Journal Article

Prognosis, treatment and outcome of childhood mesangiocapillary (membranoproliferative) glomerulonephritis

Janette C. Cansick, Rachel Lennon, Carole L. Cummins, Alexander J. Howie, Mary E. McGraw, Moin A. Saleem, E. Jane Tizard, Sally-Anne Hulton, David V. Milford and C. Mark Taylor

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 19, issue 11, pages 2769-2777
Published in print November 2004 | ISSN: 0931-0509
Published online September 2004 | e-ISSN: 1460-2385 | DOI: http://dx.doi.org/10.1093/ndt/gfh484
Prognosis, treatment and outcome of childhood mesangiocapillary (membranoproliferative) glomerulonephritis

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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 months–15 years). They were followed for a median of 3.5 years (range: 0–17 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): 9.7–14.6 years]. Five and 10 year renal survival was 92% (CI: 88–100%) and 83% (CI: 74–92%), respectively. Those with nephrotic syndrome at presentation had mean renal survival of 8.9 years (CI: 7.1–10.7 years) vs 13.6 years for those without (CI: 10.8–16.5 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

Journal Article.  4525 words.  Illustrated.

Subjects: Nephrology

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