1,720,988 research outputs found
Autosomal Dominant Polycystic Kidney Disease: Extrarenal Involvement
Full text link: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disorder, but kidneys are not the only organs involved in this systemic disorder. Individuals with the condition may display additional manifestations beyond the renal system, involving the liver, pancreas, and brain in the context of cystic manifestations, while involving the vascular system, gastrointestinal tract, bones, and cardiac valves in the context of non-cystic manifestations. Despite kidney involvement remaining the main feature of the disease, thanks to longer survival, early diagnosis, and better management of kidney-related problems, a new wave of complications must be faced by clinicians who treated patients with ADPKD. Involvement of the liver represents the most prevalent extrarenal manifestation and has growing importance in the symptom burden and quality of life. Vascular abnormalities are a key factor for patients' life expectancy and there is still debate whether to screen or not to screen all patients. Arterial hypertension is often the earliest onset symptom among ADPKD patients, leading to frequent cardiovascular complications. Although cardiac valvular abnormalities are a frequent complication, they rarely lead to relevant problems in the clinical history of polycystic patients. One of the newest relevant aspects concerns bone disorders that can exert a considerable influence on the clinical course of these patients. This review aims to provide the "state of the art" among the extrarenal manifestation of ADPKD
Comparison of Total Kidney Volume Quantification Methods in Autosomal Dominant Polycystic Disease for a Comprehensive Disease Assessment
No full textBackground: In recent times, the scientific community has been showing increasing interest in the treatments aimed at slowing the progression of the autosomal dominant polycystic kidney disease (ADPKD). Therefore, in this paper, we test and evaluate the performance of several available methods for total kidney volume (TKV) computation in ADPKD patients - from echography to MRI - in order to optimize patient classification. Methods: Two methods based on geometric assumptions (mid-slice [MS], ellipsoid [EL]) and a third one on true contour detection were tested on 40 ADPKD patients at different disease stage using MRI. The EL method was also tested using ultrasound images in a subset of 14 patients. Their performance was compared against TKVs derived from reference manual segmentation of MR images. Patient clinical classification was also performed based on computed volumes. Results: Kidney volumes derived from echography significantly underestimated reference volumes. Geometric-based methods applied to MR images had similar acceptable results. The highly automated method showed better performance. Volume assessment was accurate and reproducible. Importantly, classification resulted in 79, 13, 10, and 2.5% of misclassification using kidney volumes obtained from echo and MRI applying the EL, the MS and the highly automated method respectively. Conclusion: Considering the fact that the image-based technique is the only approach providing a 3D patient-specific kidney model and allowing further analysis including cyst volume computation and monitoring disease progression, we suggest that geometric assumption (e.g., EL method) should be avoided. The contour-detection approach should be used for a reproducible and precise morphologic classification of the renal volume of ADPKD patients
HYPOTHERMIC PERFUSION OF THE KIDNEY: FROM RESEARCH TO CLINICAL PRACTICE
No full textKidney transplantation is the treatment of choice for patients with end stage renal disease (ESRD). Donor availability is lower than demand, therefore suboptimal grafts retrieved from donors after brain death with expanded criteria donors (EC-DBD) and from donors after cardiac death (DCD) are in-creasingly used. These organs carry a higher risk of worse clinical outcomes, and subsequently need more advanced preservation systems than static cold storage (SCS). Hypothermic perfusion represents one of the aforementioned strategies.This review summarizes the main features of hypothermic perfusion: its mechanism of action through analysis of preclinical models and its clinical ef-ficacy in kidney transplantation with a focus on marginal donors. Oxygenated hypothermic perfusion was also evaluated focusing on its potential benefits on cell metabolism and graft immungenicity. Finally, as hypothermic perfu-sion not only allows to recover marginal grafts, but may also recondition grafts unsuitable for transplantation, the possible methods of graft evalu-ation and treatment options during perfusion are described in this review
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Kidney Transplant from donors after cardiac death (DCD): monocentric experience and literature review
