[Atheroembolic renal disease: analysis of clinical and therapeutic factors that influence its progression]. / Enfermedad renal ateroembólica: un análisis de los factores clínicos y terapéuticos que influyen en su evolución.

Cholesterol embolism is a disease caused by distal showering of cholesterol crystal released from disintegration of arterial atheromatous plaques. It may occur spontaneously or more often after invasive vascular procedures or thrombolytic/anticoagulant agents. Forty five cases were diagnosed between 1989 and 2005 in three Spanish hospitals. The diagnosis was confirmed by histology or diagnostic ophthalmoscopic findings. The majority were male (93.3%), elder (55.5% were older than 70 years), smoker (91.1%), had hypertension (95.6%), with high prevalence of cardiovascular risk factors. At the time of diagnosis all patients presented acute renal failure. Mean serum creatinine at diagnosis was 4.3+/- 2.4 mg/dl. The acute renal failure was accompanied with eosinophilia (64.4%) and cutanous lesions (57.7%). 20% of cases occur spontaneously and 46.7% after endovascular manipulation (coronary angiography/arteriography) and only 8% after changes in anticoagulant treatment. After a follow-up of 12 +/- 16.3 months the 55.6% of patients need chronic dialysis, 64.4% died, 8 of them after the beginning of dialysis. Nine patients recovered renal function, with a mean creatinine of 3 +/- 1.7 mg/dl at the end of follow-up. The cardiovascular comorbididy and the clinical severity of the embolism don t have impact in the renal or patient survival. Renal survival (Kaplan-Mier) were better in spontaneous than in iatrogenic cholesterol embolism. Fifteen of 45 patients were treated with steroids. In treated patients we observed a high incidence of death (73.3% versus 60%) and fewer recovery of renal function (13.3% versus 23%), without statistical significance. The mean time to dialysis was shorter in treatment patients (p= 0.017). Statins treatment was not associated with outcome (renal or individual). In summary, atheroembolic renal disease represents an acute renal failure with special characteristics. Renal and individual outcome is poor, but some patients have spontaneous recovery of renal function. Renal survival was significantly better in spontaneous disease. We don t observe beneficial effect of steroid treatment.

[Idiopathic retroperitoneal fibrosis: clinico-pathological characteristics]. / Fibrosis retroperitoneal idiopática: características clínico-patológicas.

Idiopathic retroperitoneal fibrosis is a rare disease often causing obstructive uropathy because the fibrosis entraps the ureters. The retroperitoneal tissue is constituted by a fibrous component and a chronic inflammatory infiltrate with the former characterized by miofibroblasts. The infiltrate displayed perivascular. Last years, the immunosuppressive therapy and the decompression of obstructive renal failure have improved the prognostic. We reported 9 patients with Idiopathic retroperitoneal fibrosis in a Centre. We evaluate clinical symptoms at the presentation with the signs of renal and non-renal involvement. We evaluate the achieved therapy and the follow-up. All 9 patients had a radiological and/or histological diagnosis in the absence of malignancy, previous radiotherapy or gadolinium's administration with severe renal failure. The patients were treated with 1 mg/kg/day of prednisone three months and possibly the insertion of ureteral catheters. 89% patients have a high rate of initial success without renal failure, despite frequent disease relapse. A patient developed progression of the fibrosis.

[Gadolinium-induced systemic fibrosis in advanced kidney failure]. / Fibrosis sistémica por gadolinio en insuficiencia renal avanzada.

Nephrogenic systemic fibrosis is a debilitating disease occurring exclusively in patients with severe renal failure. Originally it was described as nephrogenic fibrosing dermopathy. The pathogenesis of the disease is not yet known, but the observations suggest a close association with the exposure to gadolinium-containing contrast agents. These agents cause systemic fibrosis by releasing free gadolinium into tissues. International commissions in drug control recommend avoiding gadolinium based contrast agents ion patients with GFR <30 ml/min. The prevention is the best treatment because the observations are series with limited patients, or a case-report communication. So, we report a case with a fatal evolution, the first case in Spain, with a multisystemic involvement showed in the autopsy. The patient had repeated exposure to gadodiamide; it increases the possibility of the systemic fibrosis. We review the recent literature of the nephrogenic systemic fibrosis.

