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An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome
Background
In 2009, the European Paediatric Study Group for Haemolytic Uraemic Syndrome (HUS) published a clinical practice guideline for the investigation and initial therapy of diarrhea-negative HUS (now more widely referred to as atypical HUS, aHUS). The therapeutic component of the guideline (comprising early, high-volume plasmapheresis) was derived from anecdotal evidence and expert consensus, and the authors committed to auditing outcome.
Methods
Questionnaires were distributed to pediatric nephrologists across Europe, North America, and the Middle East, who were asked to complete one questionnaire per patient episode of aHUS between July 1, 2009 and December 31, 2010. Comprehensive, anonymous demographic and clinical data were collected.
Results
Seventy-one children were reported with an episode of aHUS during the audit period. Six cases occurred on a background of influenza A H1N1 infection. Of 71 patients, 59 (83 %) received plasma therapy within the first 33 days, of whom ten received plasma infusion only. Complications of central venous catheters occurred in 16 out of 51 patients with a catheter in-situ (31 %). Median time to enter hematological remission was 11.5 days, and eight of 71 (11 %) patients did not enter hematological remission by day 33. Twelve patients (17 %) remained dialysis dependent at day 33.
Conclusions
This audit provides a snapshot of the early outcome of a group of children with aHUS in the months prior to more widespread use of eculizumab.
Journal Article
Enterohaemorrhagic Escherichia coli and Shigella dysenteriae type 1-induced haemolytic uraemic syndrome
Haemolytic uraemic syndrome (HUS) can be classified according to the aetiology of the different disorders from which it is composed. The most prevalent form is that induced by shigatoxin producing
Escherichia coli
(STEC) and, in some tropical regions, by
Shigella dysenteriae
type 1. STEC cause a zoonosis, are widely distributed in nature, enter the food chain in different ways, and show regional differences. Not all STEC are human pathogens. Enterohaemorrhagic
E. coli
usually cause attachment and effacing lesions in the intestine. This is not essential, but production of a shigatoxin (Stx) is. Because Stx are encoded by a bacteriophage, this property is transferable to naïve strains. Laboratory methods have improved by identifying STEC either via the toxin or its bacteriophage.
Shigella dysenteriae
type 1 produces shigatoxin, identical to Stx-1, but also has entero-invasive properties that enterohaemorrhagic
Escherichia coli
(EHEC) do not. Shigella patients risk bacteremia and benefit from early antibiotic treatment, unlike those with EHEC.
Journal Article
Reform the PhD system or close it down
The academic job market collapsed in the 1970s, yet universities have not adjusted their admissions policies, because they need graduate students to work in laboratories and as teaching assistants. Prestige is measured both within and beyond institutions by the number and purported strength of a department's doctoral programmes, so, seeking competitive advantage and financial gain from alliances with the private sector, universities continue to create them.
Journal Article
Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome
This guideline for the investigation and initial treatment of atypical hemolytic uremic syndrome (HUS) is intended to offer an approach based on opinion, as evidence is lacking. It builds on the current ability to identify the etiology of specific diagnostic sub-groups of HUS. HUS in children is mostly due to infection, enterohemorrhagic
Escherichia coli
(EHEC),
Shigella dysenteriae
type 1 in some geographic regions, and invasive
Streptococcus pneumoniae
. These sub-groups are relatively straightforward to diagnose. Their management, which is outside the remit of this guideline, is related to control of infection where that is necessary and supportive measures for the anemia and acute renal failure. A thorough investigation of the remainder of childhood HUS cases, commonly referred to as “atypical” HUS, will reveal a risk factor for the syndrome in approximately 60% of cases. Disorders of complement regulation are, numerically, the most important. The outcome for children with atypical HUS is poor, and, because of the rarity of these disorders, clinical experience is scanty. Some cases of complement dysfunction appear to respond to plasma therapy. The therapeutic part of this guideline is the consensus of the contributing authors and is based on limited information from uncontrolled studies. The guideline proposes urgent and empirical plasmapheresis replacement with whole plasma fraction for the first month after diagnosis. This should only be undertaken in specialized pediatric nephrology centers where appropriate medical and nursing skills are available. The guideline includes defined terminology and audit points so that the early clinical effectiveness of the strategy can be evaluated.
Journal Article
Last works : lessons in leaving
For many today, retirement and the leisure said to accompany it have become vestiges of a slower, long-lost time. In a world where the sense of identity is tied to work and careers, to stop working often is to become nobody. In this deeply perceptive and personal exploration of last works, Mark C. Taylor explores the final reflections of writers and thinkers from Kierkegaard to David Foster Wallace. How did they either face or avoid ending and leaving? What do their lessons in ending teach us about living in the time that remains for us? Some leavings brought relief, even joy, while others brought pain and suffering. Whether the cause was infirmity, impending death, or simply exhaustion and ennui, the ways these influential voices fell silent offer poignant examples of people negotiating the challenges of ending. Throughout this profound and moving book, Taylor probes how the art of living involves learning to leave gracefully.--Jacket.
Book
