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.

Objective To study the condition of rehabilitation in Community Health Service (CHS) in rural areas after earthquake. Methods 274 medical personnels of CHS were invistigated with the questionnaire. Results There was no rehabilitation therapist in those medical personnel, and no difference of requirement, referral will and acquaintance of therapy between rehabilitation and acupuncture in CHS （P=0.056，P=0.293，P=0.654）. There was a significant difference in acquaintance of rehabilitation among different ages of medical personnel （P<0.01）, but there wasn't of acupuncture （P=0.104）. Conclusion It still lack rehabilitation therapist in CHS. Techniques and resources were the key points in the development of community-based rehabilitation.

The patients with stroke is often accompanied by malnutrition, its causes include eating less, increased catabolism, and intestinal dysfunction, etc. Malnutrition can not only aggravate the extent of brain damage in patients with stroke, but also increase the incidence of complications, and thus affecting the course and prognosis of stroke. Therefore, correct evaluation of the nutritional status in stroke patients, choosing the appropriate nutrient timing and nutritional support methods have important significance. Among a variety of nutritional assessment methods, the scored Patient-Generated Subjective Global Assessment can quickly identify malnutrition in stroke patients. Early enteral nutrition support, including oral nutritional supplements, nasogastric tube feeding and percutaneous endoscopic gastrostomy, can significantly improve the prognosis in stroke patients. Among them, nasogastric tube feeding is the first choice for nutritional support in stroke patients.

The effect of axon guidance factors ephrin-A1/EphA2 on the invasion of trophoblastic cells and the possible mechanism were investigated in this study. The expression of EphA2 in vascular endothelial cells was detected by immunohistochemistry. The proliferation and invasion of TEV-1 cells (an extravillous trophoblastic cell line) in first trimester were determined by cell counting kit-8 (CCK-8) and Transwell invasion assay. Real-time PCR was used to detect the expression of ephrin-A1 in TEV-1 cells treated with EphA2 at different concentrations (10, 50, 100, 500, 1000 and 5000 μg/L). The results showed: (1) EphA2 was expressed in the vascular endothelial cells; (2) EphA2 could promote the proliferation of TEV-1 cells. The proliferative capacity reached a peak in TEV-1 cells treated with 100 μg/L EphA2 (P<0.05); (3) EphA2 could increase the invasion of TEV-1 cells. The invasive ability was the greatest in TEV-1 cells treated with 500 μg/L EphA2 (P<0.05); (4) in the presence of EphA2 (0-500 μg/L), the expression of ephrin-A1 was increased concentration-dependently (P<0.05), but when the concentration of EphA2 was over 500 μg/L, the expression of ephrin-A1 ceased to increase (P>0.05). It was concluded that EphA2 can promote the invasion and proliferation of the human extravillous trophoblastic cells probably by regulating the ephrin-A1 ligand.

Objective To observe the effect of motor relearning program (MRP) on balance function of stroke patients.Methods Sixty stroke patients were randomly divided into the MRP group and control group with 30 cases in each group. The patients of the control group were trained with proprioceptive neuromuscular facilitation (PNF) therapy; those of the MRP group were trained with the MRP therapy. The balance function of all patients was assessed with the Fugl-Meyer Balance Function Assessment before and after training.Results After training, the scores of Fugl-Meyer Assessment of all patients increased ( P<0.05), but scores of the patients of the MRP group were significantly higher than that of the control group ( P<0.05).Conclusion The MRP training can improve the recovery of balance function of stroke patients.

Female ; Genital Neoplasms, Female ; classification ; pathology ; Humans ; Ovarian Neoplasms ; pathology ; Papillomavirus Infections ; Precancerous Conditions ; pathology ; Uterine Cervical Neoplasms ; pathology ; virology ; World Health Organization

Objective To investigate the ECG atypical changes in acute myocardial infarction(AMI). Meth-ods 352 patients with AMI were selected sequentially and examined by electrocardiogram(ECG) ,Holter of 24h,my-ocardial enzymes testing and coronary angiography, and electrocardio-monitoring was performed at the same time. Data were analyzed by statistics. Results The incidence of ECG atypical changes in patients with AMI was 14. 5%. The ECG atypical changes including Q wavelet with the elevation value of ST segment, the delays of Q wave and the only changes of ST-T. Conclusion The above several kind of ECG performane are present in atypical AMI.

Objective:To investigate the expression of transforming growth factor-?1 (TGF-?1) in mandibular condylar chondrocytes after the condylar cartilage was stimulated by compressive stress.Methods:20 condylar cartilage samples were obtained from 10 new borne SD rats and cultured for 24 h.Then,10 samples were treated by a static compressive stress with a magnitude of 10 kPa for 1 h.Another 10 samples without stress treatment were used as the controls. At end of stress stimulation, the expression of TGF-?1 in the samples of both groups was examined by immunohistochemistry and image pattern analysis.Results:The grayscale values of TGF-?1 in perichondrium lay of stress treated and control samples were 159.21?19.84 and 174.20?14.47(P

Objective To evaluate the effect of various kinds of dyslipidaemia on insulin resistance and pancreatic islet ? cell function in newly diagnosed type 2 diabetic patients. Methods 263 patients with newly diagnosed type 2 diabetes were divided into normal lipidemia group and dyslipidaemia group according to ATPⅢ standard. The dyslipidaemia group included 3 subgroups as the following: high TG group, high TC group, and mix group with high TG and TC. Homeostasis model assessment (HOMA) was applied to estimate insulin resistance (IR) and pancreatic islet ? cell function. Early insulin secretion index (EISI) and area under curve of insulin (AUCI) at all OGTT time points were calculated. Results Incidence of dyslipidaemia in the type 2 diabetic patients was 63%. BMI and WHR were significantly higher in both high TG group and mix group than in normal lipidemia group(P

Objective To understand the isolation and change of drug resistance of mucoid Pseudomonas aeruginosa in sputum from 2013 to 2015 so as to provide a scientific basis for reasonable clinical use of antibacterial drugs.Methods The isolation,culture and identification were carried out according to the fourth edition of National Clinical Laboratory Operation Rules.The drug susceptibility testing was performed with the use of Kindy-Bauer.The drug susceptibility testing results were judged according to the standards of CLSI 2014.The clinical data were analyzed by software WHONET 5.6.Results Among 1 653 strains of Pseudomonas aeruginosa isolated in the clinical sputum specimens from 2013 to 2015,including 255(15.4%)strains of mucoid Pseudomonas aeruginosa;during the three years,the drugs of highest drug resistance rate was piperacillin,which was 29.0%;the drug resistance rate less than 10.0% had amikacin and piperacillin/tazobactam;the drugs with larger increase range of resistance rate included imipenem,levofloxacin,ciprofloxacin and ciprofloxacin,their resistance rates were increased from 12.5%,9.7%,8.3% and 9.7% in 2013 to 20.5%,17.0%,19.3% and 15.9% in 2015.Conclusion The isolating rate of mucoid Pseudomonas aeruginosa in sputm is gradually increased year by year from 2013 to 2015.At the same time,the drug resistance rates show an increasing trend in recent three years.It is necessary to strengthen the surveillance of drug resistance of mucoid Pseudomonas aeruginosa.