The article sums up the experience of applying multimedia technology to Prosthodontics teaching and analyzes its necessities and problems to improve the teaching quality.

Amino Acid Metabolism, Inborn Errors ; Humans ; Mitochondrial Diseases ; Sarcosine Dehydrogenase ; deficiency

Objective To investigate the changes of ubiquitin C-terminal hydrolase-L1 (UCH-L1) and glial fibrillary acidic protein (GFAP)in acute phase of cerebral infarction. Methods From March 2011 to June 2012,95 patients with early cerebral infarction from the Neurology Clinic,the Emergency Department and the Cerebral Apoplexy Screening Project Base,and the Neurology Ward of Renhe Hospital were used as an infarction group;61 non-stroke subjects received physical examination in the Physical Examination Center of our hospital in the same period were used as a control group. The cerebral infarction group and the patients with cerebral infarction in different onset of time groups (an onset < 12 h group and an onset 12-24 h group),the different National Institutes of Health Stroke Scale (NIHSS)score groups (NIHSS 0 -4 group and NIHSS 5 -19 group),and the levels of UCH-L1 and GFAP in the control group were measured and compared among the groups. The receiver operating characteristic (ROC)curve was established. The cut-off values of the relevant parameters in the diagnosis of cerebral infarction,and the sensitivity and specificity of diagnosis were obtained. Results The UCH-L1 and GFAP values of the cerebral infarction group were all higher than those of the normal control group (0. 13[0. 09,0. 21]μg/ L vs. 0. 05[0. 02,0. 13]μg/ L,0. 030[0. 008,0. 130]μg/ L vs. 0. 004[0. 004,0. 020]μg/ L,Z values were 3. 62 and 4. 95 respectively;all P < 0. 01). The UCH-L1 and GFAP values of the NIHSS score 5 -19 group were higher than those of the NIHSS score 0 -4 group (0. 12[0. 08,0. 21]vs. 0. 09[0. 08,0. 18],0. 07 [0. 01,0. 11]vs. 0. 04[0. 01,0. 10];all P < 0. 05). There was no significant difference in the UCH-L1 and GFAP values between the onset 12 -24 h group and the onset < 12 h group (0. 12[0. 08,0. 21]μg/ L vs. 0. 09[0. 08,0. 18]μg/ L,0. 030[0. 010,0. 110]μg/ L vs. 0. 040[0. 008,0. 100]μg/ L;all P > 0. 05). The analysis results of ROC curve of UCH-L1 and GFAP for diagnosis of acute cerebral infarction showed that when the plasma UCH-L1 was ≥0. 18 μg/ L,the sensitivity and specificity of UCH-L1 were 68% and 74%respectively;When the plasma GFAP was ≥0. 11 μg/ L,the sensitivity and specificity of GFAP were 70% and 86% respectively. The area under the ROC curve of UCH-L1 and GFAP diagnosis of cerebral infarction were 0. 64 and 0. 71 respectively. Conclusions UCH-L1 and GFAP have obvious change in acute phase of cerebral infarction. UCH-L1 and GFAP may have certain correlation with the severity of stroke.

Remote ischemic postconditioning can induce cerebral ischemic tolerance and have neuroprotective effects.It has provided a novel strategy for the treatment of ischemic cerebrovascular disease.In recent years,the mechanism research of remote ischemic postconditioning and clinical applications have made significant progress and have shown encouraging prospects of clinical transformation.

