Central core disease (CCD) is a dominantly inherited congenital myopathy,manifesting as static or slowly progressive weakness ofproximal muscles.Histological characteristics on muscle biopsy are the well defined areas devoid of oxidative enzyme stains.Ryanodine receptor 1 gene mutations are associated with CCD.Great progress has been made in recent years about the RYR1 gene mutaion and the clinical feature of CCD.Genotypic and Phenotypic variations of RYR1 related CCD are reviewed.

Fluorescence quantitative PCR (FQ-PCR) is a more sensitive and specific method to diagnose and monitor cytomegalovirus(CMV) infection, and it is closely correlated with pp65 antigenemia levels. Measuring the CMV-DNA levels in infant urine and blood can be helpful to regulate the treatment and monitor the effects of the antivirus therapy.

OBJECTIVE: To provide reference for dealing with the problems arose from unlicensed and off-label drug uses.METHODS: The rationality of unlicensed and off-label drug uses and the possible problems induced by which were analyzed.RESULTS: The existence of unlicensed and off-label drug used has its rationality,and it also in line with the international practices,but it may cause some problems related to policy,laws and regulations and medication safety etc.CONCLUSION: The concerned department and trade association should attach great importance to unlicensed and off-label drug uses and establish a generally accepted principle and strategies to tackle the problems.

In order to further deepen the reform of public hospitals, to improve the qualified personnel training in Qingpu, and to enhance the overall level of medical health development, Qingpu Municipal Commission of Health and Family Planning In October 2011, a new round of disciplinary construction and personnel training project was also started.Moreover, financial support has been provided to the project.By assessing the whole process, not only has the level of discipline personnel training and medical technology shown significant improvement, the tutors who provided the training also express academic interest and have new a vision for talent cultivation.Qingpu Municipal Commission of Health and Family Planning launched a new round of comprehensive program on researcher training and disciplinary construction in 2011, aimed to further deepen the reform of public hospitals,improve the capacity of health research in Qingpu, Shanghai.We have assessed this program and found that this program has significantly improved the quality of training, and medical technology level in those hospitals;in addition, trainees has shown the increase in the interesting in conducting research and raised vision and prospects.

BACKGROUND:Hydroxyapatite-coated titanium substrates improve the biocompatibility and have the property of intimating ossteointegration with host bonebed. However, hydroxyapatite lacks the necessary mechanical strength and degrades easily in the extracelular fluids, which may affect the stability of the titanium implant.
OBJECTIVE:To study the synthesis and characterization of lanthanum-incorporated hydroxyapatite coatings.
METHODS: Lanthanum-incorporated hydroxyapatite coatings were prepared by hydroxyapatite and 10%, 20% and 30% lanthanum, respectively, by means of sol-gel, which were then deposited on titanium substrates with dip-withdrawal technique. Surface morphology and crystaline microstructure of the coatings were observed by scanning electron microscope. The presence of functional groups for the obtained samples was performed by Fourier transform infrared absorption spectroscopy and X-ray diffraction. The Ca2+ concentration released from the coatings was measured by atomic absorption spectrometry for analysis of degradation property.
RESULTS AND CONCLUSION: With the increase of lanthanum content, the diffraction peak and crystalinity of lanthanum-incorporated hydroxyapatite coatings were increased, but the whole structure of lanthanum-incorporated hydroxyapatites had little changes. The crystal structure maintained stable with charge balance. The lanthanum-incorporated hydroxyapatite coatings showed uniform and high-dense structure and were free of cracks, indicating the coatings had good bonding strength. Under the simulated biological environment, based on the determination of Ca2+ release from the coatings, we can conclude the lanthanum-incorporated hydroxyapatite coatings have a stronger acidoresistance.

Objective To visualize the pelvic floor structures by translabial three‐dimensional(3D) ultrasonography performed during labor and immediately after delivery, and to observe the morphological changes of levator hiatus in intrapartum women. Methods Totally 62 nulliparous women with singleton pregnancy and cephalic presentation in labor ward underwent intrapartum translabial 3D ultrasound examination before cervical dilatation, at 10 cm dilatation and immediately after delivery. Volume datasets were obtained at rest, biometric measurements and observation of the morphological changes of levator hiatus in different stages were determined in the axial plane. Comparative analyses of parameters in three stages were carried out. Results Translabial 3D ultrasound demonstrated the pelvic floor structures of intrapartum women well. All biometric measurements of the hiatus before cervical dilatation were the smallest and became the largest at 10 cm dilatation, showed statistically significant differences. The measurements after delivery got smaller immediately but still significantly larger than those before cervical dilatation ( P <0 0.01). Conclusions Translabial 3D ultrasound examination of the pelvic floor during labor and within the first 2h after delivery is feasible. It seems to be an effective tool to visualize pelvic floor structures in intrapartum women. Hiatal enlargement may be a common consequence of vaginal childbirth.

