Objective To report the spectrum of electron transfer flavoprotein dehydrogenase (ETFDH)gene mutations in 20 Chinese RR-LsM families.Methods Twenty-four RR-LSM patients in the First Hospital of Peking University from January 2003 to May 2010 were collected and the clinical characteristics were analyzed.These patients came from 20 families in North Mainland China.Sixteen families had 1 patient each.and the other 4 families had 2 patients.ETFDH gene analysis was performed in all patients,11 family members and 100 healthy controls.Results The mean onset age was(27.9±9.9)years.The main symptoms were limb weakness(21,87.5%),dysmasesia(15,62.5%),neck weakness (14,58.3%)and myalgia(14,58.3%).Eighteen patients had high level of acyleamitine.Fifteen of 17patients had glutaric aciduria.Seventeen ETFDH mutations,including 13 missense mutations,2 splice mutations,and 2 nonsense mutations,were identified in 19 families:c.998A>G,c.1450T>C,c.1703T>C,c.1717C>T,c.821G>A,c.643G>A,c.251C>T,c.1763A>T,c.IVS7+2T>C and c.IVS6+1G>A were Hovel mutations which were not found in 100 healthy controls.Nine families had the mutation of c.770A>G(P.Y257C)and 5 families had the mutation of c.1227A>C(P.L409F).Conclusions The numerous novel mutations in ETFDH gene indicate that Chinese RR-LSM might have special mutation pattern.c.770A>G(P.Y257C)and c.1227A>C(p.L409F)may be hot spot mutations in North Mainland China.

Objective To investigate the inductive factors, the prevention and treatment methods of pulmonary edema during pregnancy Methods The clinical materials of 21 cases of preganacy with pulmonary edema treated in our hospital from Jan 1985 to Jan 2000 were analysed retrospectively Results Among 21 patients, 26 pulmonary edema attacked, 9 of them had upper have respiratory diseases, thirteen cases caused by improper infusion Fourteen cases were well controlled quickly with antiplasm nifedipine, myocardial streng and diuretics If the symptoms could not be controlled properly, vasodilators were applicated We had 16 cesarean section under epidural anaesthesia, one vaginal birth, and the other one birth by forceps Conclusions Pulmonary edema in pregnancy usualluy followed with acute heart failure caused by sever pregnancy induced hypertension We should pay more attention to the indications of it During treatment,we should give vasodilators properly,so as to prevent pulmonary edema

Purpose:To observe the effect of thalidomide combined with chemotherapy in the treatment of newly diagnosed multiple myeloma. Methods:34 patients were divided into two groups at random. 14 cases in the treated group were initially administered thalidomide at doses ranging from 100mg~200 mg/d,and the dosage was escalated by 100mg~200 mg every two weeks until patients could not tolerate the treatment.The maximal dose did not exceed 600mg/d.MP or M 2 protocol was delivered to those patients in the treated group concomitantly.20 cases in the control group received chemotherapy consisting of MP or M 2 protocol only.Evaluation of 3-months effective(partial remission plus progress)rate,one-year event free survival and two-years overall survival was done in both groups. Results:3-months effective rate is 85.7% in the treated group and 60% in control group.One-year EFS is 71.4% in the treated group while that in the control group is 35%.Two-years OS are 57.1% and 25% respectively.The side effects of thalidomide are tolerable,and constipation is the most common. Conclusions:The combination of thalidomide and chemotherapy is an effective treatment for newly diagnosed MM,and it demonstrates an improvement in effective rate,one-year EFS and two-years OS.

Objectives:To investigate the effect of early enteral feeding on intestinal mucous energy systhesis in burned rats. Mothods:Wistar rats with 30% total body surface area Ⅲ degree burns were randomly divided into 3 groups:early feeding(EF) group,delayed feeding(DF) group,control group.The ATP,ADP,AMP of intestinal mucosa were detected by HPLC and energy charge were calculated according to formula. Results:The contents of ATP,ADP and energy charge in both experimental groups were all fluctuated at much lower level than those in control group,and the above indices exhibited significant decrease in DF group as compared with EF group at all time points except post burned 6 h.On the contrary,the contents of AMP increased more significantly in DF group than in EF group. Conclusions:Early feeding could increase the energy storage and alleviate the catabolism of energy rich phosphates of intestinal mucosa.

Response surface methodology (RSM) based on a three-level three-factor Box-Behnken design of experiments was used to optimize the extracellular polysaccharide content and the mycelium biomass by submerged cultivation using Pholiota squarrosa AS 5.245. The critical factors selected for the investigation were temperature, time of cultivation and volume of medium, based on the results of our previous Plackett-Burman design. The objectives of this present work were to locate optimum levels of these process parameters, and to find out interactions among them for enhancement of the yield of extracellular polysaccharide and mycelium biomass. By solving the regression equation and also by analyzing the response surface contour plots, the optimal process conditions were determined: under conditions of temperature, 28.07℃; cultivation time, 8.79 d and volume of medium, 68.51 mL, the prediction of extracellular polysaccharide content (EPC) by Pholiota squarrosa AS 5.245 was 1062.69 ?g per milliliter of fermentation liquor. While cultivation temperature, time and volume of medium were 27.60℃, 9.42 d and 54.20 mL respectively, the mycelium biomass expressed as dry cell weight (DCW) was 11.32 mg?mL -1. In order to simultaneously obtain the maximum yield of EPC and DCW, the above conditions would be located at 27.62℃, 9.19 d and 64.10 mL. In these conditions, the maximum predicted yield of EPC and DCW were found to be 1050.64 ?g?mL -1 and 11.10 mg?mL -1, respectively. These predicted values were also verified by validation experiment.

