NDT Advance Access originally published online on September 22, 2004
Nephrology Dialysis Transplantation 2004 19(11):2769-2777; doi:10.1093/ndt/gfh484
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Nephrol Dial Transplant Vol. 19 No. 11 © ERA-EDTA 2004; all rights reserved
Original Article
Prognosis, treatment and outcome of childhood mesangiocapillary (membranoproliferative) glomerulonephritis
1 Department of Paediatric Nephrology, Birmingham Children's Hospital, 2 Department of Paediatric Nephrology, Bristol Hospital for Sick Children, 3 Institute of Child Health, University of Birmingham and 4 Department of Pathology, University of Birmingham, UK
Correspondence and offprint requests to: Dr Janette Christine Cansick, Department of Nephrology, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK. Email: jcansick{at}doctors.org.uk
Background. Prognostic factors and outcome are incompletely known in childhood mesangiocapillary glomerulonephritis (MCGN). This study aimed to correlate renal outcome with clinical and histopathological variables.
Methods. We conducted a two-centre retrospective analysis of children with MCGN.
Results. Fifty-three children presented at a mean age of 8.8 years (range: 13 months15 years). They were followed for a median of 3.5 years (range: 017 years). Histological classification identified 31 type 1, 14 type 2, two type 3 and six undetermined type. Mean renal survival [time to end-stage renal failure (ESRF)] was projected to be 12.2 years [confidence interval (CI): 9.714.6 years]. Five and 10 year renal survival was 92% (CI: 88100%) and 83% (CI: 7492%), respectively. Those with nephrotic syndrome at presentation had mean renal survival of 8.9 years (CI: 7.110.7 years) vs 13.6 years for those without (CI: 10.816.5 years) (P = 0.047). The mean estimated glomerular filtration rate (eGFR) at 1 year in those who progressed to ESRF was 52 vs 98 ml/min/1.73 m2 in those who did not (P < 0.001). Chronic damage scored on the first biopsy in 31 children (one centre) was positively associated with adverse renal outcome at 5 years: <20% was associated with 100% and
20% with 71% 5-year renal survival (P = 0.006). In 29 children treated with steroid there was a higher proportion (76%) with reduced eGFR at presentation and a significantly higher incidence of nephrotic syndrome (P = 0.002) and hypertension (P = 0.037). There were no significant differences in outcome eGFR, hypertension or proteinuria.
Conclusions. Nephrotic syndrome at presentation and subnormal eGFR at 1 year were adverse features. The finding that structural disease at onset predicted poor renal outcome at 5 years has implications for the design of therapeutic trials. Treatment of MCGN was variable and not evidence-based.
Keywords: childhood; chronic renal damage; mesangiocapillary glomerulonephritis; prognostic factors; renal survival; treatment