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.

With the rapid development of the field of interventional therapy of cardiac valve, the innovative researches of interventional therapy of cardiac valve products have become the focus of global research. At present, there is a serious shortage of interventional valvular medical devices on the market in China, and large-scale interventional valve products are undergoing early human trials or confirmatory clinical trials. The effective quality control of clinical trials is of great significance to ensure that clinical trial data can be used to support the marketing of device products. By analyzing the problems in clinical trials quality control of interventional valvular medical devices in our hospital, and combining the characteristics of device products and diseases, we explore the key points of quality control and provide reference for the implementation and completion of high-quality clinical trials.

As 2019-nCoV vaccine is widely used in the adult population, children and adolescents have gradually become an important susceptible population to 2019-nCoV due to their low coverage of the vaccine and high contact degree. Understanding the characteristics of 2019-nCoV infection in minors is vital. This paper summarized the progress in the research of 2019-nCoV in minors by using the data from the retrieval of recently published literature. Furthermore, the key characteristics of 2019-nCoV infection in minors, including the susceptibility, transmissibility, prognosis and immune response were analyzed and the progress in child and adolescent vaccine development was introduced.

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.

Objective BAG3-related myopathy is a rare condition so far reported in twenty patients worldwide.The purpose of this study was to draw attention to this rare disease and to the fact that BAG3-related myopathy should be considered as a rare differential diagnosis of hypercapnia.Methods We report a sporadic case of a 14-year-old Chinese girl with a de novo p.Pro209Leu mutation in BAG3 and reviewed the literatures for reported cases related to this mutation.Results We described a 14-year-old Chinese girl who presented with gradually appearing symptoms of hypercapnia that required assisted ventilation.The muscle biopsy and the blood whole-exome sequencing results confirmed the diagnosis of myofibrillar myopathy with a de novo p.Pro209Leu mutation in BAG3.Totally twenty-one patients from twenty families with a confirmed diagnosis of BAG3-related myopathy were reported to date,including this patient and literature review.The male to female ratio was 11 :10 and most showed initial symptoms in the first decade of life.Most patients presented toe/clumsy walking or running as the onset symptom,followed by muscle weakness or atrophy.Creatine kinase levels were elevated in fourteen patients and were normal in three.Eighteen patients developed respiratory insufficiency during the disease course and thirteen (one could not tolerate non-invasive assisted ventilation) required non-invasive assisted ventilation for treatment.Except for one not reported,heart involvement was found in seventeen patients during the disease course and seven underwent heart transplantation.Z-disk streaming and aggregation could be observed in most of the patients' muscle histology.In the long-term follow-up,five patients died of cardiac or respiratory failure.Conclusion BAG3-associated myopathy is a rare type of myofibrillar myopathy.It should be considered as a rare differential diagnosis of hypercapnia.

Objective: To summarize the clinical characteristics and nerve conduction damage in patients with early POEMS syndrome, and to explore the value of segment nerve conduction velocity in the diagnosis of POEMS syndrome. Methods: A total of 73 patients with POEMS syndrome and 27 healthy controls in Peking Union Medical College Hospital from September 2009 to June 2019 were recruited in this study. The motor and sensory nerve conduction characteristics of median, ulnar, tibial, and peroneal nerves and the clinical features of the participants were analyzed. The analysis parameters included: (1) distal motor latency (DML), compound muscle action potential (CMAP); (2) the median velocity from elbow to wrist, the median velocity from axillary to elbow, the ulnar velocity from the site below elbow to wrist, the ulnar velocity from the site above elbow to below elbow, the ulnar velocity from axillary to the site above elbow, the tibial velocity from ankle to knee, the peroneal velocity from ankle to fibulae capitulum; (3) sensory nerve conduction velocity and amplitude of these nerves; (4) terminal latency indices (TLI) of median; (5) motor nerve conduction blocks. Results: Peripheral nerve damages were the initial symptoms in thirty-two patients in this group, accounting for 43.8% (32/73), and 81.3% (26/32) of these patients only showed numbness in lower extremities. All POEMS syndrome patients with numbness had abnormal sensory nerve conduction, and 9.5% (7/73) of patients without sensory symptoms also had abnormal sensory nerve conduction. On the other hand, the decrease of CMAP amplitude corresponded to clinical muscle strength decline and motor dysfunction. In the patients with POEMS syndrome, motor nerve conduction in the lower limbs were more likely to be affected and the damages were more severe than in the upper limbs: the proportion of CMAP disappearance in the lower limbs and upper limbs was 47.6% (112/235) vs 2.8% (7/252; χ²=133.698, P<0.01). Sensory nerve conduction damage was more severe than motor nerve conduction: the proportion of amplitude disappearance in sensory and motor conduction was 43.0% (141/328) vs 24.4% (119/487; χ²=133.698, P<0.01). The slowing of motor nerve conduction velocity was more common than the decrease of CMAP amplitude: the rate of slowing down of motor nerve conduction was 88.7% (432/487), and the rate of decrease of amplitude was 52.8% (257/487; χ²=151.905, P<0.01). The DML of median and ulnar nerve in the POEMS syndrome group was longer than that in the control group (median nerve: 4.4 (3.7, 5.0) ms vs 3.2 (3.0, 3.5) ms, U=854.000, P<0.01; ulnar nerve: 3.1 (2.7, 3.8) ms vs 2.5 (2.2, 2.7) ms, U=1 077.500, P<0.01). The TLI of median nerve in patients with POEMS syndrome was significantly higher than that of healthy controls (0.40±0.11 vs 0.35±0.06, t=3.466, P=0.001). There was no statistically significant difference in nerve conduction velocity between the forearm segment and the upper arm segment in the POEMS group. Conclusions Neurological damages were common in patients with POEMS syndrome with sensory nerve damage often being the initial clinical manifestation. In patients with POEMS syndrome, the nerve damage in the lower limbs was more severe than in the upper limbs, and sensory nerve damage was more severe than motor nerve damage. Segmental motor nerve conduction results suggested that demyelination damage occurred earlier than axonal damage, and proximal lesions were slightly heavier than distal lesions. No conduction block was detected in these patients. These characteristics of segmental nerve conduction can provide more evidence for clinical diagnosis of POEMS syndrome.

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.

Immune checkpoint inhibitors (ICIs) can cause adverse reactions in the nervous system, with the incidence rate ranging from 0.1% to 12%, and 80% occurring within the first 4 months of ICI application. It can cause lesions in various parts of the nervous system, including aseptic meningitis, meningoencephalitis, necrotizing encephalitis, brainstem encephalitis, transverse myelitis and other central nervous system diseases. It can also cause cranial peripheral neuropathy, multifocal radicular neuropathy, Guillain-Barre syndrome, spinal radicular neuropathy and myasthenia gravis, myopathy, etc. For these complications of the nervous system, diagnosis could be made by sufficient collection of disease manifestations combined with imaging, cerebrospinal fluid examinations, electro-encephalogram or electro myography to exclude infection or tumor progression. In the treatment of severe cases, ICIs should be discontinued and treated with high doses of glucocorticoid or gamma globulin with systemic support. After neurological adverse reactions, the prognosis of severe cases is poor.

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 .

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.