No abstract

Our results suggest that (a) a distinct subgroup of SLE patients exists, made up of younger patients with extensive, active lesions on renal biopsy, who are at higher risk for renal flares, (b) renal flares represent important predictors of doubling serum creatinine.

Urinary lithiasis after renal transplantation is a relatively uncommon disease; the predisposing factors and the composition of calculi are identical to those of patients with native kidneys. We present a case of a 45-year-old woman with a staghorn stone in a left-sided transplanted kidney who was treated successfully by percutaneous nephrolithotomy (PCNL). After reviewing the literature, we conclude that PCNL in transplanted kidney is a feasible and safe procedure. The technical aspects of the procedure, such as patient position and the use of the ultrasound-guided caliceal puncture, are stressed.

The aim of this study was to evaluate, in renal transplant recipients with different function of the graft, the urinary excretion of some low molecular weight proteins and tubular enzymes frequently employed as indicators of tubular dysfunction. Urinary excretion of proteins and enzymes was measured in 51 renal transplant patients and, for comparison, in 73 patients affected by different kidney diseases with various degrees of renal function. Values of urinary beta 2-microglobulin and retinol-binding protein higher than normal were found in most transplanted patients, even in those with good renal function. On the other hand, in renal patients the urinary excretion of low molecular weight proteins was high only when creatinine clearance was lower than 30 mL/min/1.73 m2. Furthermore, an increased urinary excretion of tubular enzymes was found in a higher number of transplanted patients than of renal patients. This behavior was particularly evident for lysosomal enzyme N-acetyl-beta-D-glucosaminidase. In conclusion, a tubular dysfunction occurs in the transplanted kidneys, even in those with well preserved glomerular function.

The incidence of renal flares and the long-term outcome in a group of 33 systemic lupus erythematosus (SLE) patients with diffuse proliferative glomerulonephritis (DPGN) treated with pulse steroids and a short course of pulse cyclophosphamide (CYC) are evaluated. Fifteen patients (45%) experienced a flare of renal disease at some time after the discontinuation of the immunosuppressive (IS) therapy; among these half (24%) were 'early' flares occurring shortly after the discontinuation of therapy, and the other half (21%) were 'late' flares occurring more than 2 y after the discontinuation of the treatment. Nine patients (27%) showed a poor renal outcome at the end of follow-up. On multiple regression analysis, a younger age and a high activity index (AI) on renal histology were found to be correlated with the occurrence of renal flares. Our results suggest that the combination of pulse steroids with a short course of pulse CYC (six to nine pulses) is effective in both controlling disease activity and in preventing the occurrence of renal flares in DPGN. However, short term IS therapy might not be sufficient to maintain disease control in younger patients with active lesions on renal histology. Such patients might be candidates to receive more prolonged IS treatment.

No abstract

The clinical course of primary Focal Segmental Glomerulosclerosis (FSGS) is frequently complicated by nephrotic range proteinuria and progression to renal failure. The high recurrence rate of the disease in transplanted kidney suggests the hypothesis that such patients have a circulating factor that alters glomerular capillary permeability. In recent years some authors found that serum from patients with FSGS increases glomerular permeability to albumin and partially identified the permeability factor (PF) as a protein of 30-50 Kd m.w. The removal of this protein by means of Plasma Exchange (PE) or plasma Immunoadsorption by Protein A (IA) decreased proteinuria. In this report we provide preliminary data about the prevalence of PF and the therapeutic effect of its removal by IA, in 3 pts with recurrence in the transplanted kidney, and 4 with FSGS of the native kidneys. They were resistant to corticosteroids (CS) and immunosuppressive (IS) therapy. 10 IA sessions were performed in 4 weeks: if a remission was achieved IA was gradually tapered. The level of PF in the serum was measured by an in vitro assay to determine the glomerular permeability to albumin. The FSGS was histologically proven in all cases and the degree of evolution was evaluated. PF levels, serum creatinine, daily proteinuria and serum albumin were monitored. The 3 patients with recurrent FSGS had a normalization of the PF levels; 2 had a clinical remission. In FSGS of native kidneys PF was elevated in 3/4 cases; 1 had a clinical remission; 2 with extensive sclerohyalinosis and 1 without PF levels did not improve. Our results confirm that most patients with FSGS have high PF serum levels and suggest that its removal can be beneficial.

PFAPA is an acronym for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This syndrome has been usually described in pediatric patients and it generally resolves spontaneously. The endocapillary proliferative glomerulonephritis (EPG) is a glomerular injury characterized by hypercellularity in glomerular lumen and is caused by post-infectious or autoimmune diseases. In this paper, we describe the case of a 35-year-old man affected by PFAPA and EPG. To our knowledge this association has never been reported in the literature before.