Therapeutic variability in adult minimal change disease and focal segmental glomerulosclerosis.

BACKGROUND: Variability in the management of glomerulonephritis may negatively impact efficacy and safety. However, there are little/no data on actual variability in the treatment of minimal change disease (MCD)/focal segmental glomerulosclerosis (FSGS) in adults. We assessed Spanish practice patterns for the management of adult nephrotic syndrome due to MCD or FSGS. The absence of reasonably good evidence on treatment for a disease often increases the variability substantially. Identification of evidence-practice gaps is the first necessary step in the knowledge-to-action cyclical process. We aim to analyse the real clinical practice in adults in hospitals in Spain and compare this with the recently released Kidney Disease: Improving Global Outcomes clinical practice guideline for glomerulonephritis. METHODS: Participating centres were required to include all adult patients (age >18 years) with a biopsy-proven diagnosis of MCD or FSGS from 2007 to 2011. Exclusion criteria included the diagnosis of secondary nephropathy. RESULTS: We studied 119 Caucasian patients with biopsy-proven MCD (n = 71) or FSGS (n = 48) from 13 Spanish hospitals. Of these patients, 102 received immunosuppressive treatment and 17 conservative treatment. The initial treatment was steroids, except in one patient in which mycophenolate mofetil was used. In all patients, the steroids were given as a single daily dose. The mean duration of steroid treatment at initial high doses was 8.7 ± 13.2 weeks and the mean global duration was 38 ± 32 weeks. The duration of initial high-dose steroids was <4 weeks in 41% of patients and >16 weeks in 10.5% of patients. We did find a weak and negative correlation between the duration of whole steroid treatment in the first episode and the number of the later relapses (r = -0.24, P = 0.023). There were 98 relapses and they were more frequent in MCD than in FSGs patients (2.10 ± 1.6 versus 1.56 ± 1.2; P = 0.09). The chosen treatment was mainly steroids (95%). Only seven relapses were treated with another drug as a first-line treatment: two relapses were treated with mycophenolate and five relapses were treated with anticalcineurinics. A second-line treatment was needed in 29 patients (24.4%), and the most frequent drugs were the calcineurin inhibitors (55%), followed by mycophenolate mofetil (31%). Although cyclophosphamide is the recommended treatment, it was used in only 14% of the patients. CONCLUSIONS: We found variation from the guidelines in the duration of initial and tapered steroid therapy, in the medical criteria for classifying a steroid-resistant condition and in the chosen treatment for the second-line treatment. All nephrologists started with a daily dose of steroids as the first-line treatment. The most frequently used steroid-sparing drug was calcineurin inhibitors. Cyclophosphamide use was much lower than expected.

Palliative peritoneal dialysis: Implementation of a home care programme for terminal patients treated with peritoneal dialysis (PD).

Terminal-stage patients on peritoneal dialysis (PD) are often transferred to haemodialysis as they are unable to perform the dialysis technique themselves since their functional capacities are reduced. We present our experience with five patients on PD with a shortterm life-threatening condition, whose treatment was shared by primary care units and who were treated with a PD modality adapted to their circumstances, which we call Palliative Peritoneal Dialysis.

Consensus document on the management of renal disease in HIV-infected patients.

OBJECTIVE: To update the 2010 recommendations on the evaluation and management of renal disease in HIV-infected patients. METHODS: This document was approved by a panel of experts from the AIDS Working Group (GESIDA) of the Spanish Society of Infectious Diseases and Clinical Microbiology (SEIMC), the Spanish Society of Nephrology (S.E.N.), and the Spanish Society of Clinical Chemistry and Molecular Pathology (SEQC). The quality of evidence and the level of recommendation were evaluated using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. RESULTS: The basic renal work-up should include measurements of serum creatinine, estimated glomerular filtration rate by CKD-EPI, Urine protein-to-creatinine ratio, and urinary sediment. Tubular function tests should include determination of serum phosphate levels and urine dipstick for glucosuria. In the absence of abnormal values, renal screening should be performed annually. In patients treated with tenofovir or with risk factors for chronic kidney disease (CKD), more frequent renal screening is recommended. In order to prevent disease progression, potentially nephrotoxic antiretroviral drugs are not recommended in patients with CKD or risk factors for CKD. The document advises on the optimal time for referral of a patient to the nephrologist and provides indications for renal biopsy. The indications for and evaluation and management of dialysis and renal transplantation are also addressed. CONCLUSIONS: Renal function should be monitored in all HIV-infected patients. The information provided in this document should enable clinicians to optimize the evaluation and management of HIV-infected patients with renal disease.

