Spotlight on MuSK Positive Myasthenia Gravis: Clinical Characteristics, Treatment and Outcomes

Background: To investigate the clinical characteristics, treatment and outcomes of myasthenia gravis with antibodies to the muscle-specific tyrosine kinase (MuSK-MG).Methods: We retrospectively reviewed 21 patients with confirmed MuSK-MG between January 2012 to January 2020 in our center. Detailed clinical data and long-term follow up information were summarized.Results: Females (17/21, 81%) predominated in MuSK-MG and the mean age of onset in this group was 51.86±16.16 years. MuSK-MG patients was divided into three subgroups according to the symptom of muscle weakness at onset: ocular myasthenia gravis (OMG, 47.6%), bulbar myasthenia gravis (BMG, 42.9%), and generalized myasthenia gravis (GMG, 9.5%). The mean progression time from symptoms onset to other muscle groups involvement in OMG patients was 4.38±2.54 months. Pyridostigmine bromide was adopted in 81.0% patients and 90.5% patients received corticosteroids. Compared to the usage in hospital, the median daily dose of corticosteroids decreased significantly at the last follow-up. 85.7% patients received a long-term follow-up with an average time of 1202.17 ± 976.73 days. At the end of the follow-up period: 4.8% patient received complete stable remission, 42.9% patients were in minimal manifestations, 19.0% had improved, 4.8% experienced an unchanged condition and 9.5% patients died.Conclusion: Female patients were more prevalent in this study and MuSK-MG patients had a rapid progress to a generalized state. Although about 50% MuSK-MG patients can achieve a favorable outcome with conventional immunosuppressants, complete stable remission is rare and about 15% respond poorly, more effective medications should be explored in these patients.

Coagulopathy: A Vicious Indicator of COVID-19

COVID-19 is routinely associated with coagulopathy and complications associated with thrombosis. However, the difference between the coagulopathy, which is associated with COVID-19 and the coagulopathy, which is due to different causes, is that the "COVID-19 associated coagulopathy" shows raised levels of D-Dimer and that of fibrinogen. However, it shows quite some abnormalities in the levels of prothrombin time and also in the platelet count. "Venous thromboembolism" and arterial thrombosis is frequently seen in COVID-19 associated coagulopathy as opposed to "disseminated intravascular coagulopathy". Patients suffering from COVID-19 have many have multiple factors in common for thromboembolism which is associated with "Adult respiratory distress syndrome" from different etiologies like generalized inflammation and being unambulatory. “Cytokine storm" is the hallmark of COVID-19 associated coagulopathy which is distinguished by high levels of IL-6,1, tumour necrosis factor and other cytokines. The clinical features of COVID-19 associated coagulopathy overlap that of some syndromes like antiphospholipid syndrome and thrombotic microangiopathy. Studies have shown that patients diagnosed with disseminated intravascular coagulation have a poor prognosis compared to the one's that don't get diagnosed with DIC. The advancement of the condition from coagulopathy in the vasculature of the lungs to DIC in patients who have tested positive for COVID-19 shows that the patient’s dysfunction associated with coagulation has evolved from local to generalized state. Investigating the coagulopathies will help in understanding the mechanism of COVID-19 associated coagulopathy.

MODELING OF MOBILE TMR MIXER OPERATION

A method for determining the duration of the process of preparation and distribution of feed mixtures by a mobile mixer on cattle farms, based on graph theory, is proposed. A generalized state graph of the mobile mixer is presented and its description is given. The final formula for calculating the probability of the mobile mixer being in the state of distribution of a feed mixture consisting of a different number of components is given. To check the method adequacy, time-lapse measurements were performed in production conditions. The projected durability of the mobile mixer was 92.3% (0.36 h) of the actual recorded time of 0.39 h.

Epilogue: Dilapidated

The conclusion reflects on the shifting relations between writing and stone as mediated by a common dilapidation. It reviews how aberrant stones and an unreliable Earth both trouble the novellas and novels considered in this book and perturb the ability of narrative to render a well-plotted world to the breaking point. To test the robustness of these claims the conclusion turns to the lapidary forms of the epigram and epigraph to explore how the shifting valence of stone and the lapidary might index planetary change. Walter Benjamin’s 1939 commentary on Bertolt Brecht’s War Primer epigrams, and in particular on a line written in chalk, offers a primer for commentary in the generalized state of war that is known as the Anthropocene, while also providing a vastly expanded set of coordinates for that commentary.