Unilateral Multicystic Dysplastic Kidney Management: A National Survey.

Risks of contralateral kidney abnormalities and chronic kidney disease necessitate follow-up for unilateral multicystic dysplastic kidneys (MCDK). A nationwide survey of senior UK pediatricians was conducted. Of the 60 responses obtained, 62% routinely perform a dimercaptosuccinic acid scan to confirm diagnosis. Eight percent routinely perform a cystogram to investigate contralateral vesicoureteric reflux. Sixty-two percent would routinely measure renal function (frequency ranging from once only to "every 2 years"). Twenty-five percent recalled MCDK nephrectomy being performed within the previous 5 years. Respondents voiced concerns that national guidance may result in an overcautious approach but could balance consensus and safe variation, and offer families choice and reassurance. The mean estimated cost of follow-up from birth to 18 years ranged from £258 to £3854. Results demonstrate significant variation in management, highlighting the need for a clear pathway to decrease unwanted variability and to ensure those at high risk of renal sequelae are recognized early, without undue investigatory burden.

Tolvaptan and urea in paediatric hyponatraemia.

BACKGROUND: The syndrome of inappropriate antidiuretic hormone (SIADH) is usually treated with fluid restriction. This can be challenging in patients with obligate fluid intake for nutrition or medication. Pharmaceutical treatment with tolvaptan and urea is available but minimal paediatric data are available. We review the efficacy and safety of tolvaptan and urea in paediatric patients with SIADH. METHODS: Retrospective review of paediatric inpatients with clinical diagnosis of SIADH. Patients were identified from pharmacy records based on tolvaptan and urea prescriptions. Relevant information was extracted from patient electronic records. The main outcome measures included the number of days to sodium normalisation, the daily change in plasma sodium concentration, and the maximum increase of plasma sodium concentration in 24 h. Reported side effects were captured. RESULTS: Thirteen patients received tolvaptan and six urea. Five patients had both agents (tolvaptan converted to urea). Tolvaptan led to plasma sodium normalisation in 10/13 (77%) within 6 days (median 2.5 days, range [1, 6]), with a median change of sodium concentration of 7 mmol/L (- 1, 14) within the first 24 h of treatment. Three patients experienced a change in plasma sodium > 10 mmol/l/day but had no apparent side effects. Urea led to sodium normalisation in 5/6 (83%) patients. The median number of days to normalisation with urea was 2 (1, 10) with a median change of plasma sodium concentration of 2 mmol/L (- 1, 6) within the first 24 h. All patients tolerated tolvaptan and/or urea without unexpected side effects. CONCLUSIONS: Tolvaptan and urea appear to be safe and effective when fluid restriction is challenging in paediatric SIADH. A higher resolution version of the Graphical abstract is available as Supplementary information.

A comparison of arteriovenous fistulas and central venous lines for long-term chronic haemodialysis.

BACKGROUND: Despite the Fistula First initiative there is still reluctance to use arteriovenous fistulas (AVF) for chronic haemodialysis (HD) in children. Our aim was to compare outcomes of AVFs and central venous lines (CVL) in children on chronic HD in a centre where AVF is the primary choice for vascular access. PATIENTS AND METHODS: This was a retrospective case notes analysis of access complications, dialysis adequacy and laboratory outcomes in children who underwent dialysis for at least a year by AVF (n = 20, median age 14.2 years, range (2.9-16.5) and CVL (n = 5, median age 2.4 years, range 2.0-12.2) between January 2007 and December 2010. RESULTS: Primary access failure rate (patient-months) was 1 per 78.8 for AVF (n = 5) and 1 per 15.5 for CVLs (n = 7, p = 0.3). Failure thereafter was 1 per 131.3 and 1 per 18.5 for AVF and CVLs respectively (n = 3 and 6 respectively; p = 0.2). The annualised hospitalisation rate for access malfunction was 0.44% and 3.1% for AVFs and CVLs respectively (p = 0.004). Patients with AVFs had a lower infection rate of 0.25 per 100 patient-months compared with CVL at 3.2 per 100 (p = 0.002). There was no difference in dialysis adequacy or laboratory values between AVF and CVL groups. Access survival rates (including both primary and secondary access failure) were significantly higher for AVF compared with CVL (p = 0.0002, hazard ratio = 0.15, 95% confidence interval 0.04-0.37). CONCLUSIONS: Patients with AVF spend less time in hospital than those dialysed by CVLs and have a much lower access infection rate. These findings emphasise the need to use AVF as first-line access for paediatric patients on chronic HD.