[Advance directives in chronic dialysis patients]. / Documento de voluntades anticipadas de pacientes con insuficiencia renal crónica terminal en tratamiento sustitutivo mediante diálisis.

BACKGROUND AND OBJECTIVE: Knowledge of the life-sustaining treatment preferences of the dialysis patients would be extremely helpful to substitute decision-makers and nephrologists in deciding whether to continue or stop a treatment. The population of the Mediterranean countries show this opinion with less frequency. The objective of this study is: 1) the knowledge of the patient's view for the advance directives; it may increase the likelihood to get the correct decisions of the staff when complications break the normal course of chronic dialysis, and 2) the statement of the advance directives. MATERIAL AND METHOD: We distributed 135 questionnaires to patients with chronic renal failure in dialysis treatment of the Sabadell's Hospital to explore demographic information about responders and not-responders and explore the rate of questionnaires was completed about the cardiopulmonary resuscitation, respirator, tube feeding and dialysis in case of coma, persistent vegetative state, severe dementia and terminal illness. We explore about the representative of patients and in case of not-responders about the cause to not answer. RESULTS: Sixty-four of 135 patients (47,8%) did not want cardiopulmonary resuscitation, respirator, tube feeding or dialysis in case of coma, persistent vegetative state, severe dementia or terminal illness. Compared with patients who wanted the treatments, those who did not were older (71,2 versus 62,2 years; p = 0.002). There was no difference in the other demographic questions, including sex (p=0.674), cause of kidney failure (p=0.815), comorbid conditions (p=0.824), and social status (language of questionnaire -0.155- and standard of education -0.288-). Advance care planning does not occur solely within the context of the physician-patient relationship; the respondents reported the representative in the family, essentially. The patients not-responders doesn t want to think in those situations and also they show doubt about the interpretation of their answers. CONCLUSIONS: near 50% patients in chronic dialysis want to stop certain treatments in case of resuscitation cardiopulmonary, coma, persistent vegetative state, severe dementia or terminal illness. The older patients want the limitation of treatments more frequently.

[Choosing not to dialysis in chronic renal failure in stage V (Renal Failure). Evolution of the characteristics of patients between 1992-1995 and 2000-2003]. / Elección de no diálisis en insuficiencia renal crónica en estadio V (fallo renal). Evolución de las características de los pacientes entre 1992-1995 y 2000-2003.

BACKGROUND: The incidence of chronic renal failure increase with the age. The selection of patient to dialysis has been increasing in spite of the high comorbidity. Moreover, in our clinical practice the aged patient is not contraindicated to dialysis. However, in the nephrology clinical practice not all the patients start the treatment with dialysis. OBJECTIVE The aim of our study has been to compare the characteristics of the patients who had not been dialyzed between the periods 1992-1995 and 2000-2003 to analyze the trend of the nephrology clinical practice. MATERIAL AND METHODS: Comparative study of the characteristics and the evolution of patients with chronic renal failure in stage V, (renal failure) not incorporated to dialysis in one hospital during four years between the periods the 1992-1995 ( period A) and 2000-2003 (period B). RESULTS: Start dialysis (period A versus period B): 116 patients, age 59.9+15.5 years vs. 229 patients, age 64.0+15.8 years (p<0.05). Non-dialysis (period A versus period B): 38 patients, age 77.5+9.3 years vs. 37 patients, age 81.7+6.2 years (p<0.01). Renal function: serum creatinina 7.4+2.4 mg/dl vs. 5.3+1.2 mg/dl (p<0.001), MDRD estimate glomerular filtration 6.9+2.4 mg/dl ml/min/1.73 m2 vs. 10.0+2.3 ml/min/1.73 m2 (p<0.001). Primary renal disease: unknown etiology 31.5 % vs. 24.3 %, nephroangiosclerosis 23.6 % vs. 32.4 %, diabetes 28.9 % vs. 21.6 %. Functional status: dependent patients 34.2 % vs 83.8 % (p<0.001). The principal reason for non-dialysis were: personal decision: 26.3 % vs. 35.1 %, dementia 15.8 % vs. 29.7 %, brief life expectancy because of serious co-existing diseases 13.1 % vs. 21.7 % and serious chronic illness with inability for themselves care 44.7 % vs. 13.1 %. Comorbid conditions: 2.3+1.0 vs. 3.0+1.5 (p<0.05). Survival: 55+168 days vs. 168+236 days (p<0.001). CONCLUSION: Most of the patients that don't begin dialysis are elderly together with a poor functional capacity and with more autonomy in their decisions. The identification of patients with renal failure (stage V) was detected early in the last period than in the following one. The conservative management of non-dialyzed uremic patients is a significative nephrology clinical practice due to more survival of those persons.