Objective To investigate the effectiveness of target-controlled infusion (TCI) of etomidate and remifentanil for endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA).Methods Sixtynine ASA Ⅰ or Ⅱ patients of both sexes,aged 35-71 yr,weighing 41-83 kg,scheduled for elective EBUS-TBNA,were randomly divided into 3 groups ( n =23 each).In group Ⅰ,anesthesia was induced with TCI of propofol and iv injection of fentanyl 4 μg/ml,and the target plasma concentration (Cp) of propofol was set at 3-4 μg/ml.In group Ⅱ ,anesthesia was induced with TCI of propofol ( Cp 3-4 μg/ml) and remifentanil ( Cp 5 ng/ml).In group Ⅲ ,anesthesia was induced with TCI of etomidate (Cp 0.3-0.4 μg/ml) and remifentanil (Cp 5 ng/ml).After the patients lost consciousness,laryngeal mask airway was inserted to perform mechanical ventilation.PETCO2 was maintained at 30-40 mm Hg.BIS value was maintained at 40-60.The use of vasoactive agents (perdipine,ephedrine,atropine and esmolol) and occurrence of bucking during operation,emergence time,and the occurrence of nausea and vomiting within 24 h after operation were recorded.Blood samples were collected from the femoral vein at 30 min before induction,at the end of operation and at 24 h after operation for determination of the plasma cortisol concentration.Results The incidence of bucking and nausea and vomiting was significantly lower,the emergence time was significantly shorter,and the number of patients who needed vasoactive agents during operation was significantly smaller in groups Ⅱ and Ⅲ than in group Ⅰ ( P ＜ 0.05).The number of patients who needed vasoactive agents during operation was significantly smaller in group Ⅲ than in group Ⅱ (P ＜0.05).Compared with groups Ⅰ and Ⅱ,the plasma cortisol concentration was significantly decreased at the end of operation in group Ⅲ (P ＜ 0.05).There was no significant difference in the plasma cortisol concentration at each time point between groups Ⅰ and Ⅱ (P＞0.05).Conclusion TCI of etomidate (Cp 0.3-0.4 μg/ml) and remifentanil (Cp 5 ng/ml) can provide satisfactory anesthesia for EBUS-TBNA with few adverse effects.

Objective To investigate the effect of acute irradiation by high power millimeter wave on the pathological changes of mouse lung tissue. Methods The BALB/c mice were vertically placed under the high power millimeter wave equipment with working frequency of 34. 1 GHz, and the mean output power were 5,10 and 12 W while the distance between the animal and the bottom of the irradiation horn were 10 mm and 20 mm, respectively. The mice were tied on the platform and continuously received irradiation until death. After immediate dissection, the mouse lung was quickly rinsed with 0.9% NaCl solution, fixed in 10% formaldehyde solution and mounted for paraffin section. After HE staining and image taken with a CCD camera, the Image Pro Plus software and quantitative image analysis by combining the mean optical density and area was used to determine the pathological injuries of the lung. Result Using the HA23. 16 and HA9. 92 pyramid horns with different physical parameter, the mice exposed to irradiation with high mean power of 12 W were dead most quickly, the death time was only about 110 s. Meanwhile, the death time was about 30 min after irradiation with the mean power of 5 W. There was significant hemorrhage in the mouse lung with high power millimeter irradiation, although the hemorrhage degree was different under different irradiation parameters. When the mean power were 10 and 12 W.the hemorrhage degree of lung was extremely high, where the bronchia and blood vessel of lung was markedly broken. A lot of cells of bronchia had been released. However, when the mean power was 5 W, the hemorrhage degree of lung was less observed, while the bronchia and blood vessels had not severe fracture. Conclusions High power millimeter wave wave irradiation has remarkable effect on mice lung. The damage degree of lung tissue is highly correlated with the mean power of millimeter wave irradiation. As the high power millimeter used in this study could result in significant thermal effect, the acute heat-induced response might lead to animal death by causing serious lung injury.

