PrEFiNe Plan: Strategic plan for Fabry diseases in Nephrology. / Plan PrEFiNE: Plan estratégico para la enfermedad de Fabry en Nefrología.

BACKGROUND: Renal failure is one of the main causes of death in patients with Fabry disease (FD). Due to the low prevalence of FD, delayed diagnosis and misdiagnosis, often the correct diagnosis is made when organ damage is already present. Early recognition of the disease would allow the prevention of severe complications and the premature death of patients with FD. OBJECTIVE: We present here the PrEFiNE project, which includes a wide spectrum of activities with the aim of improve knowledge and diagnosis of FD. The project is sponsored by Shire Iberia (http://shireiberica.com/) METHODS: From January 2016 to the end of 2017 several activities will be carried out, starting with a survey to evaluate current FD knowledge among nephrologists; in addition some studies to assess prevalence of this disease will be performed. One study will include patients receiving dialysis, another study will cover kidney transplant patients, and a pilot study in chronic kidney disease in stage 3-5 predialysis. Also planned is a pharmacoeconomic study to focus on burden of FD. At the same time medical education activities will be conducted both on line and on site. Plan for dissemination will include medical publications and diffusion to media. PrEFiNE Project will finish with the publication of a compilation book on FD in Nephrology including all planned activities and proposing recommendations based on results and detected unmet needs. PrEfiNE Plan will be coordinated by severa scientific committees, one at national level and 10 other regionals comittees, tha will be responsible to ensure the maximum scientific quality of proposed activities. An advisory board will supervise the project. DISCUSSION: PrEfiNE project will evaluate an action plan focused on improving FD knowledge to make necessary recommendations for an early recognition of the disease. In addition will generate a plan to improve previously undetected needs.

Economic impact of vitamin D treatment on chronic kidney disease patients.

During recent years, increasing recognition has been given to the endocrine action that vitamin D has on the extraskeletal system, and its deep involvement in CKD. This has meant that many vitamin D compounds (both nutritional and active) have been made available, with an important cost reduction. This review looks at the evidence available regarding the usefulness of different types of vitamin D (nutritional and active) for patients with stage 3-5 CKD and those undergoing dialysis. Emphasis is given to its usefulness to control hyperparathyroidism and its impact on morbidity and mortality. We also analysed pharmacoeconomic studies that have been published which compare active vitamin D metabolites. From this review, we are able to conclude that there is still not enough scientific evidence to be able to prefer one active vitamin D over another. In the meantime, doctors should follow the recommendations given in clinical practice guidelines, always taking into account their personal experience with patients. Furthermore, they must consider the economic impact that their treatment decisions may have.

The changing face of hospitalisation due to gastrointestinal bleeding and perforation.

BACKGROUND: Temporal changes in the incidence of cause-specific gastrointestinal (GI) complications may be one of the factors underlying changing medical practice patterns. AIM: To report temporal changes in the incidence of five major causes of specific gastrointestinal (GI) complication events. METHODOLOGY: Population-based study of patients hospitalised due to GI bleeding and perforation from 1996 to 2005 in Spain. We report crude rates, and estimate regression coefficients of temporal trends, severity and recorded drug use for five frequent GI events. GI hospitalisation charts were validated by independent review of large random samples. RESULTS: The incidence per 100 000 person-years of hospitalisations due to upper GI ulcer bleeding and perforation decreased over time [from 54.6 and 3.9 in 1996 (R² = 0.944) to 25.8 and 2.9 in 2005 (R² = 0.410) respectively]. On the contrary, the incidence per 100 000 person-years of colonic diverticular and angiodysplasia bleeding increased over time [3.3 and 0.9 in 1996 (R² = 0.443) and 8.0 and 2.6 in 2005 (R² = 0.715) respectively]. A small increasing trend was observed for the incidence per 100 000 person-years of intestinal perforations (from 1.5 to 2.3 events). Based on data extracted from the validation process, recent recorded drug intake showed an increased frequency of anticoagulants with colonic diverticular and angiodysplasia bleeding, whereas NSAID and low-dose aspirin use were more prevalent in peptic ulcer bleeding and colonic diverticular bleeding respectively. CONCLUSIONS: From 1996 to 2005, hospitalisations due to peptic ulcer bleeding and perforation have decreased significantly, whereas the number of cases of colonic diverticular and angiodysplasia bleeding have increased.

[Clinical experience with icodextrin. Multicenter study]. / Experiencia clínica con icodextrina. Estudio multicéntrico.

UNLABELLED: The aim of this study was to analyse our experience with icodextrin in Andalusia, Spain. The study includes 51 patients (30 women and 21 men) on peritoneal dialysis (21 on CAPD and 30 on Automated Peritoneal Dialysis) treated with icodextrin for 10.3 +/- 7 months (0-41 months). Their mean age was 57 +/- 18 years (18-86 years). We have recorded the appearance of side effects, and the evolution of several biochemical parameters at baseline and after 6, 12 ans 18 months from initiation of icodextrin. We also studied drainage fluid from 12 patients after an icodextrin exchange. RESULTS: There were side effects (all cutaneous) in 4 out of 51 patients (7.8%). Two of the affected suffered from cutaneous hypersensitivity reactions, and icodextrin had to be suspended; the other two had exfoliative dermatitis affecting hands and feet that disappeared without have to withdraws icodextrin. Biochemical parameters: Serum sodium levels decreased from baseline to six months (138 +/- 6 mEq/l vs 136 +/- 3 mEq/l; p = 0.006), and then persisted at the same levels throughout the rest of the study period. There was a slight but significant decreased of serum HDL-cholesterol at six months vs baseline (55 +/- 26 mg/dl vs 51 +/- 20 mg/dl, p = 0.04), and a further decrease at twelve months vs six months (42 +/- 15 mg/dl vs 51 +/- 13 mg/dl, p = 0.054). There were no significant variations of glucose, osmolality, cholesterol, LDL-cholesterol (tendency to increase), triglycerides, beta 2 m and weight (tendency to increase; p = 0.08). In relation with the icodextrin exchange: average ultrafiltration 296 +/- 119 ml (ranging from 104 to 480 ml), creatinine clearance 1.9 +/- 0.5 litres (20.5% of daily creatinine clearance), urea clearance 2.08 +/- 0.5 litres (18.7% of daily urea clearance), total protein losses 3.2 +/- 0.9 g, albumin losses 1.4 +/- 0.5 g; urea and creatinine clearances were negatively correlated with ratios D/P4 of urea and creatinine of PET and positively correlated with ratio G4/G0. In conclusion, side effects are scarce with the use of icodextrin. As described in other studies, there is a trend to a slight decrease in serum sodium. The long-term use of icodextrin does not-prevent weight gain or deterioration of patients on peritoneal dialysis, despite the diminution of glucose load.