Executive summary of the consensus document on the management of renal disease in HIV-infected patients.

The aim of this article is to update the 2010 recommendations on the evaluation and management of renal disease in HIV-infected patients. Renal function should be monitored in all HIV-infected patients. The basic renal work-up should include measurements of serum creatinine, estimated glomerular filtration rate by CKD-EPI, urine protein-to-creatinine ratio, and urinary sediment. Tubular function tests should include determination of serum phosphate levels and urine dipstick for glucosuria. In the absence of abnormal values, renal screening should be performed annually. In patients treated with tenofovir or with risk factors for chronic kidney disease (CKD), more frequent renal screening is recommended. In order to prevent disease progression, potentially nephrotoxic antiretroviral drugs are not recommended in patients with CKD or risk factors for CKD. The document provides indications for renal biopsy and advises on the optimal time for referral of a patient to the nephrologist. The indications for and evaluation and management of dialysis and renal transplantation are also addressed.

[Executive summary of the recommendations on the evaluation and management of renal disease in human immunodeficiency virus-infected patients]. / Resumen ejecutivo del documento de consenso sobre el manejo de la patología renal en pacientes con infección por el virus de la inmunodeficiencia humana.

The aim of this article is to update the 2010 recommendations on the evaluation and management of renal disease in human immunodeficiency virus (HIV)-infected patients. Renal function should be monitored in all HIV-infected patients. The basic renal work-up should include measurements of serum creatinine, estimated glomerular filtration rate by CKD-EPI, urine protein-to-creatinine ratio, and urinary sediment. Tubular function tests should include determination of serum phosphate levels and urine dipstick for glycosuria. In the absence of abnormal values, renal screening should be performed annually. In patients treated with tenofovir, or with risk factors for chronic kidney disease (CKD), more frequent renal screening is recommended. In order to prevent disease progression, potentially nephrotoxic antiretroviral drugs are not recommended in patients with CKD or risk factors for CKD. The document provides indications for renal biopsy and advises on the optimal time for referral of a patient to the nephrologist. The indications for and evaluation and management of dialysis and renal transplantation are also addressed.

Subphrenic abscess as a complication of hemodialysis catheter-related infection.

We describe an unusual case of subphrenic abscess complicating a central venous catheter infection caused by Pseudomonas aeruginosa in a 59-year-old woman undergoing hemodialysis. The diagnosis was made through computed tomography, and Pseudomonas aeruginosa was isolated from the purulent drainage of the subphrenic abscess, the catheter tip and exit site, and the blood culture samples. A transesophageal echocardiography showed a large tubular thrombus in superior vena cava, extending to the right atrium, but no evidence of endocarditis or other metastatic infectious foci. Catheter removal, percutaneous abscess drainage, anticoagulation, and antibiotics resulted in a favourable outcome.

Are low levels of 25-hydroxyvitamin D a risk factor for cardiovascular diseases or malignancies in renal transplantation?

BACKGROUND: Observational studies in healthy people suggest an inverse relationship between 25-hydroxy-vitamin D (25(OH)D levels) and cardiovascular diseases and malignancies. We performed an observational prospective study in renal transplant recipients to investigate the effects of vitamin D deficiency on cardiovascular and malignancy risks. METHODS: From 389 renal transplant recipients, 331 with a functioning graft at 12 months were included in the study. Mineral metabolism parameters were measured at 1, 3, 4 and 12 months. Information regarding the cardiovascular events and malignancies were collected from an electronic database. RESULTS: According to the 1-year mean of 25(OH)D levels, 75 recipients (22.7%) had a normal vitamin D status, 161 (48.6%) had insufficiency and 95 (28.7%) had deficiency in vitamin D levels. During the follow-up, 80 recipients presented at least one cardiovascular event. The total cardiovascular diseases included: 27 patients with coronary diseases, 25 with cardiac failure, 18 with arrhythmia, 11 with acute cerebrovascular events and 19 with peripheral vascular disease. Cardiovascular events were not associated with 25(OH)D levels or vitamin D status, and the 10-year cumulative incidence was 29.3% for normal vitamin D status and 31.6% for insufficiency and 51.9% for deficiency (P = 0.216). Furthermore, Cox univariate analysis showed no association between cardiovascular events and vitamin D levels or vitamin D status. In addition, 53 recipients presented at least one malignancy: 33 non-melanoma skin malignancies and 20 non-skin malignancies (5 prostate, 3 kidney and urinary tract, 2 colon, 2 lung, 2 lymphoma, 2 breast and 4 from other locations). The cumulative incidence of malignancies was 21.3% for normal vitamin D status, 22.7% for insufficiency and 16.7% for deficiency (P = 0.818). CONCLUSIONS: Our data suggested that low vitamin D levels were not associated with an increased risk of cardiovascular diseases or malignancies. However, due to the small number of patients and events, the results should not be considered as definitive. Additional studies with a higher number of patients are required to elucidate the true impact of vitamin D status on cardiovascular and malignancy risks.

