Objective To analyze the symptoms and signs in forty-five Lambert-Eaton myasthenia syndrome (LEMS) patients retrospectively. Characteristics of electrophysiological examinations were investgated. Methods Forty-five LEMS patients were reviewed and information gathered regarding clinical complains neurological symptoms, and other concomitant diseases. The records showed that repetitive nerve stimulation (RNS) and nerve conduction velocity (NCV) were performed in all patients. Needle electromyography (EMG) and skin sympathetic response (SSR) were performed in some patients. Results (1) The mean age of neurological clinical onset age was (51.2 ±6. 8) years old. The two most common symptoms were slight weakness of lower extremities ( n = 35 ) and upper extremities (n= 5). Dysarthria was found in 3 patients and neck weakness in 2 patients. Tendon reflex decreased and disappeared in 38 patients. Autonomic nervous system manifestations were presented in 30 patients. (2) RNS increasing was observed in all patients from 156% to 636%. Low frequency RNS abnormalities were found in 29 patients.Sensory nerve conduction velocity abnormalities or sensory nerve conduction velocity combined motor nerve conduction velocity abnormalities were found in 19 patients ( 42% ). Of the 30 patients who underwent a needle EMG examination, 20 had myogenic or neurological damage. Thirteen abnormal findings were observed in 25 patients who underwent SSR examination. Conclusion The most common manifestations were weakness in lower extremities and autonomic nervous system dysfunction. Many abnormal electrophysiological results were found in LEMS patients, including NCV and EMG abnormalities. These findings indicated that clinical manifestations exceed the neuromuscular junction and perhaps included the peripheral nerve and muscle.

The article discusses the curriculum truss of medical biosciences major and the curriculum setup with basic medical courses as the foundation,highlighting the life science scopes and emphasizing integrated curriculum and the training of molecular biology technology to culture practical cross-subject talents in the life sciences for society.

Objective To investigate the time and rate of generalization developed from ocular symptoms in adults to generalized myasthenia gravis (GMG).Methods The data from 456 adult patients who started on ocular symptoms during Jan 2005 to Jan 2012 were retrospectively studied,with a follow-up period of 3 months to 17 years.The data included transfer time,Osserman type,difference between steroids group and no-steroids group.Results (1) During the follow-up period,197 (43.2％) patients developed to generalized myasthenia gravis,58.4％ (115/197)in one year after onset of ocular symptoms,74.1％ (146/197) in 2 years and 80.7％ (159/197) in 3 years.86.8％ (171/197) developed to Osserman Ⅱ a or Ⅱ b.(2) Of 233 patients untreated with steroids,134 (57.5％) developed to GMG,while 63 (28.3％) of 223 patients who were treated with steroids developed to GMG (x2 =38.57,P ＜ 0.01).There were no difference between two groups in transfer time and Osserman type.Conclusion MG initiated from ocular syndromes in adults easily develops to GMG in 2 years.Patients treated with steroids have low chance of generalization.

Objective To investigate the nutritional status of children and women of childbearing age in high-risk areas of iodine deficiency disorders in Tianjin in 2008-2010 and to provide a scientific basis for iodine supplementation.Methods Hangu District was selected as survey point from 2008 to 2010,Dagang District and Jingan County were increased in 2010.Hangu District was divided into five areas,one township(town) that was not high iodine was selected from each area,four administrative villages were selected from each township(town),and 15 households were selected from each administrative village.Three township(town) were selected from Dagang and Jingan County,respectively.Two administrative villages were selected from each township(town),and 20 households were selected from each administrative village.Iodine level of salt samples from each household was determined.In 2009,one primary school was selected from each survey township(town),thyroid volume of 40 8-10-year-old students was examined,and urine iodine of 20 students was tested.In 2010,three township(town) were selected from each survey point,two primary school were selected from each survey township(town),40 8-l0-year-old students were selected from each primary school,urine iodine and thyroid volume of the students were determined.150,50 and 60 women of childbearing age from Hangu District were selected from 2008 to 2010,and 60 were selected from Dagang District and Jingan County,respectively.Urine iodine of the women was tested.Iodine content of salt was tested by a semi-quantitative test kit; diagnosis of endemic goiter was based on the Diagnostic Criteria of Endemic Goiter (WS 276-2007); urinary iodine test was based on the Arsenic Cerium Catalytic Spectrophotometry (WS/T 107-2006).Results Coverage rates of iodized salt in Hangu District in the 3 years were 65.0％ (195/300),70.3％ (211/300) and 90.8％ (274/300),respectively.Coverage rates of iodized salt in Dagang District and Jinghai County were 40.0％(48/120) and 38.3％(46/120).In 2009,the median urinary iodine of children aged 8-10 in Hangu District was 205.4 μg/L and in 2010,the medians urinary iodine of children aged 8-10 of the three survey point were 193.0,293.1 and 216.8 μg/L.Goiter rates of children aged 8-10 in Hangu District were 4.24％(9/212) and 3.59％ (9/251),and in Dagang District and Jinghai County were 3.60％ (9/250) and 5.44％(13/239).The medians urinary iodine in women of childbearing age in Hangu District in the 3 years were 134.9,196.1 and 229.5 μg/L,respectively.The medians urinary iodine in women of childbearing age in Dagang District and Jinghai County were 316.9 and 190.9 μg/L.Conclusions The nutritional status of high-risk areas of iodine deficiency disorders in Tianjin are better than the national limit of enhanced iodine requirements.Due to higher water iodine in the same areas,some people are iodine excess,to which we should pay more attention.

