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The high proportion of renewable energy integration to industrial parks has become the main way for energy-intensive industrial loads to achieve low-carbon transition. The sharp and frequent power fluctuation of tie-line is the key problem of low-carbon transition for industrial parks. Therefore, an industrial park tie-line power smoothing strategy considering production safety is proposed. First, the control model of the production equipment for electrolytic aluminum and arc furnace is analyzed, the power closed-loop feedback control model of industrial load is established based on power fluctuation feedback. Then, considering the core influencing factors of industrial production, the state of temperature with energy-intensive equipment is established, a control cost model for demand response is formed, and the control objective to each industrial load is assigned based on the optimization objective for the minimum penalty cost. Finally, the MATLAB online calculation and RTDS simulation real-time interactive example is established based on Wenshan power grid in Yunnan Province. The simulation results show that the smelting temperature is updated adaptively, the influence of power regulation on production is reduced effectively, and the economic cost of tie-line is reduced by 36.4%.

BackgroundMultiple endocrine neoplasia type 2 is an autosomal dominant inherited syndrome caused by activating mutations in the RET proto-oncogene. The RETK666N DNA variant was previously reported in two isolated medullary thyroid carcinoma (MTC) cases, but no family studies are available, and its oncogenic significance remains unknown.MethodsThe clinical features, genetic data, and family information of eight index MTC patients with a germline RETK666N variant were assessed.ResultsFour probands presented with MTC and extensive nodal metastasis, one with biopsy-confirmed distant metastasis. Two additional probands presented with localized disease. However, nodal status was not available. Of the final two probands, one had an incidental 1.5 mm MTC and C-cell hyperplasia uncovered after surgery for papillary thyroid carcinoma, and one had two foci of MTC (largest dimension 2.3 cm) detected after surgery for dysphagia. Genetic screening identified 16 additional family members carrying the K666N variant (aged 5-90 years), 11 of whom have documented evaluation for MTC. Of these, only two were found to have elevated basal serum calcitonin upon screening, and the remaining patients had calcitonin levels within the reference range. One patient who elected to have a thyroidectomy at 70 years of age was confirmed to have MTC. The other subject, 57 years old, elected surveillance. Four prophylactic thyroidectomies were performed, with one case of C-cell hyperplasia at 20 years and three cases that revealed normal pathology at ages 21, 30, and 30 years. None of the K666N DNA variant carriers had evidence of primary hyperparathyroidism or pheochromocytoma.ConclusionsFrom this case series, the largest such experience to date, it is concluded that the RETK666N variant is likely pathogenic and associated with low penetrance of MTC. However, the findings are insufficient to define its pathogenicity clearly and make firm recommendations for screening and treatment. Given the potential benefit associated with early detection of aberrant C-cell growth, and the noninvasive nature of genetic testing, "at risk" individuals should be screened, and if the K666N variant is identified, they should be managed using a personalized screening approach for detection of MTC.

Background: Multiple endocrine neoplasia type 2 is an autosomal dominant inherited syndrome caused by activating mutations in the RET proto-oncogene. The RETK666N DNA variant was previously reported in two isolated medullary thyroid carcinoma (MTC) cases, but no family studies are available, and its oncogenic significance remains unknown. Methods: The clinical features, genetic data, and family information of eight index MTC patients with a germline RETK666N variant were assessed. Results: Four probands presented with MTC and extensive nodal metastasis, one with biopsy-confirmed distant metastasis. Two additional probands presented with localized disease. However, nodal status was not available. Of the final two probands, one had an incidental 1.5 mm MTC and C-cell hyperplasia uncovered after surgery for papillary thyroid carcinoma, and one had two foci of MTC (largest dimension 2.3 cm) detected after surgery for dysphagia. Genetic screening identified 16 additional family members carrying the K666N variant (aged 5-90 years), 11 of whom have documented evaluation for MTC. Of these, only two were found to have elevated basal serum calcitonin upon screening, and the remaining patients had calcitonin levels within the reference range. One patient who elected to have a thyroidectomy at 70 years of age was confirmed to have MTC. The other subject, 57 years old, elected surveillance. Four prophylactic thyroidectomies were performed, with one case of C-cell hyperplasia at 20 years and three cases that revealed normal pathology at ages 21, 30, and 30 years. None of the K666N DNA variant carriers had evidence of primary hyperparathyroidism or pheochromocytoma. Conclusions: From this case series, the largest such experience to date, it is concluded that the RETK666N variant is likely pathogenic and associated with low penetrance of MTC. However, the findings are insufficient to define its pathogenicity clearly and make firm recommendations for screening and treatment. Given the potential benefit associated with early detection of aberrant C-cell growth, and the noninvasive nature of genetic testing, 'at risk' individuals should be screened, and if the K666N variant is identified, they should be managed using a personalized screening approach for detection of MTC. [ABSTRACT FROM AUTHOR]