No full textIl trapianto di rene da donatore dopo morte cardiaca (DCD) rappresenta una valida opzione per incrementare il numero di organi disponibili, mantenendo standard di sopravvivenza e di funzionalità del graft sovrapponibili ai trapianti da donatore per morte cerebrale (DBD). Il Centro di Riferimento Trapianti dell’Emilia Romagna ha attivato un programma di donazione da DCD a partire da Gennaio 2016. Il presente studio è stato condotto con lo scopo di analizzare la casistica dei trapianti di rene eseguiti nei primi 30 mesi di attività del programma, confrontando gli outcome dei DCD con quelli dei trapianti da donatore DBD eseguiti nello stesso periodo. Sono stati inoltre indagati eventuali fattori prognostici predittivi di funzionalità renale. Nel periodo esaminato sono stati eseguiti 16 trapianti di rene da 10 donatori DCD (5 SCD-DCD e 5 ECD-DCD). Non sono state osservate Primary Non Function(PNF). Sono state osservate 2 graft loss entro 12 mesi, entrambe conseguenti a transplantectomia per rottura dell’arteria renale in corso di complicanza infettiva. Due pazienti sono deceduti in seguito a tali complicanze infettive. La DGF ha avuto un’incidenza del 44%. In termini di funzionalità del trapianto non sono state osservate differenze nei valori di creatininemia ed eGFR alla dimissione, a 12 ed a 24 mesi rispetto ai DBD. Le caratteristiche di marginalità del donatore (ECD-DCD o KDPI >65%) correlavano con una più elevata incidenza di DGF e valori di funzione renale peggiori alla dimissione. Nessuno dei fattori analizzati, tra cui lo Score di Karpinsky, ha mostrato correlazione con i valori di creatiniemia e filtrato glomerulare a 12 e 24 mesi.Kidney transplant from donor after circulatory death (DCD) represents a valid choice to increase the incidence of renal transplantation, presenting recipients' and grafts' survival rates comparable to those from brain dead donors (DBD). In January 2016, the Transplant Referral Center in the Emilia Romagna region has started a DCD program. In the present study we report on the first 30 months of the program as far as our own Center in Bologna is concerned, and we provide a comparison with DBD transplants performed over the same period. From January 2016 to September 2018, 16 kidney transplants from 10 DCD donors (5 SCD-DCD and 5 ECD-DCD) have been performed, with two graft-loss at 12 months of follow-up, both due to renal artery rupture caused by infectious arteritis with consequent transplantectomy. Two patients died due to sepsis. Seven (44%) delay graft function (DGF) have been reported. No differences have been found between DCD and DBD in terms of kidney function (serum creatinine and eGFR evaluated at discharge, 12 and 24 months of follow-up). Kidney from marginal donors (ECD-DCD or KDPI >65%) were associated with a higher rate of DGF and worst graft function at discharge. All the predicting factors that have been analysed, including Karpinsky Score, failed to show an association with serum creatinine and eGFR at 12 and 24 months of follow up
[Membranous glomerulonephritis (MGN), ongoing studies]
No full textLa glomerulonefrite membranosa rappresenta il 20% dei casi di sindrome nefrosica dell’adulto, con una incidenza annuale di 1/100.000 pazienti/anno. Negli ultimi 10 anni è stato identificato nel podocita il vero responsabile del processo patologico. In risposta a possibili triggers ambientali in pazienti geneticamente predisposti, il podocita espone epitopi antigenici (recettore della fosfolipasi A2, trombospondina tipo 1) che diventano bersaglio di autoanticorpi in grado di attivare il complemento e determinare danno della membrana basale glomerulare. Rimane incerto il meccanismo effettivamente patogenetico in queste complesse interazioni. Da queste acquisizioni sono derivati nuovi trattamenti focalizzati sui meccanismi specifici di blocco delle vie di attivazione della malattia con l’ipotesi di superamento dei farmaci convenzionali ad attività meno specifica. ll rituximab (Rtx), anticorpo monoclonale diretto contro il CD20 espresso dai linfociti B, è utilizzato in numerosi trials con lo scopo di bloccare la produzione di anticorpi. Il presupposto degli studi in corso deriva dal GEMRITUX trial, in cui Rtx si è dimostrato in grado di indurre remissione della sindrome nefrosica in circa il 65% dei pazienti senza un maggior rischio di eventi avversi; resta da definire lo schema terapeutico e posologico più efficace. Altri trials evidenziano nuovi orientamenti della ricerca su meccanismi di blocco specifico (belimumab) ed aspecifico (ACTH) e più occasionali segnalazioni prendono in considerazione nuove possibili opzioni terapeutiche quali l’ofatumumab, il bortezomib e l’eculizumab. Queste novità in campo patogenetico e terapeutico hanno determinato un impulso alla ricerca sui processi patologici implicati nella genesi delle nefropatie glomerulari ed avviato nuove prospettive di trattamento.The membranous nephropathy (MN) is the major cause of nephrotic syndrome in in the adult, account for 20% of cases with annual incidence is 1 in 100.000. In the past 10 years, the role of podocytes has been identified; environmental triggers in genetically predisposed patients can activate podocytes to exhibit antigenic epitopes (receptor of phospholipase A2, thrombospondin type 1) that become targets of specific autoantibodies with subsequent complement activation. The discovery of this mechanisms has opened new horizons in the therapy of MN and novel drugs are available with more specific mechanism of action. Rituximab, a monoclonal antibody directed against CD20 expressed by lymphocytes B, has been used in several trials and appears able to induce remission of nephrotic syndrome in 60% of patients (GEMRITUX trial) with similar risk profile. Nowadays it remains to define the most effective therapeutic pattern. In MN, the concept of targeting disease control, has permit novel therapies with specific blocking mechanisms (belimumab) and non-specific (ACTH) and new therapeutic options, such as ofatumumab, bortezomib and eculizumab, that have allowed to recognize pathological processes involved in the glomerular diseases
Appropriate Similarity Measures for Author Cocitation Analysis
Full text linkWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
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