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This paper, considering risk aversion and fair concern, establishes a dynamic price game model of a dual-channel supply chain in which dual-channel retailer sells products through traditional channel and online channel and the online retailer only sells products through online channel. The stability of the system and the influences of different parameter values on utilities are analyzed emphatically using game theory and nonlinear dynamic theory, such as 2D and 3D bifurcation diagram, parameter plot basin, chaos attractor, and sensitivity to initial value. The results find that the system is more likely to lose stability and fall into chaos with the customer demand fluctuating greatly. The system enters into chaos through flip bifurcation with the increase of the price adjustment speed; adjusting the risk-aversion levels or the fairness concern levels of the two retailers can make the system be in a stable state or delay the occurrence of system instability. When the system is in chaos, the average utility of the online retailer will decrease and one of the dual-channel retailers will increase. Using the state feedback control method, the system can return to a stable state from chaos by selecting appropriate control parameters. The research of this paper is of great significance to the decision-makers’ price decision and supply chain operation management.

We study a dual-channel supply chain which consists of one dual-channel manufacturer and one traditional retailer considering fairness concern. The manufacturer takes the market share as one of its business objectives in the competition game. We are devoted to establishing a vertical Nash game model and analyzing the price evolution of the model via the method of complexity theory and take adaptive adjustment control method to control the system’s chaotic state. The results show that an excessive price adjustment speed will hurt the system’s stability as well as profit of the supply chain. A high level fair caring for retailer will push the system to fall into chaos earlier, while a higher level fair caring for manufacturer will enlarge the stability region of the system. Setting the different objectives by the manufacturer will cause drastic competition in dual-channel supply chain. The research of this paper is of great significance to the decision-makers’ price decision and supply chain operation management.

Background: Myotonic dystrophy type 1 (DM1), one of the most common forms of adult-onset muscular dystrophy, is caused by abnormally expanded CTG repeats in the 3′ untranslated region of the DMPK gene. The CUG repeats transcribed from the expanded CTG repeats sequestrate a splicing factor, MBNL1, causing the clinical symptoms in DM1. Nowadays, only symptomatic treatments are available for DM1, and no rational therapy is available. Recently, upregulation of MBNL1 expression has been found to be one of the promising therapies for DM1. Methods: All experiments were conducted in the C2C12 myoblasts and HSALR mice, a DM1 mouse model. Real-time PCR and western blot were used to detect the mRNA and protein level, respectively. The rotarod exercise, grip strength and hanging time were used to evaluate the muscle strength of mice. Results: In this study, we demonstrated that calcitriol, an active form of vitamin D3, increased MBNL1 in C2C12 mouse myoblasts as well as in HSALR mice model for DM1. In HSALR mice model, calcitriol improved muscle strength, and corrected aberrant splicing in skeletal muscle. Besides, calcitriol reduced the number of central nuclei, and improved muscle histopathology in HSALR mice. In addition, we identified that calcitriol upregulated MBNL1 expression via activating the promoter of Mbnl1 in C2C12 myogenic cells. Conclusion: Our study suggests that calcitriol is a potential pharmacological strategy for DM1 that enhances MBNL1 expression. [ABSTRACT FROM AUTHOR]

This article reports two cases of childhood-onset nemaline myopathy diagnosed by muscle pathology and genetic diagnosis. The two patients had onset in early childhood, with muscle weakness as the first manifestation, as well as long disease duration and slow progression. Gomori staining and hematoxylin-eosin staining showed red-stained rods in the sarcoplasmic cytoplasm and sarcolemma under a light microscope. Electron microscopy showed that the dense nemaline rods were located under the muscle fiber sarcolemma and parallel to the long axis of the muscle fibers, and some muscle fiber myofilaments were dissolved and necrotic. Gene testing found that one of the two patients had heterozygous mutation (c.1013A>C) in the ACTA1 gene, and the other had compound heterozygous mutation (c.18676C>T and c.9812C>A) in the NEB gene. The two mutations were more common in nemaline myopathy. Nemaline myopathy is a recessive or dominant inheritance myopathy, in which the nemaline rod in the cytoplasm of myocytes is a characteristic muscle pathological change. Pathological and genetic diagnosis is the gold standard for diagnosis of nemaline myopathy.

