RESUMEN
Most cases associated with Hereditary Severe Insulin Resistance Syndrome (H-SIRS) are linked to mutations in the insulin receptor (INSR) gene. Patients with H-SIRS typically manifest symptoms of hyperinsulinemia, insulin resistance, and diabetes mellitus. Other symptoms include impaired glucose regulation, hyperandrogenism, and the presence of acanthosis nigricans (AN). In this report, we present two cases of H-SIRS in female children exhibiting various symptoms, such as hyperinsulinemia, fasting hypoglycemia, postprandial hyperglycemia, overweight, fatty liver, hyperandrogenism, and varying degrees of AN. One patient also presented with mental retardation. Gene sequencing identified specific mutations in the INSR gene for both patients: c.2663A > G (p.Tyr888Cys) and c.38_61del (p.Pro13_Ala20del). These mutations have the potential to disrupt the interaction between INSR and insulin, leading to abnormal insulin signaling, insulin resistance, and various clinical manifestations.
RESUMEN
Red tourism is a distinctive form of tourism in China. Its network attention serves as a typical indicator to measure the level of promotion and publicity for red tourism, as well as an important reflection of its influence. Understanding the network structure of red tourism is of significant importance for optimizing the spatial pattern of tourism and promoting the development of the tourism industry. Based on this, this study takes the classic red tourism attractions in Shaanxi province, China as an example and constructs a multi-source data network attention evaluation index. Additionally, it employs social network theory to explore the network attention and tourist flow characteristics of the case study area. Research shows that: (1) Overall, the network attention to case-based destinations is relatively low, and there are significant differences in network attention among different attractions. Spatially, the distribution of network attention is uneven. This is manifested by higher network attention to attractions in Yan'an city and lower network attention to attractions in other regions. (2) There are differences in the network attention of different types of attractions. High-level attractions have a higher level of online attention, while low-level attractions have a lower level of network attention. Additionally, archaeological sites tend to receive a higher level of online attention. (3) The network density of tourist flow is low, and the tourism connections between nodes are not closely linked. The linkage between core nodes and edge nodes in tourism is poor. Developed tourism routes only exist in core nodes. (4) Nodes such as Zaoyuan revolution site, Yangjialing revolution site, and Wangjiaping revolution site have a significant influence in the network structure. In addition, the integration and development between red nodes and non-red nodes have been achieved. (5) There is a correlation between network attention and tourist flow, as well as a 'misplacement' feature. Based on the characteristics of attractions, they can be divided into four types: bright-star attractions, cash-cow attractions, thin-dog attractions, and question attractions. Based on the above conclusions, this study proposes targeted development recommendations.
