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Artículo escrito por un elevado número de autores, solo se referencian el que aparece en primer lugar, el nombre del grupo de colaboración, si le hubiere, y los autores pertenecientes a la UAM, Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations., Hay una corrección posterior a este artículo: http://hdl.handle.net/10486/703679, Background X-linked hypophosphatemia (XLH) is a hereditary rare disease caused by loss-of-function mutations in PHEX gene leading tohypophosphatemia and high renal loss of phosphate. Rickets and growth retardation are the major manifestations of XLH in children, but there is a broad phenotypic variability. Few publications have reported large series of patients. Current data on the clinical spectrum of the disease, the correlation with the underlying gene mutations, and the long-term outcome of patients on conventional treatment are needed, particularly because of the recent availability of new specific medications to treat XLH. Results The RenalTube database was used to retrospectively analyze 48 Spanish patients (15 men) from 39 different families, ranging from 3 months to 8 years and 2 months of age at the time of diagnosis (median age of 2.0 years), and with XLH confirmed by genetic analysis. Bone deformities, radiological signs of active rickets and growth retardation were the most common findings at diagnosis. Mean (± SEM) height was − 1.89 ± 0.19 SDS and 55% (22/40) of patients had height SDS below—2. All cases had hypophosphatemia, serum phosphate being − 2.81 ± 0.11 SDS. Clinical manifestations and severity of the disease were similar in both genders. No genotype—phenotype correlation was found. Conventional treatment did not attenuate growth retardation after a median follow up of 7.42 years (IQR = 11.26; n = 26 patients) and failed to normalize serum concentrations of phosphate. Eleven patients had mild hyperparathyroidism and 8 patients nephrocalcinosis. Conclusions This study shows that growth retardation and rickets were the most prevalent clinical manifestations at diagnosis in a large series of Spanish pediatric patients with XLH confirmed by mutations in the PHEX gene. Traditional treatment with phosphate and vitamin D supplements did not improve height or corrected hypophosphatemia and was associated with a risk of hyperparathyroidism and nephrocalcinosis. The severity of the disease was similar in males and females, This research has been partially funded by Kyowa Kirin Farmacéutica S.L.U., project PI17/01745 from Instituto de Salud Carlos III, Acción Estratégica en Salud 2017-2020 and FEDER funds, Fondo de Investigaciones Sanitarias (FIS), Fundación Nutrición y Crecimiento (FUNDNYC), Instituto de Investigación Sanitaria del Principado de Asturias (ISPA) and Fundación para la Investigación y la Innovación Biosanitaria del Principado de Asturias (FINBA)

Background: Chronic fluid overload is frequent in hemodialysis patients (P) and it associates with hypertension, left ventricular hypertrophy (LVH) and higher mortality. Moreover, echocardiographic data assessing fluid overload is limited. Our aim was to evaluate the relationship between fluid overload measured by bioimpedance spectroscopy (BIS) and different echocardiographic parameters. Methods: Cross-sectional observational study including 76 stable patients. Dry weight was clinically assessed. BIS and echocardiography were performed. Weekly time-averaged fluid overload (TAFO) and relative fluid overload (FO/ECW) were calculated using BIS measurements. Results: Based on TAFO three groups were defined: A- dehydrated, TAFO < -0.25 L 32 P (42%); B- normohydrated, TAFO between -0.25 and 1.5 l: 26 (34%); C- overhydrated, TAFO>1.5 l: 18 (24%). We found significant correlation between TAFO and left atrial volume index (LAVI) (r: 0.29; p = 0.013) but not with FO/ECW (r: 0.06; p = 0.61). TAFO, but not FO/ECW kept a significant relationship with LAVI (p = 0.03) using One-Way ANOVA test and linear regression methods. LVH was present in 73.7% (concentric 63.2%, eccentric in 10.5%). No differences between groups in the presence of LVH or left ventricular mass index were found. Conclusions: We found that left atrial volume index determined by echocardiographic Area-length method, but not left ventricle hypertrophy or dimensions of cavities, are related on hydration status based on bioimpedance measured time-averaged fluid overload (TAFO), and not with FO/ECW.

