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Objective. Tubular atrophy is one of the factors predicting poor outcome of renal function in primary immunoglobulin A nephropathy (IgAN). However, the development of tubular atrophy is a late phenomenon during the disease progression. It would be useful to identify early factors that potentially result in renal damage and could be used as early predictors of renal outcome. Material and methods. Forty-eight patients with IgAN were examined retrospectively. All patients had a renal biopsy at the beginning of the study. Histological parameters were reviewed and immunohistochemistry was performed on cryostat sections. Monoclonal antibodies used were against C5b-9, α3β1-integrin, transforming growth factor-β1 (TGF-β1) and α-smooth muscle actin (α-SMA), and the results were correlated with histological data and long-term outcome of renal function. Results. In the glomeruli the extent of C5b-9 deposition had significant positive correlations with the degree of focal glomerulosclerosis (p=0.005), tubular atrophy (p=0.003), interstitial inflammation (p=0.005) and tubular expression of α3β1 (p=0.0001). α3β1 tubular expression correlated positively with the severity of proteinuria (p=0.01), number of glomerular and tubulointerstitial myofibroblasts, and the degree of tubular atrophy (p=0.0001) and interstitial monocyte infiltration (p=0.005). Tubular α3β1 expression and the degree of tubular atrophy had significant implications in the development of renal failure at the beginning and at the end of follow-up, respectively. Conclusions. Glomerular deposition of C5b-9 may participate in the development of glomerulosclerosis in IgAN. Furthermore, its positive correlation with the intensity of tubular α3β1-integrin suggests a possible implication in the development of tubulointerstitial fibrosis. [ABSTRACT FROM AUTHOR]

Strumpell's familial spastic paraplegia is a rare hereditary disease, clinically characterized by progressive disturbance of gait. Focal Segmental Glomerulosclerosis (FSGS) is a frequent glomerulopathy, with an extremely rare familial subtype. The cases of two brothers with Strumpell's disease are reported, who also developed glomerular renal disease, most probably familial FSGS. The genetics of the two disorders, Strumpell's paraplegia and familial FSGS, are discussed, together with the possibility of a parallel transmission. [ABSTRACT FROM AUTHOR]

Background. The effect of a“very low dose” of purified omega-3 fatty acids(PFA) in the progression of severe IgA nephropathy(IgAN) was tested, in a randomized, prospective, controlled trial. METHODS: Fourteen patients were assigned to receive a“very low dose” of PFA(0.85 g EPA and 0.57 g PHA) and 14 patients were treated symptomatically and used as controls. Both groups were similar in terms of serum creatinine(Scr) and glomerular filtration rate(GFR) at baseline. Patients were treated for 4 years. The primary end-points were an increase of 50% or more in Scr or a decrease of 50% or more in GFR at the end of the study. RESULTS: During treatment, 1 patient(7%) in the PFA group and 6(43%) in the control group had an increase of 50% or more in their Scr(p < 0.01). Also, 1 patient(7%) in the PFA group and 7(50%) in the control group had a decrease of 50% or more in GFR(p < 0.007). The mean annual change in Scr was 0.2 mg/dL in the PFA group and 1.0 mg/dL in the control group(p < 0.01). The mean annual change in GFR was− 1.4 mL/min in the PFA group and− 3.0 mL/min in the control group(p < 0.001). One patient in the PFA group(7%) and 6 patients in the control group(43%; p < 0.01) developed end-stage renal disease during the period of observation. CONCLUSIONS: A“very low dose” of PFA is also effective in slowing renal progression in high-risk patients with IgAN and particularly those with advanced renal disease. [ABSTRACT FROM AUTHOR]