Early presentation of membranoproliferative glomerulonephritis in Arab children

Idiopathic membranoproliferative glomer-ulonephritis (MPGN) is a relatively uncommon cause of progressive renal disease characterized by immune complex deposition resulting in mesangial proliferation and endocapillary inflammation with capillary wall thickening. It has a variable clinical expression and usually thought of as a disease of older children and young adults. In this study we report the spectrum of MPGN in Arab children.

Eight Arab patients with MPGN type I and type II were described and studied retrospectively. This study was carried out at King Abdul-Aziz University Hospital, Jeddah, Kingdom of Saudi Arabia during a 6 year period, 1996-2002.

Their mean age at presentation was 2.4 +/- 1.2 years. All patients presented with a steroid resistant nephrotic syndrome. None had macroscopic hematuria. However 5 (62.5%) were hypertensive at presentation. Complements were low in 3 patients (37.5%). The mean follow-up between presentation and last visit was 1.1 +/- 0.7 years; range 0.1-2. Three patients were siblings and their parents were 2nd-degree cousins. Another patient had a brother who had a renal failure following steroid resistant nephrotic syndrome (SRNS), but the histological cause of his SRNS was not known. Four patients were on dialysis within 2 years of follow-up, one patient progressed to chronic renal failure with creatinine of 240 umol/l, one patient died and 2 patients were lost follow-up.

Membranoproliferative glomerulonephritis seems to present at earlier age in Arab children and tends to have a severe course with rapid progression to end stage renal disease.