Long-term outcomes of IgA nephropathy presenting with minimal or no proteinuria.

The long-term outcome of patients with IgA nephropathy who present with normal renal function, microscopic hematuria, and minimal or no proteinuria is not well described. Here, we studied 141 Caucasian patients with biopsy-proven IgA nephropathy who had minor abnormalities at presentation and a median follow-up of 108 months. None of the patients received corticosteroids or immunosuppressants. We reviewed renal biopsies using the Oxford classification criteria. In this sample, 46 (32%) patients had mesangial proliferation, whereas endocapillary proliferation, focal glomerulosclerosis, and tubulointerstitial abnormalities were uncommon. Serum creatinine increases >50% and >100% were observed in five (3.5%) patients and one (0.7%) patient, respectively; no patients developed ESRD. After 10, 15, and 20 years, 96.7%, 91.9%, and 91.9% of patients maintained serum creatinine values less than a 50% increase, respectively. Using Cox proportional hazards regression, the presence of segmental glomerulosclerosis was the only factor that significantly associated with a >50% increase in serum creatinine. Clinical remission occurred in 53 (37.5%) patients after a median of 48 months. Proteinuria>0.5 and >1.0 g/24 h developed in 21 (14.9%) and 6 (4.2%) patients, respectively. Median proteinuria at the end of follow-up was 0.1 g/24 h, with 41 (29.1%) patients having no proteinuria. At presentation, 23 (16.3%) patients were hypertensive compared with 30 (21.3%) patients at the end of follow-up; 59 (41.8%) patients were treated with renin-angiotensin blockers because of hypertension or increasing proteinuria. In summary, the long-term prognosis for Caucasian patients with IgA nephropathy who present with minor urinary abnormalities and normal renal function is excellent.

Asymptomatic large extracapsular renal pseudoaneurysm following kidney transplant biopsy.

Vascular complications after kidney biopsy include hematomas, arteriovenous fistulas, and pseudoaneurysms. Ultrasonography is a useful tool for the diagnosis of these complications, and color Doppler scan is effective at distinguishing among them. We describe a transplant patient who underwent percutaneous kidney biopsy in whom echography performed after biopsy showed a pulsatile hypoechoic perinephric mass of 4.4 cm. This collection illuminated with color Doppler and connected to the transplant. Color Doppler scanning of the mass showed high-velocity turbulent flow within the cavity and a jet of blood from an intrarenal segmental artery. A typical pattern biphasic flow ("to-and-fro" waveform) at the pseudoaneurysm neck on color Doppler confirmed the diagnosis of postbiopsy pseudoaneurysm. Pseudoaneurysms usually are asymptomatic, but when they cause clinical signs or risk rupture, interventional treatment is required. Supraselective coil embolization of the artery feeding the pseudoaneurysm was performed successfully in our patient. Pseudoaneurysm can mimic renal cysts on gray-scale ultrasound. We suggest that Doppler sonography be performed in cystic areas detected after biopsy to exclude pseudoaneurysm.

A retrospective study on outcome of microscopic polyangiitis in chronic renal replacement therapy.