[Secondary amyloidosis (AA-type) due to localized cutaneous vasculitis]. / Amiloidosis secundaria asociada a vasculitis cutánea localizada.

We report a case of a 49 year old man, diagnosed soon after the outcome of casual proteinuria, of AA-type amyloidosis in relation to small and medium vessel cutaneous vasculitis without systemic involvement. This combination is a rare entity and only two cases of cutaneous hypersensibility vasculitis complicated with AA-type amyloidosis had been reported. We describe the results of the use of several immunosuppressive drugs during four years follow up with temporally total remission of the disease.

[Ischemic renal disease: revascularization or conservative treatment?]. / Nefropatía isquémica: revascularización o tratamiento médico conservador?

Ischemic nephropathy is recognized as a distinct cause of renal insufficiency and it is defined as a significant reduction in glomerular filtration rate in patients with hemodynamically significant renovascular occlusive disease. We argue the epidemiologic and clinical manifestations of atherosclerotic renovascular disease, and we evaluate the pronostic agents. Published studies of the outcome of revascularization for renal-artery stenosis have been excellent, offering a durable patency and functional improvement but they have had numerous limitations. The atherosclerosis is a systemic disease and it provides the general prognosis of patients. We conclude that ischemic renal disease is a nephropathy of smoker men, with proteinuria excretion similar to nephropathy with unilateral stenosis. The age of patients is the clinical feature that decide the treatment: surgery, angioplasty/stent or medical management. Comparative analysis of percutaneous transluminal angioplasty and operation for renal revascularization and medically treated patients have proved that the advanced chronic renal insufficiency is associated with an unfavourable response of treatment of the ischemic nephropathy. But, in this nephropathy the revascularization can be the better therapy for selected patients. The revascularization with angioplasty/stent for patients with unilateral renal stenosis and chronic renal insufficiency has a doubtful effectiveness, as the chronic renal failure is result of nephroangiosclerosis.

[Diagnosis and prognosis of atheroembolic disease]. / Diagnóstico y pronóstico de la enfermedad ateroembólica.

Atheroembolic disease is recognized as an iatrogenic complication from an invasive vascular procedure, such as manipulation of the aorta during angiography or vascular surgery, and after anticoagulant and fibrinolytic therapy. Cholesterol crystal embolism is caused by showers of cholesterol crystals from an atherosclerotic aorta that occlude small arteries. The kidney is a frequent target organ for cholesterol emboli because of proximity of the renal arteries to abdominal aorta and it receive an enormous amount of blood flows. We describe the epidemiologic agents of 19 cases that were diagnosed by histologic sections of the affected tissues; the eosinophilia and the renal failure are the clinical features that guide to the diagnosis, in patients with ischemic nephropathy and general atherosclerosis. 53% among patients had a previous invasive procedure and 26% occurred spontaneously. We remark the importance of the kidney's biopsy in diagnosis of the atheroembolic disease and their bad prognosis with 63% of death rate in 18 months of average follow-up. We report patients with the multiple cholesterol emboli syndrome mimicking systemic vasculitis: they died by multivisceral acute failure. The subacute presentation of atheroembolic disease with progressive renal failure treated with hemodialysis is a sign of bad prognosis. The knowledge of the disease and their prevention are the better treatment.

[Diagnostic role of magnetic resonance in the renal involvement in nocturnal paroxysmal hemoglobinuria]. / Papel diagnóstico de la resonancia magnética en la afectación renal en la hemoglobinuria paroxística nocturna.

