Los cinco tipos más importantes de glomerulonefritis primarias son las lesiones mínimas, la glomeruloesclerosis focal y segmentaria, la nefropatía membranosa, la nefropatía IgA y la glomerulonefritis membranoproliferativa. Las tres primeras se presentan típicamente con un síndrome nefrótico o proteinuria importante: su pronóstico depende de la remisión de la proteinuria, más fácil de conseguir en las lesiones mínimas que en la glomeruloesclerosis o en la membranosa. La nefropatía IgA presenta hematuria macro o microscópica de manera prácticamente constante y proteinuria de rango variable. Su pronóstico depende fundamentalmente de la cuantía de la proteinuria. La membranoproliferativa es muy variable en sus manifestaciones clínicas y su pronóstico guarda relación con la gravedad de estas. El tratamiento con esteroides es eficaz en las lesiones mínimas y en algunos casos de glomeruloesclerosis. En la membranosa, IgA y membranoproliferativa, los inmunosupresores están indicados, en diferentes tipos y combinaciones, siempre que el tratamiento conservador no controle la enfermedad.

There is no a clear guidelines concerning with the treatment of post-transplant membranous glomeulonephritis (MGN). We have analyzed our renal transplant patients with renal biopsy diagnosis of MGN and its evolution in relation with the treatment received. From January 1980 to December 2010, 25 patients were diagnosed of MGN: four of them were eliminated for this analysis after received Ciclophosphamide or were lost for follw-up and the remanining 21 were included in our study (33.3% MGN recurrence, 47.6% MGN de novo, and the rest undetermined):66.7% men, age 51.3 ± 13.3 years, 95.2% first kidney transplant and 42.8% with hepatitis C antibodies and RNA+ Patients were divided according to whether they received treatment with steroids: Group 1 (7p) received no steroid treatment, Group 2 (14p) were treated with steroids, 6p with oral steroids (0.7 mg / kg / day for an average of 20 days) and 8p received pulses of steroids (mean dose 1.25 g). They were followed during 12 months. Concerning to the baseline characteristics, higher serum creatinine and proteinuria in patients of group 2 was observed. Patients treated with steroids achieved a significantly higher rate of complete remission (CR) or partial remission (PR) compared to untreated patients, 85.7 vs 28.6%, p 0.009. Renal function in patients treated with steroids was also significantly better 1.8 ± 0.9 vs 3.2 ± 2 mg / dl, p 0.04 at the end of 12 months. 11 of the 25 patients were HCV virus-positive, 9 of them were included in our study; 66.6% (6p) received oral or intravenous steroid treatment, four patients achieved a complete remission and the remaining two patients did not response. During the follow-up none of these patients showed acute liver disease. In Conclusion: Our data suggest that treatment with high doses of steroids in these patients may be effective in increasing the percentage of remission and improving graft survival. In patients HCV virus-positive, the treatment may be beneficial too and there was no episode of acute liver disease. Diccionario

BACKGROUND:Several cases of obesity-associated focal segmental glomerulosclerosis (OB-FSG) have been reported but little is known about the clinico-pathological features of this entity and its long-term outcomes.METHODS:We studied 15 obese patients (BMI 35+/-5.2 kg/m(2)) with biopsy-proven FSG. They were compared with a control group of 15 non-obese patients with idiopathic FSG (I-FSG).RESULTS:Mean proteinuria at the time of renal biopsy was 3.1+/-2 g/24 h in OB-FSG; it reached the nephrotic range (> or =3.5 g/24 h) during follow-up in 12 patients (80%), but none of them had oedema, hypoproteinaemia, or hypoalbuminaemia. Proteinuria was more marked amongst I-FSG (6.5+/-4.2 g/24 h) and most of them developed oedema and biochemical nephrotic syndrome. Glomerulomegaly was observed in all renal biopsies from OB-FSG patients (mean glomerular diameter 256+/-24 microm in OB-FSG vs 199+/-26 microm in I-FSG, P<0.001). Twelve OB-FSG patients (80%) were treated with ACE inhibitors (ACEI) and proteinuria significantly decreased within the first 6 months of treatment but showed a later increase. None of the obese patients achieved a sustained weight loss. Seven (46%) patients with OB-FSG experienced a progressive renal insufficiency and five of them started intermittent dialysis. Kaplan-Meier estimated probabilities of renal survival after 5 and 10 years were 77 and 51%, respectively, in OB-FSG patients, and 52 and 30% in I-FSG (P<0.05). The risk of developing progressive renal failure among OB-FSG patients was statistically correlated with serum creatinine and creatinine clearance at presentation.CONCLUSIONS:OB-FSG indicates a poor prognosis with almost one-half of patients developing advanced renal failure. Knowledge of the clinico-pathological features of this entity (obesity, FSG lesions with glomerulomegaly, absence of nephrotic syndrome despite nephrotic-range proteinuria) should be helpful in establishing an accurate and early diagnosis.

