MP824
BILATERAL NEPHRECTOMY IN CHILDREN WITH CONGENITAL NEPHROTIC SYNDROME - IS IT STILL THE WAY TO GO Free

INTRODUCTION AND AIMS: Congenital nephrotic syndrome (CNS) remains one of the most challenging conditions within the field of paediatric nephrology. The management of these children is controversial and includes different approaches ranging from conservative management only, unilateral nephrectomy or bilateral nephrectomies with dialysis and transplantation. However, due to the rarity of the disease clinical trials are scarce and clinicians depend on single centre experiences and guidelines.

METHODS: We conducted a 6-year survey across members of the European Society for Paediatric Nephrology Dialysis Working Group to compare different management strategies (bilateral nephrectomies versus conservative management) in children with CNS.

RESULTS: 82 children (52% male) were included, across 19 tertiary nephrology units in Europe. Median age at presentation was 9.5 (inter quartile range 0-164) days with serum albumin 11 (4-29) g/L and creatinine 27 (2-480) μmol/L. Nephrectomy was performed in 39 (48%) patients - unilateral in 5 (13%), bilateral in 2 steps in 11 (28%) and bilateral in 1 step in 23 (59%) - at a median age of 8 (1-13) months. After unilateral nephrectomy (or first kidney removal in stepwise approach) serum albumin improved from 22.5 to 30 g/L (p=0.012), with a significant decrease in albumin infusion requirement from 6.5 to 0 g/kg/week (p<0.05). To compare outcomes of bilateral nephrectomies versus no nephrectomy, we studied children with NPHS1 mutations and at least 12 months follow-up (n=40); Nephrectomised patients (n=25) presented earlier (2 vs 29 days; p<0.05), but with similar serum albumin (8 vs 10 g/L, p=0.75) and creatinine (20 vs 18 μmol/l, p=0.21) compared to non-nephrectomised patients (n = 15). Long-term dialysis was required in all patients with nephrectomy versus 5 non-nephrectomised children (p<0.05). There was no difference in the number of septic or thrombotic episodes. Growth was comparable between the two groups. At final follow up (median age of 35 and 33 months respectively) in the nephrectomy group 1 patient had died versus 2 patients in the non-nephrectomised group (p=0.28). In the nephrectomy group a significantly higher number were transplanted (80% vs 20%, p<0.01). In the non-nephrectomy group 8 (53%) patients have not required renal replacement therapy at time of final follow-up (median age 2.75 yrs).

CONCLUSIONS: An individualised, stepwise approach, with prolonged conservative management, followed by unilateral nephrectomy may be a reasonable alternative to early bilateral nephrectomies in children with CNS. This approach requires further study in large prospective cohorts.