The United States is the first country that has introduced legislation designed to stimulate orphan drug research and development .In this paper , we analyze three major amendments of the Orphan Drug Act ( ODA) since 1983 and its incentive system .We then discuss the success of the ODA in encouraging orphan drug research and development and boosting the biopharmaceutical industry .Following that , we investigate the high price of orphan drugs caused by market exclusivity and drug repositioning .Finally, we draw experience from the ODA to make some suggestions on how orphan drug legislation can be devised in China with the aim of improving the health of rare -dis-ease patients and facilitating the development of Chinese biopharmaceutical industry .

Objective To investigate the relationship between cerebrovascular accident and abnormal electrocardiogram. Methods The ECG abnormalities and clinical data of 314 patients with cerebrovascular accident were analyzed. Results 56.7% patients developed arrhythmia. Among them, the morbility of arrhythmia in patients with cerebral hemorrhage was higher than that of cerebral infarction ( P

OBJECTIVETo investigate the effects of recombinant human growth hormone on serum lipid in aged male patients with chronic heart failure (CHF).

METHODSEighty seven patients with chronic heart failure(> or = 60 years old) were randomly divided into 2 groups: the CHF control group (n = 46) who received regular therapy and the CHF experimental group (n = 41) who received regular therapy and recombinant human growth hormone. The treatment would be continued for 3 months. Another group was normal control group (n = 10). The detection of serum growth hormone (GH), insulin-like growth factor (IGF-1), total cholesterol (TC), triglyceride (TG), low-density lipoprotein cholesterol (LDL-C), high density lipoprotein cholesterol (HDL-C) was carried out before and after treatment in the participants.

RESULTSBefore treatment, the levels of GH and IGF-1 were not significantly different among groups. After treatment, the levels of GH (0.71 +/- 0.34 vs 0.96 +/- 0.48) and IGF-1 (95.64 +/- 21.11 vs 111.64 +/- 23.14)in CHF experimental group were higher than those before the treatment. In CHF control group, the levels of GH(0.81 +/- 0.32 vs 0.79 +/- 0.29) and IGF-1 (97.82 +/- 19.74 vs 99.65 +/- 20.11) had no significant change after the treatment. After treatment, the levels of GH (0.96 +/- 0.48 vs 0.79 +/- 0.29) and IGF-1 (111.64 +/- 23.14 vs 99.65 +/- 20.11) in CHF experimental group were higher compared with that of CHF control group. Before treatment, the serum levels of LDL-C, HDL-C, TC and TG had no significant difference among groups. After treatment,the levels of LDL-C (2.11 +/- 0.82 vs 1.76 +/- 0.51) and TC (3.78 +/- 1.34 vs 3.21 +/- 1.17) in CHF experimental group were lower than those before the treatment. However, the levels of HDL-C (1.10 +/- 0.31 vs 0.99 +/- 0.28)and TG (1. 89 +/- 1.07 vs 1.66 +/- 0.95) had no significant change after the treatment compared with before treatment. In CHF control group, the serum lipid levels had no significant change after the treatment.

CONCLUSIONAs the treatment of rhGH for aged male patients with chronic heart failure, GH influences lipid metabolism, which reduces the level of LDL-C, TC. However GH has no effects on the serum HDL-C and TG level. With the treatment of rhGH for long-term, lipid metabolism should be paid attention,and the treatment for blood lipid reduction should be adjusted in time.

Aged ; Chronic Disease ; Heart Failure ; blood ; therapy ; Human Growth Hormone ; pharmacology ; Humans ; Lipids ; blood ; Male ; Recombinant Proteins ; pharmacology

Objective To investigate the clinical features and therapy of fournier's gangrene.Methods The clinical data of 12 patients with fournier's gangrene who were treated at the Third Affiliated Hospital of Nanjing University of Chinese Medicine from January 2010 to February 2012 were retrospectively analyzed.Eleven patients had perianal abscess and 1 patient had perianal foreign body,most of the patients were presented with perianal pain,fever (＞ 38.5 ℃) and tachycardia.The mean interval between the onset of symptoms and admission to the hospital was 8 days (range,3-20 dyas).Seven patients were complicated with diabetes.Only 2 patients were administered hypoglycemic agents,but the effects were poor.Six patients were complicated with hypertension.One patient had the history of resection of sigmoid colon cancer 2 months before operation.Debridement and drainage were applied to all the patients after examination.Antimicrobial therapy applied to all the patients according to the results of drug sensitivity test.The incisions were washed by hydrogen peroxide solution postoperatively.Redebridement was applied if the disease was progressed.All patients were followed up via out-patient examination and phone call till June 2012.Results Results of clinical features:Perianal tissues necrotized,with the color of black.The scrotum or labia majora of 8 patients were invaded by the necrotic tissues.Results of laboratory test:the white blood cell count was (3.8-27.6) × 109/L,the white blood cell count of 6 patients was above 10 × 109/L,3 was above 20 × 109/L.The ratio of neutrophil granulocytes was 0.61-0.93.Four patients received single debridement,5 received redebridement,and 3 patients received debridement for more than 2 times.Two patients had sapremia and infectious shock,and they received treatment for 3 days and 4 days at the intensive care unit.Results of bacterial culture:6 patients were infected by the escherichia coli,4 by klebsiella pnenmoniae,and 1 by pseudomonas aeruginosa.The mean duration of hospital stay was (25± 14)days (range,1-49 days).All patients were followed up for 4-29 months,1 patient was discharged at postoperative day 2,and the prognosis of the 11 patients was good,no mortality was observed.Conclusions The clinical features of fournier's gangrene include severe pain and rapid spread of infections caused by necrosis of perianal subcutaneous tissues.Diabetes might be the risk factor of fournier's gangrene.Early diagnosis and prompt and aggressive surgical debridement are critical for improving survival.

