Clinical manifestations of IgA nephropathy combined with thin glomerular basement membrane nephropathy in children.

BACKGROUND: Immunoglobulin A nephropathy (IgAN) and thin glomerular basement membrane nephropathy (TBMN) are the most common causes of persistent hematuria during childhood. The objective of this study is to determine the difference in clinical features and laboratory findings between pediatric patients with IgA deposited TBMN and IgAN alone. METHODS: Between January 2000 and March 2009, 95 children were diagnosed with IgAN by renal biopsy. Clinical features and laboratory findings of patients with isolated IgAN and with IgAN plus TBMN were compared; the children diagnosed with IgAN were compared to 127 children who had been diagnosed with TBMN alone during the same period. RESULTS: There were 71 (74.7%) of a total 95 patients that were diagnosed with isolated IgAN (Group1); in 24 (25.3%) of the 95 patients IgAN was combined with TBMN (Group 2). There was marked difference in the gender distribution between Group 2 and isolated TBMN patients. The degree of proteinuria and pathologic severity was higher in Group 1 compared with Group 2. Gross hematuria was present in both groups. There were no distinguishing features in the other laboratory parameters. CONCLUSION: Patients with both IgAN and TBMN seem to have similar clinical features to patients with isolated IgAN; however, the latter tend to have better pathologic and laboratory findings, compared to the patients with IgAN alone.

A Case of Idiopathic Membranous Glomerulonephritis in association with Thin Glomerular Basement Membrane Nephropathy / 대한신장학회잡지

We report a rare case of the idiopathic membranous glomerulonephritis (IMGN) in association with the thin glomerular basement membrane nephropathy (TGBMN) in a 63-year-old female with hematuria. This is the first case reported in Korea. In renal biopsy of this case, direct immunofluorescence demonstrated anti-IgG Ab along the glomerular capillary wall with granular pattern. The basement membrane was thin, about 170-220 nm and small epimembranous electron dense deposits were observed by electron microscopy. As this case, the combination of TGBMN and IMGN is very uncommon because the IMGN is characterized morphologically by diffuse global thickening of the glomerular capillary wall, while the TGBMN is defined as an extreme thinning of the glomerular basement membrane, less than 200 nm. Our case showed no renal function deterioration and benign prognosis as other reports showed.