Aims To describe the presentation and clinical course of children with MPGN II in a regional paediatric nephrology centre in the past 25 years (1986-2011).
Methods Retrospective case note review of patients with renal biopsy proven MPGN II
Results Twelve patients were identified of which nine were female. The average age at diagnosis was 8.5 years (4-12 years). At presentation 4 children had isolated macroscopic haematuria, 1 child had nephritic syndrome, 2 children had nephrotic syndrome and 3 children had mixed nephritic/nephrotic features (2 missing data) One child had bilateral drusen detected 18 months before developing renal problems. Low C3 levels and normal C4 levels were found in 9/10 patients. C3 nephritic factor was positive in 3/9 patients. A preceding illness was found in 9 patients with 7 having upper respiratory tract infections (URTIs). Four children had hypertension requiring medication. Two children required dialysis at presentation - 1 child remained on dialysis until renal transplant 3 years later and 1 child required dialysis on 2 subsequent occasions due to relapses each triggered by viral URTIs but subsequently recovered normal renal function. Methylprednisolone was given to 4 patients and half of these were also treated with cyclophosphamide. Six children were treated with ACE inhibitors. Four patients had renal transplants - one 3 years after diagnosis, one 4 years after diagnosis and one 25 years after diagnosis. One patient has had two renal transplants - the first within 5 years of diagnosis and the second 15 years later.
Discussion The clinical presentation of MPGN II varied widely including one child who had bilateral drusen before any renal problems presented. The vast majority had C3 hypocomplementaemia but C3 nephritic factor was positive in only 3/9 patients which is considerably less than the >80% CS nephritic factor positivity in MPGN II reported elsewhere. The majority were female. The vast majority had viral URTIs prior to initial presentation. Four patients required transplantation (3-25 years after diagnosis). MPGN is a rare disease with considerable variation in the presentation and clinical course.