ObjectiveTo investigate the clinical feature peripheral neuropathy and vasculopathy after acrylamide toxication. Methods2 young male patients with peripheral neuropathy who had exposed to acrylamide for job more than one year were reported.ResultsNeuroelectrophysiological examination showed marked abnormalities in both peripheral and central nerve conduction in both patients. Sural nerve biopsies revealed axonal degeneration, Wallerian degeneration and giant axon with accumulated neurofilaments. Additonally, vasculopathies including prominant thickness of arterial intesma and basal membrane of capillary as well as apoptosis of vascular pericyte, were evident. ConclusionAxonal degeneration and vascular involvement has been found in acrylamide toxication. Vascular impairment maybe plays an important role in the pathogenesis of neuropathy.

Objective To report the development of clinical symptoms in a Chinese family with autosomal dominant progressive external ophthalmoplegia(adPEO).Methods Electromyologram and muscle biopsy were performed in the proband and 4 family members with the disease.Results The proband was a 57 year-old woman,who developed bilateral ptosis after the age of 30,external ophthalmoplegia after the age of 35 years old,weakness of extremities at the age of 37 years old and bulb palsy with palmus at the age of 47 years old.In the family there were 20 male and female members from five generations.All of them complained about bilateral ptosis between 26—33 years old,external ophthalmoplegia(12/15)and weakness of all extremities(14/15)between 35—45,facial and masticatory weakness(9/9)as well as dysphagia(8/9)between 44—60,accompanied with heart lesions(4/7)after 50 years old.Some patients died due to cardiac impairment.Electromyologram showed myopathic abnormalities in the examined patients. The main myopathological changes were ragged red fibers,cytochrome c oxidase negative fibers and ragged blue fibers in succinate dehydrogenase staining.Conclusions The adPEO started from extra-ocular muscles to limbs,finally facial and bulbar muscles.Heart lesions were presented in late stage and lead to death in some members.The developing process of symptoms suggested that we should pay more attention to cardiac manifestations in this disease.

Objective To detect the expression of heat shock protein 47(HSP47) in renal proximal epithelial cell lines (HK-2) and to investigate the role of HSP47 in the progress of transforming growth factor β1 (TGF-β1) induced epithelial-mesenchymal transdifferentiation (EMT) in HK-2 cells.Methods HK-2 cells were exposed to TGF-β1 (0,2.5,5,10 μg/L) for different time (0,12,24,48 h).The expression of HSP47 was examined by Western blotting.Then HK-2 cells were exposed to 10 μg/L TGF-β1,the expressions of vimentin,zona occludens-1 (ZO-1) were examined by Western blotting and real-time PCR.Furthermore,the expressions of p-Smad3 and Smad3 were examined by Western blotting.HK-2 cells were transfected with HSP47 siRNA and siRNA negative control before exposing to TGF-β1.Then the expressions of vimentin,ZO-1 were detected by Western blotting and real-time PCR,meanwhile Western blotting for HSP47,p-Smad3 and Smad3.Results Stimulating HK-2 with TGF-β1 resulted in a significant increased expression of HSP47 in time-and concentration-dependent manner (P ＜ 0.05).Meanwhile,TGF-β1 up-regulated the protein and mRNA expression of vimentin (P ＜ 0.05),and down-regulated the protein and mRNA expression of ZO-1 (P ＜ 0.05),all in time-dependent manner.Stimulating HK-2 with TGF-β1 resulted in phosphorylation of Smad3,which was peaked at 30 min,slightly decreased at 1 h,and then increased again between 24 and 48 h (P ＜ 0.05).Compared to the TGF-β1 group,inhibition of HSP4.7 expression in HK-2up-regulated the protein and mRNA expression of ZO-1,down-regulated the protein and mRNA expression of vimentin (P ＜ 0.05) and down-regulated the ratio of p-Smad3/Smad3.HSP47 siRNA negative control had no significant effect on the expressions of ZO-1,vimentin and p-Smad3/Smad3 (P ＞ 0.05).Conclusion HSP47 can promote the EMT of renal tubular epithelial cell which is possibly via the TGF-β1-Smad3 pathway.

