Objective To investigate the clinical value of contrast-enhanced ultrasound (CEUS) in the diagnosis of postoperative complications after pancreas transplantation.Methods Eighty-six post pancreastransplantation patients were examined by CEUS to observe the perfusion of contrast agent,plot timeintensity curve (TIC),and compare to the results of enhanced CT,MRI and pathology.Results Eleven cases of thrombosis,13 cases of rejection and 13 cases of pancreatitis were observed among 86 patients.Result from CEUS showed that:① Distribution of normal pancreatic grafts vascular and parenchyma was even,TIC follows positive skewed distribution.②Thrombosis:there was no perfusion in the embolic vessels and no enhancement or low uneven enhancement in the parenchyma.③ Rejection:perfusion of the parenchyma was slow,peak value was decreased,rising and falling slope of the TIC was reduced with blunt peak.④Pancreatitis:perfusion of the parenchyma was uneven,the regional low enhanced area was visible,clear of the contrast agent was relatively slow,rising slope of TIC was reduced and the peak value was decreased.Conclusions CEUS can monitor the perfusion of the pancreatic grafts vascular and parenchyma,and get useful perfusion parameters.It has been proven as an effective method to definitely diagnose postoperative complications after pancreas transplantation.

Objective To evaluate the expression of human kallikrein10 (KLK10) in different endometrioid tissues and analyse the relationship of KLK10 with ER and PR in endometrioid adenoearcinoma.Methods The expression of KLK10 protein in 12 normal endometria,19 endometrial hyperplasia and 34 endometrial carcinoma were detected by immunohistochemistry.The correlations of the expression of KLK10 protein,ER and PR were analyzed.Results The expression of KLK10 in stage Ⅰ,Ⅱ and Ⅲ were 64.3 ％(9/14),30.0 ％ (3/10),10.0 ％ (1/10),and the difference was statistically significant (P ＜ 0.05).The expression of KLK10 in endometrial carcinoma,normal endometria,endometrial hyperplasia were 66.7 ％ (8/12),33.3 ％(4/12),10.0 ％ (1/10),and the difference was statistically significant (P ＜ 0.05).The expression of KLK10 in G1,G2,G3 were 66.7 ％,33.3 ％,10.0 ％,and the difference was statistically significant (P ＜ 0.05).The positive rate of KLK10 expression was 38.2 ％ and the positive rates of ER and PR expression were 67.6 ％and 55.9 ％,respectively,in 34 endometrial carcinoma.The expression of KLK10 was positively correlated with ER and PR expression (x2 =0.448,P ＜ 0.01).Conclusion Absence or down-regulated expression of KLK10 may play an important role in the formation and development of endometrioid adenocarcinoma.The low expression of KLK10 is correlated with low expression of ER and PR in endometrioid adenocarcinoma.The positive expression of KLK00,ER and PR predicts a better prognosis.

Objective To investigate the efficacy and safety of rituximab in the treatment of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.Methods Three patients with anti-NMDAR antibodies in cerebrospinal fluid and serum hospitalized from May 2012 to July 2014 were retrospectively reviewed.The clinical syndrome,investigations,and therapeutic interventions by rituximab when first line immunotherapy failed were evaluated.Results All 3 patients were females with median age of 17 years (12,17,and 22 years).One patient had ovarian teratoma.All 3 patients presented with psychiatric symptoms and movement disorders,2 of which developed into a state of unresponsiveness.Brain magnetic resonance imaging of 2 patients was unremarkable,and 1 showed T2 and FLAIR hyperintensity among the areas of medulla,pons,caudex cerebri and callosum.Fluoro-2-deoxy-D-glucose-PET showed variable multifocal cortical and subcortical abnormalities that changed during the course of the disease.Electroencephalograms were abnormal in all patients,showing non-specific,slow,and disorganised activity,1 showing extreme delta brush.The cerebrospinal fluid showed lymphocytic pleocytosis.All patients showed no response to treatment with first line immunotherapy (corticosteroids,intravenous immunoglobulin (400 mg · kg-1 · d-1 × 5 d,2-3 courses of treatment)).After the administration of rituximab,1 patient responded slower,whereas the other 2 patients who recovered dramatically (375 mg/m2 every week for 3-4 weeks) continued immunosuppression with mycophenolatemofetil for 1 year.Relapse occurred in 1 patient when the immunotherapies discontinued 6 months later.During the treatment of rituximab,2 patients had grade 3 infectious adverse events (hospitalization and intravenous administration of antibiotics).Conclusions Rituximab is effective for the patients with anti-NMDAR encephalitis who fail to respond to the first line immunotherapy.However the utility of rituximab is still a challenge due to the risk of infectious complications and off-label use.