In this paper a new type of cerebral circulation is introduced, including the basic principal, parameter algorithms and equipment design. The analyzer is developed on the basis of previous cerebral circulation analyzer and combined with the latest development of hemodynamics. It has the advantages of previous analyzer and overcomes its shortcomings frequently encountered in clinical that unable to finish the analysis without detection of all the intracranial vessels. It provides new functional module and adds indices such as hydraulic power, carotidshear stress, comprehensive index etc. This analyzer can be used for cerebral circulation dynamic analysis and auxiliary diagnosis of cerebrovascular diseases.
Algorithms ; Cerebrovascular Circulation ; Cerebrovascular Disorders ; diagnosis ; Equipment Design ; Hemodynamics ; Humans

Endomyocardial Fibrosis ; Fibroblasts ; Humans ; Myocardium ; pathology

Cardiovascular System ; Pharmacogenetics

Objective Intramuscular ketamine is often used for premedication in children. Premeditation can also be administered perorally in children. The aim of this study was to evaluate the efficacy of different compounds of ketamine given perorally as premedication in children. Methods Seventy-five ASA Ⅰ- Ⅱ pediatnc patients weighing 10-30 kg undergoing urologic operation were randomly divided into 5 groups of 15 patients each : (1) control group received atropme 0.015 mg ? kg-1 im 30 min before surgery; (2) DA group received intramuscular diazepam 0.2 mg?kg-1 and atropine 0.015 mg?kg-1 30 min before operation; (3) (4) (5) KMA groups received ketamine 3 mg?kg-1 (K3MA) or5mg?kg-1 (K5MA) or 8mg?kg-1 ( K8 MA) + midazolam 0.5 mg?kg-1 + atropine 0.03 mg?kg per os 30 min before operation. SpO2 and heart rate (HR) were monitored and recorded before premedication and at 0, 5, 10, 15, 20, 30 and 40 min after premedication. Peak effect time, duration of operation and emergence time were also recorded. Sedation, anxiolysis and behaviour at separation from parents, during venepuncture and induction were graded and assessed. Results There was no significant difference in duration of operation among the five groups. The peak effect time in the three KMA groups was shorter than that in control and DA group and was shortest in K8MA group. The three KMA groups were significantly better than control and DA group and the K8 MA group was the best in terms of sedation, anxiolysis and analgesia. The incidence of adverse effects like diploplia headache and agitation was higher in K8MA group. Conclusion K5MA group provides satisfactory sedation and analgesia similer to Kg MA group with less side-effects, so is the oral ketamine compound of choice for premedication in children.

Objective: To explore cognitive dysfunction in patients wi th schizophrenia Methods: 21 first onset patients with schizophr e niform disorder and 26 patients with chronic schizophrenia were studied with Wis consin Card Sorting Test (WCST), Semantic Fluency Test, Association Learning, Co mprehension, Similarity and Digit Span Test 22 normal controls also received t h ese tests Results: Both patients' groups did worse on above te st s than normal control The first onset patients were impaired cognitively as th e chronic schizophrenics in all tests except for Association Learning No correl a tion was found between result of WCST and other neuropsychological tests Conclusion: Schizophrenic patients are cognitively impaired early in t he course of psychosis

OBJECTIVE To understand the clinical distribution and drug resistance of Enterococcus to provide evidence for the rational use of antibiotics. METHODS The Enterococcus isolated form clinic from Jan 2006 to Dec 2007 and the sensitivity to 13 kinds of antibiotics were tested by K-B method. RESULTS All of 122 strains of Enterococcus were isolated which included 76 strains of E.faecalis(62.3%),29 strains of E.faecium(23.8%) and 17 strains of other enterococcus(13.9%).Most enterococcus were detected from vaginal secretion,urine,drainage fluid,cervical secretion and throat swab.Drug sensitivity test showed that the sensitivity to vancomycin and teicoplanin was high while the resistance to rifampicin,erythromycin,tetracycline and ciprofloxacin was the highest,and the total resistance rate of E.faecium was higher than E.faecalis. CONCLUSIONS The detection rate and resistance rate of Enterococcus present a rising trend,which should be paid for more attention by clinic.Bateria cultivation is necessary for suspected enterococcus infection and the drugs selected according to sensitivity result to improve the curative effect.