Objective To explore the value of Color Doppler Flow Imaging in diagnosis of superfi-cial substantial tumors.Methods Retrospective analysis of 742 cases of superficial substantial masses ( all confirmed by operation and pathology) of Patients'ultrasonography , and summarized its features of benign and malignant, and made a comparison.Results Of the 742 patients, ultrasound detection rate of 100%, including 12 cases of malignant lesions, ultrasound diagnosis of 12 malignant cases, the accuracy rate of 100%;730 cases of benign lesions, ultrasound diagnosis of 711 cases of benign, qualitative accuracy rate 97.4%.Conclusions Color Doppler Flow Imaging has an important value in the diagnosis of benign and malignant of superficial substantial tumors.

Objective To observe the quantity change of autophagosomes in podocytes and expressions of autophagy-related gene Beclin-1 and microtubule-associated protein 1 light chain 3 (LC3) in different pathological stages of idiopathic membranous nephropathy (IMN),and to explore how autophagy is related to podocyte injury,the occurrence of proteinuria and the disease progression in IMN.Methods Clinical data of 26 patients who were diagnosed as IMN (14 IMN stage 1 and 12 IMN stage 2) admitted to Zhejiang Provincial people's Hospital from January 2013 to December 2014 were retrospectively analyzed.Normal renal tissue from 15 cases of kidney neoplasms with nephrectomy was collected as control.The changes of kidney tissue pathology were detected after PAS and PASM staining by light microscope.The autophagosomes of podocyte were detected by transmission electron microscopy.Expressions of Beclin-1 and LC3 protein were detected by immunohistochemistry.Expressions of LC3 and synaptopodin were detected by immunofluorescence.The correlation of autophagosomes and clinical pathologic factors in IMN patiens was analyzed.Results There were fewer autophagosomes of podocytes and lower expression of Beclin-1 and LC3 protein in IMN group than those in control group (P=0.034,P=0.011,P=0.013,respectively).Moreover,these effects were more obvious with the development of IMN.Compared with those in control group,autophagosomes,Beclin-1 and LC3 protien were reduced in IMN stage 2 group (P=0.009,P=0.030,P=0.015);the number of autophagosomes and the expressions of LC3 and Beclin-1 were decreased in IMN stage 1 group as well,however statistically insignificant (P=0.352,P=0.087,P=0.128);Comparisons between IMN stage 2 patients and IMN stage 1 patients shown significant difference in the number of autophagosomes (P=0.030),but no significant difference in expressions of Beclin-1 and LC3 (P=0.355,P=0.181).Autophagosomes number was not correlated with serum creatinine,serum urea nitrogen,24-hour urinary protein and eGFR (all P ＞ 0.05).The expressions of synaptopodin and LC3 protein were lower in IMN group than those in control group.Conclusion Autophagy may contribute to podocyte injury and the production of protein urine in IMN,and may be closely related to the progression of disease.

Objective To investigate clinical and pathological features of idiopathic membranous nephropathy (IMN) accompanied by mesangial dense deposit.Methods Clinical data of 46 patients who were diagnosed as IMN accompanied by mesangial dense deposit admitted to Zhejiang Provincial People's Hospital from January 2013 to December 2014 were retrospectively analyzed.They were compared with those of 29 patients who were diagnosed as IMN without mesangial dense deposit during the same period in the hospital.Analysis of their clinical and pathological features was conducted.Results The IMN accompanied by mesangial dense deposit accounted for IMN 61.3％,and had more hyaline changes of arteriole (43.5％ vs 6.9％,P=0.001) and more obvious arteriolar wall thickening (78.2％ vs 51.7％,P=0.016) than IMN without mesangial dense deposit.Furthermore,the positive rate of IgA deposition in IMN accompanied by mesangial dense deposit was greatly higher than that in IMN without mesangial dense deposit (21.7％ vs 0,P=0.007).In other index,such as serum biochemical parameters,urine protein,glomerular lesion,tubulointerstitium pathological damage and other immunopathologic changes,no statistically significant differences were found between these two groups.Conclusions IMN patients accompanied by mesangial dense deposit have severe intrarenal artery lesions,and high positive rate of IgA deposition.

Objective To investigate the clinical and pathological features of idiopathic membranous nephropathy (IMN) in young patients.Methods Clinical data of 20 young patients, 16 to 44 years, who were diagnosed as IMN admitted to the Zhejiang Provincial People's Hospital from January 2013 to December 2014 were retrospectively analyzed, comparing to 55 mid-aged patients who were diagnosed as IMN during the same period in the hospital.Clinical and pathological features of above mentioned patients were analyzed.Results Young patients with IMN accounted for 26.7％ of IMN patients.Compared to mid-aged patients, young patients with IMN had lower proportion of hypertension (P=0.003), lower blood glucose level (P=0.010), higher estimated glomerular filtration rate (eGFR) and low-density lipoprotein (LDL) (P=0.012, P=0.038), and lower levels of T3 and T4 (P=0.030, P=0.034).Furthermore, there were less sclerosis glomeruli (P＜ 0.001), hyaline change of arteriole (P=0.040) and arteriolar wall thickening (P ＜ 0.0001), lower positive ratios of IgA (P=0.008),and more without renal tubulointerstitial lesions (P=0.018) in young patients.There were no statistically significant differences between these two groups in other index.Conclusions Compared to mid-aged patients, young patients with IMN have better blood pressure and blood glucose level, higher glomerular filtration rate and LDL.Moreover, thyroid function is significantly affected, meanwhile the lesions of glomerular, interstitial and vascular are mild in young patients.