The Role of Zinc in the Treatment of Wilson's Disease.

Wilson's disease (WD) is a hereditary disorder of copper metabolism, producing abnormally high levels of non-ceruloplasmin-bound copper, the determinant of the pathogenic process causing brain and hepatic damage and dysfunction. Although the disease is invariably fatal without medication, it is treatable and many of its adverse effects are reversible. Diagnosis is difficult due to the large range and severity of symptoms. A high index of suspicion is required as patients may have only a few of the many possible biomarkers. The genetic prevalence of ATP7B variants indicates higher rates in the population than are currently diagnosed. Treatments have evolved from chelators that reduce stored copper to zinc, which reduces the toxic levels of circulating non-ceruloplasmin-bound copper. Zinc induces intestinal metallothionein, which blocks copper absorption and increases excretion in the stools, resulting in an improvement in symptoms. Two meta-analyses and several large retrospective studies indicate that zinc is equally effective as chelators for the treatment of WD, with the advantages of a very low level of toxicity and only the minor side effect of gastric disturbance. Zinc is recommended as a first-line treatment for neurological presentations and is gaining acceptance for hepatic presentations. It is universally recommended for lifelong maintenance therapy and for presymptomatic WD.

Modelling the long-term evolution of worst-case Arctic oil spills.

We present worst-case assessments of contamination in sea ice and surface waters resulting from hypothetical well blowout oil spills at ten sites in the Arctic Ocean basin. Spill extents are estimated by considering Eulerian passive tracers in the surface ocean of the MITgcm (a hydrostatic, coupled ice-ocean model). Oil in sea ice, and contamination resulting from melting of oiled ice, is tracked using an offline Lagrangian scheme. Spills are initialized on November 1st 1980-2010 and tracked for one year. An average spill was transported 1100km and potentially affected 1.1 million km2. The direction and magnitude of simulated oil trajectories are consistent with known large-scale current and sea ice circulation patterns, and trajectories frequently cross international boundaries. The simulated trajectories of oil in sea ice match observed ice drift trajectories well. During the winter oil transport by drifting sea ice is more significant than transport with surface currents.

Deflector designs for fish habitat restoration.

Paired current deflectors are structures that are installed on each bank of a river to locally reduce the width of the channel, thereby creating flow acceleration and promoting scouring. These instream habitat structures have been used extensively in restoration projects to create pool habitat for fish, but there are many discrepancies in deflector design recommendations in terms of orientation, height, and length. Our objectives were to (1) examine how the angle, height, and length of paired deflectors affect scour hole dimensions and potential for bank erosion; and (2) test the applicability to paired deflectors of existing equations for scour hole depth and volume. Three deflector angles (45 degrees, 90 degrees, and 135 degrees), two deflector heights (with flow under and over the deflector height), and two lengths (reducing the width by 25% and 50%) were investigated using uniform sand in a laboratory flume. Results showed a 26-30% smaller scour depth resulting from 45 degrees deflectors than from 90 degrees deflectors and a 5-10% smaller scour depth for 135 degrees deflectors compared to 90 degrees deflectors. The volume of scour and the potential for bank erosion were greater when flow was under the height of the deflectors rather than overtopping and when the length of deflector was increased. When flow was under the deflector height, 135 degrees deflectors had the highest amount of bank erosion; whereas during overtopping flow conditions, 90 degrees deflectors had the greatest bank erosion potential. Values predicted by the model of Kuhnle and others were closest to observed scour depth and volume measurements. The assumption that upstream-oriented deflectors always generate the largest scour should be revised.