Membranoproliferative Glomerulonephritis, Type 1, Pediatric

Abstract

Membranoproliferative glomerulonephritis (MPGN) Type I is most common among a group of rare glomerular diseases historically classified according to findings on electron microscopy, and is characterized by subendothelial immune deposits. Most of what was traditionally characterized as MPGN type I falls under the category of immune complexmediated MPGN, while fewer cases of MPGN type I may be attributed to complement dysregulation. In children, MPGN type I is associated with approximately 1.3% of end stage kidney disease (ESKD) patients. Hypocomplementemia, hematuria and proteinuria are common at presentation, with nephrotic syndrome in MPGN associated with a worse prognosis. While there have been various approaches to treatment of MPGN type I in children, the mainstay of therapy is alternate day prednisone.