3 research outputs found

    Kinetics of Gravity-Driven Water Channels Under Steady Rainfall

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    We investigate the formation of fingered flow in dry granular media under simulated rainfall using a quasi-2D experimental set-up composed of a random close packing of mono-disperse glass beads. Using controlled experiments, we analyze the finger instabilities that develop from the wetting front as a function of fundamental granular (particle size) and fluid properties (rainfall, viscosity).These finger instabilities act as precursors for water channels, which serve as outlets for water drainage. We look into the characteristics of the homogeneous wetting front and channel size as well as estimate relevant time scales involved in the instability formation and the velocity of the channel finger tip. We compare our experimental results with that of the well-known prediction developed by Parlange and Hill [1976]. This model is based on linear stability analysis of the growth of perturbations arising at the interface between two immiscible fluids. Results show that in terms of morphology, experiments agree with the proposed model. However, in terms of kinetics we nevertheless account for another term that describes the homogenization of the wetting front. This result shows that the manner we introduce the fluid to a porous medium can also influence the formation of finger instabilities.Comment: 13 pages, 7 figure

    JAK inhibition in Aicardi-Goutières syndrome: a monocentric multidisciplinary real-world approach study

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    International audienceThe paradigm type I interferonopathy Aicardi-Goutières syndrome (AGS) is most typically characterized by severe neurological involvement. AGS is considered an immune-mediated disease, poorly responsive to conventional immunosuppression. Premised on a chronic enhancement of type I interferon signaling, JAK1/2 inhibition has been trialed in AGS, with clear improvements in cutaneous and systemic disease manifestations. Contrastingly, treatment efficacy at the level of the neurological system has been less conclusive. Here, we report our real-word approach study of JAK1/2 inhibition in 11 patients with AGS, providing extensive assessments of clinical and radiological status; interferon signaling, including in cerebrospinal fluid (CSF); and drug concentrations in blood and CSF. Over a median follow-up of 17 months, we observed a clear benefit of JAK1/2 inhibition on certain systemic features of AGS, and reproduced results reported using the AGS neurologic severity scale. In contrast, there was no change in other scales assessing neurological status; using the caregiver scale, only patient comfort, but no other domain of everyday-life care, was improved. Serious bacterial infections occurred in 4 out of the 11 patients. Overall, our data lead us to conclude that other approaches to treatment are urgently required for the neurologic features of AGS. We suggest that earlier diagnosis and adequate central nervous system penetration likely remain the major factors determining the efficacy of therapy in preventing irreversible brain damage, implying the importance of early and rapid genetic testing and the consideration of intrathecal drug delivery
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