BACKGROUND: Pauci-immune vasculitis is a heterogeneous disorder with an unfavourable prognosis. Renal involvement is frequently observed in antineutrophil cytoplasm autoantibody (ANCA)-associated small-vessel vasculitis and is an important cause of end-stage renal disease (ESRD). Renal replacement therapy (RRT) is frequently required. Although better prognosis under dialysis is well known, the long-term follow-up of pauci-immune renal vasculitis with RRT is rarely reported. METHODS: We described 24 patients with pauci-immune vasculitis and requirement of dialysis who were admitted in our institutions from January 1989 to December 2008. Mean age was 65 ± 12 years at the beginning of dialysis. There were 12 males and 12 females. Patients with Wegener's granulomatosis, Churg-Strauss syndrome or evidence of anti-glomerular basement membrane were excluded. The study group was formed by patients with a diagnosis of necrotizing extracapillary glomerulonephritis and microscopic polyangiitis. RESULTS: The distribution according to ANCAs was 14 p-ANCA (58%), 5 c-ANCA (21%) and 5 ANCA-negative (21%) pauci-immune renal vasculitis. Pulmonary renal syndrome (PRS) was observed in 10 patients at the onset of vasculitis. Corticosteroids and daily cyclophosphamide were administered to 18 patients, and one patient had intravenous cyclophosphamide. Five patients received isolated corticosteroid therapy. Early reduction in cyclophosphamide dosage was required in five patients due to leucopaenia. Mean follow-up after first dialysis was 89 ± 66 months (range 2-208). Twenty patients were included in haemodialysis (HD), and four patients were included in peritoneal dialysis (PD). At the end of the study, nine patients had received a cadaveric kidney transplant (KT). Relapses rate after the onset of dialysis was 0.03 episode/patient/year. PRS-associated relapses after beginning dialysis were observed in four patients. Main therapy in relapses was also corticosteroids and cyclophosphamide. Survival rates for year 1, 2 and 5 was 91%, 91% and 85%, respectively. Overall mortality at the end of the study was 31.8%. Five patients died in the PRS group, but only one death was associated with progressive pulmonary fibrosis. Higher mortality was observed in PRS vasculitis present at the onset of RRT (50% vs 16.7%, P = NS). Better outcome in patients who received a renal transplantation was observed (88.8% vs 53.8%, P = NS). Conclusions. Despite a low number of patients in this series, pauci-immune vasculitis prognosis under dialysis seems equal to other causes of chronic kidney disease. This study observed a low rate of relapses after beginning dialysis. Poor prognosis is related to severe complications at the beginning of RRT. Today, kidney transplantation is an important therapeutic option for these patients.

Effect of hypertension before beginning dialysis on survival of hemodialysis patients.

BACKGROUND: The role of hypertension as a predictor of mortality in hemodialysis patients is controversial. The purpose of this study is to investigate the effect of hypertension before starting hemodialysis therapy on survival of patients without diabetes during renal replacement therapy. METHODS: We reviewed 184 patients starting hemodialysis therapy. Variables studied were age, sex, renal disease, hypertension, comorbidity, vascular calcifications, left ventricular hypertrophy, body mass index, and albumin, cholesterol, and alkaline phosphatase levels. Regarding blood pressure control, three groups were considered: normotensive (NH), controlled hypertensive (c-HT), and uncontrolled hypertensive (uc-HT). RESULTS: The Cox model was performed considering all-cause and cardiovascular mortality. The model was adjusted for age, sex, serum albumin level, vascular calcifications, history of hypertension, and comorbidity. Comorbidity included cardiovascular comorbidity. For all-cause mortality, comorbidity and history of uncontrolled hypertension were independent risk factors (comorbidity relative risk, 1.95; 95% confidence interval, 1.26 to 3.1; P = 0.003; uncontrolled hypertension relative risk, 1.79; 95% confidence interval, 1.15 to 2.8; P = 0.01). For cardiovascular mortality, uncontrolled hypertension was the main risk factor (relative risk, 2.93; 95% confidence interval, 1.68 to 5.12; P = 0.000). Mortality rates were 7.9/100 patient-years for NH, 8.7/100 patient-years for c-HT, and 14.1/100 patient-years for uc-HT patients. CONCLUSION: This study suggests that uncontrolled hypertension in renal patients before starting dialysis therapy is a major risk factor for cardiovascular mortality during hemodialysis. Because hypertension usually starts in the initial stages of renal disease, we emphasize the importance of prompt and adequate control of blood pressure in this population.