Background: Mycophenolate (MF) is effective as induction and maintenance treatment in patients with lupus nephritis (LN). This study evaluates the efficacy and safety of MF in patients with refractory and relapsing LN. Methods: Data were retrospectively obtained for 85 patients (35 refractory and 50 relapsing) from 11 nephrology departments in Spain. The primary endpoints were the incidence and cumulative number of renal responses and relapses and their relationship with baseline clinical and analytical data. The secondary endpoint was the appearance of side effects. Results: The main clinical and analytical variables were similar both in refractory and relapsing LN. Most of the patients had received cyclophosphamide, and all of them switched to MF. 74 patients (87%) achieved a response (69% partial, 31% complete). Age at starting MF, gender, pathological classification, body mass index, blood pressure, baseline renal function, and proteinuria were not associated with achieving response. After stopping MF, 3 of 19 patients (15.7%) relapsed, all at 6 months of follow-up. No differences were found between clinical and analytical variables and number of relapses. Side effects were unremarkable, except for 1 patient, who died of thrombocytopenia and ovarian hemorrhage. Conclusions: Switching to MF from other immunosuppressive treatments is effective and safe in refractory and relapsing LN.

Spontaneous remission is a well known characteristic of idiopathic membranous nephropathy, but contemporary studies describing predictors of remission and long-term outcomes are lacking. We conducted a retrospective, multicenter cohort study of 328 patients with nephrotic syndrome resulting from idiopathic membranous nephropathy that initially received conservative therapy. Spontaneous remission occurred in 104 (32%) patients: proteinuria progressively declined after diagnosis until remission of disease at 14.7 ± 11.4 months. Although spontaneous remission was more frequent with lower levels of baseline proteinuria, it also frequently occurred in patients with massive proteinuria: 26% among those with baseline proteinuria 8 to 12 g/24 h and 22% among those with proteinuria >12 g/24 h. Baseline serum creatinine and proteinuria, treatment with angiotensin-converting enzyme inhibitors or angiotensin receptor antagonists, and a >50% decline of proteinuria from baseline during the first year of follow-up were significant independent predictors for spontaneous remission. Only six patients (5.7%) experienced a relapse of nephrotic syndrome. The incidence of death and ESRD were significantly lower among patients with spontaneous remission. In conclusion, spontaneous remission is common among patients with nephrotic syndrome resulting from membranous nephropathy and carries a favorable long-term outcome with a low incidence of relapse. A decrease in proteinuria >50% from baseline during the first year predicts spontaneous remission.

Background: Our aim was to analyze the longitudinal changes in cardiac biomarker levels in hemodialysis patients with high comorbidity treated in our special hospital unit. We hypothesize that strict volume control strategy (salt-restricted diet, extended dialysis sessions and dry weight clinical assessment and reassessment in every session) could prevent progression of left-ventricular damage and, therefore, progressive increment in cardiac biomarker levels over time. Methods: This is a longitudinal cohort study including 46 dialysis patients in which a strategy of strict volume control has been adopted. N-terminal pro-B-type natriuretic peptide (NT-proBNP), troponin T and C-reactive protein (CRP) levels were measured at baseline and prospectively at 4, 8 and 12 months. The possible association between volume control and cardiac biomarker levels was analyzed. Results: Dry weight could be reduced (p < 0.01) over time. A reduction in systolic BP (p < 0.05) and in CRP levels (p < 0.05) was observed, whereas NT-proBNP and troponin T values remained stable. However, patients in the high quartile of NT-proBNP at baseline showed a reduction (p = 0.02) in troponin T over time with no significant trend (p = 0.08) to progressive reduction in NT-proBNP values. Conclusions: Strict volume control in dialysis patients may prevent progressive increment in cardiac biomarker levels over time. The impact seems to be higher among patients with higher levels at baseline in whom strict volume control can even reduce cardiac biomarker levels on follow-up.

Visceral leishmaniasis due to Leishmania Infantum is an endemic parasitic infection in the Mediterranean area. Since 2009, Europe's largest outbreak of Leishmaniasis has been reported in the region of Madrid (Spain). Renal involvement is an unusual complication. Different forms of renal disease have been described: interstitial, glomerular, and vascular damage. Direct invasion of renal parenchyma by the parasite has been described as a mechanism of kidney damage, especially in the immunocompromised. Immune complex deposition and T cells adhesion molecules activation have demonstrated that a pathogenic role in glomerulonephritis related to visceral leishmaniasis. The association between mixed cryoglobulinemia and visceral leishmaniasis has been previously reported in six patients. Renal involvement is only described in one of them. From July 2009 to October 2012, 4 patients with membranoproliferative glomerulonephritis and mixed cryoglobulinemia with negative serology for hepatitis B and C were diagnosed in our hospital. Serology of Leishmania in serum bank samples was performed; it was positive in 3 patients. Leishmania parasite was confirmed by other tests. We present 3 patients with mixed cryoglobulinemia and membranoproliferative glomerulonephritis as first clinical manifestation of visceral leishmaniasis.

