Plasma exchange for two patients with autoimmune GFAP astrocytopathy with rapid progression to respiratory failure: a case report

BackgroundPatients with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy can present with early neurological deterioration, but rapidly progressive respiratory failure is rarely reported. We present the cases of two patients with autoimmune GFAP astrocytopathy who experienced rapid progression to respiratory failure and were effectively treated using plasma exchange therapy.Case reportTwo patients were diagnosed with autoimmune GFAP astrocytopathy. Their initial symptoms were consistent with those of previously observed cases of autoimmune GFAP astrocytopathy. However, they experienced rapid progression to respiratory failure due to their lesion location. Specifically, case 1 had lesions in the medulla oblongata, and case 2 had lesions in the high cervical spinal cord, which are both common sites of lesions causing respiratory failure. The patients did not respond well to intravenous methylprednisolone and intravenous immunoglobulin initially and could not be withdrawn from ventilator support. Fortunately, subsequent plasma exchange therapy led to significant clinical improvements and successful withdrawal from ventilator support.DiscussionPatients with autoimmune GFAP astrocytopathy can present with rapidly progressive respiratory failure. Early treatment with plasma exchange can be beneficial in withdrawing patients from ventilator support

Soft Roof Failure Mechanism and Supporting Method for Gob-Side Entry Retaining

To study the soft roof failure mechanism and the supporting method for a gateway in a gently inclined coal seam with a dip angle of 16° kept for gob-side entry retaining, and through the methodology of field investigation and numerical and analytical modeling, this paper analyzed the stress evolution law of roof strata at the working face end and determined that the sharp horizontal stress unloading phenomenon along the coal wall side did not appear after the working face advanced. Conversely, the horizontal stress along the gob side instantly decreased and the tensile stress produced, and the vertical stress in the central part of the roof had a higher reduction magnitude as well. An in-depth study indicates that the soft roof of the working face end subsided and seriously separated due to the effect of the front abutment pressure and the roof hanging length above the gob line, as well as certain other factors, including the rapid unloading of the lateral stress, tension and shear on the lower roof rock layer and dynamic disturbance. Those influencing factors also led to rapid crack propagation on a large scale and serious fracturing in the soft roof of the working face end. However, in the gob stress stabilized zone, the soft roof in the gob-side entry retaining has a shearing failure along the filling wall inside affected by the overburden pressure, rock bulking pressure, and roof gravity. To maintain the roof integrity, decrease the roof deformation, and enable the control of the working face end soft roof and the stabilization of the gob-side entry retaining roof, this study suggests that the preferred bolt installation angle for the soft roof situation is 70° based on the rock bolt extrusion strengthening theory

Mesomechanics coal experiment and an elastic-brittle damage model based on texture features

To accurately describe damage within coal, digital image processing technology was used to determine texture parameters and obtain quantitative information related to coal meso-cracks. The relationship between damage and mesoscopic information for coal under compression was then analysed. The shape and distribution of damage were comprehensively considered in a defined damage variable, which was based on the texture characteristic. An elastic-brittle damage model based on the mesostructure information of coal was established. As a result, the damage model can appropriately and reliably replicate the processes of initiation, expansion, cut-through and eventual destruction of microscopic damage to coal under compression. After comparison, it was proved that the predicted overall stress-strain response of the model was comparable to the experimental result. Keywords: Mesomechanics experiment, Image processing, Texture feature, Damage variable, Damage mode

Genome-Wide Identification and Comparative Analysis of Albumin Family in Vertebrates

Albumins are the most well-known globular proteins, and the most typical representatives are the serum albumins. However, less attention was paid to the albumin family, except for the human and bovine serum albumin. To characterize the features of albumin family, we have mined all the putative albumin proteins from the available genome sequences. The results showed that albumin is widely distributed in vertebrates, but not present in the bacteria and archaea. The phylogenetic analysis of vertebrate albumin family implied an evolutionary relationship between members of serum albumin, α-fetoprotein, vitamin D–binding protein, and afamin. Meanwhile, a new member from the albumin family was found, namely, extracellular matrix protein 1. The structural analysis revealed that the motifs for forming the internal disulfide bonds are highly conserved in the albumin family, despite the low overall sequence identity across the family. The domain arrangement of albumin proteins indicated that most of vertebrate albumins contain 3 characteristic domains, arising from 2 evolutionary patterns. And a significant trend has been observed that the albumin proteins in higher vertebrate species tend to possess more characteristic domains. This study has provided the fundamental information required for achieving a better understanding of the albumin distribution, phylogenetic relationship, characteristic motif, structure, and new insights into the evolutionary pattern