Paroxysmal nocturnal haemoglobinuria or Machiafava-Micheli disease is an acquired clonal stem cell disorder characterized by defective haematopoiesis, which results in an increased sensitivity of the erythrocytes to complement-mediated intravascular haemolysis. Renal damage is rare but it can lead to chronic renal failure. Micro-infarctions due to repeated episodes of microvascular thrombosis and cortical haemosiderosis are thought to be the main contributors to the development of chronic renal failure in paroxysmal nocturnal haemoglobinuria. Magnetic resonance imaging provides characteristic images of the kidney. We describe a patient with cortical haemosiderosis and show the magnetic resonance features.

[40-year-old man with primary antiphospholipid syndrome]. / Varón de 40 años de edad con síndrome antifosfolópido primario.

We report the case o a 40-year-old male patient with "primary antiphospholipid syndrome" who developed ischemic cerebral infarctions and renal microangiopathy with infarction. A review of the literature on renal involvement in the primary antiphospholipid syndrome disclosed the differences from the antiphospholipid syndrome in the systemic lupus erythematosus. We describe the evolution of the patient at eight years, and we emphasize the importance of the treatment with warfarin. Also, we review the pathophysiology of severe secondary arterial hypertension.

[Arteriovenous fistula: complication of renal biopsy. Superselective embolization]. / Fístula arteriovenosa: complicación de biopsia renal. Embolización supraselectiva.

The arteriovenous fistula can be a major and late complication of percutaneous renal biopsies of native kidneys. The incidence of arteriovenous fistulas appears to be low and has been infrequently mentioned in large series of biopsies although there are a number of individual reports. In most part of cases, no systemic effects of the arteriovenous fistulas were observed, so they close by themselves. Optimization of renal biopsy techniques aims not only at obtaining kidney tissue technically adequate for diagnosis, but at reducing biopsy-induced complications. When these complications are done, the new transvascular techniques can get the healing of fistula with the embolism by catheters. We describe the case of a 37-year-old woman who had chronic renal failure by a possible chronic glomerulonephritis. She developed deterioration of renal function after the percutaneous renal biopsy. An arteriovenous fistula of high flow was detected by Doppler ultrasound. Then, it was confirmed angiographically and closed by embolism's catheter without damage of renal tissue.

[An outbreak of gram-negative bacteremia (GNB), especially enterobacter cloacae, in patients with long-term tunnelled haemodialysis catheters]. / Brote epidémico de bacteriemias por bacilos gram-negativos (BGN), especialmente enterobacter cloacae, en una unidad de hemodiálisis, en pacientes portadores de catéteres permanentes tunelizados.

Vascular access through a venous catheter for haemodialysis is associated with increased risk of thrombosis, central venous stenosis, short access survival and inadequate dialysis. The most important catheter-related complications, which determine method survival, are infection and dysfunction. In particular, infectious episodes are in some studies the leading cause for untimely catheter removal and for catheter-related morbidity but also for morbidity in dialysis patients. Double-lumen central venous catheters used for haemodialysis, are common causes of septicaemia. Most cases are caused by staphylococci. Episodes of gram-negative bacteriemia have been traced to bacterial contamination of water and/or dialysate, errors in dialyzer reprocessing, and improper setup procedures. In this paper, we describe and outbreak of gram-negative bacteremia, firstly E. cloacae, in an outpatients haemodialysis unit, in the patients with long-term tunnelled haemodialysis catheters. We describe the epidemic investigation that we achieved to identify the source of contaminating bacteria and the route by which bacteria gained access to the bloodstream. We prove the contamination by gram-negative bacterium of the water-distribution lines and haemodialysis machines. Moreover, E. cloacae strains isolated from the lines and machines are genotypically identical to the isolated from the patients. Also, we prove that the hands of health care personnel are unintentional carriers. The outbreak was finished when decontamination of dialysis machines was enhanced and dialyzer-priming fluid was modified.

[Patients with end-stage chronic renal insufficiency on programmed withdrawal from dialysis]. / Pacientes con insuficiencia renal crónica terminal retirados de diálisis bajo protocolización.