The morphological, immunohistochemical, and immunopathological analyses of muscle biopsy are essential for the diagnosis of idiopathic inflammatory myopathies (IIMs). However, they are also one of the most common causes of misdiagnosis. Although several diagnostic criteria have been proposed for the diagnosis of IIMs, misdiagnosis still remains common in clinical practice. The present study aims to characterize the inflammatory profile of IIMs, including the expression of MHC-I, MHC-II, MAC and infiltrating cells. We also investigated the sensitivity and specificity of MHC-I and MHC-II immunostaining for the diagnosis of IIMs. We found that the expression of MHC-I and MHC-II was both higher in IIMs than in non-inflammatory myopathies (NIMs). The distribution of MHC-I in IIMs is different from that of MHC-II. MHC-I is mainly located in the sarcoplasms, while MHC-II is located mostly on the sarcolemmas. Moreover, our findings suggest that MAC may be a potential marker to diagnose DM, and the combination of MHC-I and MHC-II immunostaining results in a higher sensitivity and specificity for IIM diagnosis, especially for DM. In addition, infiltrating cells in PM were mainly CD8+ cells, but we found in DM and NIMs they were primarily CD4+ cells, which is consistent with previous studies. Lastly, glucocorticoid treatment and disease duration have little effect on the MHC-I and MHC-II expression pattern. Our findings indicate that the immunostaining of inflammatory markers such as MHC-I, MHC-II, CD4, CD8, CD303 and MAC are of diagnostic value for IIMs regardless of the immunosuppression regime and disease duration., Competing Interests: None., (IJCEP Copyright © 2018.)

Objective: To study the clinical and pathological features of children with Duchenne muscular dystrophy (DMD), with the aim of increasing the possibility of early diagnosis., Methods: The clinical data of 50 children who were definitely diagnosed with DMD, based on clinical manifestations and the results of skeletal muscle biopsies and monoclonal antibody immunohistochemical staining, was reviewed., Results: The children showed similar clinical manifestations, including running slowly in the toddler period, muscle weakness when climbing stairs and standing up followed by squatting down and walking abnormalities a predominant increase in serum creatine kinase level increased dominantly, and myopathic lesions seen on electromyography. Hematoxylin-eosin staining showed similar pathological presentations in all 50 children, including different-sized muscle fibers with rounding, degeneration and necrosis in various degrees, and proliferation of connective tissues. There was some inflammatory cell infiltration in muscle fibers and interstitial tissues. Dystrophin expression was completely absent at the sarcolemma in all 50 children, and sarcoglycan-α,-β, -',-δ expression was reduced to various degrees in 33 of them., Conclusions: For children with the clinical manifestations mentioned above, skeletal muscle biopsies and monoclonal antibody immunohistochemical staining are recommended as these examinations contribute to a definite diagnosis of DMD by demonstrating dystrophin deficiency at the sarcolemma.

Non-progressive congenital myopathy is a group of muscle diseases occurring at birth or during teenage years. A number of new reports of congenital myopathy, such as homogeneous bodies myopathy, muscle quality control myopathy and type 1 fiber predominance have recently been reported, but they lack of sufficient quantity and constant clinico-pathologic manifestations. This paper reports two cases of congenital myopathy with type 1 fiber predominance confirmed by muscle biopsy. The clinical manifestations of the two children (a 4.5-year-old girl and an 11-year-old boy) included non-progressive symptoms of muscle weakness, skeletal deformities and other clinical features of congenital myopathy. The physical examinations showed a long face or figure and funnel chest or kyphosis/scoliosis, high palatal arch and wing-like shoulder. Serum levels of creatine kinase were normal but slightly elevated serum lactate dehydrogenase levels were noted in the two children. The skeletal muscle biopsy by ATPase staining showed that type 1 fibers accounted for more than 90% of the total number of muscle fibers. No other abnormal pathological changes, such as central cores, muscle tube and central nuclei, were found in the two children.