Paula López-Sánchez

Peritoneal Dialysis Can Be an Option for Dominant Polycystic Kidney Disease: an Observational Study

♦ Background: Autosomal dominant polycystic kidney disease (ADPKD) has been considered a relative contraindication for peritoneal dialysis (PD), although there are few specific studies available. ♦ Methods: A multicenter historical prospective matched-cohort study was conducted to describe the outcome of ADPKD patients who have chosen PD. All ADPKD patients starting PD (n = 106) between January 2003 and December 2010 and a control group (2 consecutive patients without ADPKD) were studied. Mortality, PD-technique failure, peritonitis, abdominal wall leaks and cyst infections were compared. ♦ Results: Patients with ADPKD had similar age but less comorbidity at PD inclusion: Charlson comorbidity index (CCI) 4.3 (standard deviation [SD] 1.6) vs 5.3 (SD 2.5) p < 0.001, diabetes mellitus 5.7% vs 29.2%, p < 0.001 and previous cardiovascular events 10.4% vs 27.8%, p < 0.001. No differences were observed in clinical events that required transient transfer to hemodialysis, nor in peritoneal leakage episodes or delivered dialysis dose. The cyst infection rate was low (0.09 episodes per patient-year) and cyst infections were not associated to peritonitis episodes. Overall technique survival was similar in both groups. Permanent transfer to hemodialysis because of surgery or peritoneal leakage was more frequent in ADPKD. More ADPKD patients were included in the transplant waiting list (69.8 vs 58%, p = 0.04) but mean time to transplantation was similar (2.08 [1.69 – 2.47] years). The mortality rate was lower (2.5 vs 7.6 deaths/100 patient-year, p = 0.02) and the median patient survival was longer in ADPKD patients (6.04 [5.39 – 6.69] vs 5.57 [4.95 – 6.18] years, p = 0.024). ♦ Conclusion: Peritoneal dialysis is a suitable renal replacement therapy option for ADPKD patients.

A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome

Pregnancy-associated atypical hemolytic uremic syndrome (aHUS) refers to the thrombotic microangiopathy resulting from uncontrolled complement activation during pregnancy or the postpartum period. Pregnancy-associated aHUS is a devastating disease for which there is a limited clinical understanding and treatment experience. Here we report a retrospective study to analyze the clinical and prognostic data of 22 cases of pregnancy-associated aHUS from the Spanish aHUS Registry under different treatments. Sixteen patients presented during the first pregnancy and as many as nine patients required hemodialysis at diagnosis. Identification of inherited complement abnormalities explained nine of the 22 cases, with CFH mutations and CFH to CFHR1 gene conversion events being the most prevalent genetic alterations associated with this disorder (66%). In thirteen of the cases, pregnancy complications were sufficient to trigger a thrombotic microangiopathy in the absence of genetic or acquired complement alterations. The postpartum period was the time with highest risk to develop the disease and the group shows an association of cesarean section with pregnancy-associated aHUS. Seventeen patients underwent plasma treatments with a positive renal response in only three cases. In contrast, ten patients received eculizumab with an excellent renal response in all, independent of carrying or not inherited complement abnormalities. Although the cohort is relatively small, the data suggest that pregnancy-associated aHUS is not different from other types of aHUS and suggest the efficacy of eculizumab treatment over plasma therapies. This study may be useful to improve prognosis in this group of aHUS patients.

