Objective To explore the significance of serum myoglobin detection in the diagnosis and evaluation of Kennedy`s disease (KD).Methods The level of serum myoglobin (Myo) was detected in 60 KD patients and 30 amyotrophic lateral sclerosis (ALS) patients.Results The serum Myo level and abnormal rate in KD group were significantly higher than those in ALS group (all P<0.001).The serum Myo level was positively related with the disease course of KD (r=0.665,P<0.01).There was no significant difference of serum Myo level and disease course of ALS (r=-0.047,P>0.05).There was no overlap of serum Myo level between the two groups.There was no significant difference of serum Myo level and CAG repeated number of ALS (r=-0.193,P>0.05).Conclusion Myo is likely as an easy and sensitive biomarker, used to identify the KD and special type of ALS, and used in the evaluation of the KD condition in the future.

AIM: To explore the effects of sitai tablet on blood pressure, blood lipid,blood sugar and insulin of rats with insulin resistance syndrome(IRS).METHODS: A total of 40 male clean SD rats were divided into normal control group, model group, sitai group and captopril group by randomized block design. The rats were fed with high-fat high sugar diet for 8 weeks to induce IRS. Then, sitai tablets were given to rats with IRS to observe the influence of sitai tablet on blood pressure, blood sugar, blood lipid, insulin levels and insulin sensitivity index(ISI) of them.RESULTS: The systolic pressure reduced from ( 158 ± 8), ( 157 ± 7) mm Hg before treatment to (128 ± 4), (110 ± 2)mm Hg after treatment in sitai group and captoril group respectively. There was also significant difference on systolic pressure of between sitai group, captopril group and model group [ ( 165 ± 6) mm Hg] ( t = 11.86 - 28.99, P < 0.01 ). In sitai group, the total cholesterol (TC), triacylycerol (TG)decreased clearly than those in model group( t = 44.27, 9. 14, P < 0.01 ), but high density lipoprotein cholesterol (HDL-C) increased( t = 703, P < 0. 01 ) . There were increased fasting insulin(FINS) and decreased ISI in model group. There was significant difference on these indes between model group and normal control group( t= 7.14, 9.35, P < 0.01 ) . The insulin resistance was evident so that the model was successfully established. The fasting blood sugar and FINS all reduced when comparing it between sitai group, captopsil group with model group, there was significant difference( t = 11.95, 6.71, P < 0.01). When comparing the fasting blood sugar in captoril group and sitai group, there was also difference( t = 8.33, P < 0.01 ) . The ISI all increased in sitai group and captoril group, there was difference between them and model group (t=9.82,9.35, P <0.01).CONCLUSION: Sitai tablet can remarkably reduce the blood pressure,blood sugar and blood fat, and increase the sensibility of insulin so that it can improve the general effects of IRS.

[Objective]To report the result of percutaneous biopsy in bone tumor and analyze the interrelated influence factors.[Method]A total of 171 patients were subjected to trocar puncture for biopsy of bone tumor in pre-operation,and the results of all cases were reviewed.[Result]Totally 155 cases in 171 cases showed positive outcome,the positive rate was 90.64%.The accuracy of 98 cases was comfirmed in 122 postoperative pathology diagnoses,and the acuracy rate was 80.33%.There were 110 cases of tumor.Biopsy sensitivity was 95.41%,and biopsy diagnostic particularity was 100%.[Conclusion]Percutaneous biopsy is both effective and valuable for the diagnosis of bone tumor in pre-operation,but the biopsy positive rate and accuracy,and postoperative complication was highly related to the operator cognitive level of bone tumor and meticulous manipulation and so on.

Objective To develop a scale to assess Kennedy's disease (KD) based on patients' clinical characteristics and to validate its application in patients.Methods We developed KD1234 Scale following the model of Amyotrophic Lateral Sclerosis-Functional Rating Scale.Using this new scale,we assessed patients with genetic diagnosis of KD and evaluated the reliability and validity of the new scale.Reliability was analyzed using Cronbach' s α coefficient and split-half reliability.Validity was analyzed by content validity and structure validity.Results The Cronbach' s α coefficient of the total scale was 0.789 and split-half reliability coefficient was 0.868.The α of breathing with total score was 0.3-0.4,α of written,language and swallowing with the total score was 0.4-0.5,α of other items with the total score was ＞ 0.5 (P ＜ 0.01 in each of above α).Four common factors extracted by exploratory factor analysis had well correspondence between the scale construction and the theoretical construction.Factor loading was ranged from 0.541 to 0.864 for each item.The duration of the disease showed some correlation with the score of KD1234 Scale.Conclusions The KD1234 Scale demonstrates high reliability and validity.The duration of the disease indicates negative correlation with KD1234 Scale scores.The KD1234 Scale can be used in clinical research to evaluate the condition of KD patients quantitatively.