Objective To determine whether peripheral nerve ultrasound can differentiate CharcotMarie-Tooth type 1 (CMT1) from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).Methods Eighteen patients with CIDP,13 patients with CMT1 and 16 healthy controls were recruited prospectively from Peking Union Medical College Hospital between January 2014 and July 2015 for this study.Ultrasonographic tests were performed via nerve tracing from wrist to axilla on median and ulnar nerve with a 10 MHz linear array probe.The cross sectional areas (CSAs) were measured at 10 defined sites of the nerves,respectively.Results CSAs (mm2) at all sites of median nerve were significantly increased in CMT1 than in CIDP (10.5 ±5.3 vs7.8 ±2.4,10.9 ±3.6 vs 6.8 ±1.9,11.5 ±5.0 vs7.3 ±1.8,13.5 ± 4.4vs7.2±2.5,16.0±4.5vs7.2±2.1,17.1±5.1vs7.0±2.8,21.0±4.5vs9.5±4.8,24.3±6.9 vs 9.5 ±4.3,23.9 ±6.0 vs 10.2 ±4.3,22.4 ±6.7 vs 9.8 ±2.1;t=2.141,4.766,2.935,4.858,6.715,6.602,7.148,7.100,8.078,6.498,respectively,all P ＜ 0.05).CSAs (mm2) at all sites of ulnar nerve were significantly increased in CMT1 than in CIDP (7.9 ± 1.8 vs 4.0 ± 1.3,8.9 ± 2.0 vs 4.9 ± 1.3,13.5±1.9 vs6.5±2.4,15.0±4.3 vs 6.5 ±1.5,15.8 ±4.4 vs 6.8 ±3.3,11.6±2.3 vs6.9± 3.1,10.2±3.2vs7.6±2.8,14.0±3.0vs6.6±2.1,19.2±3.7vs7.6±4.4,18.1±3.6vs6.3± 2.5;t =7.652,7.414,9.194,6.893,6.443,4.766,2.561,7.897,8.113,11.554,respectively,all P ＜ 0.05).CSAs at 8 sites of median nerve and 8 sites of ulnar nerve were significantly increased in CIDP than in healthy controls.Receiver operation characteristic curve analysis revealed that CSA was suited for differentiating CMT1 from CIDP,and the area under curve in 8 sites of median nerve and 9 sites in ulnar nerve was more than 0.9.Conclusions CSAs measured at different sites by peripheral nerve ultrasound in CMT1 were significantly increased than in CIDP.Measurement of CSAs by peripheral nerve ultrasound can be used for differentiating CMTI from CIDP.

Objective To investigate the clinical value of contrast-enhanced ultrasound (CEUS) in the diagnosis and grading of acute rejection of the transplanted pancreas after simultaneous pancreas-kidney transplantation.Methods Seventy cases pancreas grafts underwent gray scale ultrasound,color Doppler flow imaging(CDFI) and CEUS examination,the contrast agent perfusion processes were observed,and the parameters of time intensity curves(time-intensity curve,TIC) were calculated.The CEUS results were compared with the pathological findings.Results Twenty-one cases were acute rejection in 70 cases,of which 10 cases were mild,8 cases were moderate and 3 cases were severe;and 49 cases were non-rejection.①Gray scale and CDFI ultrasound:The pancreatic grafts of acute rejection were edema and enlarged,the parenchyma echo were decreased.The artery resistance index(RI) were significant different between acute rejection group and non-rejection group (0.77 ± 0.05 vs 0.74 ± 0.10,P ＜0.05),but there were no significant differences between mild,moderate and severe group (P ＞0.05).②CEUS:TIC curves showed a significantly longer time to reach peak [TTP,(21.7 ± 4.3)s vs (13.0 ± 2.9) s,P ＜0.01] and significantly reduced peakintensity(PI,18.8 ± 7.9 vs 29.6 ± 2.4,P ＜0.05).There was no significant difference between the mild and moderate groups (P ＞0.05) but statistically difference was found when the severe group compared with the other two groups (P ＜0.05).Conclusions CEUS can be used to observe the perfusion of the vascular and parenchyma of the pancreas,the results also can be quantitative analyzed.It is an effective method for the diagnosis of pancreas acute rejection of simultaneous pancreas-kidney transplantation.

Objective To investigate the value of ultrasound multiplex mode in the evaluation ot liver damage caused by brain death.Methods Brain death model were established in 12 Ba-Ma pigs by gradual intracranial compression method.Color Doppler flow imaging (CDFI),contrast-enhanced ultrasonography (CEUS) and real-time elastography (RTE) were performed respectively before the model had been established which defined as control group,and at 0 h,3 h,6 h,9 h after brain death,the data were analyzed.According to the pathological changes of liver,the livers were divided into different groups for ROC curve analysis,and the effectiveness of single mode ultrasound and multiplex were compared.Results ①Compared with control group,velocity of portal vein (PVV) decreased,and resistance index(RI),hepatic artery systolic velocity/diastolic velocity (S/D),pulsatility index (PI) increased in experimental group (all P ＜ 0.05);arrival time of the hepatic artery(ATHA),arrival time of the portal vein(ATPV) and arrival time of the inferior vena cava(ATIVC) increased with the extension of brain death time (all P ＜0.05);mean value (MEAN),standard deviation(SD) of the relative strain value within the ROI,area of low strain within the ROI (％ AREA),complexity of low strain area within the ROI (COMP),skewness (SKEW),correlation(CORR) were significant different between experimental group and control group (all P ＜0.05).②RI,ATHA and MEAN demonstrated the highest diagnostic accuracy (AUC =0.518,0.833,0.917) among CDFI,CEUS and RTE indexes.③Combined with ATHA and MEAN had the highest effectiveness in ultrasound multiplex mode to evaluate liver injury caused by brain death.Conclusions Ultrasound multiplex mode combining CEUS and RTE in evaluation of liver injury caused by brain death has higher sensitivity and accuracy.

