The orphan drug management system can significantly improve the quality of care and that of life of patients suffering from rare diseases, and accelerate the progress of medicinal science. With experiences of practicing the orphan drug management system in developed countries and regions,including certification of orphan drugs, supports for developing orphan drugs, market protection and clinic application management. Based on such studies, the authors discussed the feasibility and methodology of establishing an orphan drug management system in China, along with the significance of such a system.

Objective To investigate the efficacy of preemptive analgesia with parecoxib, a novel intravenous cyclooxygenase type-2 inhibitor, far acute postoperative pain management after intracranial tumor resection.Methods Sixty ASA I or II patients of both sexes aged 18-60 yr with body mass index < 30 kg/m~2 were randomized into 2 groups ( n = 30 each) : control group (group C) and parecoxib group (group P) . In group P, parecoxib 40 mg in 2 ml of normal saline ( NS) was injected iv over 2 min before induction of anesthesia. In group C NS 2 ml was injected instead of parecoxib. Patient controlled intravenous analgesia (PCIA) with fentanyl (bolus dose 0.05 μg/kg, lockout interval 15 min, background infusion 0.2μg·kg~ (-1)·h~(-1), 24 h maximum dose 9.6μg /kg) was used after operation. The number of successfully delivered doses and the number of attempt were calculated. If PCIA did not provide satisfactory analgesia (VAS < 3) , iv bolus of fentanyl 1μg /kg or tramadol 12 mg/kg was given as rescue medication. VAS (0 = no pain, 10 = worst pain) was used to measure pain intensity and recorded at 2, 6, 12 and 24 h after operation. Patient's satisfaction, nausea and vomiting were recorded, and activated coagulation time (ACT), coagulation rate (CR) and platelet function (PF) were measured before and 2 h after parecoxib administration. Results The consumption of fentanyl, the number of successfully delivered doses and the number of attempt, the number of rescue medication administration and degree of nausea and vomiting were significantly lower while the level of patient's satisfaction was higher in group P than in group C. There was no difference in ACT, CR and PF between the two groups. Conclusion Parecoxib given before induction of anesthesia can improve the efficacy of PCIA with fentnayl and decrease side effects.

Objective To observe the expression of neuron specific enolase (NEC) to evaluate the neuroprotective effect of a cell-penetrating Bax-inhibiting peptide (BIP) on neonatal rats with hypoxic-ischemic brain damage (HIBD). Methods Wi-star rats (7-day old) were randomly divided into Sham group, BIP group and HIBD group. After modeling HIBD, the histologi-cal (HE staining) and immunohistochemistry methods were used to determine the apoptotic pathological changes and the NSE expression levels in the brain at different time points. Results Compared to the Sham group, the rats of HIBD group showed significant apoptotic pathological changes. The histological changes and the brain damages were improved significantly in BIP group at each sampling point. The number of NSE-positive cells was significantly decreased in HIBD and BIP groups over time (P<0.05). The number of NSE-positive cells had significant difference among different groups at 48 h, 96 h and 7 d after opera-tion (F=45.35-81.66, P<0.01). The number of NSE-positive cells in the HIBD group was smaller than that of the Sham group and BIP group 48 h after operation (P<0.05). The number of NSE-positive cells in the BIP group was smaller than that of the Sham group 96 h after operation (P<0. 05). Conclusions BIP can decrease the apoptosis of cortex nerve cells in 7-day old HIBD rat model, and may have neuroprotective effect on the early stage of HIBD.

Objective To compare the accuracy of jugular venous oxygen saturation (SjvO2),somatosensory evoked potentials (SSEPs) and motor evoked potentials (MEPs) in estimation of the occurrence of intraoperative cerebral ischemia in patients undergoing clipping of intracranial aneurysm.Methods Forty-three ASA Ⅰ or Ⅱ patients of both sexes,aged 18-64 yr,with a body mass index of 20-25 kg/m2,undergoing clipping of intracranial aneurysm,were studied.Anesthesia was induced with sufentanil,rocuronium and propofol.The patients were tracheal intubated and mechanically ventilated.Anesthesia was maintained with remifentanil and propofol.Blood samples were taken from the jugular bulb for detection of SjvO2 before aneurysm clipping or temporary occlusion of parent artery and at 1,3,10,20 and 30 min after clipping aneurysm or temporary occlusion of parent artery.The amplitude and latency of SSEPs and MEPs were recorded simultaneously.The occurrence of cerebral ischemia estimated by SjvO2,SSEPs and MEPs was recorded.The condition of nerve defect was recorded within 3 days after operation and the gold standard of cerebral ischemia was defined as the occurrence of nerve defect.Results Among 43 patients,14 cases were diagnosed as having brain ischemia.The sensitivity and specificity of SjvO2 in estimation of the occurrence of intraoperative brain ischemia were 71％ and 93％,respectively (P ＜ 0.01).The sensitivity and specificity of SSEPs in estimation of the occurrence of intraoperative brain ischemia were 71％ and 62％,respectively (P ＜ 0.05).When the diagnostic criterion of cerebral ischemia was defincd as a decrease in the amplitude of MEPs or prolongation of the latency MEPs,the sensitivity and specificity of MEPs in estimation of the occurrence of intraoperative brain ischemia were 79 ％ and 52 ％,respectively (P ＞ 0.05).When the diagnostic criterion of cerebral ischemia was defined as a loss of the amplitude of MEPs,the sensitivity and specificity of MEPs in estimation of the occurrence of intraoperative brain ischemia were 57％ and 93％,respectively (P ＜0.05).Conclusion The sensitivity of SjvO2 and SSEPs in estimation of the occurrence of intraoperative brain ischemia is higher,however,the specificity of SjvO2 and MEPs is higher,indicating that SjvO2 is a reliable criteria for estimation of the occurrence of intraoperative brain ischemia in patients undergoing clipping of intracranial aneurysm.

Objective To evaluate the agreement of bispectral index values recorded from tumor side and the contralateral areas during supratentorial craniotomy.Methods Thirty-five patients (16 males, 19 females, aged 18-65 years, ASA physical status Ⅱ or Ⅲ) scheduled for supratentorial tumor resection were enrolled in this study.Bispectral index (BIS) sensors were placed at bilateral frontal areas.The patients were anesthetized with propofol.From each BIS monitor, we collected data at each of four time stages: before the induction of anesthesia, before dura opening, removal of tumor and recovery of conscious.These data were compared using Bland-Altman analysis.Results Bland and Altman analysis revealed a BIS negative-bias (limits of agreement) of before induction-0.8(-7.2-5.7), before dura opening 0.6 (-8.3-9.5), and removal of tumor 1.5(-6.9-9.9), recovery of conscious 0.2 (-9.3-9.8).Conclusion There are significant agreement for BIS values between the frontal area of tumor side and the contralateral areas.BIS values can be used interchangeably between bilateral frontal.