Neonatal-onset multisystem inflammatory disease responsive to interleukin-1 beta inhibition

BACKGROUND:Neonatal-onset multisystem inflammatory disease is characterized by fever, urticarial rash, aseptic meningitis, deforming arthropathy, hearing loss, and mental retardation. Many patients have mutations in the cold-induced autoinflammatory syndrome 1 (CIAS1) gene, encoding cryopyrin, a protein that regulates inflammation.METHODS:We selected 18 patients with neonatal-onset multisystem inflammatory disease (12 with identifiable CIAS1 mutations) to receive anakinra, an interleukin-1-receptor antagonist (1 to 2 mg per kilogram of body weight per day subcutaneously). In 11 patients, anakinra was withdrawn at three months until a flare occurred. The primary end points included changes in scores in a daily diary of symptoms, serum levels of amyloid A and C-reactive protein, and the erythrocyte sedimentation rate from baseline to month 3 and from month 3 until a disease flare.RESULTS:All 18 patients had a rapid response to anakinra, with disappearance of rash. Diary scores improved (P<0.001) and serum amyloid A (from a median of 174 mg to 8 mg per liter), C-reactive protein (from a median of 5.29 mg to 0.34 mg per deciliter), and the erythrocyte sedimentation rate decreased at month 3 (all P<0.001), and remained low at month 6. Magnetic resonance imaging showed improvement in cochlear and leptomeningeal lesions as compared with baseline. Withdrawal of anakinra uniformly resulted in relapse within days; retreatment led to rapid improvement. There were no drug-related serious adverse events.CONCLUSIONS:Daily injections of anakinra markedly improved clinical and laboratory manifestations in patients with neonatal-onset multisystem inflammatory disease, with or without CIAS1 mutations

Shoaling of nonlinear internal waves in Massachusetts Bay

Author Posting. © American Geophysical Union, 2008. This article is posted here by permission of American Geophysical Union for personal use, not for redistribution. The definitive version was published in Journal of Geophysical Research 113 (2008): C08031, doi:10.1029/2008JC004726.The shoaling of the nonlinear internal tide in Massachusetts Bay is studied with a fully nonlinear and nonhydrostatic model. The results are compared with current and temperature observations obtained during the August 1998 Massachusetts Bay Internal Wave Experiment and observations from a shorter experiment which took place in September 2001. The model shows how the approaching nonlinear undular bore interacts strongly with a shoaling bottom, offshore of where KdV theory predicts polarity switching should occur. It is shown that the shoaling process is dominated by nonlinearity, and the model results are interpreted with the aid of a two-layer nonlinear but hydrostatic model. After interacting with the shoaling bottom, the undular bore emerges on the shallow shelf inshore of the 30-m isobath as a nonlinear internal tide with a range of possible shapes, all of which are found in the available observational record.A. Scotti began this project as a Postdoctoral Scholar at the Woods Hole Oceanographic Institution, with support from the Johnson Foundation and the USGS. Further support was provided to Scotti by the Office of Naval Research under grants N00014-01-1-0172, N00014-03-1-0553, and N00014-05-1-0361, and by NSF under grant OCE 07-29636. R. Beardsley was supported by ONR under grants N00014-98-1- 0059, N00014-00-1-0210, and the Smith Chair in Coastal Physical Oceanography. J. Pineda was supported by ONR under grants N00014-01-1-0172, and by a WHOIOcean Life Institute Fellowship

Riverine photosynthesis influences the carbon sequestration potential of enhanced rock weathering

As climate mitigation efforts lag, dependence on anthropogenic CO2 removal increases. Enhanced rock weathering (ERW) is a rapidly growing CO2 removal approach. In terrestrial ERW, crushed rocks are spread on land where they react with CO2 and water, forming dissolved inorganic carbon (DIC) and alkalinity. For long-term sequestration, these products must travel through rivers to oceans, where carbon remains stored for over 10,000 years. Carbon and alkalinity can be lost during river transport, reducing ERW efficacy. However, the ability of biological processes, such as aquatic photosynthesis, to affect the fate of DIC and alkalinity within rivers has been overlooked. Our analysis indicates that within a stream-order segment, aquatic photosynthesis uptakes 1%–30% of DIC delivered by flow for most locations. The effect of this uptake on ERW efficacy, however, depends on the cell-membrane transport mechanism and the fate of photosynthetic carbon. Different pathways can decrease just DIC, DIC and alkalinity, or just alkalinity, and the relative importance of each is unknown. Further, data show that expected river chemistry changes from ERW may stimulate photosynthesis, amplifying the importance of these biological processes. We argue that estimating ERW’s carbon sequestration potential requires consideration and better understanding of biological processes in rivers

Generation and propagation of nonlinear internal waves in Massachusetts Bay

During the summer, nonlinear internal waves (NLIWs) are commonly observed propagating in Massachusetts Bay. The topography of the area is unique in the sense that the generation area (over Stellwagen Bank) is only 25 km away from the shoaling area, and thus it represents an excellent natural laboratory to study the life cycle of NLIWs. To assist in the interpretation of the data collected during the 1998 Massachusetts Bay Internal Wave Experiment (MBIWE98), a fully nonlinear and nonhydrostatic model covering the generation/shoaling region was developed, to investigate the response of the system to the range of background and driving conditions observed. Simplified models were also used to elucidate the role of nonlinearity and dispersion in shaping the NLIW field. This paper concentrates on the generation process and the subsequent evolution in the basin. The model was found to reproduce well the range of propagation characteristics observed (arrival time, propagation speed, amplitude), and provided a coherent framework to interpret the observations. Comparison with a fully nonlinear hydrostatic model shows that during the generation and initial evolution of the waves as they move away from Stellwagen Bank, dispersive effects play a negligible role. Thus the problem can be well understood considering the geometry of the characteristics along which the Riemann invariants of the hydrostatic problem propagate. Dispersion plays a role only during the evolution of the undular bore in the middle of Stellwagen Basin. The consequences for modeling NLIWs within hydrostatic models are briefly discussed at the end

