A Case Report

“…Congenital solitary kidney (CSK) is the anatomical or functional absence of one kidney from birth [ 5 ]. This can occur due to a failure of embryonic renal tissue formation (renal agenesis), or due to extreme forms of dysplasia (renal aplasia and multicystic dysplastic kidney) [ 5 ]. It is a relatively common occurrence with an incidence of 1 in 2000 to 1 in 4300 live births [ 6 , 7 ].…”