2014年 · 第94卷第41期

At present, many disasters in the world have serious consequences. Once a disaster occurs, the most important thing is to save human lives. How to carry out scientific and efficient emergency medical rescue has become a hot topic in various countries. Especially in recent years, developed countries such as the United States, Canada, Britain and Japan have taken the information transformation as an opportunity in the field of emergency medical rescue, and continuously increased their fiscal budgets every year to build a more agile and solid emergency mechanism and management system, aiming at effectively responding to various emergencies that threaten human normal production and life. The United States has built a relatively complete Emergency "Emergency Management System" (EMS), five European countries have developed a "Major Emergency Intelligent Management System" (MEMS) under the Eureka Plan, and Japan has also built a "Disaster Response System" (DRS) for natural disasters. The established system, such as Phoenix Disaster Management System used in Hyogo Prefecture, Japan, is a disaster prevention response system based on the practical experience and lessons of Hanshin earthquake. Phoenix Disaster Management System is the core system of Hyogo Disaster Prevention Center and the most perfect disaster prevention emergency system in Japan. These projects and systems have played a positive role in human response to a variety of disasters. At present, China has not established a complete injury assessment and rescue decision-making system for dealing with sudden disasters. In the face of complex sudden major disasters, how to define the number of casualties, injuries and types of injuries, whether there are enough local rescue forces to deal with them, and how to draw reasonable personnel and equipment for emergency rescue teams often rely more on comprehensive plans and empirical decision-making. The preparations for emergency medical rescue teams lack predictability and pertinence. After the disaster, a large number of rescue organizations are integrated into the incident site, resulting in congestion of rescue routes, surge of local load, lack of effective preparation and scattered rescue operations. In order to solve the above problems and improve the efficiency of emergency rescue, it is necessary to develop a major disaster injury assessment and rescue decision support system. The system is implemented by JavaEE platform, and Struts, Spring and Hibernate frameworks are applied to the emergency rescue model, and WebGIS, web crawling and cloud push technologies are adopted to realize data acquisition and user interaction. It can realize the general assessment of casualties caused by emergencies such as earthquakes, floods, fires, mudslides, infectious diseases, violent terrorist activities, etc., and on the premise that the destructive force of disasters exceeds the local emergency medical force and needs external support, it can generate emergency plans of users' personnel, equipment, medicinal materials and routes in a targeted manner, which can not only provide auxiliary decision-making for rescue preparation, but also integrate with the "emergency medical rescue platform" to provide early data management services for the whole rescue operation.

A 15-year-old male was diagnosed with dermatomyositis. He was admitted to the hospital due to rash, facial edema and fatigue for 1 week. Physical examination: Body temperature 37.1 ℃. He likes to lie on the right side, with large erythema on the face, not higher than the skin surface, and does not fade when pressed, edema on the face, and a surgical scar of about 15 cm can be seen in the middle of the sternum. The breathing sounds of both lungs were slightly thick, no dry or wet rales were heard, no abnormalities in cardiac signs, no palpation of the liver and spleen under the costs, small superficial lymph nodes, no tenderness in the muscles of the limbs, and weakened muscle strength, grade 3. Previous ventricular septal repair. On admission, CT from outside hospital reported pulmonary infectious lesions, which may be fungal infection. The chest radiograph was basically normal 3 days before admission (FIG. 1A), CT showed that the scope of the lesion was significantly enlarged at admission, showing exudative foci and small nodular foci with vague margins, accompanied by mild emphysema, mainly distributed in the right upper lobe and right lower lobe, and there were almost no lesions in the left lobe (FIG. 1B，Figure 1C，Figure 1D), the lesions have the characteristics of gravity distribution, which is not in line with the common leaf segmental distribution characteristics of infectious lesions. Patients with dermatomyositis have aspiration factors due to esophageal muscle involvement. Patients like to lie in the right lateral position with postural factors. At the same time, there are no obvious infection symptoms such as cough, expectoration and fever. Therefore, imaging combined with clinical diagnosis was dermatomyositis complicated with aspiration pneumonia. A gastric tube was inserted to correct the aspiration factors, and the patient was instructed to perform postural drainage in the left lateral recumbent position to correct the postural factors. No antibiotics were used. After 3 days, CT was re-examined. CT showed that the right lung lesions almost completely disappeared, and a few cord foci were seen in the lingual segment of the left upper lobe (Figure 1E，Figure 1F)。 Discharge diagnosis: (1) dermatomyositis, (2) aspiration pneumonia.

FHL1 belongs to a member of the LIM protein family. The LIM protein family of human FHL includes five members, namely FHL1, FHL2, FHL3, FHL4 and ACT (also known as FHL5)^[1,2,3,4]。 The expression levels of FHL1 and FHL3 were relatively high in skeletal muscle, but they were also expressed in myocardium; FHL2 is mainly expressed in the heart and at relatively low levels in skeletal muscle; FHL4 and ACT are specifically expressed in the testis^[5,6,7]。 Differential expression of FHL family members in different tissues may determine their biological function in these tissues. The FHL protein family is characterized by containing a specially arranged LIM secondary structure (C – X2 – C – X16 – 21 – H – X2 – C – X2 – C – X17 – C – X2 – C). All FHL proteins are made up of four complete LIM domains arranged in tandem and separated by eight different amino acid residues^[4]。 FHL1 is one of the more studied members of the FHL family. The human FHL1 gene is located at Xq27.2 and contains 5 exons. The cDNA is 2441 bases in length and encodes 280 amino acids. The splicing variants of FHL1 are: FHL1A (also known as Slim1, KyoT1, or transcriptional variant 3 in mice), FHL1B (Slimmer, KyoT3, or transcriptional variant 1), and FHL1C (KyoT2, or transcriptional variant 4), consisting of four-half, three-half, and two-half LIM domains, respectively^[8,9]。 Different splicing results in different nuclear localization sequences and formation of RBP – J junction domains at the C-terminus of FHL1B, FHL1C subunits. On the one hand, FHL1 can function as a co – factor and protein interaction; On the other hand, FHL1 can act as a transcription factor to regulate the expression of downstream target genes. For example, FHL1 plays a role in cytoskeletal rearrangement, fiber formation, and assembly of sarcomeriae, and plays an important role in regulating homeostasis and oxidative capacity within mitochondria. To date, FHL1 mutations in 27 humans have been found to be associated with the occurrence of at least 6 different muscular dystrophies. Therefore, this paper reviews the role of FHL1 gene in skeletal muscle development and its relationship with human muscle diseases.