Large internal waves in Massachusetts Bay transport sediments offshore

This paper is not subject to U.S. copyright. The definitive version was published in Continental Shelf Research 26 (2006): 2029-2049, doi:10.1016/j.csr.2006.07.022.A field experiment was carried out in Massachusetts Bay in August 1998 to assess the role of large-amplitude internal waves (LIWs) in resuspending bottom sediments. The field experiment consisted of a four-element moored array extending from just west of Stellwagen Bank (90-m water depth) across Stellwagen Basin (85- and 50-m water depth) to the coast (24-m water depth). The LIWs were observed in packets of 5–10 waves, had periods of 5–10 min and wavelengths of 200–400 m, and caused downward excursions of the thermocline of as much as 30 m. At the 85-m site, the current measured 1 m above bottom (mab) typically increased from near 0 to 0.2 m/s offshore in a few minutes upon arrival of the LIWs. At the 50-m site, the near-bottom offshore flow measured 6 mab increased from about 0.1 to 0.4–0.6 m/s upon arrival of the LIWs and remained offshore in the bottom layer for 1–2 h. The near-bottom currents associated with the LIWs, in concert with the tidal currents, were directed offshore and sufficient to resuspend the bottom sediments at both the 50- and 85-m sites. When LIWs are present, they may resuspend sediments for as long as 5 hours each tidal cycle as they travel westward across Stellwagen Basin. At 85-m water depth, resuspension associated with LIWs is estimated to occur for about 0.4 days each summer, about the same amount of time as caused by surface waves.MBIWE98 was supported by the USGS and the Office of Naval Research (ONR). The long-term observations at LT-A and LT-B were conducted under a Joint Funding Agreement between the USGS and the Massachusetts Water Resources Authority and an Inter-Service Agreement with the US Coast Guard. A. Scotti received support from the WHOI Postdoctoral Scholar program, the Johnson Foundation, the USGS, and ONR through grant N00014-01-1-0172; R. Beardsley through ONR grants N00014-98-1-0059, N00014-00-1-0210 and the WHOI Smith Chair in Coastal Physical Oceanography; and S. Anderson through ONR grant N000140-97-1-0158

Shoaling of nonlinear internal waves in Massachusetts Bay

The shoaling of the nonlinear internal tide in Massachusetts Bay is studied with a fully nonlinear and nonhydrostatic model. The results are compared with current and temperature observations obtained during the August 1998 Massachusetts Bay Internal Wave Experiment and observations from a shorter experiment which took place in September 2001. The model shows how the approaching nonlinear undular bore interacts strongly with a shoaling bottom, offshore of where KdV theory predicts polarity switching should occur. It is shown that the shoaling process is dominated by nonlinearity, and the model results are interpreted with the aid of a two-layer nonlinear but hydrostatic model. After interacting with the shoaling bottom, the undular bore emerges on the shallow shelf inshore of the 30-m isobath as a nonlinear internal tide with a range of possible shapes, all of which are found in the available observational record

Examining the Impact of Stream Permanence on Headwater Stream Carbon Emissions

Quantifying headwater stream carbon emissions is important for our understanding of the global carbon cycle because these emissions (an estimated 0.93-1.15 Pg C year) can be substantial compared to the terrestrial flux. Headwater stream networks can have high emissions due to their coupling with the terrestrial environment and high turbulence with some estimates predicting headwater stream networks can contribute 70% of the global riverine stream emissions. These carbon emissions are challenging to predict, especially with regards to headwater stream network spatiotemporal heterogeneity. The majority of headwater streams exhibit changes in stream network area on a seasonal basis, and these locations and extents are not often well documented because they are based on topographic maps with limited spatial accuracy. Research suggests 50-80% of river networks are comprised of non-perennial stream segments. Physically based models are a potential solution to both mapping streamflow permanence and carbon dioxide emissions by accounting for the spatiotemporal heterogeneity that can occur in stream networks.In this study, we modeled stream permanence at three streams across the United States in different ecosystems using the Watershed Erosion Prediction Project (WEPP) hydrological model to simulate changes in stream network area over the year. We then used these results to inform a process-based stream network model to predict carbon emission from these networks throughout the year. We calibrated these network model with longitudinal data collected at the three sites during both low and high flow. Our results show the importance of considering stream permanence when predicting stream network carbon emissions, and how some ecosystems may emerge as hotspots for these emissions during high flow periods

A modified beam-to-earth transformation to measure short-wavelength internal waves with an acoustic Doppler current profiler

The algorithm used to transform velocity signals from beam coordinates to earth coordinates in an acoustic Doppler current profiler (ADCP) relies on the assumption that the currents are uniform over the horizontal distance separating the beams. This condition may be violated by (nonlinear) internal waves, which can have wavelengths as small as 100-200 m. In this case, the standard algorithm combines velocities measured at different phases of a wave and produces horizontal velocities that increasingly differ from true velocities with distance from the ADCP. Observations made in Massachusetts Bay show that currents measured with a bottom-mounted upward-looking ADCP during periods when short-wavelength internal waves are present differ significantly from currents measured by point current meters, except very close to the instrument. These periods are flagged with high error velocities by the standard ADCP algorithm. In this paper measurements from the four spatially diverging beams and the backscatter intensity signal are used to calculate the propagation direction and celerity of the internal waves. Once this information is known, a modified beam-to-earth transformation that combines appropriately lagged beam measurements can be used to obtain current estimates in earth coordinates that compare well with pointwise measurements