BACKGROUND The age limit of the cadaver kidney donors is increasing in response to the growing demand for renal transplantation. Simultaneous double kidney transplantation (SDKT) with kidneys obtained from elderly adults has been proposed to increase the transplantation number and improve its results. However, if SDKT is performed when there are no clear indications, a negative effect could be produced on the total number of transplanted patients as both kidneys would be used for only one recipient. MATERIAL AND METHODS In December 1996 we designed a transplantation protocol to be able to extend the selection of cadaver kidney donors with normal serum creatinine levels without establishing any age limit. A pregraft renal biopsy was always performed to analyze the glomerulosclerosis (GE) percentage whenever the donors were 60 years of age or older. A SDKT was performed in a single recipient when the donor age was 75 years or older or when the donors between 60 and 74 years old had a GE rate of more than 15%. On the contrary, a single kidney transplantation was performed in two different recipients for kidneys from donors between 60 and 74 years of age with a GE rate of less than 15%. Kidneys having GE rates of more than 50% were discarded for transplantation. Donor kidneys from subjects younger than 60 years of age were always used for a single kidney transplantation. RESULTS Based on the above mentioned protocol, from December 1996 to May 1998, 181 patients received a kidney transplantation in our hospital. These patients were divided into three groups: group I which included the SDKT recipients (n=21), group II or single kidney recipients from 60- to 74-year-old donors (n=40), and group III or recipients from <60-year-old donors (n=120). The mean follow-up time was 15+/-5 months (range 6-24). Mean donor age was 75+/-7 years in group I, this was significantly higher than in group II (67+/-4, P<0.001) and group III (37+/-15, P<0.001). The primary nonfunction rate was low in the three groups, there being no statistically significant differences (5, 5, and 4%, respectively). A significantly greater percentage of patients from group I (76%) presented immediate renal graft function as compared with group II (43%, P<0.01) and III (50%, P<0.05). The acute rejections rate was very low in all three groups (9.5, 7.5, and 22%, respectively) with significant differences between groups II and III (P<0.05). No significant differences between the different groups were observed for one year actuarial patient survival (100, 95, and 98%, respectively) or graft survival rates (95, 90, and 93%, respectively). The 6-month serum creatinine levels were excellent in the three groups, although there were significant differences between groups I and II (1.6+/-0.3 vs. 1.9+/-0.6 mg/dl, P<0.05), II and III (1.9+/-0.6 vs. 1.4+/-0.4 mg/dl, P<0.001), and I and III (P<0.05). CONCLUSIONS Simultaneous double kidney transplantations make it possible to use kidneys from extremely elderly donors (>75 years) or those whose GE>15%. In addition, kidneys from donor 60-74 years old in which the GE<15% can be used for single kidney transplantations in two different recipients with excellent results.

We observed that some patients do not develop hypoalbuminemia despite the presence of massive proteinuria. To investigate whether the absence or presence of hypoalbuminemia could be a marker in the distinction between idiopathic focal segmental glomerulosclerosis (FSG) and FSG secondary to hyperfiltration, we reviewed all our patients with biopsy-proven FSG and persistent nephrotic-range proteinuria (>3.5 g/24 h). Patients who met these conditions were then separated into those with hypoalbuminemia (serum albumin level <3 g/dL; group I; n = 19) and those with normoalbuminemia (>3.5 g/24 h; group II; n = 18). All group I patients had nephrotic edema in contrast with the absence of edema in all group II patients. Serum cholesterol and triglyceride levels were significantly greater in group I. All group I patients had been diagnosed with idiopathic FSG. The diagnoses of group II patients were FSG secondary to massive obesity in eight patients (44%), vesicoureteral reflux in five patients (27%), and renal mass reduction in three patients (16%); only two patients (11%) in this group had idiopathic FSG. The case histories of 19 other patients with nephrotic-range proteinuria associated with hyperfiltering disorders (reflux nephropathy, massive obesity, renal mass reduction), but without renal biopsy, were also reviewed; despite massive proteinuria (5.8 +/- 3.1 g/24 h), serum albumin and total protein levels were always within normal values. In conclusion, patients with FSG secondary to hyperfiltration do not develop hypoalbuminemia or the other characteristic complications of nephrotic syndrome, despite the presence of massive proteinuria values.