Objective To study the pathological characteristics of skeletal muscle in patients with polymyalgia rheumatica(PMR).Methods Thirteen patients were diagnosed PMR according to the diagnostic criteria described by Bird in 1979.The onset of disease was between 49-78 years of age (median 60.3 years).All of them showed continuous symmetric myalgia with markedly elevated erythrocyte sedimentation rate(ESR).Five patients had mild fever and 8 patients had elevated C-reactive protein(CRP).Electromyogram(EMG)showed myogenic pattern in 2 patients.Only 1 patient showed anemia and 2 patients had mild elevated creatinine phosphokinase.None of them showed evidence of temporal arteritis.Biceps brachii muscle biopsies were performed in all patients before corticosteroid therapy.Results The typeⅡmuscle fiber atrophy was observed in all patients.Moth-eaten changes appeared in 8 cases,increased intramuscular lipid drops in 8 cases and ragged red fibers(RRF)in 3 cases of them.After steroid therapy,all patients showed improvement of myalgia and normal ESR as well as CRP.Conclusions The main symptoms of the patients are myalgia and abnormal ESR.Some patients have high level of CRP and abnormal EMG.Anemia and temporal arteritis are less frequent.The main myopathological changes were typeⅡmuscle fiber atrophy.Some patients have moth-eaten changes.More lipid drops and RRF indicate abnormal metabolism of skeletal muscle.

Objective To analysis the clinical manifestations of mtDNA A3243G mutation in adulthood.Methods The clinical features were investigated in 36 cases (28 patients from 5 families with the mutation and 8 sporadic cases),in whom mtDNA A3243G mutation was confirmed genetically in 23 cases (15 cases from 5 mutation families and 8 sporadic cases).Cranium radiology was performed in 14 cases.Muscal biopsies were performed in l0 cases.Results Among 28 cases in the 5 family,there were 9 cases (32.1%) with stroke like episodes,17 cases (60.7%) with diabetic mellitus and 16 cases (57.1%) with deafness.Such symptoms usually combined with each other and rarely existed alone. Cardiomyopathy and renal failure were uncommon.In the 23 cases with mtDNA A3243G mutation,14 cases (61.0%) had mitochondria] myopathy,encephalopathy,lactic acidosis,and stroke-like episodes (MELAS),mostly presenting cognitive abnormalities,dysarthria or aphasia and headache,3 cases (13.0%) were asymptomatic carriers,2 cases (8.7%) had autonomic dysfunction,2 cases (8.7%) had diabetic mellitus with or without nerve deafness,1 case (4.3%) had diabetic mellitus with infertilitas and cardiomyopathy,respectively.Cranial radiological images revealed the changes more commonly in the temporal and occipital lobes and less frequently in the frontal lobes.Ragged red fibers were confirmed in 9 of 10 cases with muscle biopsies.The proportion of mutant mtDNA A3243C was not significantly different between MEALS (28.75%?13.69%) and non-MELAS (25.08%?11.54%).Conclusions mtDNA A3243G mutation mainly results in the lesions in the central nerve system,pancreatic island and acoustic nerve in adulthood.Heart and kidney are less frequently involved.Cognitive abnormalities,aphasia and headache are the major symptoms of adult MELAS.Families have with more than 1 patient with diabetic mellitus and deafness,indicating that the mutation is other than MELAS mutation.We should pay more attention to the non-MELAS symptoms in the families with mtDNA A3243G mutation.