Background: The prevalence of inflammation is high among patients with chronic renal failure but the reason of inflammation is unclear. We test the hypothesis that inflammation in chronic renal failure could be the consequence of an increased left-ventricular wall tension related to ventricular dysfunction, hypervolemia or both. Methods: For assessing left-ventricular filling pressure, plasma level of N-terminal pro-B-type natriuretic peptide (N-BNP) was used, as B-type natriuretic peptide is secreted from the cardiac ventricles in response to increased wall tension. N-BNP levels and C-reactive protein (CRP) were measured on the same day in 75 pre-dialysis patients. A previous history of cardiomiopathy with systolic dysfunction was present in 27 (36%) of them. Results: The levels of N-BNP were not normally distributed (mean: 2,589 ± 4,514 pg/ml; median: 789 pg/ml). The distribution of CRP levels was also not normal (mean: 15 ± 27 mg/l; median: 5 mg/l). Both parameters correlated significantly (r: 0.41; p < 0.005). N-BNP was higher (p < 0.001) in patients with known ventricular dysfunction. Excluding these patients, the correlation between N-BNP and CRP was stronger (r: 0.88; p < 0.001). Univariate analysis in these patients without known cardiomyopathy showed that N-BNP levels also correlated with systolic and diastolic blood pressure (r: 0.54; p < 0.005) and inversely with creatinine clearance (r: –0.43; p < 0.01), serum albumin (r: 0.6; p < 0.001) and hemoglobin levels (r: 0.37; p < 0.05). CRP levels correlated significantly (p < 0.01) with the same parameters as N-BNP in univariate analysis. However, in multiple stepwise regression analysis in which CRP was the dependent variable, only the association with N-BNP remained significant (r: 0.87; p < 0.001). Conclusions: Our results suggest a link between left-ventricular filling pressure and inflammation in patients with advanced renal insufficiency. The importance of strict volume control in these patients, in order to reduce left-ventricular pressure and therefore inflammation, should be considered.

Background/Aims: Lower serum sodium levels have been associated with increased mortality among patients with chronic kidney disease (CKD). Our aim was to analyze the independent factors associated with lower sodium levels among nondialysis patients with advanced CKD and to evaluate the evolution of these patients in comparison to those with higher plasma sodium over a 1-year period. Methods: We included 72 patients with CKD stages 4 and 5 without clinically evident cardiopathy or liver disease. Bioelectrical impedance and echocardiography were performed to analyze the possible relation between plasma sodium and volume status and subclinical left ventricular (LV) dysfunction. During follow-up, we compared the evolution of patients with lower baseline plasma sodium (low quartile: Results: At baseline, the independent predictors of lower plasma sodium were C-reactive protein (CRP; OR 0.96; 95% CI 0.91-0.99) and body mass index (OR 0.89; 95% CI 0.78-0.99). An inverse correlation between plasma sodium and CRP was observed (r = -0.32; p = 0.01). Plasma sodium did not correlate with extracellular water and was not different between patients with or without echocardiographic data of LV dysfunction (p = 0.7). During follow-up, patients with lower sodium at baseline showed persistently lower sodium values (p = 0.04), higher CRP (p = 0.05), lower serum albumin (p < 0.01) and higher erythropoietin-stimulating agent resistance index (p = 0.05). Conclusions: Our results suggest an association between lower plasma sodium and a microinflammatory state among patients with advanced CKD. Inflammation could be an underlying confounding factor explaining the increased mortality in these patients.

