1,720,967 research outputs found
L'alluvione in Romagna: l'impatto sulle attivitá nefrologiche, i provvedimenti organizzativi e la lezione da imparare
No full textabstract not availabl
Author Response: Teaching NeuroImage: Partially Reversible Widespread Leukoencephalopathy Associated With Atypical Hemolytic Uremic Syndrome
No full textWe greatly appreciated the comment by Dr. Graber on our case.1 To date, kidney involvement associated with TREX1 sequence variations has been sparsely reported in the context of retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S). Specifically, different subtypes of kidney involvement have been described in RVCL-S, including thrombotic microangiopathy (TMA) and glomerulosclerosis.2-4
As for our case, unfortunately, TREX1 analysis was not included in the genetic panel. However, a diagnosis of RVCL-S was unlikely, given the optimal response to eculizumab. Indeed, TREX1-related TMA and leukoencephalopathy have been suggested to be driven by dysregulated type-1 interferon pathways.5 Therefore, the pathophysiologic association between TREX1 sequence variations and endothelial damage is supposed to be independent of complement and should not improve after anticomplement treatment. In addition, after 14 months from onset, no other episodes of leukoencephalopathy occurred in our patient, and she was totally weaned from hemodialysis.
To sum up, even if in our case RVCL-S was a remote possibility, we agree with Dr. Graber's suggestion to consider TREX-1 analysis in patients presenting with acute leukoencephalopathy and small vessel kidney disease. Close collaboration between neurologists and nephrologists remains the key point to promptly recognize such a critical condition
Teaching NeuroImage: Partially Reversible Widespread Leukoencephalopathy Associated With Atypical Hemolytic Uremic Syndrome
No full textA 43-year-old woman presented with altered mental status and hypertension. She had a 3-day history of oliguria. The blood test detected microangiopathic hemolytic anemia, thrombocytopenia, and severe kidney injury. The absence of shiga-like toxin, ADAMTS13 autoantibodies, and normal ADAMTS13 activity were consistent with a diagnosis of atypical hemolytic uremic syndrome (aHUS) and then confirmed by a renal biopsy. Genetic tests (CFH, CFHR1-5, MCP/CD46, CFI, C3, CFB, THBD, and DGKE) were unremarkable. Nevertheless, a history of anemia and kidney failure in her younger brother suggested a genetic etiology.
Brain MRI revealed extensive T2 FLAIR hyperintensities. Treatment with eculizumab and twice-weekly hemodialysis resulted in prompt mental recovery and improvement of MRI abnormalities. aHUS is an ultra-rare complement-mediated kidney disease occasionally associated with neurologic involvement.1 Less extensive T2 FLAIR abnormalities involving every CNS structure have also been reported in diarrhea-associated HUS.2 Here, we presented a case of aHUS-related widespread leukoencephalopathy partially reverting on treatment with eculizumab and hemodialysis
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
Bilateral loin pain and fever in a young woman: yet another pyelonephritis?
No full textNo abstract availabl
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
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
Dispelling the Myths Behind First-author Citation Counts
Full text linkWe conducted a full-scale evaluative citation analysis study of scholars in the XML research field to explore just how different from each other author rankings resulting from different citation counting methods actually are, and to demonstrate the capability of emerging data and tools on the Web in supporting more realistic citation counting methods. Our results contest some common arguments for the continued
use of first-author citation counts in the evaluation of scholars, such as high correlations between author rankings by first-author citation counts and other citation
counting methods, and high costs of using more realistic citation counting methods that are not well-supported by the ISI databases. It is argued that increasingly available digital full text research papers make it possible for citation analysis studies to go beyond what the ISI databases have directly supported and to employ more
sophisticated methods
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