AIM: To investigate the mutation type in the IDUA gene of Liaoning district mucopolysaccharidosis I (MPS-I) patients. METHODS: The mutation type and polymorphism site in the IDUA gene of Liaoning district MPS-I patients were detected by PCR-RFLP, SSCP and DNA sequencing. RESULTS: ① There is a new mutation (1278-g-a) in the IDUA gene of Liaoning district MPS-I patients. ② There is no the common mutation (W402X and Q70X) of European patients and the common mutation (R89Q) of Japanese patients in the 10 families we studied. CONCLUSION: The mutation type in the IDUA gene of Liaoning district MPS-I patients is different from that of other countries and districts.

Objective To evaluate regenerative nerve and functional recovery of target muscle in rats with sciatic nerve defect bridged by acellular nerve allograft made through chemical extraction.Methods Sciatic nerve of SD rats was processed in a volume fraction of 3％ Triton X-lO0 solution and 40 g/L sodium deoxycholate solution.Morphology of myelin sheath,axons and basal lamina tubes of sciatic nerve segments was observed under the light microscopy before and after the chemical processing.Twenty-five Wistar rats were divided into acellular nerve allograft group (n =10),autograft group (n =10) and normal control group(n =5) according to the random number table.A 1 cm sciatic nerve defect was created in acellular nerve allograft group and autograft group,and was respectively bridged by acellular nerve allograft and autograft.Sciatic nerve function index (SFI) was measured every two weeks.Twelve weeks after surgery,nerve conduction velocity (NCV),recovery rate of compound muscle action potential (CMAP) and recovery rate of muscle force were measured in each group.Results Cellular components including myelin sheath and axons were removed thoroughly,but the basal lamina tubes were preserved completely.At postoperative 2,4,6,8,10 and 12 weeks,SFI in normal control group (-1.7±5.9,-0.3 ±2.5,0.8 ±4.1,-1.4±3.6,-2.5 ±5.7 and-2.1±3.2) was superior over autograft group (-94.3±3.7,-90.1±4.1,-63.7±7.8,-51.9±8.2,-48.8±8.6 and -44.3 ± 10.5) and acellular nerve allograft group (-97.1 ± 5.3,-91.2 ± 6.1,-70.6 ± 5.5,-60.4±6.2,-58.2 ±10.2 and-56.4 ±8.0) (P ＜0.01).At postoperative 6,8,10 and 12 weeks,SFI in autograft group were better than those in acellular nerve allograft group (P ＜0.05).NCV [(61.6 ± 8.1) m/s],recovery rate of CMAP[(98.7 ± 5.9) ％] and recovery rate of muscle force [(101.8 ± 6.6) ％] in normal control group were higher than those in acellular nerve allograft group [(22.3 ± 4.7) m/s,(40.3 ± 9.2) ％ and (43.8 ± 9.3) ％] and those in autograft group [(29.0 ±5.5) m/s,(52.5 ± 10.6) ％ and (54.3 ± 10.5) ％] (P ＜ 0.01).NCV,recovery rate of CMAP and recovery rate of muscle force in autograft group were better than those in acellular nerve allograft group (P ＜ 0.05).Conclusions Acellular nerve segments are harvested satisfactorily by chemical extraction.Sciatic nerve defect in rats can be cured with the acellular nerve allograft,but the repair effect of autograft is relatively better.

AIM: To investigate the mutatation type and polymorphism site in the ?-L-iduronidase (IDUA) gene of Liaoning district MPS-I patients. METHODS: The mutation type and polymorphism site in the IDUA gene of Liaoning district mucopolysaccharidosis type I (MPS-I) patients were detected by PCR-SSCP and DNA sequencing. RESULTS: ① 2 new mutations: R363H, 880+g-c in the IDUA gene of Liaoning district MPS-I patients were found. ② There were 3 polymorphism sites: R105Q、L118 and A361T in the IDUA gene of Liaoning district MPS-I patients. CONCLUSIONS: The mutation type in the IDUA gene of Liaoning district MPS-I patients is different from that of other countries and districts, while the polymorphism site in the IDUA gene of Liaoning district MPS-I patients is the same as that of other countries. [