UNLABELLED: The voluntary discontinuation of dialysis by patients is a common mode of death in dialysis programmes. Unfortunately the Spanish experience has not been related in the nephrological literature. Initiation of, and withdrawal from, dialysis pose ethical questions for medicine in the 21st century. The dialysis population is aging and they have multiple medical problems. The choice may be between prolongation of quantity or quality of life. We evaluated a protocol for initiation of dialysis in patients with end stage renal failure and their subsequent withdrawal. We determined the factors predicting withdrawal of dialysis and revised the protocol to take account of these. We carried out an opinion poll of doctors and nurses about the effectiveness of the protocol. We studied prospectively the reasons for death of patients in the last seven years. RESULTS: Thirty patients were withdrawn from dialysis out of 116 who died during treatment by hemodialysis or continuous ambulatory peritoneal dialysis (CAPD) in the last seven years. Vascular nephropathy is the principal disease predicting withdrawal from dialysis; the main precipitating cause is mental incapacity. The availability of a protocol for withdrawal of dialysis is well received by doctors and nurses and it engenders moral and legal calm when facing difficult decisions. Twenty-six per cent of deaths on regular dialysis are the result of withdrawal of treatment.

[Pseudomononucleosis induced by diphenylhydantoin (author's transl)]. / Pseudomononucleosis inducida por defenilhidantoína.

The authors report a patient who developed a mononucleotic syndrome after 2 months treatment with diphenylhydantoin. The clinical and biological criteria needed to establish the diagnosis of pseudomononucleosis are listed, while other possible causes described in the literature have been excluded. Complications which may occur during anti-convulsant therapy with diphenylhydantoin are reviewed and the few cases of pseudomononucleosis in the literature noted. Diagnosis was established through eliminating other possible causes, as the only definite test-drug induced reaction-is hazardous to the patient.

[Validation, adaptation and translation of the MacCAT-T into Spanish: a tool to assess the ability to make health decisions]. / Validación, adaptación y traducción al castellano del MacCAT-T: herramienta para evaluar la capacidad en la toma de decisiones sanitarias.

INTRODUCTION: Capacity assessment is an essential element of the informed consent process and is the duty of the physician. The MacCAT-T instrument explores four skills needed to consent a treatment. There is no Spanish version, and the main objective of this work is to validate, adapt and translate the MacCAT-T into Spanish. MATERIAL AND METHODS: The MacCAT-T was translated into Spanish and then back-translated into English. It was validated as regards its appearance and content (by 15 experts), construct (inter-rater reliability and internal consistency) and criteria (the validity of an instrument by comparing it to some external criterion, in this case the Mini Examen Cognoscitivo de Lobo). Ninety medical and surgical outpatients over 18 years were included with no deficits of expression and/or severe disorders of consciousness that did not allow them to be interviewed. RESULTS: They have been optimal considering different types of validity. The average application time was between 9 and 13minutes. DISCUSSION: Data are consistent with those obtained in other applications of MacCAT-T in the English language and facilitate the provision of a Spanish tool for assessing capacity.

Exploring the opinion of CKD patients on dialysis regarding end-of-life and Advance Care Planning.

Advance care planning (ACP) and the subsequent advance directive document (ADD), previously known as "living wills", have not been widely used in Spain. The Ethics Group from the Spanish Society of Nephrology has developed a survey in order to investigate the opinion of dialysis patients regarding the ADD and end-of-life care. Patients received documentation explaining ACP and filled out a survey about their familiarity with and approval of the ADD. Seven hospital dialysis centres participated in the study for a total of 416 active dialysis patients. Questionnaires were distributed to 263 patients, 154 of which answered (69.2% completed them without assistance). The rates for ADD implementation (7.9%) and designation of a representative person (6.6%) were very low. Most of the patients clearly expressed their wishes about irreversible coma, vegetative state, dementia and untreatable disease. More than 65% did not want mechanical ventilation, chronic dialysis, tube feeding or resuscitation if cardiorespiratory arrest occurred. They reported that an ADD could be done before starting dialysis but most thought that it should be offered only to those who requested it (65% vs 34%). In conclusion, patients have clear wishes about end-of-life care, although these desires had not been documented due to the very low implementation of the ADD.