Case Report: Ofatumumab treatment in a patient with rituximab-intolerant refractory autoimmune GFAP astrocytopathy

BackgroundOfatumumab, a fully humanized anti-CD20 monoclonal antibody, has shown promising efficacy in limited cases of neuromyelitis optica spectrum disorder, but there is a lack of studies on its use in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. We present a case of refractory GFAP astrocytopathy with poor response to conventional immunosuppressants and rituximab who responded well to subcutaneous ofatumumab.Case reportThe patient is a 36-year-old woman with a diagnosis of GFAP astrocytopathy and high disease activity. She experienced five relapses over three years despite immunosuppressive treatment with oral prednisone, azathioprine, mycophenolate mofetil, and intravenous rituximab. Additionally, her circulating B cells were not completely depleted during the second administration of rituximab and an allergic reaction occurred. Based on insufficient B cell depletion and allergic reaction to rituximab, subcutaneous ofatumumab was introduced. After twelve injections of ofatumumab without injection-related reactions, she had no further relapses and was sufficiently depleted of the circulating B cells.DiscussionThis case illustrates the effective use and good tolerance of ofatumumab in GFAP astrocytopathy. Further studies are needed to investigate the efficacy and safety of ofatumumab in refractory GFAP astrocytopathy or those intolerant to rituximab

Elasto-plastic analysis of a circular borehole in elastic-strain softening coal seams

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Load Bearing and Deformation Characteristics of Granular Spoils under Unconfined Compressive Loading for Coal Mine Backfill

The load bearing capacity and deformation response of granular spoils under uniaxial compression are numerically and experimentally investigated, aiming to shed light on the performance of back filled waste spoils while controlling ground subsidence after coal extraction. In numerical study, the particles are assembled in PFC commercial code in light of the digitized real shape of spoils with image technique, which is proved to be consistent with the physical test. The results from numerical and laboratory experiments showed that the complete compressive process of spoils tended to have spatial and temporal characteristics. The load-strain curves of investigated specimens could be divided into three stages (stage I, rearranging stage; stage II, breaking stage; stage III, consolidating stage) and three zones (I, rearranging zone; II, interlocking zone; III, consolidated zone) from outside to inside. During stage I, the load increasing rate of smaller spoils is relatively low, but it increases faster than larger ones in stages II and III. In addition, spoils with Talbot’s gradation are greater than single gradations. The magnitude of the density in consolidated zone is maximum, indicating that it is the main part holding the overlying strata weight

Experimental investigation on the dynamic response of an innovative semi-submersible floating wind turbine with aquaculture cages

Floating offshore wind turbines integrating with other functional devices can not only produce green electricity but also realize three-dimensional utilization of ocean space. Therefore, an innovative concept of semi -submersible floating wind turbine with aquaculture cages is proposed here. Correspondingly, its dynamical behaviors are required to be examined and investigated while various oceanic conditions are considered, including but not limited to an irregular wave condition and combined wind and wave conditions. Hence, a series of model tests in 1:40 scale is carried out in Shanghai Jiao Tong University Ocean Engineering Basin. Firstly, the scaling law and experimental setup are interpreted in detail. Calibrations of the wind generator and wave makers are carefully performed to ensure the accuracy and reliability of model tests. Then the dynamic responses of the new floating wind-aquaculture platform (FWAP) are studied experimentally, including the periods, damping, motions, accelerations and tower top loads. The results show that the existence of nets mainly has a damping effect on the dynamics of the new FWAP under different sea states. The motions, accelerations and tower top loads can be significantly increased when the FWAP undergoes 50-year extreme sea state. When the new FWAP is subjected to combined wind and wave, more complicated dynamic responses can be detected due to the rotation of wind turbine. The results can serve as a reference basis for the improvement of the new FWAP