OBJECTIVE:To establish the quality standard for ShutonganⅠgranule.METHODS:TLC was conducted for the qual-itative identification of Citrus aurantium and Forsythia suspensa. HPLC was conducted for the content determination of naringin in the preparation;the column was Agilent C18 with mobile phase of acetonitrile-water(20:80,V/V)at a flow rate of 1.0 ml/min,the detection wavelength was 283 nm,the column temperature was 30 ℃,and the injection volume was 10 μl. HPLC was also used for the content determination of phillyrin;the column was Agilent TC-C18 with mobile phase of acetonitrile- water(23:77,V/V)at a flow rate of 1.0 ml/min,the detection wavelength was 277 nm,the column temperature was 30 ℃,and the injection volume was 10 μl. RESULTS:The C. aurantium and F. suspensa of TLC showed clear spots and good separation. The linear range was 57.843-289.214 μg/ml for naringin(r=0.999 9)and 37.434-187.169 μg/ml for phillyrin(r=0.999 6);RSDs of precision,stability and reproducibility tests were less than 2%;recoveries were 97.20%-102.04%(RSD=1.7%,n=9)and 97.14%-102.27%(RSD=2.0%,n=9),respectively. CONCLUSIONS:The standard can be used for the quality control of ShutonganⅠgranule.

Objective To explore the quantitative characteristics of pancreatic ductal adenocarcinoma(PDAC) in single-source dual energy spectral CT imaging. Methods From January 2013 to December 2014, 113 patients underwent dual phase contrast-enhanced gemstone spectral imaging(GSI) on Discovery CT 750 HD. All diagnoses were pathologically confirmed by surgery or biopsy. The spectral HU curves of PDAC were observed, the monochromatic CT values, the effective atomic number(Zef ), the iodine concentration(IC), water concentration(WC), and the corresponding normalized values(normalized monochromatic CT values, normalized Zef , normalized IC, normalized WC)of the lesion and the pancreatic parenchyma in late arterial phase(AP) and portal venous phase(PP) were recorded . The measurements were performed three times repeatedly. Paired t test (normal distribution) or Wilcoxon test (non-normal distribution) were used for analyzing the differences between the two phases and between PDAC and pancreatic parenchyma. Results The monochromatic CT values of PDAC in AP were lower than in PP at each energy level and the difference was more marked at lower energy. The normalized monochromatic CT values increased with the increase of energy level in both AP and PP and the difference was more distinct at lower energy. The Zef , IC and normalized IC of PDAC all had significant differences(P<0.05), while the WC, normalized Zef , and normalized WC had no difference between AP and PP. The Zef and IC of pancreatic parenchyma had significant differences(P<0.05), while the WC had no difference between AP and PP. The differences of Zef , IC, and WC between PDAC and pancreatic parenchyma were significant in both two phases (P<0.05). Conclusions Dual phase CT spectral imaging showed characteristic quantitative parameters of pancreatic ductal adenocarcinoma. The monochromatic CT values, Zef , and iodine concentration of PDAC were lower than those of pancreatic parenchyma in both AP and PP. The monochromatic CT values, Zef , and iodine concentration of PDAC in late arterial phase were lower than those in portal venous phase. The differences were all more distinct at lower energy.

Objective To compare the clinical features between radiological isolated syndrome (RIS) and classical multiple sclerosis (CMS), in order to improve the understanding of the RIS. Methods All 35 patients with RIS and 32 patients with CMS were selected. The epidemiological and clinical findings, cerebrospinal fluid, neural electrophysiological examination and magnetic resonance imaging (MRI) data were analyzed. Results There were no statistical differences in sex ratio and onset age between RIS patients and CMS patients (P>0.05). The main symptoms of in patients with RIS were headache (45.7%, 16/35), dizziness (40.0%, 14/35), hypomnesis (20.0%, 7/35) and psychiatric disorders (11.4%, 4/35). But the main symptoms of in patients with CMS were limb weakness (75.0%, 24/32), sensory abnormalities (68.8%, 22/32) and ocular symptoms (34.4%,11/32). The incidences of limb weakness, sensory abnormalities and ocular symptoms in patients with CMS were significantly higher than those in patients with RIS:75.0%(24/32) vs. 0, 68.8%(22/32) vs. 0 and 34.4%(11/32) vs. 0, and there were statistical differences (P<0.01). The 18 patients with RIS and 21 patients with CMS underwent the examination of cerebrospinal fluid, and there was no significant difference in leukocyte between patients with RIS and patients with CMS (P>0.05). The cerebrospinal fluid protein and the incidences of IgG index>0.7 in patients with RIS were significantly lower than those in patients with CMS:0.175 (0.03-0.69) g/L vs. 0.440 (0.04-1.09) g/L and 3/18 vs. 47.6%(10/21), and there were statistical differences (P<0.05). The 15 patients with RIS and 22 patients with CMS underwent the examination of neural electrophysiological, and the abnormality rates of visual evoked potential (VEP) and brain stem auditory evoked potential (BAEP) in patients with RIS were significantly lower than those in patients with CMS:4/15 vs. 63.6%(14/22) and 3/15 vs. 54.5%(12/22), and there were statistical differences (P<0.05). But there was no statistical difference in the abnormality rate of somatosensory evoked potential (SEP) between patients with RIS and patients with CMS (P>0.05). On MRI, the demyelinating lesions of RIS and CMS were both mainly distributed in the periventricular, semi-oval center, infratentorial white matter, partly involving corpus callosum or cortical. The rates of demyelinating lesions in brainstem and cerebellum in patients with RIS were significantly lower than those in patients with CMS:5.7%(2/35) vs. 34.4% (11/32) and 2.9% (1/35) vs. 25.0% (8/32), and there were statistical differences (P<0.01 or <0.05). Comparison with CMS lesions, RIS lesions mainly showed patching and stippled, and there were statistical differences (P<0.01 or <0.05). The rates of lesions enhancement and spinal cord injury in patients with RIS were significantly lower than those in patients with CMS: 2/17 vs. 45.0% (9/20) and 1/14 vs. 43.5% (10/23), and there were statistical differences (P<0.05). Conclusions There are differences in clinical findings, cerebrospinal fluid, neural electrophysiological examination and MRI appearances between RIS and CMS.