POEMS syndrome is a rare paraneoplastic syndrome due to plasma cell neoplasm. It is a disorder involving multiple organs, mainly manifesting as demyelinating peripheral neuropathy. The major diagnostic criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder, sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. A good history and physical examination followed by appropriate testing-radiographic assessment of bones, measurement of vascular endothelial growth factor, bone marrow biopsy and electromyography can differentiate this syndrome from other conditions such as chronic inflammatory demyelinating polyradiculoneuropathy, other plasma disorders (including amyloidosis, myeloma, and monoclonal gammopathy of undetermined significance neuropathy). Early diagnosis and treatment could greatly improve the prognosis.

Objective To investigate the application value of the tissue diffusion quantitative analysis technique of real-time elastic( RTE) imaging in evaluation of liver damage caused by brain death . Methods Fifty cases of brain dead donor liver were examined by ultrasound elastography ,at the same time , 11 parameters were obtained by the tissue dispersion quantitative analysis software ,included MEAN ( mean relative strain value within the ROI) ,SD( standard deviation of the relative strain value within the ROI) ,％AREA (area of low strain within the ROI) ,COMP(complexity of low strain area within the ROI) ,KURT (kurtosis) ,SKEW(skewness) ,CONT (contrast) ,ENT(entropy) ,IDM(inverse difference moment) ,ASM (angular second moment) ,CORR(correlation) ,and the pathologic specimens in the process of operating were obtained . According to pathological findings ,50 patients were divided into damaged group and non-damaged group ,and the results were compared and analyzed . The correlation analysis of electronic speculum results and RTE were carried out . According to the ROC curve ,the RTE parameters'cut-off value and AUC were obtained .Results Among 11 parameters of tissue diffusion analysis ,MEAN and IDM in damaged group were higher than those in non-damaged group( all P < 0 .05) ;and SKEW and ENT were lower in damaged group than those in non-damaged group ( all P <0 .05) ;there was no significant difference in the other parameters between the two groups(all P >0 .05) . The MEAN ,SKEW ,COMP ,ENT and IDM had correlativity with electronic speculum( r = -0 .633 , P =0 .000 ;r =0 .388 , P =0 .005 ;r =0 .315 , P =0 .026 ;r = 0 .324 , P = 0 .022; r = -0 .314 , P = 0 .026 ,respectively ) . The diagnostic efficiency and sensitivity of MEAN were the highest , the critical value of MEAN was 125 .84 , AUC was 0 .907 . Conclusions The tissue dispersion quantitative analysis technique is a noninvasive method to preliminary evaluation of liver damage caused by brain death . M EAN had the highest effectiveness in eleven parameters .

Peripheral nerve hyperexcitability syndromes (PNHS) encompass a spectrum of a heterogeneous condition with clinical as well as electrophysiological manifestations of peripheral nerve hyperexcitability. The PNHS consist of Isaacs syndrome, Morvan syndrome and Cramp-fasciculation syndrome, which cause widespread symptoms and signs while without evident peripheral nerve disease. Probably the most well-known condition of PNHS is Isaacs syndrome, often called acquired neuromyotonia. Clinical symptoms of PNHS are characterized by muscle twitching, cramps, stiffness, and neuropathic pain. The electrophysiological findings that are very useful in the diagnosis of PNHS are spontaneous myokymic, neuromyotonic, and cramp discharges. An overview of the history, clinical manifestations, pathophysiology, electrophysiological findings and management of PNHS is presented.

The clinical manifestations and examination results of a case of Creutzfeldt-Jakob disease (CJD) admitted to the Department of Neurology of Peking Union Medical College Hospital,Chinese Academy of Medical Sciences in August 2020 were analyzed, and a comprehensive neuropsychological assessment and assessment of apraxia were conducted. The neuropsychological characteristics of apraxia in CJD patient and the progress in the research and evaluation of apraxia were reviewed. The patient was a 65-year-old male with insidious onset and progressive symptoms, whose clinical manifestations were apraxia, rapidly progressing dementia, and extrapyramidal symptoms. The magnetic resonance imaging showed hyper-intense signal in diffusion weighted imaging in bilateral cerebral hemispheres, and 14-3-3 protein in cerebrospinal fluid was positive, which were consistent with the probable CJD diagnostic criteria. The patient exhibited prominent signs and symptoms of ideomotor apraxia. It has been reported in the literature that apraxia can also be the main neuropsychological manifestation of CJD. It is necessary to pay attention to the standard evaluation and timely identification of apraxia in clinical diagnosis.