To report a dramatic and immediate clinical and biochemical response during treatment with octreotide in a patient with a functioning mesenteric paraganglioma (PGL).
A 44-year-old woman was admitted ...with a severe hypertensive crisis and a blood pressure reaching 260/150 mm Hg. She was 2 months postpartum and had been previously diagnosed with pre-eclampsia. Secondary hypertension was suspected. This was confirmed by finding a 6 × 5-cm2 retroperitoneal mass located using 68-Gallium DOTA–octreotate positron emission tomography/computed tomography and a grossly elevated plasma catecholamine level of 93 000 pmol/L (normal reference range: 650-2433 pmol/L). Treatment was immediately started with high doses of long- and short-acting octreotide. After 6 weeks and before surgery, the patient was normotensive, with a blood pressure of 120/70 mm Hg and a norepinephrine level of 6000 pmol/L. The tumor resection was uneventful, and histology confirmed the diagnosis. Following the surgery, the patient remained normotensive without any medications.
PGLs and pheochromocytomas are neuroendocrine tumors, and most have receptors for octreotide. This case and another patient previously reported responded dramatically to treatment with a high dose of octreotide. Earlier reports of patients failing to respond are likely to have been the result of using a smaller octreotide dose.
We conclude that high doses of short- and long-acting octreotide are valuable in severely hypertensive patients. Our experience suggests that octreotide is of value in other patients with PGLs and pheochromocytomas. The response is rapid, sustained, effective, and with minimal reported side effects. To the best of our knowledge, this is the first report of a hypertensive crisis in a functional mesenteric PGL.
One hundred and fourteen boys with posterior urethral valves were treated between 1966 and 1975. Four died during the first hospital admission, 6 died from renal failure during childhood, 1 died from ...other causes and 15 were lost to follow-up. Eighty-eight were reviewed 11 to 22 years after diagnosis and the renal outcome of 98 patients is therefore known. Approximately one-third of patients presented under 1 month of age, between 1 month and 1 year, and over 1 year respectively. Bilateral vesicoureteric reflux was observed in one-quarter of the boys, more frequently in those presenting in the first month of life. Half of the patients were treated by primary valve ablation and half underwent temporary upper tract diversion: the outcome was worse for the diverted group. One-third of the boys had a long-term bad outcome for renal function. This outcome was associated with early presentation, bilateral vesicoureteric reflux and day-time urinary incontinence after the age of 5 years. The association of bad outcome with incontinence points to continuing bladder dysfunction as a major determinant of long-term outcome for renal function.
•Sets out evolution of international water policy over the past three decades.•Reviews key debates on scale, participation, markets and water governance goals.•Discusses “scarcity” narratives that ...inform “universal” models of water governance.•Context-specific water governance experience challenges normative universal models.
Since the UN water conference at Mar del Plata in 1977, there have been international debates about how water governance could and should respond to the challenges of sustainable development. New global institutions were established to promote universal norms of governance based on the 1992 “Dublin Principles” and its version of “Integrated Water Resource Management” (IWRM). Many of these prescriptions were contested, not least because of their advocacy of market-based approaches to address what were posed as challenges of scarcity and environmental sustainability.
The paper examines the drivers that have informed different conceptualisations of water governance. It shows how “scarcity” has become central to narratives that sought to focus governance at the river basin scale, to restrict water use in favour of the protection and restoration of water resource ecosystems and to prioritize economic efficiency through market mechanisms. It then reviews the experience of a diverse set of countries, some of which have implemented systemic governance reforms and others whose trajectories have been more evolutionary, driven by domestic contexts.
These practical experiences, supported by a growing understanding of polycentric approaches and how networks cross and link a range of geographic and administrative scales, have given rise to alternatives to the normative IWRM, river basin-focused approaches to water governance. Despite continuing concerns about “planetary environmental boundaries” and transboundary security, these are proving to be weak motivations for adoption of formal global systems of water governance. Instead, new narratives emphasise locally-diverse approaches that see water governed within “problem-sheds” rather than “water-sheds”.
Water governance remains a scene of contestation between local and “global” criteria and developmental and environmental goals. But, in the face of challenges of complexity and diversity and the emerging understanding of network governance, emerging practitioner-oriented guidance is focusing on general principles and explicitly avoiding normative approaches.
The British Society of Gastroenterology (BSG) and the Association of Coloproctology for Great Britain and Ireland (ACPGBI) commissioned this update of the 2002 guidance. The aim, as before, is to ...provide guidance on the appropriateness, method and frequency of screening for people at moderate and high risk from colorectal cancer. This guidance provides some new recommendations for those with inflammatory bowel disease and for those at moderate risk resulting from a family history of colorectal cancer. In other areas guidance is relatively unchanged, but the recent literature was reviewed and is included where appropriate.
The availability of large amounts of high-throughput genomic, transcriptomic and epigenomic data has provided opportunity to understand regulation of the cellular transcriptome with an unprecedented ...level of detail. As a result, research has advanced from identifying gene expression patterns associated with particular conditions to elucidating signalling pathways that regulate expression. There are over 1,000 transcription factors (TFs) in vertebrates that play a role in this regulation. Determining which of these are likely to be controlling a set of genes can be assisted by computational prediction, utilising experimentally verified binding site motifs. Here we present CiiiDER, an integrated computational toolkit for transcription factor binding analysis, written in the Java programming language, to make it independent of computer operating system. It is operated through an intuitive graphical user interface with interactive, high-quality visual outputs, making it accessible to all researchers. CiiiDER predicts transcription factor binding sites (TFBSs) across regulatory regions of interest, such as promoters and enhancers derived from any species. It can perform an enrichment analysis to identify TFs that are significantly over- or under-represented in comparison to a bespoke background set and thereby elucidate pathways regulating sets of genes of pathophysiological importance.
A continent urinary diversion was formed for 16 patients using the Mitrofanoff principle for continence. As originally described, this system used the appendix tunnelled into the bladder to form a ...continent catheterisable vesicostomy. We have expanded the technique and have used all available narrow tubes as continent conduits (ureter 10 cases, appendix 5, Fallopian tube 1). The urine container was made of large and small intestine and bladder in several combinations. The system has been very satisfactory: 14 patients were continent and able to catheterise; 1 required a revision to achieve continence and 1 awaits revision; 3 patients required revision procedures for stricture.
The western United States is experiencing a severe multiyear drought that is unprecedented in some hydroclimatic records. Using gridded drought reconstructions that cover most of the western United ...States over the past 1200 years, we show that this drought pales in comparison to an earlier period of elevated aridity and epic drought in AD 900 to 1300, an interval broadly consistent with the Medieval Warm Period. If elevated aridity in the western United States is a natural response to climate warming, then any trend toward warmer temperatures in the future could lead to a serious long-term increase in aridity over western North America.
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