Objective To explore the effect of different scan parameters and algorithm on detection of iron deposition with coexsiting fat by dual soure CT.Methods One ml homogenate of normal rat liver tissues and 1 ml iron dextran solution with different concentrations (50.000,25.000,12.500,6.250,3.125,and 1.560 mg/ml) were fully mixed respectively.The 6 test tubes were scanned by dual source CT in the mode of dual energy to gain the iron specific slope.Different concentrations of dextran iron(50,40,30,20,10,and 5 mg/ml),triglyceride(volume percent were 60％,30％,and 10％,respectively),and homogenate of normal rat liver tissues were mixed.All 18 tubes were scanned by dual source CT with 80-140 kVp and 100-140 kVp,and all these images were reconstructed by filtered back projection (FBP) and singogram affirmed iterative reconstruction(SAFIRE).Raw data were reconstructed by filtering and iterative method.△H value (△H =CT value low kVp-CT valuehigh kVp) and overlay value of three substances decomposition algorithm were measured and analyzed by t test.The Spearmen correlation was applied in iron concentration and measured values.Results Iron specific slope was found to be 1.9392.When 80-140 kVp and 100-140 kVp were applied,△H values were (198 ± 28) and (152 ± 18) HU by using FBP reconstruction,and △H values were (191 ± 23) and (149 ± 17) HU by using SAFIRE reconstruction.The differences were statistically significant(t =-2.934 and-3.492,P =0.032 and 0.017).Overlay values were (381 ±54) and (236 ±31) HU by using FBP reconstruction,and overlay values were (399 ±53) and (276 ± 43) HU by using SAFIRE reconstruction.The differences were also statistically significant (t =-5.739 and-7.949,P =0.002 and 0.001).Under the same energy portfolio but different reconstruction,both △H and overlay value showed no differences (P ＞ 0.05).Except for the conditions with 100-140 kVp tube voltage,△H had no correlation with iron solution concentration (P ＞ 0.05) under either FBP or SAFIRE reconstruction.When other reconstruction methods and tube voltage combination were used,overlay or △H value showed correlation with iron solution concentrations (r value ranged from 0.959 to 0.997,P values ＜ 0.01).Conclusions Different energy portfolios of dual source CT will act on the measured value of iron deposition.The measured values will not be affected by ways of reconstruction.For the detection of iron coexisting in fat,overlay values of material decomposition algorithm is better.