Background. Familial persistent microhematuria with normal renal function is the most common presentation of thin basement membrane nephropathy (TBMN). Gross hematuria episodes and loin pain attacks are other manifestations of the disease. On the other hand, it has been shown that hypercalciuria (HC) and hyperuricosuria (HU) can produce both gross or microscopic non-glomerular hematuria, in addition to their role in renal stone formation.Methods. We studied the prevalence of HC, HU and nephrolithiasis in a group of 27 biopsy-proven TBMN as well as in 19 non-biopsied first-degree relatives with persistent microhematuria and 25 first-degree relatives without microhematuria. A group of 27 patients with IgA nephropathy (IgAN) and persistent microhematuria, and another group of 20 healthy subjects without known renal diseases were selected as control groups.Results. Ten (37%) patients with TBMN and 8 (42%) relatives with microhematuria showed HC and/or HU at presentation; relatives without microhematuria, IgAN patients and normal controls showed a significantly lower prevalence of HC and HU. The prevalence of previous nephrolithiasis among TBMN patients (25%) was significantly higher than in IgAN patients (3%; P < 0.05). Family history of nephrolithiasis was recorded in 14 (51%) of the 27 TBMN families, in contrast with 2 of 27 (7%) with IgAN and 1 of 20 (5%) in normal controls (P < 0.05). The prevalence of nephrolithiasis, gross hematuria bouts and loin pain episodes among TBMN patients and microhematuric relatives showing HC and/or HU at presentation (44%, 44% and 27%, respectively) were significantly higher than those of TBMN patients and microhematuric relatives with normal calcium and uric acid urinary excretions (10%, 7% and 3%, respectively; P < 0.05). At the end of follow-up (8.8 +/- 4.1 years in TBMN patients and 9.1 +/- 4.2 years in relatives with microhematuria), all the cases maintained normal renal function.Conclusions. We found a high prevalence of HC, HU, and nephrolithiasis among TBMN patients and relatives with microhematuria. Our study also shows a significant relationship between the presence of HC and/or HU and the prevalence of nephrolithiasis, gross hematuria bouts and loin pain episodes.

AimsWe report a case study to elucidate the pathogenesis of polyarteritis nodosa (PAN) type vasculitis, a rare complication of familial mediterranean fever (FMF).Methods and resultsA woman with amyloidosis complicating FMF underwent a cadaveric renal transplantation and 5 years later suffered an acute myocardial infarction secondary to an isolated coronary vasculitis.ConclusionsThe histopathological findings of the vasculitis were not in keeping with PAN. We postulated that the pathogenesis of vasculitis in FMF is different from that of the classic PAN and might be similar to the mechanism of the serosal inflammation.

Background. Hepatitis C virus (HCV) infection has been described in association with various types of glomerular diseases, usually type I membranoproliferative glomerulonephritis and rarely membranous glomerulonephritis (MGN). In this article, we describe the first series of MGN exhibited in renal transplant patients and associated with HCV infection. Methods. From January 1980 to December 1994, 2045 kidney transplantations were performed in our renal transplant units. A retrospective analysis demonstrated an overall 20% prevalence of HCV virus-positive patients; 409 transplanted patients were HCV positive (ELISA and RIBA). Results. Fifteen patients developed an allograft MGN (3.66%) 24 months after renal transplantation. MGN appeared in the form of significant proteinuria (>1.5 g/24 h) with stable renal function. In all cases, graft biopsy demonstrated a thickening of the capillary wall, subepithelial electron-dense deposits, and IgG and C3 diffuse granular deposits along the basal membrane. Ten cases were considered de novo, two cases were considered recurrent MGN, and three cases were considered undetermined because the primary renal disease was chronic glomerulonephritis. All patients showed negative antinuclear antibodies and cryoglobulins, normal complement, and negative rheumatoid factors. During follow-up (an average of 2 years), 12 patients developed a progressive worsening of renal function, with increased serum creatinine and persistent proteinuria; 8 of the 12 patients returned to dialysis. Of the remaining three cases, two patients showed partial remission of nephrotic syndrome after high doses of steroids, and one patient persisted with stable renal function and proteinuria (<2 g/24 h.). Conclusions. In summary, HCV is preferentially associated with MGN in renal transplant patients, rather than with membranoproliferative glomerulonephritis as in the normal adult population. MGN associated with HCV infection has a similar clinical picture and outcome to posttransplant idiopathic de novo MGN, with persistent massive proteinuria and progressive deterioration of renal function.

