Objectives Steroid-resistant nephrotic syndrome (SRNS) remains a challenge facing pediatric nephrologists. We have studied the pattern of the histopathology of SRNS in children presented to King AbdulAziz University Hospital (KAUH).

Patients and Methods We reviewed the notes of all primary nephrotic children with SRNS, who presented to the pediatric renal unit at KAUH between 2002 and 2007. We have recorded the age of presentation, and histopathology results. We have excluded patients with congenital nephrotic syndrome, lupus or sickle cell disease. Only patients who had a renal biopsy were included in the study.

Results 36 children fulfilled the inclusion criteria, 25 girls and 11 boys. Their mean age at presentation +SD was 4.3 +3.0 (range 1–12) years. Their mean serum albumin at presentation was 15.6 +7.1 g/l and all of them had 4+ proteinuria. Five children had high creatinine at presentation while the mean serum creatinine was 50.4 +45.6. Three children had low complements at presentation and none have positive hepatitis surface antigen or positive antinuclear antibody (ANA). The renal histopathology was compatible with focal segmental glomerulosclerosis (FSGS) in 50% (18 children), IgM nephropathy in 38% (10 children), minimal change disease in 5% (2 children), mesengioproliferative disease in 8% (3 children), and C1q nephropathy in 8% (3 children).

Conclusion FSGS was the most common underlying histopathology in children presented with SRNS to KAUH. IgM nephropathy and other variants of MCD such as mesingioproliferative disease were the second most common cause. C1q nephropathy was the underlying cause in some children.