Objective To study diagnosis and treatment of rhabdomyosarcoma (RMS) in children.Method The clinical data of 43 children with RMS was retrospectively reviewed from Nov 2004 to May 2012.Their long-term survival was followed up.Forty-three children with RMS(male 26,female 17) in Beijing Tongren Hospital were enrolled.Their median age on diagnosis was 6 years old.We confirmed the diagnosis according to pathological diagnosis.Comprehensive treatment included chemotherapy,surgery and implantation of radioactive seeds,and so on.Statistical analysis was used on clinical data and treatment efficacy.Result The median follow up time of 43 cases with RMS in children was 31 mouths until May 2012.Four cases abandoned,39 cases were followed up.The cases of complete remission were 27(69％),and the cases of partly remission were 7 (18％),and the cases of dead were 5 due to cerebral metastasis.Overall survival rate was 87％ (34/39).Eight cases were treated with 125Ⅰ particles implants into its location,7 cases responded,1 case was invalid.Conclusion Although anatomic structure of RMS of head and neck was very complex,and the prognosis of it with metastatic disease was inferior,the muttidisciplinary treatment can still improve the clinical remission rate.In particular,high risk RMS with 125Ⅰ particles implants and large dose chemotherapy would be beneficial.

Three human leucine-rich repeats and immunoglobulin-like domains (LRIG) genes and proteins, named LRIG1-3, has been previously characterized and it was proposed that they may act as suppressors of tumor growth. The LRIG1 protein can inhibit the growth of tumors of glial cells and the down-regulation of the LRIG1 gene may be involved in the development and progression of the tumor. Real-time reverse transcription-polymerase chain reaction (RT-PCR) is a recently developed technique for quantitative assessment of specific RNA levels. In the current study, it was demonstrated that LRIG1-3 and EGFR mRNA was detected in human pituitary adenoma cell lines and a normal pituitary sample, with differences in the expression levels. Compared to the normal pituitary samples, the expression of LRIG1-3 in HP75 cell line was lower, but the expression of EGFR in HP75 cell line was higher. The results are consistent with LRIG1-3 being tumour suppressor genes, and LRIG genes decreasing the expression of EGFR. The ratio of EGFR/LRIG1 was increased at least 13-fold in HP75 cells compared with the normal pituitary cells, which was also the case for the ratio of EGFR/LRIG2 (14-fold increase in HP75) and EGFR/LRIG3 (11-fold increase in HP75). Further studies were needed to elucidate the explicit role of LRIG genes as negative regulators of oncogenesis in human pituitary adenoma.

From May, 2000 to June, 2001, 27 patients with Parkinson disease (PD), including 10 cases of rigidity, 13 cases of tremor, 4 cases of rigidity and tremor, were treated by microelectrode-guided technique. Among them, phlebotomy was carried out in 17 cases and thalamotomy in 10 cases. All the targets of lesion were anatomically located by using MR and neurophysiological signals on microelectrode. Our results showed that the efficiency of microelectrode-guided technique for treatment of PD was 98%. The postoperative unified parkinson disease rating scale were 12.3 +/- 9.1 and 13.2 +/- 8.9 respectively, which significantly improved as compared with those before operation. It was concluded that by recognizing special electrical signals in neurons microelectrode-guided neuropsychological techniques can locate target at cellular level, which overcomes the individual difference in anatomy and function, and allow more accuracy, safety and efficiency of operation. This is especially true of PD patients who fail to respond to medical treatment.
Magnetic Resonance Imaging ; *Microelectrodes ; Parkinson Disease/*surgery ; Stereotaxic Techniques ; Thalamus/surgery

Objective To evaluate the value of intraduetal ultrasonography (IDUS) in diagnosing biliary and pancreatic disorders. Methods The findings by endoscopic retrograde cholangiopancreatography (ERCP) and IDUS from 19 patients with suspected biliary and pancreatic disorders from July 2006 to August 2007 in our hospital were analyzed retrospectively. Results Of the 19 patients, 17 had obstructive jaundice (including 6 eases of cholangiocarcinoma, 2 pancreatic adenocareinoma, 2 gallbladder carcinoma, 2 chole-docholithiasis with bile duct stricture, 2 autoimmune pancreatitis, 1 papillary adenocarcinoma, 1 papillary adenoma, and 1 sclerosing cholangitis) and 2 intraduetal papillary mueinous tumor (IPMT). The diagnosis was confirmed by surgery and pathological findings in 11 patients. The diagnostic accuracy of ERCP and IDUS was 73. 7% (14/19) and 84. 2% (16/19), respectively, and that of ERCP combined with IDUS was 89. 5% (17/19). The sensitivity and specificity of ERCP to differentiate benign bile duct strictures from ma-lignant ones were 100. 0% (11/11) and 83.3% (5/6), respectively; and those of IDUS were 100. 0%(11/11) and 100. 0% (6/6), respectively. The sensitivity and specificity of ERCP in diagnosing cholan-gioeareinoma were 83.3% (5/6) and 60% (3/5), respectively; and those of IDUS were 100. 0% (6/6) and 40. 0% (2/5), respectively. Conclusion Combination of ERCP with IDUS can improve the diagnostic accuracy of pancreaticobiliary disorders. Additionally, IDUS shows higher sensitivity and specificity in differ-entiation between benign and malignant bile duct strictures, but it is still difficult to identify the etiologic factors of malignant bile duet strictures by IDUS.

