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.

Prostate cancer is the most prevalent malignancies in elderly men. At present, gene therapy is the most po-tential treatment for late-stage prostate cancer. However, virus vector or nonvirus vector are less safety, weaker targeting function and higher immunogenic. Ultrasound microcapsular contrast agent as a drug transporter is a new type of disease tar-geted treatment strategies. It has provided a high-targeted, an effective and a safety gene therapy for prostate cancer, and brings a new hope for late-stage prostate cancer patients. This article aims to summarize the recent progress in using the mi-crobubble technology in prostate cancer.

Objective To explore the effect of FTY720 on human hepatocellular carcinoma cell line HepG-2 of invasion ability. Methods HepG-2 cells were cultured,and divided into 4 groups,namely control and FTY720 (0.05 g/ml,0.10 g/ml,0.15 g/ml) groups.The cell invasion ability was observed by the cell invasion assay.mRNA expression of MMP-2,MMP-9 was measured by RT-PCR.MMP-2,MMP-9 protein expression was measured by ELISA method.Results The invasive ability of cancer cells in each group significantly weaken,with the FTY720 concentration increased.The number of control group was (110±3)(P<0.05),the number of O.05 g/ml FRY720 treatment group cells was (74±4),while the number of cells of O.10 g/ml,0.15 g/ml the drug treated group,Was (42±3),(25±5)(P<0.01).Compared with control group,the MMP-2,MMP-9 expression of different concentrations of FTY720 groups decreased significantly (P<0.05).As the expression of FTY720 concentration increased,the MMP-2,MMP-9 expression decreased (P<0.01).Compared with control group,the MMP-2,MMP-9 protein expression of different concentrations of FTY720 groups decreased significantly (P<0.05), but the protein expression and concentration of FTY720 is inversely proportional in a certain range. Conclusion The invasive ability of HepG-2 cells can be inhibited by FTY720,and with FTY720 concentration increased,the invasive ability decreased gradually.One of the mechanisms may be related to reduce the MMP-2,MMP-9 protein and gene expression.

Objective To introduce a new surgical method of reconstructing A ch illes tendon by transfer of the soleus tendon flap. Methods The method was desig ned on the basis of the anatomical characteristics of the superficial muscles as sociated with Achilles tendon. It was applied to treat 12 patients with Achilles tendon defects. These patients were followed up for 0.5 to 6 years (average 39 months). Results The curative effect of the method were assessed according to Ar ner-Lindholms evaluation criteria. 9 cases were rated as excellent, 2 fine an d 1 poor, with the total excellent and fine rate being 92%. Conclusions ①Misdi agnosis and improper initial treatment are the major causes of Achilles tendon d efects. ②Systematic training after operation is very important for good curativ e effects. ③The method mentioned above brings about less trauma, less negative effects on blood supply to Achilles tendon, and good healing after operation.

Objective:To observe the effect of Chinese herbal fumigation combined with three-step tuina manipulation on concentration of endothelin (ET) and calcitonin gene-related peptide (CGRP) and vertigo in patients with vertebral artery cervical spondylosis (VACS).
Methods:A total of 120 eligible cases were randomly allocated into an observation group and a control group, 60 in each group. Cases in the observation group were treated with Chinese herbal fumigation combined with three-step tuina manipulation, whereas cases in the control group were treated with oral Flunarizine Hydrochloride Capsules.
Results: After treatment, vertigo in both groups was alleviated; there were intra-group significant differences in ET decrease and CGRP increase (P<0.01, P<0.05);and there were also inter-group significant differences (both P<0.05).
Conclusion: Chinese herbal fumigation combined with three-step tuina manipulation can regulate the levels of ET and CGRP and improve vertigo in patients with VACS. Its therapeutic efficacy is superior to oral Flunarizine Hydrochloride Capsules.

Objective To investigate the anterior trans-injured vertebral short segment limited fixation and fusion in treating thoracic and lumbar spinal tuberculosis.Methods One hundred and three patients with spinal tuberculosis from 2010 to 2012 were operated by trans-anterior approach(trans-thoracic above thoracic spine 12,trans-retroperitoneal below waist spine 1)tuberculosis focus clearance and spinal canal decompression,intervertebral bone graft of vertebral structure,and short segment internal fixation for residual disease spine.Results The nerve function of the patients was effectively improved after surgery,and the imaging re-sults showed that the average lesion kyphosis angle was significantly decreased(P <0.05).The postoperative follow-up found that the bone graft was fused after half a year,and the average local kyphosis angle was still 13°,which had no obvious change compared with that after operation.In addition,the internal fixation position had no looseness and fracture,the grafted bone had no displace-ment and detachment,the four limbs movement was normal without local percussion pain or tenderness.Conclusion The anterior trans-injured vertebral short segment limited fixation and fusion has the satisfactory short term clinical efficacy in the treatment of thoracic and lumbar spinal tuberculosis.