Background Although tacrolimus is recommended by KDIGO Clinical Practice Guideline for Glomerulonephritis for the treatment of idiopathic membranous nephropathy (MN), little is known about factors that influence response and relapse of the disease after tacrolimus therapy. Methods Multicentre study that collected 122 MN patients with nephrotic syndrome and stable renal function treated with tacrolimus. Duration of treatment was 17.6 ± 7.2 months, including a full-dose and a tapering period. Results The percentage of remission was 60, 78 and 84% after 6, 12 and 18 months of treatment, respectively. The amount of proteinuria at baseline significantly predicted remission, the lower the baseline proteinuria the higher the probability of remission. Only 10 patients (8%) received concomitantly corticosteroids, and their rate of remission was similar (80% at 18 months). Among responders, 42% achieved complete remission (CR) and 58% partial remission (PR). Almost half (44%) of the responder patients relapsed. The amount of proteinuria at the onset of tacrolimus tapering was significantly higher in relapsing patients. By multivariable analysis, the presence of a PR versus CR at the onset of tacrolimus tapering and a shorter duration of the tapering period significantly predicted relapses. Tolerance was good and the number of adverse events low. Conclusions Tacrolimus monotherapy is an effective and safe option for the treatment of MN with stable renal function. Relapses are frequent in patients with PR and can be partially prevented by a longer tapering period.

Background/Aim: Sodium and water retention is common in peritoneal dialysis patients and contributes to cardiovascular disease. As peritoneal sodium removal depends partly on dwell time, and automated peritoneal dialysis (APD) often uses short dwell time exchanges, the aim of this study was to compare the 24-hour peritoneal sodium removal in APD and standard continuous ambulatory peritoneal dialysis (CAPD) patients and to analyze its possible influence on blood pressure control. Methods: A total of 53 sodium balance studies (30 in APD and 23 in CAPD) were performed in 36 stable peritoneal dialysis patients. The 24-hour net removal of sodium was calculated as follows: M = ViCi – VdCd, where Vd is the 24-hour drained volume, Cd is the solute sodium concentration in Vd, Vi is the amount of solution used during a 24-hour period, and Ci is the sodium concentration in Vi. Peritoneal sodium removal was compared between APD and CAPD patients. Residual renal function, serum sodium concentration, daily urinary sodium losses, weekly peritoneal Kt/V and creatinine clearance, 4-hour dialysate/plasma creatinine ratio, proportion of hypertonic solutions, net ultrafiltration, systolic and diastolic blood pressures, and need for antihypertensive therapy were also compared between the groups. Results: Peritoneal sodium removal was higher (p < 0.001) in CAPD than in APD patients. There were no significant differences in residual renal function, serum sodium concentration, urinary sodium losses, peritoneal urea or creatinine clearances, 4-hour dialysate/plasma creatinine ratio, or proportion of hypertonic solutions between groups. The net ultrafiltration was higher in CAPD patients and correlated strongly (r = 0.82; p < 0.001) with peritoneal sodium removal. In APD patients, peritoneal sodium removal increased significantly only in those patients with a second daytime exchange. The systolic blood pressure was higher (p < 0.05) in APD patients, and the proportion of patients with antihypertensive therapy was also higher in APD patients, although no significant relationship between blood pressure values and amount of peritoneal sodium removal was found. Conclusions: The 24-hour sodium removal is higher in CAPD than in APD patients, and there is a trend towards better hypertension control in CAPD patients. As hypertension control and volume status are important indices of peritoneal dialysis adequacy, our results have to be considered in the choice of the peritoneal dialysis modality.

Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) is an autosomal recessive tubular disease caused by mutations in the CLDN16 or CLDN19 gene. Previous studies using microsatellite markers flanking the CLDN19 locus estimated that p.G20D (c.59G>A), a recurrent mutation in Spanish families, is a founder mutation. In the present study, we assessed the haplotype of Spanish patients using single nucleotide polymorphisms (SNPs). Twenty-seven FHHNC patients were included in this study. We analyzed four SNPs located in CLDN19 introns 3 and 4 by polymerase chain reaction amplification and DNA sequencing. Three new patients with homozygous p.G20D were identified. The SNP genotyping analysis showed that alleles carrying this mutation shared a common SNP haplotype. Our findings suggest the existence of a founder effect responsible for FHHNC in our cohort. Testing for the presence of mutation p.G20D should be the first genetic screening in Spanish patients.

In patients older than 75 years with advanced chronic kidney disease (CKD), the decision between treatment with dialysis [intention to treat with dialysis (ITD)] or conservative care (CC) is a challenge. Geriatric assessment can be helpful. The aim was to identify which factors had had an influence on decision-making. Methods: We recruited 56 patients. At baseline we analyzed age, frailty (defined following the criteria of Fried et al. [J Gerontol A Biol Sci Med Sci 2001;56:146-156]), dependence for activities of daily living (ADL), cognitive impairment, depression, comorbidity, cardiovascular disease, and diabetes. After full information about prognosis and treatment options, the preferences of the patients and families were taken into consideration as determinants in the decision-making process. During the follow-up, we evaluated clinical and laboratory parameters, hospitalization, mortality and reevaluated frailty. Results: Twenty patients opted for CC, and 36 patients opted for ITD. On univariate analysis, the predictive factors of the election of CC were age, prefrailty, cognitive impairment, and dependence for ADL. In the multivariate analysis, age and prefrailty remained as predictors for the choice of CC. Hospitalizations were more frequent in CC. Survival was similar in both groups (p = 0.098). Conclusions: Frailty assessment could be useful for decision-making about the treatment in elderly patients with CKD. CC may be a good treatment option.