Objective To investigate the axonal lesion in chronic inflammatory demyelinating polyneuropathy(CIDP).Methods Eighteen patients had undergone sural nerve biopsy.The clinical and electrophysiological distinction based on the different pathological changes were analyzed.Results Five patients with demyelination predominance which presented myelinated fiber with thin myelin.Three of them showed also mild axonal degeneration.Eight patients with axonal lesion predominance which presented Wallerian degeneration and regeneration of myelinated fibers.Three patients with mixed myelin and axon lesion of myelinated fibers and two with mild lesion.There was no significant difference between CIDP predominantly with axonal lesion and demyelination.Electrophysiological examination shows both axonal lesion and demyelination feature in some of the 2 types patients at the same time.Conclusions Axonal lesion is a common pathological change in CIDP and should not be considered as an exclusive criterion in diagnosis of the disease.Infiltration of macrophages is a common change.

In view of the anatomical and physiological barrier of the ocular surface and the intraocular structure, the conventional ophthalmic agents cannot efficiently reach the lesion site.Currently, the different types of nanomaterials possess great advantages in delivering drugs due to their characteristics of small size, easy preparation, degradability, strong targeting and less irritation to biological tissue.As drug delivery carriers, nanomaterials have been widely used in ocular drug delivery so as to treat different types of eye diseases.In this paper, the applications of nanomaterials as drug delivery carriers in treating eye diseases are briefly reviewed.

OBJECTIVETo observe the differential effect of joint ultrasound on the syndrome differentiation of rheumatoid arthritis (RA) by observing the high frequency ultrasound performances among inactive stage and different syndromes in active stage.

METHODSTotally 83 RA patients in the active stage were assigned to the dampness heat syndrome group (DHS, 59 cases)and the cold dampness syndrome group (CDS, 24 cases) according to Chinese medicine (CM) syndrome typing. Besides, 20 RA patients in the remission stage were recruited as the control group (abbreviated as the remission group). By using high frequency ultrasound and power Doppler ultrasound technology, a comparative observation of synovitis, tenosynovitis, synovial blood flow, and bone erosion in the 2nd-5th metacarpophalangeal (MCP) joints, proximal interphalangeal (PIP) joints, wrist joints, knee joints, the second and the fifth metatarsophalangeal (MTP) joints (a total of 24 joints) was performed in all patients. Correlation analyses were performed between the ultrasound performance, laboratory indices, and the disease activity. Ultrasound data of each RA patient were analyzed by their total scores. Χ2 test was used for enumeration data. The measurement data was expressed as x ± s. One-way ANOVA was used for data of normal distribution, while non- parametric test was used for data of non-normal distribution. Correlation analysis of two variables was performed for clinical indicators and ultrasound indicators. Its significance was detected using Pearson correlation.

RESULTSCompared with the remission group, the severity degree of synovitis, tenosynovitis, synovial blood flow, and bone erosion significantly increased in the DHS group (P < 0.01). There was statistical difference in ESR, CRP, anti-CCP, DAS28 score, and the positive rate of RF (P < 0.05, P < 0.01). There was statistical difference in the severity degree of synovitis and synovial blood flow, and DAS28 score in the CDS group (P < 0.05). Compared with the CDS group, there was statistical difference in the four ultrasound indices (P < 0.05, P < 0.01), ESR, CRP, anti-CCP, DAS28 score, and the positive rate of RF in the DHS group (P < 0.05, P < 0.01). There was no statistical difference in G, IgG, IgA, or IgM among the three groups (P > 0.05). There existed positive correlation between ESR and the synovitis degree, synovial blood flow, and bone erosion in the DHS group (r = 0.444, 0.397, 0.486, P < 0.05).There existed positive correlation between ESR and the synovitis degree, bone erosion, and synovial blood flow in the DHS group (r = 0.378, 0.270, P < 0.05). There existed positive correlation between the DAS28 score and the synovitis degree and synovial blood flow in the DHS group (r = 0.304, 0.351, P < 0.05).

CONCLUSIONSThe inflammation degree was the most severe in RA patients of DHS. High frequency ultrasound could provide better evidence for Chinese medical syndrome differentiation of RA patients.