Objective To study Baizhu's inhibitory effect on S180 sarcoma growth and its effect on Bcl-2 expression in tumor-transfected mice. Methods S180 cells were subcutaneously injected into 60 healthy Kunming mice. Meanwhile the mice were dealt with MTX or Baizhu, separately; the weight of tumor was measured in the following two weeks; the expression of Bcl-2 was detected by RT-PCR. Results The tumor's weight in Baizhu group was lower than that in model group, but higher than in MTX group (P<0.05), but Baizhu's inhibition was not associated with its dose (P>0.05), and Baizhu's rate of tumor growth inhibition was lower than that of MTX. Compared with that in control group, Bcl-2 expression was lower in Baizhu group obviously. Conclusion Baizhu can inhibit tumor growth and serve as an adjuvant drug in tumor therapy.

Adolescent ; Alleles ; Asian Continental Ancestry Group ; genetics ; Erythropoietin ; genetics ; Ethnic Groups ; genetics ; Female ; Genotype ; Humans ; Male ; Polymorphism, Single Nucleotide ; Young Adult

BACKGROUNDMediastinoscopy has experienced a renaissance due to the introduction of neoadjuvant treatment protocols and recognition of the limitations of noninvasive mediastinal staging of lung cancer by CT and PET. The aim of this study is to determine the value of video-mediastinoscopy in mediastinal staging of lung cancer.

METHODSA total of 60 patients who underwent video-mediastinoscopy for known or suspected lung cancer were retrospectively reviewed. There were 52 cervical mediastinoscopies, 2 parasternal mediastinoscopies and 6 combined procedures. All the patients were found to have enlarged mediastinal lymph nodes on radiographic examination of the chest (more than 1.0cm on its shortest axis).

RESULTSOf the 60 patients, 42 patients were found with N2 or N3 disease on video-mediastinoscopy and then were confirmed pathologically. The other 18 mediastinoscopy-negative patients underwent thoracotomy for pulmonary resection and mediastinal lymph node dissection in the same operative session, in which thoracotomy-proven N0 was found in 17 patients, and N2 in 1 patient (false-negative result by video-mediastinoscopy). The sensitivity, specificity, and accuracy of video-mediastinoscopy for mediastinal staging of lung cancer were 97.7%, 100% and 98.3%, respectively. In the entire group of 60 patients, there was 1 complication (1.7%) , and no perioperative death.

CONCLUSIONSVideo-mediastinoscopy is a highly effective and safe procedure. It can be used routinely in mediastinal staging of lung cancer.

Objective To study risk factors of cardiovascular disease (CVD) and status of bone mineral density (BMD) in women with hypoestrogenism.Methods From Jul 2011 to April 2013,a total of 256 women with hypoestrogenism in the First Affiliated Hospital of Shanxi Medical University were enrolled in this retrospective study,which were divided into four groups:133 women in ppausal group,25 women in premature ovarian failure (POF) group,67 women in menopausal transition group and 31 women in premature ovarian failure transition group.General statue,CVD risk factors and BMD were compared among four groups.General statue include menopausal period,menopausal symptoms (Kupperman Index),CVD risk factors include body mass index,blood pressure,waist circumference,waist-hip ratio,blood lipids and glucose,BMD include left hip,lumbar spine bone mineral density and T or Z value.Results (1) The median menopausal period were 3.4 years in postmenopausal group and 3.6 years in premature ovarian failure group,which did not show no statistical difference (P ＞ 0.05).Kupperman Index in four groups were 12 in postmenopausal group,9 in POF group,9 in menopausal transition group and 8 in premature ovarian failure transition group,which reached statistical difference (P ＜ 0.05).(2) The difference of body mass index (BMI),waist circumference,waist-hip ratio,diastolic blood pressure were no statistically significant among four groups(P ＞ 0.05) ; the systolic blood pressure in four groups were 120,110,110,110 mm Hg (1 mm Hg =0.133 kPa),their differences were statistically significance (P ＜ 0.05); the high-density lipoprotein (HDL-C) was 1.6 mmol/L in postmenopausal group,and 1.3 mmol/L in premature ovarian failure transition group,their differences were all statistically significance (P ＜ 0.05) ; the difference of the fasting plasma glucose (FPG) was not statistically different in 4 groups (P ＞0.05).(3) The abnormal rate of lower bone mass in lumbar spine were 57％ (46/81) postmenopausal group,8/15 in POF group,32％ (9/28) in menopausal transition group,12/19 in premature ovarian failure transition group,and osteoporosis was 9％ (7/81),3/15,1％ (3/28)and 0 respectively,their differences were statistically different (P ＜ 0.05) ; the abnormal rate of BMD of left hip and lumbar spine of 11/15 and 12/16 in POF group was higher than 65％ (53/81) in postmenopausal group.In the mean time,the abnormal rate of BMD of left hip and lumbar spine were,12/19 and 10/20 in premature ovarian failure transition group,which were significantly higher than 43％ (12/28) and 39％ (12/31) in the menopausal transition group.Conclusions The menopausal symptoms resulting from hypoestrogenism in natural postmenopausal women are mostly remarkable.The decrease of BMD in lumbar spine is more significant than that of left hip among postmenopausal women.Women with earlier menopause was prone to cause the changes of blood fat and abnormal of BMD,especially HDL-C decreased significantly compared with those natural postmenopause,it is more likely to cause CVD and osteoporosis.