Objective To summarize the clinical experiences of liver transplantation.Methods Of the nine patients, four operation was standard orthotopic liver transplantation,the latter five were the piggyback liver transplantation.The immunosuppressive protocols included methylprednisolone FK506 and mycophenolatemofeti. Meanwhile intravenous antihepatitis B immunoglobulin and Lamivudine were used to prevent hepatitis B recurrence.Results All patients were cured.Conclusion Liver transplantation can be employed for liver disease both cirrhosis and carcinoma as a conventional surgery.It is an effective way for the treatment of no metastatic liver carcinoma.The immunosuppressive protocols included methylprednisolone FK506 and mycophenolatemofeti,it can prevent immune rejection.

Objective To investigate the underlying mechanisms of Miller-Fisher syndrome (MFS) and Bickerstaff' s brainstem encephalitis (BBE) by studying their clinical and electrophysiological characteristics.Methods The clinical and electrophysiological characteristics of 13 MFS and 7 BBE cases in Peking Union Medical College Hospital between 2000 and 2011 were retrospectively analyzed.The electrophysiological parameters included sensory and motor nerve conduction,electromyography,F wave,sympathetic skin response and brainstem auditory evoked potential and blink reflex.Results MFS and BBE had similar clinical characteristics:respiratory symptoms were the most common infectious symptoms before disease onset; Ophthalmoplegia,facial palsy and bulbar symptoms were common; They both had cerebrospinal fluid albuminocytological dissociation and positive serum anti-GQ1b antibody.However,BBE had more central nervous system lesion signs clinically such as conscious disturbance,positive Babinski' s sign and central facial palsy.Electrophysiologically,MFS and BBE also had similar electrophysiological features:sensory nerve abnormality ratios were 6/13,2/7 respectively,with prominently reduced sensory nerve active potential amplitude,normal or slightly slowed sensory conduction velocity; Motor nerves abnormality ratios were 2/13,1/7 respectively,with slightly prolonged distal motor latency and normal compound muscle action potential; Electromyography abnormality ratios were 1/7,0/4 respectively; F wave frequency abnormality ratios were 3/13,5/7 respectively,and in some cases,F wave frequency would restore; Sympathetic skin response abnormality ratios were 1/2,1/3 respectively; Blink reflex abnormalityratios were 1/2,1/1 respectively,with central involvement in BBE; Brainstem auditory evoked potential abnormality ratios were 3/5,1/4 respectively,with wave Ⅰ latency or amplitude abnormality.Conclusion The similarities of clinical and electrophysiological features suggest that MFS and BBE have the same mechanism and they form a continuous spectrum with variable central nervous system and peripheral nervous system involvement.

Objectives:To observe the anatomical location and mechanism of axis ring fractures (ARF) using 3-D CT scans, and propose a new classification for such fractures.Methods:By reviewing prospectively maintained database collecting ARF from 7 medical centers in China, 202 patients were included in this study. According to anatomical location, ARFs were classified into axis arthrosis fracture (AAF) and axis bony damage (ABD). The axis ring was divided into anterior, middle, and posterior rings, based on the border of the pars interarticularis (or pedicle) of axis. According to the features of ARF and previous study, a new classification was proposed based on the anatomical features of different fracture patterns, which was divided into three types and six subtypes (A1, A2, B1, B2, C1 and C2). The incidence of AAF and ABD and their distribution in different location of axis ring and the new classification, were observed.Results:In 202 patients with ARF, 501 anatomical structures were involved. 288 AAFs were found in 178 patients (288/501, 57%), while 213 ABDs were found in 149 patients (213/501, 43%). In anterior ring, 304 structures (304/501, 61%) were involved in injury, with 225 AAF and 79 ABD. In middle ring, 99 structures (99/501, 20%) were involved in injury, and all of them were ABD. In posterior ring, 98 structures (98/501, 19%) were involved in injury, with 63 AAF and 35 ABD. The anterior ring injuries (61%) were more common than middle (20%) or posterior ring (19%). In anterior ring, AAF (84%) were morecommon than ABD (16%); In middle ring, all the injuries were ABD; In posterior ring, AAFs (64%) were more common than ABD (36%). Type A fractures were featured with pedicle fractures and were identified in 30 patients (30/202, 15%). Type A1 fractures were bilateral pedicle fracture lines symmetrically or asymmetrically and identified in 12 (6%) patients; Type A2 fractures were pedicle fracture lineson one side and inferior articular facet injuries or lamina fractures on the otherside and identified in 18 (9%) patients. Type B fractures were featured with superior articular facet injuries or posterior wall of C2 body fractures on one side and identified in 136 patients (67%). Type B1 fractures were superior articular facet injuries or posterior wall of C2 body fractures on one side and pedicle fracture on the other side and identified in 57 (28%) patients; Type B2 fractures were superior articular facet injuries or posterior wall of C2 body fractures on one side and inferior articular facet injuries or lamina fractures on the otherside and identified in 79 (39%) patients. Type C fractures were featured with bilateral superior articular facet injuries or posterior wall of C2 body fractures and identified in 36 patients (18%). Type C1 fractures were bilateral superior articular facet injuries or posterior wall of C2 body fractures symmetrically and identified in 22 (11%) patients; Type C2 fractures were bilateral superior articular facet injuries or posterior wall of C2 body fractures asymmetrically and identified in 14 (7%) patients.Conclusion:ARF could occur in different anatomical locations, and most of these fractures were caused by hyperextension and axial load on superior articular facet on one or two sides. The new CT classification of ARF with three types and six subtypes might provide all fracture patterns, which could be useful for the choice of proper diagnosis and treatment for such fractures.

