Tuberous sclerosis complex (TSC) is a genetic multisystem disorder that has an impact on many organ systems.It is a common neurocutaneous syndrome in children, the main clinical manifestations characterized by impairment of skin and nervous system.The most frequent neurological symptoms are seizures,which occur in up to 90％ of patients and often are intractable,followed by autism spectrum disorders, intellectual disability, attention deficit-hyperactivity disorder, and sleep, et al.Conventional treatment has frequently proven insufficient for neurological and behavioral symptoms, particularly seizure control.Currently, there are many studies focus on the mechanism-based treatment in TSC.Therefore, this review will focus on the genetic mechanism of TSC, further exploring personalized medicine and prognosis for TSC shows good prospects.

Cone-bine CT(CBCT)images of 2 130 maxillary third molars were divided into 3 groups according to the impaction position. The relationship between the floor of maxillary sinus and the roots of maxillary third molar was analyzed and compared.In low,median and high impaction groups the probability of the root tip of maxillary third molar higher than the floor of maxillary sinus was 4.5%,10.3% and 14.6% respectively.

Covered in the paper are reform efforts made by the hospital on the HIS system amid the pilot programs total prepaid and mcdicine-drug separation trials in Beijing.Findings of the programs revealed defects of the HIS system,for example measures for refusal of payment of non-compliance items,and real-time reimbursement for patients.In view of these setbacks,the hospital made further attempts to the system in such aspects of the fundamental maintenance,rational drug usage and new problems,for the purpose of successful completion of the pilot programs at the hospital.Discussions were also made as to how to adapt the HIS system to total prepaid and the separation reforms,aiming at helping those not yet initiated with the reforms,and the information sharing of patient diagnosis/treatment records and social insurance payment at all hospitals in Beijing.

OBJECTIVE:To provide reference for small interfering ribonucleic acid (SiRNA) R&D and registration in China. METHODS:The situation of SiRNA R&D at home and abroad were introduced,and problems encountered in the evaluation and approval process were summed up to put forward suggestions. RESULTS & CONCLUSIONS:SiRNA has developed slowly in Chi-na,with a gap of 5-10 years at least compared with foreign developed countries. The registration category of SiRNA is not clear;applicants lack of guidance documents;production workshop which meet requirement is in shortage;the efficiency of drug registra-tion allows of no optimism. It is recommended that pharmaceutical registration departments carry out the pilot reform for SiRNA registration,publish SiRNA registration guide,relax regulations for IND gradually,encourage applicants to participate in the inter-national multicenter clinical trials in order to improve the efficiency of SiRNA evaluation and approval efficiency.

Objective To compare the effectiveness of the forced-air warming system and the conventional warming care practice in preventing hypothermia in patients undergoing major surgeries.Methods A total of 62 patients were enrolled in this study. Patients of both sexes,aged≥18 years,and with an ASA physical status Ⅰ-Ⅲ were included and allocated randomly into intervention group(n=30)or control group(n=32). Patients in the intervention group were provided forced-air warming,either prior to induction or during the surgery procedure depending on core temperature monitoring results. In the control group,patients were warmed with conventional warming care without any intervention. Core temperature was measured by Spot-On sensor and tympanic membrane thermometer,blood pressure,the electrocardiogram,heart rate,pulse oxygen staturation,and partial pressure of end-tidal COwere also monitored continuously in the two groups.Crystalloid,colloid,and allogeneic blood were also recorded by strict protocol. Results Intraoperative hypothermia monitored with tympanic thermometer was observed in 23 patients(71.87%)in the control group,which was significantly higher than that (0) in the intervention group(P=0.00). In addition,the incidence of hypothermia monitored by Spot-On sensor was 81.25%(n=26)in control group and 13.33%(n=4)in intervention group(P=0.00). Conclusion Compared with the conventional warming care,forced-air warming system is more effective in preventing hypothermia during major surgeries.

Diagnostic Errors ; Humans ; Myoclonus ; diagnosis ; Tics ; diagnosis

Objective To longitudinally analyze the unit costs and technical efficiency of the model of male circumcision in the different kinds of persons .Methods Unit costs were calculated by the person and period using longitudinal data from 3 kinds of persons ,and then technical efficiency and Malmquist indices were measured with an approach to data envelopment analysis . Results Theunit costs for changing the willingness to accept surgery changed dramatically ,decreasing from 7 166 .67 yuan(mean) to 737 .31 yuan ,while the costs for changing the ratio of the surgery increased from 666 .64 yuan (mean) to 744 .58 yuan ,and its technical efficiency was averaging between 0 .95-0 .96 .Conclusion The time series of unit costs for changing the willingness to ac-cept surgery dramatically dropped ,while changing the ratio of the surgery formed a U-shape curve with an inflection point before which unit costs dramatically dropped and another inflection point beyond which unit costs went up .These findings can inform pro-gram managers of the changing unit costs when extending or expanding the program .

The incidence rate of erectile dysfunction (ED) is reportedly as high as 30-90% after radical prostatectomy for prostate cancer, which seriously affects the patients' quality of life. Penile rehabilitation is defined as the use of any drug or device at or after radical prostatectomy to maximize erectile function recovery. A variety of treatment options are available for post-prostatectomy ED patients, including oral phosphodiesterase 5 inhibitors (PDE5I) , intracorporal injections, vacuum erection device, and penile prosthesis. This article presents an overview of the currently used methods for the drug treatment and penile rehabilitation of the ED patients after radical prostatectomy. It seems proper to recommend daily use of a vacuum erection device plus oral PDE5I in the early postoperative period. For those who fail to respond to this therapy, intraurethral alprostadil, intracorporal injections, or a penile prosthesis could be considered.
Alprostadil ; administration & dosage ; Combined Modality Therapy ; Erectile Dysfunction ; etiology ; therapy ; Humans ; Male ; Penile Erection ; Penile Prosthesis ; Phosphodiesterase 5 Inhibitors ; administration & dosage ; Prostatectomy ; adverse effects ; Quality of Life ; Recovery of Function ; Vacuum

Objective To describe a new surgical approach to treatment of scapular neck and body fractures and evaluate the clinical outcomes. Methods From December 2002 to October 2004, 13 cases of the scapular neck and body fractures including 9 males and 4 females with age range of 22-48 years (mean 31 years) were admitted into our hospital. There were seven cases with neck fractures, two with associated clavicle fractures, six with mere body fractures, seven with associated subluxation of humeral head and three with associated rib fractures. Internal fixation and open reduction reconstruction using titanium plate and screws were for treatment of scapular neck and body fractures in all 13 cases by making straight incision along lateral border of the scapula. Results The operation lasted for 40-80 minutes, with intraoperative blood loss for 30-80 ml. The follow up for 4-24 months showed average fracture healing time for 6-8 weeks and satisfactory results in 10 cases. Conclusion The surgical approach with straight incision along the lateral border of the scapula can attain perfect reduction and internal fixation of scapular neck and body fractures as well as satisfactory functional recovery with minor (trauma.)