Defective migration of neuroendocrine GnRH cells in human arrhinencephalic conditions

Patients with Kallmann syndrome (KS) have hypogonadotropic hypogonadism caused by a deficiency of gonadotropin-releasing hormone (GnRH) and a defective sense of smell related to olfactory bulb aplasia. Based on the findings in a fetus affected by the X chromosome–linked form of the disease, it has been suggested that hypogonadism in KS results from the failed embryonic migration of neuroendocrine GnRH1 cells from the nasal epithelium to the forebrain. We asked whether this singular observation might extend to other developmental disorders that also include arrhinencephaly. We therefore studied the location of GnRH1 cells in fetuses affected by different arrhinencephalic disorders, specifically X-linked KS, CHARGE syndrome, trisomy 13, and trisomy 18, using immunohistochemistry. Few or no neuroendocrine GnRH1 cells were detected in the preoptic and hypothalamic regions of all arrhinencephalic fetuses, whereas large numbers of these cells were present in control fetuses. In all arrhinencephalic fetuses, many GnRH1 cells were present in the frontonasal region, the first part of their migratory path, as were interrupted olfactory nerve fibers that formed bilateral neuromas. Our findings define a pathological sequence whereby a lack of migration of neuroendocrine GnRH cells stems from the primary embryonic failure of peripheral olfactory structures. This can occur either alone, as in isolated KS, or as part of a pleiotropic disease, such as CHARGE syndrome, trisomy 13, and trisomy 18

Seeds of good anthropocenes: developing sustainability scenarios for Northern Europe

Scenario development helps people think about a broad variety of possible futures; however, the global environmental change community has thus far developed few positive scenarios for the future of the planet and humanity. Those that have been developed tend to focus on the role of a few common, large-scale external drivers, such as technology or environmental policy, even though pathways of positive change are often driven by surprising or bottom-up initiatives that most scenarios assume are unchanging. We describe an approach, pioneered in Southern Africa and tested here in a new context in Northern Europe, to developing scenarios using existing bottom-up transformative initiatives to examine plausible transitions towards positive, sustainable futures. By starting from existing, but marginal initiatives, as well as current trends, we were able to identify system characteristics that may play a key role in sustainability transitions (e.g., gender issues, inequity, governance, behavioral change) that are currently under-explored in global environmental scenarios. We suggest that this approach could be applied in other places to experiment further with the methodology and its potential applications, and to explore what transitions to desirables futures might be like in different places

Impacts of stronger winds and less sea ice on Canadian Beaufort Sea shelf ecosystems since the late 1990s

Continuous and multi-decadal records of faunal abundance and diversity helping to identify the impacts of ongoing global warming on aquatic ecosystems are rare in the coastal Arctic. Here, we used a 50-year-long microfaunal record from a sediment core collected in the Herschel Basin (YC18-HB-GC01; 18 m water depth) to document some aspects of the environmental responses of the southern Canadian coastal Beaufort Sea to climate change. The microfaunal indicators include benthic foraminiferal assemblages, ostracods and tintinnids. The carbonate shells of two foraminiferal species were also analyzed for their stable isotope signatures (δ13C and δ18O). We compiled environmental parameters from 1970 to 2019 for the coastal region, including sea ice data (break-up date, freeze-up date, open season length and mean summer concentration), the wind regime (mean speed, direction of strong winds and the number of storms), hydrological data (freshet date, freshet discharge and mean summer discharge of the Firth and the Mackenzie rivers), and air temperature. Large-scale atmospheric patterns were also taken into consideration. Time-constrained hierarchal clustering analysis of foraminiferal assemblages and environmental parameters revealed a near-synchronous shift around the late 1990s. The microfaunal shift corresponds to an increased abundance of taxa tolerant to variable salinity, turbulent bottom water conditions, and turbid waters towards the present. The same time interval is marked by stronger easterly winds, more frequent storms, reduced sea-ice cover, and a pervasive anticyclonic circulation in the Arctic Ocean (positive Arctic Ocean Oscillation; AOO+). Deeper vertical mixing in the water column in response to intensified winds was fostered by increased open surface waters in summer leading to turbulence, increased particle loading and less saline bottom waters at the study site. Stronger easterly winds probably also resulted in enhanced resuspension events and coastal erosion in addition to a westward spreading of the Mackenzie River plume, altogether contributing to high particulate-matter transport. Increase food availability since ∼2000 was probably linked to enhanced degradation of terrestrial organic carbon, which also implies higher oxygen consumption. The sensitivity of microfaunal communities to environmental variations allowed capturing consequences of climate change on a marine Arctic shelf ecosystem over the last 50 years