Primer episodio de infección peritoneal: descripción y factores pronósticos

La peritonitis es una de las complicaciones mas frecuentes y graves asociadas a la dialisis peritoneal (DP), siendo la primera causa de perdida de cateter y de finaliza- cion de la tecnica. La incidencia y etiologia de cada episodio varia en funcion de cada region y evoluciona a lo largo del tiempo. Por esta razon, es de vital importancia conocer los factores de riesgo y pronosticos de peritonitis en cada mo- mento. Metodos: Se realizo un estudio observacional de cohorte, multicentrico, prospectivo, con un seguimiento ma- ximo de 7 anos (2003-2010) que incluyo 1177 pacientes, con 476 episodios de primera peritonitis (total: 1091 peritonitis). Resultados: Se presenta la descripcion de las caracteristicas del primer episodio de peritonitis en una serie amplia y ac- tual. Los factores que se asociaron a un menor tiempo hasta la primera peritonitis en analisis multivariante fueron la co- morbilidad cardiovascular previa ( hazard ratio [HR] 1,25 [1,04-1,58]), la procedencia de hemodialisis (HR 1,39 [1,10- 1,76]) o trasplante renal previo (HR 1,38 [1,10-1,93]), iniciar DP con tecnica manual (HR 1,39 [1,13-1,73]) y la edad de ini- cio > 70 anos (HR 1,53 [1,23-1,90]). El primer episodio de pe- ritonitis presenta una tasa de recidiva del 7,8 %, retirada de cateter del 11,7 % y una mortalidad en el primer mes tras el episodio del 1,3 %. La evolucion de la peritonitis depende fundamentalmente del tipo de germen. Se estima un riesgo mayor para gramnegativos ( odds ratio [OR] 5,31 [2,26-12,48]) y el agregado de peritonitis polimicrobianas, por hongos o por micobacterias (OR 38,24 [13,84-105,63]), comparados con la referencia de grampositivos. Conclusion: El desarrollo del primer episodio de peritonitis depende de las caracteristicas del paciente al inicio de dialisis, la comorbilidad y la tecnica utilizada. Los pacientes que presentan mayor riesgo deben recibir una atencion especial en los procesos de entrena- miento y seguimiento

Cumplimiento de objetivos de calidad y evolución de los pacientes incidentes en diálisis peritoneal

INTRODUCTION In 2007 the Scientific Quality-technical and Improvement of Quality in Peritoneal Dialysis was edited. It includes several quality indicators. As far as we know, only some groups of work had evaluated these indicators, with inconclusive results. AIM To study the evolution and impact of guidelines in Peritoneal Dialysis. METHODS Prospective cohort study of each incident of patients in Peritoneal Dialysis, in a regional public health care system (2003-2006). We prospectively collected baseline clinical and analytical data, technical efficacy, cardiovascular risk, events and deaths, hospital admissions and also prescription data was collected every 6 months. RESULTS Over a period of 3 years, 490 patients (53.58 years of age; 61.6% males.) Causes of ERC: glomerular 25.5%, diabetes 16%, vascular 12.4%, and interstitial 13.3%. 26.48% were on the list for transplant. Dialysis efficacy: Of the first available results, the residual renal function was 6.37 ml/min, achieving 67.6% of all the objectives K/DOQI. 38.6% remained within the range during the entire first year. Anaemia: 79.3% received erythropoietic stimulating agents and maintained an average Hb of 12.1 g/dl. The percentage of patients in the range (Hb: 11-13 g/dl) improved after a year (58.4% vs 56.3% keeping in the range during this time of 25.6%). Evolution: it has been estimated that per patient-year the risk of: 1) mortality is 0.06 IC 95% [0.04-0.08]; 2) admissions 0.65 [0.58-0.72]; 3) peritoneal infections 0.5 [0.44-0.56]. CONCLUSION Diabetes Mellitus patients had a higher cardiovascular risk and prevalence of events. The degrees of control during the follow-up in many topics of peritoneal dialysis improve each year; however they are far from the recommended guidelines, especially if they are evaluated throughout the whole study.

Cómo debemos analizar y describir la mortalidad de nuestros pacientes: Experiencia del Grupo Centro Diálisis Peritoneal

INTRODUCTION There are different strategies to analyse mortality in peritoneal dialysis (PD) with different definitions for case, event, time at risk, and statistical tests. A common method for the different registries would enable proper comparison to better understand the actual differences in mortality of our patients. METHODS We review and describe the analysis strategies of regional, national and international registries. We include actuarial survival, Kaplan-Meier (KM) and competitive risk (CR) analyses. We apply different approaches to the same database (GCDP), which show apparent differences with each method. RESULTS A total of 1,890 incident patients in PD from 2003-2013 were included (55 years; men 64.2%), with initial RRF of 7ml/min; 25% had diabetes and a Charlson index of 3 [2-4]; 261 patients died, 380 changed to haemodialysis (HD) and 682 received a transplant. Annual mortality rates varied up to 20% in relative numbers (6.4 vs. 5.2%) depending on the system applied. The estimated probability of mortality measured by CR progressively differs from the KM over the years: 3.6 vs. 4.0% the first year, then 9.0 vs. 11.9%, 15.6 vs. 28.3%, and 18.5 vs. 43.3% the following years. CONCLUSIONS Although each method may be correct in themselves and express different approaches, the final impression left on the reader is a number that under/overestimates mortality. The CR model better expresses the reality of PD, where the number of patients lost to follow-up (transplant, transfer to HD) it is 4 times more than deceased patients and only a quarter remain on PD at the end of follow up.