Objective:To study the effects of arsenic exposure on necroptosis pathway and inflammatory response of mouse myocardial cells.Methods:Sixty male C57BL/6J mice were randomly divided into control group (group C) and low, medium, and high dose arsenic exposure groups (groups L, M, H) based on body weight using a random number table method. Each group had 15 mice, and they drank 0.00, 0.15, 1.50, and 15.00 mg/L arsenic trioxide (As 2O 3) solution prepared with deionized water. The exposure period was 12 weeks. Hematoxylin-eosin (HE) staining and Masson trichrome staining of paraffin-embedded heart tissues were used to observe the histopathology changes of the heart. Transmission electron microscopy (TEM) was used to evaluate the ultrastructural changes of myocardial cells. The quantitative real-time PCR (qRT-PCR) was used to detect the mRNA expression of inflammatory genes [tumor necrosis factor (TNF)-α and interleukin(IL)-6] and the genes involved in necroptosis pathway [receptor-interacting protein (RIP) 1, RIP3 and mixed-lineage kinase domain-like protein (MLKL)]. Protein expressions of RIP1 and RIP3 in the heart were assessed by western blotting. Results:Histopathological examination results showed there were myocardial necrosis, inflammatory cells infiltration and fibroblasts hyperplasia and other changes in groups M and H. TEM analysis revealed marked ultrastructural changes in groups M and H, including fractured myofibril, fractured Z lines of sarcomere, and swollen mitochondria with fractured cristae. Compared with group C (1.00 ± 0.00), the mRNA expression of RIP1 in group H was significantly up-regulated (1.41 ± 0.06, P < 0.05); the mRNA expressions of RIP3 (1.29 ± 0.14, 1.56 ± 0.08), MLKL (1.23 ± 0.05, 1.36 ± 0.07), TNF-α (2.20 ± 0.10, 2.23 ± 0.18) and IL-6 (1.87 ± 0.16, 1.63 ± 0.15) were significantly up-regulated in groups M and H ( P < 0.05). The protein expressions of RIP1 (0.43 ± 0.04, 0.50 ± 0.04) and RIP3 (0.68 ± 0.02, 0.84 ± 0.05) in groups M and H were higher than those in group C (0.25 ± 0.01, 0.45 ± 0.04, P < 0.05). Conclusion:Subchronic arsenic exposure induces histopathological changes such as myocardial necrosis and fibrosis in mice, inducing necroptosis and inflammatory reactions in myocardial cells.

Objective: To investigate the differentially expressed genes (DEGs) associated with the occurrence and development of breast cancer and to screen the molecular markers for breast cancer by bioinformatic analysis. Methods: Three breast cancer microarray datasets were downloaded from Gene Expression Omnibus (GEO) database. GEO2R was used to identify DEGs. The differentially co-expressed genes in the three datasets were screened by Venn diagram. GO function enrichment analysis and KEGG signal pathway analysis were performed using DAVID. The protein-protein interaction (PPI) network of DEGs was constructed using STRING. The most important modules in the PPI network were analyzed using Molecular Complex Detection (MCODE), and the genes with degree≥10 were identified as Hub genes. Hierarchical clustering analysis of hub genes was conducted using UCSC Cancer Genomics Brower. The survival curve and the co-expression network of hub genes were constructed using cBioPortal. Results: A total of 65 DEGs were screened from the three data sets. Eight hub genes, CTNNB1, CDKN1A, CXCR4, RUNX3, CASP8, TNFRSF10B, CFLAR and NRG1, were finally obtained, which exerted important roles in cell adhesion, proliferation and apoptosis regulation etc. Clustering analysis showed that the differential expression levels of CTNNB1, CFLAR, NRG1 and CXCR4 were associated with the occurrence of breast cancer. The overall survival analysis indicated that the patients with elevated CDKN1Aexpression had significantly shorter overall survival time (P<0.01). Conclusion: The hub genes identified in the present study can be used as molecular markers for breast cancer, providing candidate targets for diagnosis, treatment and prognostic prediction of breast cancer.