Objective To explore the value of CT spectral imaging in the demonstration of pancreatic ductal adenocarcinoma (PDAC).Methods 113 patients were scanned by CT spectral,and gemstone spectral imaging (GSI) was performed in late arterial phase (AP) and portal venous phase (PP).All diagnosis were pathologically confirmed.The ROIs were placed on the lesion and on the pancreatic parenchyma.The ROI files including the CTmono values and the normalized CTmono values (normalized to pancreatic parenchyma) were saved.The works were performed three times repeatedly.CNR values ranged from 40 keV to 140 keV and the optimal keV in AP and PP were calculated.The differences of CTmono values, normalized CTmono values,and CNR were compared between the optimal keV and 70 keV(equivalent to conventional 120 kVp energy level).Paired t-test and Wilcoxon signed rank test were performed.P<0.05 was considered statistically significant.Results The optimal monochromatic energy of PDAC were 40 keV in both AP and PP.The optimal CNR values(mean±standard) were 2.31±1.02 and 2.38±1.02 in AP and PP,while the corresponding values of 70 keV were 2.08±0.98 and 2.12±0.96.The CNR of 40 keV was higher than that of 70 keV in both AP and PP.The CTmono values of PDAC were (58±13) HU and (71±19) HU at 70 keV and were (111±44) HU and (155±57) HU at 40 keV in AP and PP.The CTmono value in PP was higher than in AP.The median of normalized CTmono values of PDAC at 40 keV were 47.0% and 53.9% in AP and PP, and were lower than those of 70 keV,which were 57.7% and 61.8%.The differences of normalized CTmono values between 40 keV and 70 keV were significant.Conclusion CT spectral imaging manifests that PDAC is hypovascular both in AP and PP and is progressively enhanced form AP to PP.There is maximal conspicuity of tumor in AP, and the optimal monochromatic imaging can improve the conspicuity of PDAC lesion.

Objective To study whether abnormalities can be detected by MR diffusion tensor imaging (DTI) technology in radiologically isolated syndrome (RIS) patients with normal-appearing white matter (NAWM).Methods Twenty-seven patients who met diagnostic criteria for RIS were collected.Sixteen age-and sex-matched healthy controls with normal neurologic examination findings and no history of neurologic or psychopathic disorders were included.All subjects were examined by both conventional scan and DTI scan on GE Signa 3.0 T MRI.All the images were transmitted to the Advantage Workstation 4.2P and postprocessed using functool software.Anisotropic maps and average diffusion coefficient (ADC) maps were reconstructed.Two ROIs were selected in the genu and splenium of the corpus callosum separately at the trigone of lateral ventricle level.Other 2 ROIs were selected in the anterior and posterior body of the corpus callosum separately at the level of the lateral ventricle body,and 1 ROI was selected in the white matter of bilateral frontal and occipital lobe separately,then the FA and MD values were measured.The diffusion indices (FA and MD)were analysed by SPSS 13.0.Independent-sample t test was performed to examine the group differences in each subregion.Intraclass correlation coefficient analysis was performed to assess the diffusion indices of two measurements in each subregion.Results The ICC of 2 measurements was 0.934-0.989 (P ＜ 0.01),which showed favorable consistency.The FA values were decreased obviously in the genu,anterior and posterior body of the corpus callosum of the RIS patients compared with controls (0.705 ±0.040 vs 0.738 ±0.045,0.632 ±0.043 vs 0.675 ±0.042,0.628 ±0.043 vs 0.666 ± 0.045,t =-3.526,-4.487,-3.890,P ＜0.01),but the FA values of the two groups did not show any significant difference in the splenium,the white matter of the frontal and occipital lobe respectively (t =-1.387,-0.683,-1.243,P ＞0.05).In comparison with controls,the RIS patients had increased MD values in the genu,anterior and posterior body of the corpus callosum.(0.891 ±0.038 vs 0.874 ±0.035,0.839 ± 0.047 vs 0.794 ± 0.031,0.833 ± 0.039 vs 0.792 ± 0.057,t =2.101,5.836,5.146,P ＜ 0.05),but the MD values of the two groups did not show any significant difference in the splenium,the white matter of the frontal and occipital lobe respectively (t =1.671,1.702,1.624,P ＞ 0.05).Conclusion The NAWM abnormalities in the patients with radiologically isolated syndrome could be detected by DTI.

Though achieved great success as the only curative treatment for end-stage liver diseases, liver transplantation has been severely restricted by the shortage of donors. The adoption of extended criteria donor (ECD) tackles the donor shortage problem to some extent, thus became an issue we must face in China at present stage. As the transplantation effect and recipient safety is largely decided by the quality of liver graft, ECD graft should be cautiously selected and adopted because of its inherent defects. This relies on individualized decisions made for recipients by transplant surgeons who have a good understanding of the risk factors and evaluation criteria of ECD. In this article, we investigate the risk factors, evaluation criteriaand allocation of ECD liver grafts as well as the application of mechanic perfusion, and we discuss the fundamental issues and prospects of ECD study.