Arthritis, Rheumatoid ; diagnostic imaging ; Humans ; Medicine, Chinese Traditional ; Metacarpophalangeal Joint ; ultrastructure ; Syndrome ; Synovitis ; diagnostic imaging ; Ultrasonography

Objective: To observe the effects of electroacupuncture (EA) on the protein and gene expressions of Bax, Caspase-3 and Bcl-2 in cerebral cortex of type 2 diabetic rats with cognitive impairment (CI), and to explore the mechanism of EA in improving the learning and memory abilities. Methods: A total of 100 Sprague-Dawley (SD) rats were divided into a normal group (n=10) and a model group (n=90) by random number table method. Rats in the model group were intraperitoneally injected with a small dose of streptozotocin (STZ) to establish the type 2 diabetic models, after being fed with high-fat and high-sugar diet for 1 month. Twenty CI rats were selected from the 50 successful model rats by the Morris water maze (MWM) test and randomly divided into a model group and an EA group according to the blood glucose level and MWM data (n=10). Rats in the EA group received acupuncture at Zusanli (ST 36), Neiting (ST 44) and Yishu (Extra), of which Zusanli (ST 36) and Neiting (ST 44) were stimulated by EA apparatus, 20 min/time, once a day for 6 d a week and 4 consecutive weeks. The rats in the model and the normal groups were fixed without treatment. After 4-week treatment, the random blood glucose level of the rats was measured; the learning and memory abilities of rats were measured by MWM; terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL) assay was used to detect apoptotic cells; Western blot (WB) and real-time quantitative polymerase chain reaction (RT-qPCR) were used to detect the protein and gene expressions of Bax, Caspase-3 and Bcl-2 in cerebral cortex. Results: After modeling, the random blood glucose level and the escape latency tested by MWM were significantly increased, and the number of crossing the platform tested by the MWM was decreased in the EA and model groups, and were significantly different from those in the normal group (P<0.05 or P<0.01), while the differences between the model group and the EA group were not statistically significant (all P>0.05). After 4-week treatment, the random glucose level and the escape latency tested by MWM were significantly increased (both P<0.05), and the number of crossing the original platform tested by the MWM was significantly reduced (P<0.01), the protein and gene expressions of Bax and Caspase-3 were significantly increased (all P<0.001), the protein and gene expressions of Bcl-2 were significantly reduced (both P<0.001), and the number of neuron apoptosis was significantly increased (P<0.001) in the model group than in the normal group; the random blood glucose level was significantly reduced (P<0.05), the escape latency tested by MWM was significantly shortened (P<0.05), and the number of crossing the original platform tested by MWM was significantly increased (P<0.05), the protein and gene expressions of Bax and Caspase-3 were significantly reduced (all P<0.001), the protein and gene expressions of Bcl-2 were significantly increased (both P<0.001), and the number of neuron apoptosis was significantly reduced (P<0.001) in the EA group than in the model group. Conclusion: EA can improve the learning and memory damages induced by type 2 diabetic model rats with CI; the action mechanism may be achieved via anti-apoptosis.

Objective To analyze the developments and frontiers of systemic lupus erythematosus (SLE) research in order to make possible breakthroughs in subsequent studies.Methods Information visualization software Citespace V was applied to draw the mapping knowledge domain based on the database of SLE literature in Science Citation Index Expanded of Web of Science from 1975 to 2016.In this way,it was possible to analyze the basis of knowledge,network relationships between countries or regions,hot spots and courses frontier evolution in the recent 40 years.Results Totally 39 847 articles of literature were collected.The USA,UK,Spain,Italy and Australia were known as the leading countries in the study of SLE.The relationships between SLE and other autoimmune diseases,autoantibody detection and significance,anticardiolipin antibody expression,gene expression,classification criteria,disease activity were hot spots of SLE.Depression of SLE patients,management of SLE and differentiation of SLE were eye-catching research frontiers in recent years.Conclusion The mapping knowledge domain can display the main hot spots in research of SLE,show the frontier spheres and evolution course of related branches of learning,reveal the potential information in complex giant data networks,and guide future studies.