Objective To observe the effect of systemic chemotherapy on conditions of tumor infiltrating,metastasis and disease-specific survival (DSS) for advanced retinoblastoma (RB).Methods Forty-one patients with advanced RB who received enucleation were enrolled in this study.There were 26 males and 15 females,age at diagnosis was ranged from 2 to 72 months,with a mean of 23.08 months.There were 16 bilateral patients and 25 unilateral patients;13 group D eyes and 28 group E eyes.16 patients received enucleation as the primary treatment (operation group),25 eyes received chemotherapy before enucleation (chemotherapy group).There was no significant statistical difference between two groups for the gender,unilateral and bilateral,international staging or diagnostic age (P＞0.05).The histopathology report was performed to assess the risk of postoperative tumor-node-metastasis staging (pTNM) in each patient,and the extent of tumor invasion in the optic nerve,choroid and anterior chamber was divided into 3 levels of low risk,medium risk and high risk.Five deaths were all in the group E with chemotherapy before enucleation.Using R software survival analysis software package survfit function,the application of Kaplan-Meier estimation method,DSS of RB children was calculated from the time of diagnosis,up to the date of the death of patient.DSS differences between chemotherapy,operation group and eye removal time (more than 3 months,less than 3 months) in group E RB children were analyzed.Results The proportion of high risk pTNM stage in chemotherapy group was significantly lower than the operation group.But there was no significant difference between the two groups in the overall risk classification (x2 =3.130,P=0.077).For group D eyes,the overall risk classification in chemotherapy group was significantly lower than the operation group (x2 =5.870,P=0.015).There was no significant difference between the two groups in the overall risk of group E eyes (x2 =0.020,P=0.889).The DSS in chemotherapy group and operation group were 0.71 and 1.00,respectively;the difference was significant (x2 =3.700,P=0.05).The DSS in children whose enucleation delayed for more than 3 months and children whose enucleation performed within 3 months were 0.64 and 1.00,respectively;the difference was significant (x2 =4.800,P=0.028).Conclusion Systemic chemotherapy did not reduce the risk of tumor invasion and metastasis in patients with advanced RB.Instead,it will reduce the DSS in group E eyes of RB.

BACKGROUND: Navigation systems all can improve the accuracy rate in cervical pedicle screw insertion, but cannot achieve desired outcomes because of some shortcomings, such as poor imaging quality, complicated operation, poor real-time performance, and invasive navigation.OBJECTIVE: To study the accuracy of X-ray fluoroscopy and CT three-dimensional image navigation in cervical pedicle screw insertion.METHODS: Totally 90 patients undergoing cervical pedicle screw insertion from the First Affiliated Hospital of Nanyang Medical College were enrolled, and randomly divided into control and experimental groups (n=45 per group). 132 cervical pedicle screws were inserted into the patients in the control group manually under X-ray fluoroscopy, and 128 ones were inserted into the patients in the experimental group assisted with CT three-dimensional image navigation. The operation time and intraoperative blood loss in the two groups were recorded. Postoperative CT three-dimensional reconstruction was performed to compare the placement accuracy between two groups.RESULTS AND CONCLUSION: The excellent and good rate of placement in the experimental group (95.3%) was significantly higher than that in the control group (88.6%, P < 0.05). (2) The operation time and intraoperative blood loss in the experimental group were significantly higher than those in the control group, and the navigation matching time and radiant quantity in the experimental group were significantly less than those in the control group (P < 0.05). (3) The Japanese Orthopedic Association scores showed no significant difference between two groups before surgery (P > 0.05),and were significantly improved in the experimental group compared with the control group at 3 and 6 months postoperatively (P < 0.05). The incidence of complications in the experimental group was significantly lower than that in the control group (7% versus 16%, P < 0.05). (5) These results suggest that compared with X-ray fluoroscopy, CT three-dimensional image navigation can improve the accuracy of cervical pedicle screw insertion, showing higher safety and precision. However, it needs complicated operation skills, and long operation time results in massive intraoperative blood loss; thereafter, choosing which placement method depends on the patient condition.