In Caucasians, maturity-onset diabetes of the young (MODY) is mostly caused by mutations in the hepatocyte nuclear factor (HNF)-1a(MODY3) and glucokinase (MODY2) genes. Most Japanese MODY patients, however, are not linked to known MODY genes. In this study, we examined the genetic and clinical characteristics of Chinese subjects with MODY. The study included 146 unrelated families fulfilling the minimum criteria for MODY: two consecutive generations of type II diabetes with at least one member diagnosed under the age of 25. We screened for mutations in the HNF-4a(MODY1), MODY2 and MODY3 genes by direct sequencing. Antibody to glutamic acid decarboxylase (GAD-Ab) was measured in subjects with MODY of unknown cause (MODYX). Insulin resistance index and other clinical data were compared in sex-, age- and duration-matched MODY3 and MODYX patients. In all, 13 families had MODY3 mutations and two had MODY2 mutations. No MODY1 mutation was found. Four of the 12 different MODY3 mutations were newly identified novel mutations (Q243E, A311D, P379R and P488fsdelC). In subjects with MODYX, 3%were GAD-Ab positive and 60%were overweight. Compared to MODY3 patients, MODYX patients had higher body mass index (P<0.02), higher insulin resistance index (P=0.001) and triglyceride level (P<0.02), lower HDL level (P=0.001) and more hypertension (P<0.05), but no significant difference in the prevalence of diabetic complications. In conclusion, MODY3 and MODY2 account for only 9 and 1%, respectively, of Chinese MODY. A majority of Chinese MODY patients are due to defects in unknown genes and appear to be characterized by insulin resistance.European Journal of Human Genetics (2005) 13, 422-427. doi:10.1038/sj.ejhg.5201347 Published online 15 December 2004 [ABSTRACT FROM AUTHOR]

Working rights enjoyed by laborers or other entities, with the right to get hired and the right to join a union as their core, refer to the rights which originate from or are closely related to working. The position of working rights refers to the position or level at which the working rights are locate in the system of rights in a country. The working rights of the citizens, the basic human rights empowered by the Constitution, are in the upper position in the whole system of rights, and when they conflict with other rights or powers, they should be protected with priority by the law. Regarding the relationship between public rights and working rights, it should be reasonably established as guaranteeing and guaranteed, servicing and serviced, enhancing and enhanced, compulsive and free (in terms of labor standards and contracts). On the one hand, the enforcement of working rights should not violate labor standards and other compulsive legal regulations. And on the other hand, it has become an undisputed fact that the enforcement of public rights is more and more restricted and controlled and gradually compromising with and giving way to working rights. Regarding the relationship between the working rights and the private rights, except very specific situations, the position of working rights should be put in a higher position than common private rights such as real rights, creditor's rights and intellectual property rights, especially when contradictions exists between the working rights of a laborer and the management rights of an entity. There are many problems in this aspect in the present legislation in China and some suggestions concerning the improvement on them should be put forward. Regarding the relationship between the working rights and the other social rights such as social security rights, environment rights, and consumer's rights, because they are at the same level and in the same position with working rights, therefore, when they are conflicted, we can only decide which right should prevail by judging which social interests are bigger according to each individual case. [ABSTRACT FROM AUTHOR]