Hypophosphatasia (HPP) is caused by loss-of-function mutation(s) of the gene that encodes the tissue-nonspecific isoenzyme of alkaline phosphatase (TNSALP). Its manifestations are variable, including impaired skeletal mineralization, altered calcium and phosphate metabolism, recurrent fractures, pain, impaired mobility, premature tooth loss, developmental delay, and seizures.There are different attempts for HPP.Asfotase alfa (Strensiq?), is a bone-targeted recombinant TNSALP which has shown significant improvements in morbidity and mortality in patients with perinatal and infantile hypophosphatasia, and it can improve growth, mobility function and quality of life.This enzyme replacement therapy has generally been well-tolerated, with most adverse reactions being mild-to-moderate in nature.This article reviews recent advances in the treatment of the disease.

Polymyositis (PM) and dermatomyositis (DM) are inflammatory myopathies and slowly advanced of unknown etiology that affect the skeletal muscles.With the advancement of the constant research on the disease and use of new technology,people have a penetrating realization about the pathogenesis. The disease is thought to be associated with autoimmune as well as nonimmune mechanism. By now, there is no overall summary on the newest advancement of the pathogenesis.We summarized the progress on the pathogenesis of the disease in order to make it more clear to physicians.

OBJECTIVE:To establish the ultraviolet visible spectrophotometry(UV-VIS)for the determination of Fe content in iron dextran preparations.METHODS:Under the acidic condition,Fe~(3+)was dissociated from iron dextran solution by heating,then Fe~(3+)was deoxidized into Fe~(2+)by hydroxylamine hydrochloride,and red Fe~(2+)-orthophenanthroline complex was formed through combination with o-phenanthroline.The content of the Fe~(2+)-orthophenanthroline complex was determined by UV-VIS at a wavelength of 510 nm.RESULTS:The linear range of Fe~(2+)was 0.5～4.0?g?mL~(-1)(r=0.999 9). The average recoveries of two preparations(tablets and injection)of iron dextran were 99.8%(RSD=0.52%)and 99.7% (RSD=0.44%),respectively.CONCLUSION:The UV-VIS method is simple,accurate in results and applicable for the determination of iron dextran preparations.

Ultrasound molecular imaging has become one of the hotspots of molecular imaging because of its advantages of non-invasion, non-radiation, repeatability and real-time dynamic imaging. The key of imaging is to select the appropriate target and ligand to bind to the ultrasound contrast agent. The formation of blood vessels in the development of tumor is a significant feature. Vascular endothelial growth factor receptor (VEGFR) is an important specific molecule on tumor vascular endothelial cells, and which can be used as a target for tumor ultrasound imaging. In recent years, many scholars have carried out related studies on the imaging of ultrasound molecules targeting VEGFR. This paper reviewed the mechanism and application of tumor ultrasound molecular imaging with VEGFR as target in recent years.

Objective To observe the clinical efficacy of S-1 plus oxaliplatin (SOX) in neoadjuvant chemotherapy for patients with local advanced gastric carcinoma.Methods Clinicopathological data of 68 patients with local advanced gastric cancer were retrospectively analyzed and enrolled as treatment group.All the patients in the treatment group achieved 3-4 cycles chemotherapy of SOX regimen preoperatively.Outcomes of tumor remission after neoadjuvant chemotherapy were evaluated according to RECISI1.1 criteria.Meanwhile,70 patients who underwent operation directly after pathological diagnosis of local advanced gastric cancer during the same period were recruited as control group.Resection rate,R0 resection rate,postoperative complications rate and postoperative progression free survival(PPFS) time were compared between the two groups.Results In the treatment group,after neoadjuvant chemotherapy,4 cases (5.9％) achieved complete renission,36 cases (52.9％) achieved partial remission.Meanwhile,the numbers of cases with stable disease and progressive disease were 15 cases(22.1％) and 13 cases (19.1％) respectively.The objective remission rate and disease control rate in the treatment group were 58.8％ and 80.9％ respectively.The tumor resection rate,R0 resection rate,postoperative complications rate in the treatment group were 92.6％,85.3％ and 14.7％ respectively.The R0 resection rate in the treatment group was higher than that in the control group,the difference was statistically significant(x2 =8.042,P ＜ 0.05).However,the differences of tumor resection rate and postoperative complications rate between the two groups were not statistically significant (x2 =1.711,0.673,all P ＞ 0.05).The median time of PPFS in the treatment group and the control group was 36 months and 30 months respectively,there was statistically significant difference in PPFS between the two groups (x2 =4.049,P ＜ 0.05).Conclusion The neoadjuvant chemotherapy of S-1 plus oxaliplatin for local advanced gastric carcminoma has satisfactory short-term effect and can also improve PPFS.However,further investigation is required to observe the effect of this neoadjuvant chemotherapy regimen on postoperative overall survival.

Objective To learn the present acceptance of patients from outside Tianjin by local hospitals, for improving the management of their medical services.Methods A study of the hospitals′ network system identified 5 306 inpatients from other places in 2016 as evidenced by their hospital settlement account, with analysis of the patient flow from other places by the hospitals surveyed.Medical workers of eight medical institutions which signed the online settlement contract for non-local patients were subject to questionnaire survey.Results Of the 876 respondents,the number of patients willing to receive non-local insured patients accounted for 78.08%.Non-local patients aged 60 or above accounted for 82.31% among the total non-local patients, of whom 48.55% selected hospitals with national key disciplines, and 30.53% chose those with municipal key disciplines.Conclusions An analysis is required for the demand of non-local patients in terms of the social background and population characteristics.Based on such, non-local patient population needs a better management while those with irrational needs should be discouraged with rules and regulations.

The new medical reform policy situation,the professional development of health management should follow the principle of scientific development to achieve three-dimensional models of development.Three-dimensional-style development should focus on the realization of the subject-object interaction,full management,all-round construction,double-entry-style positioning,and compound training of personnel.And through the three-dimensional development strategy we should seek to promote the health management leaps and bounds.

Objective To discuss the application effect of TCM constitution recognition on TCM chronic disease refining health management for type 2 diabetes mellitus.Methods Totally 140 outpatients with type 2 diabetes mellitus from Chaoyangmen Community Health Center were selected and divided evenly into two groups after TCM constitution assessment. The study lasted for 2 years (1st August 2013–31st July 2015). The control group (70 cases) received diabetes community health management. In addition to routine community health management, TCM constitutional management was also administrated in the intervention group. The latter group received medicine, food, sport guidance according to the results of TCM constitution assessment. The blood glucose, blood lipid, BMI and waist-hip ratio of the two groups were monitored, and the self-management and life quality of patients were evaluated. Results After the intervention, significant improvement was found for fasting blood glucose, 2 h postprandial blood glucose, BMI, waist-hip ratio and blood lipid both in the control group and the intervention group, and the improvement in the intervention group was more obvious than the control group (P<0.05). The whole level of self-management and life quality was greatly increased in both groups, while improvement of the intervention group was much higher than the control group (P<0.05).Conclusion Integrated application of TCM constitutional theory in TCM chronic disease refining health management for type 2 diabetes mellitus will contribute to improving patients’ self-management, changing their clinical symptoms and finally improving their life quality.

Moyamoya disease is a progressive cerebrovascular occlusive disease, which is characterized by the formation of abnormal vascular network at the brain base. It is an important cause of stroke in children and adults. Now, the etiology of moyamoya disease is not clear. This article reviews its pathogenesis from 3 aspects, including genetic factors, angiogenesis, and immune inflammation.

Child, Preschool ; Humans ; Lymphatic Abnormalities ; diagnosis ; therapy ; Male ; Middle Aged