Overweight and obesity are associated to a higher cardiovascular risk and mortality in the general population and conflicting findings exist in the dialysis population. Adipokines (Leptin, adiponectin) produced in adipocytes may play a role in that process, and inflammatory parameters (CRP, IL-6) may be markers for it. Nevertheless, obtaining dry weight is today one of the main aims of adequacy in dialysis because overhydration is a clear mortality predictor.The aim of this study was to analyse body composition using an impedance spectroscopy technique in patients on haemodialysis (HD): and evaluate overweight and overhydration prevalence and its possible relation with adipokines, inflammatory and nutritional parameters, HD technique (Conventional [CHD], on-line haemodiafiltration [OL-HFD]) and erythropoietin needs.In a cross-sectional observational study, a pre-HD multifrequency bioimpedance spectroscopy (BIS) was performed in the middle of the week on 77 HD outpatients: 56 CHD and 21 OL-HFD. Patients were considered overweight when Body Mass Index (BMI) was ≥25kg/m² and overhydrated when overhydration normalised for extracellular water was higher than 0.15l. Clinical and biochemical parameters were analysed and IL-6, leptin and adiponectin levels were determined. This information was analysed in overweight and non-overweight, regular and overhydrated patients and both HD techniques.50% of patients fulfilled overweight criteria and 21% were pre-HD overhydrated. Overweight patients had a superior fat and extracellular water content (P.001). Leptin (P=.001) and CRP (P=.036) levels were higher and adiponectin levels were lower (P=.003). An inverse correlation did exist between BMI and lean mass (P=.01). Nutritional markers (prealbumin, albumin, total proteins, creatinine and transferrin) were related to lean mass (P=.05). Comparing both HD techniques, a lower fat content was observed in OL-HFD (P=.049) without overhydration differences. In the univariate analysis, age, fat, extracellular/intracellular water ratio, leptin, hipoadiponectinaemia, lower lean mass and CHD technique were predictors of overweight. In the multivariate analysis, hipoadiponectinaemia (OR: 0.86; IC: 0.76-0.98), lean mass (OR: 0.89; IC: 0.84-0.94) and OL-HFD technique (OR: 0.200; IC: 0.04-0.99) predicted the absence of overweight.This observational study emphasises the high prevalence of overweight in the outpatient haemodialysis population, as long as overweight is related with fat and extracellular water. Furthermore, it is accompanied by higher inflammation and leptin levels and lower levels of adiponectin. The use of the OL-HFD technique is associated to less overweight and fat content. Bioimpedance may prove to be a valuable ally for decisions regarding weight changes in dialysis patients.

Background: Mycophenolate (MF) is effective as induction therapy for lupus nephritis (LN) in patients with normal renal function; however, little is known about its role in patients with impaired renal failure. The purpose of this study was to evaluate the response to MF in LN and its association with baseline renal function. Methods: Data were obtained for 90 patients from 12 Spanish renal units who were receiving MF as induction therapy for LN. Patients were classified into 2 groups: group 1 (estimated glomerular filtration rate [eGFR] ≥60 ml/min/1.73 m2) and group 2 (eGFR 2). The primary outcome measure was the percentage of patients who achieved any response and its relationship with initial eGFR. The secondary outcome measures were the percentage of patients who achieved a complete response (CR) or partial response (PR) and the appearance of relapses during treatment and side effects. Results: At initiation of MF treatment, there were no differences in the main parameters between group 1 (n = 63; eGFR 87 ± 23 ml/min/ 1.73 m2) and group 2 (n = 27; eGFR 44 ± 12 ml/min/1.73 m2). Exposure to prednisone and MF was similar. The percentages of patients who achieved a response in groups 1 and 2 were, respectively, 69.2 and 43.8% at 6 months and 81.3 and 73.7% at 12 months. CR was more frequent in group 1, whereas PR was similar in both groups. Four patients relapsed and side effects were unremarkable. Conclusions: MF is effective and safe as induction therapy for LN, and response is even achieved in patients with baseline renal impairment.