Aged ; Coronary Disease ; Coronary Sinus ; pathology ; Female ; Heart Atria ; pathology ; Humans ; Middle Aged

OBJECTIVE To investigate and analyze the reasons of nosocomial infection(NI) in patients in intensive care unit( ICU),to find effective countermeasures for preventing NI and reducing the incidence rate of NI in ICU,and to enhance the management of ICU.METHODS All data of patients who suffered MRSA in ICU from Dec 2005 to Feb 2006,were analyzed by prospective monitoring and retrospective studies.RESULTS The nine patients were with lower respiratory tract.All were infected by the same MRSA.The same MRSA strain was found from hand,mouth and nose among the treating doctor and nurses based on the sample-analysis.CONCLUSIONS The incidence rate of NI in ICU is much higher than that in other departments.Risk factors depend on the severity of underlying diseases,invasive procedure,the quality of disinfection and sterilization,the incorrect use of the antibiotic,and patients′ immunity status especially among elderly.The key way to reduce incidence rate of NI is to take comprehensive measures and strengthen the antibiotic management.

Objective To investigate the effects of glucagon like peptide 2(GLP 2) on the mRNA expressions of GLP 2 receptor (GLP 2R) and proglucagon gene(PG) in postburn rats. Methods A total of 55 Wistar rats were randomly divided into three groups including burn group, GLP 2 treatment group(treated with GLP 2, 200 ?g/kg, b.i.d ) and normal control group. Rats were sacrificed at 6, 12 h and 1, 3 and 5 d after burn injury. The proliferating cell nuclear antigen(PCNA) expression and the histological changes of the intestinal mucosa were determined by immunohistochemical staining. mRNA expressions of GLP 2R and PG were detected by RT PCR at 6, 12 h and 1, 3 and 5 d after burn injury. Results The expression of PCNA in rat intestinal mucosa decreased at 1 d after burn injury, but it was stronger in GLP 2 treatment group than that in burn group. Histological observation revealed that intestinal villi in GLP 2 treatment group were regularly arranged without obvious epithelial shedding. PG mRNA expression peaked at 12 h, 1 and 3 d after burn injury. No change of PG mRNA expression was found after treatment with GLP 2. Decreased GLP 2R mRNA expression was found in postburn rats, but after treatment with GLP 2, increased GLP 2R mRNA expression was found. Conclusion GLP 2 supplementation can keep the structure of the postburn rat intestinal mucosa without affecting PG gene expression. The mechanism may probably be related to the promotion of the mRNA expression of GLP 2R of the intestinal mucosa in rats.

ObjectiveTo compare the effect and safety of ropivacaine mesylate with ropivacaine hydrochloride in epidural anesthesia of patients make hypogastric region and lower extremity operation.Methods126 patients with epidural anesthesia were divided into two groups,each group 63 cases.The observation group was administered with ropivacaine masylate,and the control group was administered with ropivacaine hydrochloride.The sense of pain block plane and effective-acting period and effect time,sports block rating and effect-acting period and duration,vital signs,adverse events and serious adverse events were observed.ResultsThere were no significant differences between the two groups on sense of pain block plane and effective-acting period and effect time,sports block rating and effect-acting period and duration,Bp,HR,SpO2,chang of ECG,bleeding in operation( t =13.23,10.52,10.64,12.21,13.23,10.52,10.64,12.21,6.11,5.34,5.23,6.05,all P ＞ 0.05 ).ConclusionThe results of ropivacaine mesylate used for anesthesia epidural was satisfied and had no obvious side effects as well as ropivacaine hydrochloride.

Objectives To analyse the clinical and MRI features of cerebral autosomal dominant arteriopathy with subcortical infarct and leucoencephalopathy (CADASIL) in mainland China and compare these features with those reported in other countries.Methods All 26 CADASIL families were collected in First Hospital of Peking University from January 2003 to October 2009, and the diagnosis was confirmed by ultrastructural examination or Notch3 gene analysis.The age of onset, initial symptoms, main symptoms in 102 patients were described and the features with those reported in Germany, Japan, Arab and France were compared using x2 test.The cranial MRI changes in 35 patients were analysed and compared with the British and French patients.Results 102 patients had their initial symptoms between 22-80 years with the mean age of onset at (43.9 ± 11.0) years.There was no significant difference in the frequency of ischemic stroke or TIA (79.41% ) between our patients and the patients in other countries.Dementia rate (50.00% ) was significantly greater than that of Arab (21.05% ,x2 =5.513, P =0.020) and French patients(31.11%,x2 =4.517, P =0.034).The frequency of mood disturbances ( 14.71% ) was significantly lower than that of German patients (30.39% ,x2 =7.185, P =0.007).The frequency of migraine ( 13.73% ) was similar to that of France but lower than that of Japan (40%, x2 = 12.658, P = 0.000), Germany (38.24%, x2 =15.932, P=0.000) and Arab (42.11% ,x2 =6.869, P=0.009).Cranial MRIs were abnormal in all 35patients, but no lesion was seen in the medulla oblongata.Lacunar infarcts in the basal ganglia (82.86% )were significantly more frequent than that of French patients (60% , x2 = 5.663, P = 0.017).The frequency of leukoaraiosis in the cerebrum was similar to that of French patients.Anterior temporal involvement (68.57%) was significantly lower than of British patients ( 95%, x2 = 5.211, P = 0.022 ).Conclusion The clinical symptoms and MRI changes of CADASIL in different countries were not identical.Abnormal anterior temporal pole signal on MRI is not a sensitive marker for diagnosis of CADASIL in Chinese patients.