Long-term renal survival and related risk factors for primary membranous nephropathy in Chinese children: a retrospective analysis of 217 cases.

BACKGROUND

Primary membranous nephropathy (PMN) is a rare pathological finding in paediatric patients. Data on PMN in children have been restricted to studies with small samples and fairly short follow-up periods. Therefore, we conducted this single-centre study to evaluate the long-term renal survival and related risk factors for PMN in children, and the clinical and histological characteristics were also described.

METHOD

Two hundred and seventeen children with PMN were enrolled from July 2008 to September 2017. Patients with follow-up durations < 12 months were excluded, except for patients who progressed to end-stage kidney disease (ESKD) or experienced a related death within 12 months. Long-term renal survival and related risk factors were analysed.

RESULT

The sex ratio was 1.33:1 (male vs female), and the median age was 15.0 (14.0-17.0) years old. The most prominent clinical manifestation was nephrotic syndrome (130 59.9%), which was accompanied by various degrees of oedema (142 65.4%), hyperlipidaemia (151 69.6%), hypoalbuminemia (130 59.9%), and nephrotic proteinuria (135 62.2%). Hypertension occurred in 36.4% of children with PMN. After a median follow-up of 45.0 (23.5-74.0) months, 11 patients (5.1%) developed ESKD, and the cumulative kidney survival rates of ESKD at 5 and 10 years after renal biopsy were 95.3% and 67.8%, respectively. The cumulative kidney survival rates of the combined event of ESKD and/or 30% decline in estimated glomerular filtration rate (eGFR) at 5 and 10 years after renal biopsy were 92.6% and 59.5%, respectively. Cox multivariate regression and Kaplan-Meier analysis demonstrated that hypertension and proteinuria ≥ 50 mg/kg/day were associated with renal outcome.

CONCLUSION

In this study, the 5-year and 10-year cumulative renal survival rates of ESKD in children with PMN were reported for the first time as 95.3% and 67.8%, respectively. In addition, this is the first report to find that hypertension and proteinuria  ≥ 50 mg/kg/day are associated with renal outcome in children with PMN.