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Aim: To investigate the changing of idiopathic membranous nephropathy (iMN) in China., Methods: This study retrospectively analyzed renal disease spectrum of 6049 patients who underwent renal biopsy at Peking University First Hospital from January 2003 to December 2012. The patients were grouped into two periods at a 5-year interval: 2003-2007 (period 1) and 2008-2012 (period 2)., Results: Among 6049 renal biopsied patients, 3831 (63.3%) patients were diagnosed as primary glomerular disease (PGD). The proportion of nephrotic syndrome (NS) in PGD was significantly higher in period 2 than that in period 1 (1016/2214 [55.0%] vs 682/1617 [42.2%], P = 0.022). The proportion of iMN in PGD increased from 16.8% (217/1617) in period 1 to 29.35% (646/2214) in period 2 in all groups of age (P < 0.001). There was no significant difference of clinical characteristics including age, gender, hypertension, serum cholesterol and proteinuria between the two periods (P > 0.05). However, in young patients with iMN (14-44 years old), the percentage of renal histopathology stage I and stage II of iMN in PGDs was significantly higher in period 2 than that in period 1 (101/1240 [8.15%] vs 210/1340 [15.7%], P < 0.001)., Conclusion: Our study suggests that the frequency of iMN in PGD in our referral diagnostic centre has doubled over the past 10 years. The increase of adult iMN is mainly due to the increase of early stages of iMN in young patients., (© 2015 Asian Pacific Society of Nephrology.)

Objective: To study the clinical and pathological features of Denys-Drash syndrome (DDS)., Method: Three DDS cases who were treated in our department from December 2009 to June 2011 were subjected to this study by reviewing of literature., Result: Both case 1 and case 2 were female, with karyotype 46, XX. Case 3 was male with bilateral cryptorchidism. The ages of nephropathy onset of the three cases were 1 year and 9 months, 2 years and 8 moths, and 3 months respectively. Proteinuria in case 2 and case 3 were evidenced to be resistant to steroid. Case 1 was partially responsive to tacrolimus, plasma albumin and cholesterol were improved, although proteinuria was persistent after Tacrolimus was administered. Remission was achieved in case 2 after administration of cyclosporine A and later tacrolimus, and her renal function remains normal till present (4 years and 9 months). Residue renal histology revealed diffused mesangial sclerosis (DMS) in all three patients. All of the three patients had developed right unilateral Wilms tumor. A novel WT1 missense mutation exon 9 c.1213C > G was detected in case 1. WT1 exon 9 c.1168C > T nonsense mutation and exon 8 c.1130A > T missense mutation were detected in case 2 and case 3, respectively., Conclusion: The clinical manifestation of nephropathy in DDS is variable. The majority present with early onset nephropathy and reach renal failure before the age of 4 years. But in a few patients, nephropathy can also be present much later and progress slowly. Proteinuria in DDS is resistant to steroid but is responsive to calcineurin inhibitors, including Cyclosporine A. The effectiveness of tacrolimus was also observed in this study. DDS is evidently caused by WT1 mutation. DMS is the characteristic renal pathological change in DDS.

Background: Nephrotic syndrome is caused by a variety of glomerulopathy. The current study investigated the renal histopathological spectrum of patients with nephrotic syndrome who received a renal biopsy in our department within the last 15 years., Methods: One thousand five hundred and twenty-three consecutive patients (≥14 years old at renal biopsy) with nephrotic syndrome were recruited. Patients were divided into four groups according to age at the time of renal biopsy. The renal histopathological spectrum was also compared between nephrotic-range proteinuria patients with and without hypoalbuminaemia., Results: Among the 1523 patients, the most common cause of nephrotic syndrome was idiopathic membranous nephropathy (IMN) (20.7%), followed by minimal change disease (MCD) (20.4%). Among the patients aged 14-24, 25-44, 45-59 and ≥60 years, the most common cause of nephrotic syndrome was MCD (33.0%), lupus nephritis (LN) (23.0%), IMN (37.9%) and IMN (42.3%), respectively. Among the female patients aged 14-24 and 25-44 years, LN was the leading cause of nephrotic syndrome (35.8 and 36.2%, respectively). The proportion of patients with renal amyloidosis increased in parallel with patient age. The comparison between nephrotic patients with and without hypoalbuminaemia suggests that patients with MCD, LN or renal amyloidosis were more likely to develop hypoalbuminaemia., Conclusions: The renal histopathological spectrum of nephrotic syndrome differs between ages. MCD, LN and IMN were the main cause of nephrotic syndrome among younger patients, and IMN was the main cause of nephrotic syndrome among older patients. The proportion of patients with renal amyloidosis increased in parallel with patient age.

Background: Massive proteinuria is often associated with hypoalbuminaemia in glomerulopathy. However, patients may have normal levels of serum albumin despite heavy proteinuria in many circumstances. This study analysed factors affecting serum levels of albumin in primary glomerulopathy patients with nephrotic-range proteinuria., Methods: The renal histopathological data of 780 consecutive adult patients (age ≥ 18 years old) with primary glomerulopathy and nephrotic-range proteinuria, who received native renal biopsies in Peking University First Hospital from 1998 to 2007, were retrospectively analysed., Results: Compared with patients with hypoalbuminaemia (serum albumin < 30 g/L), patients without hypoalbuminaemia were significantly younger (P < 0.001) and had significantly lower levels of proteinuria (P < 0.001). Patients without hypoalbuminaemia had a significantly higher proportion of IgA nephropathy (66.0% vs. 17.2%, P < 0.001). The independent predictors of hypoalbuminaemia in nephrotic-range proteinuria patients included age, gender, interval between onset of the disease and renal biopsy, proteinuria level, and pathological type of glomerulopathy. A serum level of albumin ≥ 35 g/L could predict IgA nephropathy with a specificity of 95.8%, and specificity increased with age., Conclusions: Among patients with primary glomerulopathy and nephrotic-range proteinuria, normoalbuminaemia is associated with IgA nephropathy.

Background: Idiopathic membranous nephropathy (IMN), a common cause of nephrotic syndrome in adults, is usually treated with corticosteroids in combination with cyclophosphamide or cyclosporine. A recent placebo-controlled study suggested that tacrolimus monotherapy was effective in IMN. However, the effectiveness of tacrolimus versus classic regimen and its potential nephrotoxicity remain inconclusive. This study evaluated the efficacy and safety of tacrolimus plus prednisone in patients with nephrotic IMN., Methods: Seventy-three patients with nephrotic IMN were recruited in this multicenter randomized controlled trial, 39 receiving tacrolimus and prednisone, while 34 receiving cyclophosphamide and prednisone. Tacrolimus was given at 0.1 mg/kg/d initially and adjusted to a blood trough level at 5 to 10 ng/mL for 6 months and then reduced to 2 to 5 ng/mL in the subsequent 3 months., Results: Intention-to-treat analysis suggested that the remission rate at the end of the sixth month was significantly higher in tacrolimus group than that in cyclophosphamide group (85% versus 65%, P < 0.05). The decrease of proteinuria was significantly greater in tacrolimus group. At the end of the 12th month, the remission rates were comparable between these 2 groups. Patients treated with tacrolimus were more likely to develop glucose intolerance (or diabetes mellitus), infection, and hypertension. No obvious nephrotoxicity of calcineurin inhibitor was found in repeat renal biopsy., Conclusions: Tacrolimus plus corticosteroids is an alternative therapeutic regimen for nephrotic IMN. The short-term efficacy might be better than cyclophosphamide plus prednisone.