We evaluated the use of telemedicine in the long-term control of stable patients undergoing peritoneal dialysis at home. From September 2003 to August 2005, patients were randomly selected from current cases and invited to join study group A, in which they had telemedicine support. Patients not selected for this group, or who refused the invitation, were placed in study group B, and used for comparison. There were 25 patients in group A and 32 patients in group B. Videoconferencing equipment was installed in each patient's home, connected to a videoconferencing unit at the hospital by three ISDN lines. Patients in group A were followed for a mean of 8 months (range 3–24) with alternate months of teleconsultations and hospital visits. A total of 172 teleconsultations were conducted. A mean of 22 min (SD 9) were spent on each teleconsultation, significantly less than in hospital consultations, which took a mean of 33 min (SD 8) ( P

Although hypoalbuminemia is a fundamental characteristic of nephrotic syndrome (NS), there are many patients with massive proteinuria that do not develop hypoalbuminemia. We have studied the clinical and biochemical characteristics of 19 patients with persistent massive proteinuria (greater than 5 g/d) and normal serum albumin (group I) in comparison with 16 patients with similar proteinuria excretion, but persistent hypoalbuminemia (group II). Most of group I patients had diagnoses suggesting glomerular hyperfiltration (focal glomerulosclerosis [FGS] associated with vesicoureteral reflux [VUR], reduction of renal mass, proteinuria associated with obesity, sclerotic phase of idiopathic crescentic glomerulonephritis [GN] in contrast with those of group II, in which membranous GN was the most frequent diagnosis. We prospectively investigated differences in the antiproteinuric effect of captopril, an antiotensin-converting enzyme inhibitor (ACEI); after 6 months of treatment, proteinuria decreased clearly in group I (7.1 +/- 1.7 to 3.7 +/- 1.7 g/d; P less than 0.001), whereas no significant changes were observed in group II (8.1 +/- 2.4 to 8.8 +/- 4 g/d). Serum creatinine (Scr) remained stable during captopril treatment in group I, whereas three patients in group II showed a worsening of renal function.(ABSTRACT TRUNCATED AT 250 WORDS)

In order to elucidate whether or not the natriuretic properties of calcium entry blockers persist during the long-term administration of the drug, we have investigated the renal capacity to excrete an intravenous sodium load (2,000 ml of isotonic saline in 4 h) in a group of eight mild to moderate essential hypertensive patients, before and after 1, 8, and 24 weeks of treatment with nitrendipine. The following parameters were measured before and hourly during saline infusion: blood pressure, and plasma and urine sodium and potassium. For 5 days prior to the performance of the test, the patients received a diet containing 120 mEq of sodium daily. Nitrendipine induced a significant fall of both systolic and diastolic blood pressure (p less than 0.05-0.01) that was maintained throughout the study. A significant increase of the cumulative renal sodium output (microEq/min/h) was observed after 1, 8, and 24 weeks (p less than 0.05-0.001) of therapy with the calcium entry blocker. Meanwhile, plasma sodium and potassium and the kaliuresis did not change significantly. These results indicate that the natriuretic effect of calcium entry blockers is still present after long-term treatment.

We studied the long-term effect of an angiotensin converting enzyme (ACE) inhibitor, captopril, on the progression of chronic renal failure and on the rate of urinary protein excretion. When compared with standard triple therapy, captopril slowed the progression of renal failure. Captopril was also able to reduce the proteinuria of non-diabetic glomerular origin. This reduction was not dependent on the presence or absence of arterial hypertension but was limited by the presence of low serum albumin levels, and only occurred in patients with proteinuria in excess of 3 g/24 h.

• Three episodes of acute renal failure, all of them requiring hemodialysis, were observed in two patients. The renal biopsy specimens showed a massive periglomerular granulomatosis with crescentic glomerulonephritis. With cyclophosphamide therapy, a dramatic improvement in renal function was observed in both patients. Although the histologic findings and the response to cyclophosphamide therapy were characteristic of Wegener's granulomatosis, there was no evidence of respiratory involvement or extrarenal manifestations throughout the patients' clinical course. We think that these cases could represent limited forms of Wegener's granulomatosis with exclusive renal involvement. (Arch Intern Med1988;148:1377-1379)