Opinion article: Neurosurgical treatment for neuro-ophthalmologic conditions

A significant role of the neuro-ophthalmologist is to counsel patients on appropriate management and anticipated visual prognosis for conditions affecting the afferent and efferent visual systems, including those requiring neurosurgical treatment. However, the literature regarding anticipated neuro- ophthalmologic prognosis after neurosurgical intervention for cerebral aneurysms, sellar lesions, optic pathway tumors, and elevated intracranial pressure is limited with many key questions unanswered. For example, if a cerebral aneurysm is equally amenable to clipping or endovascular coiling, is there a preferred approach in terms of visual prognosis based on aneurysm location? Is dural venous sinus stenting (VSS) for idiopathic intracranial hypertension (IIH) superior, equivalent or inferior to shunting in terms of visual recovery and safety profile? Landmark studies on pituitary tumors using pre-operative optical coherence tomography (OCT) imaging of the optic nerve head to predict visual recovery after surgical decompression of the optic chiasm have changed neuro-ophthalmologic practice and enabled patients to be better informed regarding expected visual outcomes. 1,2 In order to optimize an interdisciplinary team approach to patient care, further studies of visual outcomes for neuro- ophthalmologic conditions requiring neurosurgical intervention are needed

The Pre-Modern Manuscript Trade and its Consequences, ca. 1890-1945

This collection brings together current research into the development of the market for pre-modern manuscripts. Between 1890 and 1945 thousands of manuscripts made in Europe before 1600 appeared on the market. Many entered the collections in which they have remained, shaping where and how we encounter the books today. These collections included libraries that bear their founders’ names, as well as national and regional public libraries. The choices of the super-rich shaped their collections and determined what was available to those with fewer resources. In addition, wealthy collectors sponsored scholarship on their manuscripts and participated in exhibitions, raising the profile of some books. The volume examines the collectors, dealers, and scholars who engaged with pre-modern books, and the cultural context of the manuscript trade in this era

Abnormal plasma polyunsaturated fatty acid pattern in non-active inflammatory bowel disease

An abnormal plasma polyunsaturated fatty acid pattern (PUFA) (increased n3 and decreased n6 PUFA) has been reported in active inflammatory bowel disease (IBD). The possibility of a primary defect in the PUFA metabolism in IBD was hypothesised. The aim of this study was to assess plasma PUFA pattern in inactive inflammatory bowel disease and to ascertain whether patients who had had a colectomy and who were suffering from ulcerative colitis have a similar PUFA pattern than those patients with non-active ulcerative colitis and who had not had a colectomy. Plasma fatty acids were analysed by semi-capillary column gas-liquid chromatography in three groups of patients with inactive IBD (24 patients with inactive ulcerative colitis who had not had a colectomy, 15 patients with ulcerative colitis who had had a colectomy, and 27 patients with Crohn's disease). Plasma concentration and percentage of C22:6n3 and unsaturation index were significantly higher in patients with inactive ulcerative colitis without a colectomy and the Crohn's disease group (p < 0.0001) than in controls. Plasma concentration and percentage of C22:6n3 and the unsaturation index remained significantly higher, in both the operated and non-operated ulcerative colitis patients when compared with controls (p < 0.0001). These results suggest that in inactive IBD, an increased PUFA biosynthesis might be the cause of the high values of n3 compounds. These findings although seen in active disease, are more noticeable in remission because of the lack of artefactual factors (malnutrition, steroids, inflammation). In addition, persistence of high values in both groups of ulcerative colitis patients--that is, those who had had a colectomy and those who had not suggests the existence of a primary abnormality in the PUFA metabolism in IBD

Regional and annual variability in common eider nesting ecology in Labrador

Nesting densities are often used to estimate breeding population size and with other measures of reproductive performance can be useful indicators of population status. These aspects of breeding biology often show considerable spatial and temporal variation. Between 2000 and 2003, we surveyed nesting common eiders (Somateria mollissima) on 172 islands in three archipelagos (Nain, Hopedale, Rigolet) on the Labrador coast. Rigolet was the largest archipelago (2834 km2) followed by Nain then Hopedale, and island density varied inversely with archipelago size. Overall means were: nest density 52.0 ± 141.9 (SD) nests/ha; nest initiation 12 June ± 12 days; clutch size 3.7 ± 1.2 eggs/nest; egg volume 98.8 ± 10.4 cm3; and clutch volume 392.3 ± 135.0 cm3. Rigolet had the highest average egg volumes and nest densities, the highest single island nest density of 1053 nests/ha, and the earliest average nest initiation date. We found significant differences in nest densities among archipelagos and across years; significant archipelago and year interactions were detected for nest initiation date and clutch size. Signifi cant differences were found among individual islands for all response variables except egg volume. For egg volume, within-archipelago island differences were not signifi cant, but between-archipelago differences were signifi cant. Thus egg volume may be a useful diagnostic to identify population affi liation