Bed level motions and sheet flow processes in the swash zone: Observations with a new conductivity-based concentration measuring technique (CCM+)

Detailed measurements of bed level motions and sheet flow processes in the lower swash are presented. The measurements are obtained during a large-scale wave flume experiment focusing on swash zone sediment transport induced by bichromatic waves. A new instrument (CCM+) provides detailed phase-averaged measurements of sheet flow concentrations, particle velocities, and bed level evolution during a complete swash cycle. The bed at the lower swash location shows a clear pattern of rapid erosion during the early uprush and progressive accretion during the middle backwash phase. Sheet flow occurs during the early uprush and mid and late backwash phases. Sheet flow sediment fluxes during these instances are highest in the pick-up layer. Sediment entrainment from the pick-up layer occurs not only during instances of high horizontal shear velocities but also in occurrence of wave–backwash interactions. As opposed to oscillatory sheet flow, the pivot point elevation of the sheet flow layer is time-varying during a swash event. Moreover, the upper sheet flow layer concentrations do not mirror the concentrations in the pick-up layer. Both differences suggest that in the lower swash zone the dynamics of the upper sheet flow layer are not only controlled by vertical sediment exchange (such as in oscillatory sheet flows) but are strongly affected by horizontal advection processes induced by the non-uniformity of the flow

Meta-analysis of the clinical and immunopathological characteristics and treatment outcomes in epidermolysis bullosa acquisita patients

Background: Epidermolysis bullosa acquisita (EBA) is an orphan autoimmune disease. Several clinical phenotypes have been described, but subepidermal blistering is characteristic of all variants. Limited data on clinical and immunopathological characteristics and treatment outcomes in EBA are available. To fill this gap, we collected this information from EBA cases, meeting current diagnostic criteria, published between 1971 and 2016. Results: We identified 1159 EBA cases. This number must be, however, interpreted with caution, as it is not possible to check for multiple reporting. The analysis of all cases indicated that EBA affects all age groups (median: 50 years, range: 1 to 94 years) at an equal gender distribution. Non-mechanobullous (non-MB) forms of EBA were observed in 55% of patients, whereas the mechanobullous variant (MB-EBA) or a combination of both variants was described in 38 or 7% of patients, respectively. Type VII collagen (COL7)-specific autoantibodies were primarily of the IgG isotype, but anti-COL7 IgA, IgM and IgE were also documented. Comparison of the 2 clinical EBA types showed a higher frequency of IgA deposits in non-MB EBA as opposed to MB EBA. Mucous membrane involvement was observed in 23% of patients, and 4.4% of cases were associated with other chronic inflammatory diseases. Of note, IgA deposits were more frequently observed in cases with mucous membrane involvement. Our analysis indicated that EBA is difficult to treat and that the choice of treatment varies widely. Chi square was applied to identify medications associated with complete remission (CR). Considering all EBA cases, intravenous immunoglobulin (IVIG, p = 0.0047) and rituximab (p = 0.0114) were associated with CR. Subgroup analysis demonstrated that no treatment was associated with CR for non-MB EBA, while IVIG (p = 0.003) was associated with CR in MB EBA. Conclusions: Within the limitations of the study, we here document the clinical and immunopathological characteristics and treatment outcomes in a large cohort of EBA patients. The observed associations of single drugs with treatment outcome may serve as a guide to develop clinical trials