The first well-documented case of recurrent type III membranoproliferative glomerulonephritis after kidney transplantation is reported in this article. A 48-year-old man was admitted to the hospital because of nephrotic syndrome and moderate renal failure. The renal biopsy showed double-contour images at light microscopy. Electron microscopy revealed electrondense deposits in the mesangium and in both the subepithelial and subendothelial sides of the basement membrane. Subepithelial deposits were sometimes hump-like and produced an irregular disruption of the lamina densa. A diagnosis of type III membranoproliferative glomerulonephritis was suggested. The patient had a rapid decrease in renal function and received dialysis in 3 months. Three years later, he received a cadaveric kidney transplant, and subsequently recovered normal renal function. Proteinuria appeared after 13 months, and a biopsy of the graft demonstrated recurrence of the original disease. Seven years after transplantation, he returned to hemodialysis.

We evaluated the pathogenic role of Corynebacterium urealyticum in the development of encrusted pyelitis (EP) and encrusted cystitis (EC), and their clinical consequences in renal transplant recipients. During a 4-year period, we studied seven renal transplant recipients with EP and two with EC. The records of 320 other renal transplant patients studied during the same period were used as a control group. C urealyticum (> or = 10(5) CFU/ml) was isolated from 4 patients with EP (urine 3, blood 1) and from 1 patient with EC (urine). Alkaline urines with struvite crystals, microscopic hematuria, and sterile conventional urine cultures were present in all our cases. All the patients with EP developed obstructive uropathy with deterioration of the renal function and pyelonephritis (4 patients) or renal abscesses (3 patients). Chronic urinary discomfort and macroscopic hematuria were present in the 2 patients with EC. Long-term vesical and ureteral catheterization were considered the most important risk factors for the development of EC and EP, respectively. Vancomycin was successfully used in 5 cases, but all the patients required a derivative procedure or a surgical resection of the incrustations to improve. We conclude that EP and EC should be investigated in renal transplant patients who develop pyelonephritis, obstructive uropathy, or chronic urinary symptoms. EP and EC could lead to the loss of their grafts. C urealyticum appears to have a pathogenic role in these entities.

Several cases of acute renal failure associated with episodes of macroscopic hematuria (EMH) in IgA nephropathy (IgAN) have been reported; in every case renal function returned to normal. We report 2 cases with previous normal renal function that developed acute renal failure during EMH of 17 and 30 days duration, respectively. Renal biopsies showed mesangial proliferation with IgA deposits, crescents in 10-15% of glomeruli and a marked tubular necrosis with red blood cell casts obstructing the lumen in 30-45% of tubules. Although renal function improved after EMH cessation, it did not recover previous normal values. Thus, EMH can produce permanent loss of renal function in patients with IgAN.

We report a 35-year-old man with adult polycystic kidney disease (APKD) that developed nephroptic syndrome accompanied by a rapid worsening of renal function. Histologic examination showed marked tubulointerstitial chronic abnormalities and focal glomerulosclerosis (FGS) lesions in 24% of the glomeruli. With captopril, an renal function continued to deteriorate. No other cases of nephrotic-range proteinuria were detected among 65 APKD patients with renal insufficiency. Histologic examination of an other 12 kidneys removed from patients with APKD showed striking interstitial lesions, most of the glomeruli being normal. However, those patients with higher amounts of proteinuria had more glomeruli (14-32%) with FGS lesions.

We analyzed the clinical course of 120 patients who were diagnosed as having primary hypertension and subsequently given standard stepped-care therapy (diuretic, beta-blocker and vasodilator) for 9 years. At the end of the follow-up period, 21 patients (17.5%) had developed overt proteinuria. The initial study showed no difference in systolic blood pressure, age, sex, serum creatinine and its clearance, glucose, cholesterol and triglycerides between these patients and those who had not become proteinuric, but uric acid levels and diastolic blood pressure were higher (both P less than 0.01). An adequate control of blood pressure was obtained and maintained in all patients, who had similar therapeutic needs. During the follow-up period, uric acid levels remained significantly elevated (P less than 0.01) in the proteinuric patients, while changes in serum glucose, cholesterol and triglycerides were similar in all patients. These results indicate that long-term treatment of primary hypertensives does not fully protect kidney function and that initially elevated uric acid levels could be a predictor of a poor prognosis.

We present two sisters with nephronophthisis and pigmentary retinopathy (Senior-Loken syndrome) and associated liver fibrosis. Clinical and histological findings are discussed, as well as the importance of family studies. A comparative analysis with previous published cases is made; we found only three other references with this triad. Our report underlines the need to investigate liver disorders in all patients with nephronophthisis and the existence of liver fibrosis as an element of the hereditary 'nephronophthisis complex.'