Cleidocranial dysplasia is a rare autosomal-dominant condition that affects ossification. The main symptoms of this cleidocranial dysplasia patient include craniofacial abnormalities, hypoplasia of clavicles, narrow thorax, retarded exfoliation of deciduous teeth, retarded eruption of permanent teeth, and multiple impacted supernumerary teeth. The aim of this treatment was to correct the craniofacial abnormalities and provide an adequate functional reconstruction of the occlusion. The patient was treated with a multidisciplinary therapeutic protocol including removal of supernumerary teeth, surgical exposure of impacted teeth, orthodontic, and orthognathic surgery. After 6 years’ follow-up, the treatment objectives of acceptable occlusion, normal function, and acceptable profile were achieved.
Ciliated and secretory cells are two major cell types that comprise the oviduct epithelia. Accumulating evidences support a role of oviductal multiciliated epithelia for embryo transport, however the mechanisms underlying this specialized cell type differentiation remain elusive. Here, we report that CDC42 depletion in oviduct epithelia hampers the morphogenesis of multiciliated cell, and results in embryo retention, leading to early pregnancy failure. Utilizing the oviduct organoid model, we further observed that CDC42 guides secretory cells transition into multiciliated cells independent of its GTPase activity and the well-known Notch pathway. Further exploration uncovered the AKT as a novel indispensable regulator for multiciliated cells differentiation, whose activity was maintained by CDC42 through interacting with the p110β. Consistently, re-activating AKT partially incites multiciliated cells differentiation in Cdc42 knockout oviductal organoids. Finally, low levels of CDC42 and phospho-AKT with reduced multiciliated cells in the oviduct are observed in women with ectopic pregnancy. Collectively, we provide previously unappreciated evidence that CDC42-AKT signaling is a critical determinant for morphogenesis of oviduct multiciliated cell, which possesses the clinical application in understanding the pathology of ectopic pregnancy and facilitating the development of prevention strategies.
The S region on the full characteristic curve of pump-turbine is one of the important key features that affect its operation stability. In S region, one specific rotational speed value ( n11) corresponds to three specific flow rate values ( Q11), which causes the unit to fluctuate between different operation conditions. In this paper, focusing on the special region of pump-turbine, based on the combination of model experiment and numerical simulation, the S-region dynamic model of pump-turbine is established. The internal flow mechanism of pump-turbine is analyzed in detail from the perspective of Flow Energy Dissipation (FED). The results show that the high FED component is different at different operating points, but the flow energy maintain balance in the pump-turbine unit. High FED area displayed in near-wall or inter-channel of different components under different flow conditions. Besides, the generation of S region is related to the dynamic balance of energy especially in the no-load region. The analysis results of this paper provide theoretical basis for scientific and stable operation of pump-turbine.
The axial-flow turbomachinery is important in water supply and drainage. However, there is a gap between the blade tip and the casing, which is easy to cause tip leakage vortex. Tip leakage of axial-flow turbomachinery has bad impact on the operation stability. In this case, the numerical simulation is used to study the simplified hydrofoil model with clearance. Then the change of tip leakage vortex core position and the pressure distribution are analyzed. Results show that with the fluid flows downstream, the longitudinal position of the tip leakage vortex core first drops and then rises. On different streamwise planes, the vortex core has the lowest pressure. From the downstream to the vortex generating position, the vortex core pressure decreases gradually, and the cavitation is most likely to occur at the location where the vortex occurs.
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