Objective: To analyze the clinical and genetic features of progressive cavitating leukoencephalopathy (PCL). Method: The data of clinical and genetic features of 4 PCL patients diagnosed by Beijing Children′s Hospital between January 2015 and January 2016 were analyzed. The cases with complete clinical data retrieved on literature search at China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform and PubMed (up to August 2016) by using search terms of"NDUFV1" ,"NDUFS1" , or"leukoencephalopathy" , were summarized. Result: There were three females and one male, two of which were compatriots. The age of onset ranged from 6 months to 15 months. All four children′s first symptoms were motor development regression, and the developmental milestones were almost normal before the onset. Of the 4 patients, 3 had cognitive impairment, 1 had seizures, 4 had dystonia and pyramidal impairment, 2 had emaciation, and 1 had nystagmus. The lactate concentrations of 4 patients were normal in blood. One patient had lactaciduria in the urinary organic acid analysis. Cranial magnetic resonance imaging (MRI) of all patients showed leukoencephalopathy, involved in the corpus callosum, and three patients accompanied by cystic lesions. Follow up for 2-13 years showed that the physical and language development were improved. Genetic analysis revealed that mutations in NDUFS1 were found in three patients and NDUFV1 mutation was found in one patient. All six mutations (p.Arg377Cys and p. Arg377His in NDUFV1; p. Arg482Glyfs^*5, p.Thr368Pro, p.Tyr454X and p. Asp565Gly in NDUFS1) are novel. Five English case reports including 10 PCL patients were collected. Together with this group of 4 cases, a total of 14 cases were involved. All 14 children patients had motor development regression, 11 cases had cognitive impairment and dystonia, 6 cases had pyramidal impairment, 5 cases had irritability, 4 cases had epilepsy and nystagmus, 3 cases had strabismus and swallowing difficulty. Cranial MRI showed patchy leukoencephalopathy with cavities, involved in the corpus callosum. Follow up for 19 months-15 years that the neurology development were improved slowly in all patients. Conclusion NDUFS1 and NDUFV1 gene mutation screening should be performed firstly in patients with PCL clinical and imaging feature.

OBJECTIVETo analyze the clinical characteristics of pediatric neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD).

METHODA retrospective analysis was performed evaluating clinical and laboratory characteristics of ten NMO and NMOSD children who were seen in our hospital from December 2010 to May 2014. Median age at onset was 8.9 years (range 0.8-13.8 years). Seven cases were female and three were male. Median disease duration was 1.5 months (range 1-18.5 months).

RESULTEight patients fulfilled diagnostic criteria for NMO and two patients fulfilled diagnostic criteria for NMOSD. The two NMOSD patients had recurrent longitudinally extensive transverse myelitis. Four cases had a monophasic disease course, and six cases had a recurrent course. In eight NMO patients, neuritis was the initial presentation. The two NMOSD patients had no neuritis in the first attack. Nine cases had clinical manifestations of myelitis, one case had asymptomatic spinal cord MRI anomaly. Among the ten patients, seven cases had brain lesions, wherein, four cases had the midbrain involvement and in four cases extensive hemispheric white matter was involved. Three cases had medullary involvement. And two cases had posterior limb of the internal capsule involvement, two cases had thalamus involvement. In one case there was pons, cerebellum or corpus callosum involvement, respectively. One case had accompanied brain symptoms. Of the five patients who had symptomatic brain lesions, four cases had encephalopathy accompanied by large hemispheric lesions on MRI, having a presentation similar to acute disseminated encephalomyelitis. And one case had multiple sclerosis like brain lesion. Of the ten patients tested, nine were seropositive for anti-aquaporin-4 autoantibody. One-patient was complicated with systemic lupus erythematosus. Oligoclonal bands were negative in all cases. All patients received treatment for acute attacks with high-dose intravenous methylprednisolone and intravenous gammaglobulin. The symptoms of 8 cases mitigated. Two cases whose symptoms showed no sign of improvement received plasmapheresis for acute attacks. Seven of the patients were followed up. The median duration of follow-up was 19 months (ranged from 13 months to 30 months). The median Expanded disability status (EDSS) score was 3 (range 1-7).

CONCLUSIONPediatric NMO and(or) NMOSD have a diverse clinical presentation which are more than just optic neuritis and transverse myelitis, including brain symptom. So it may be difficult to distinguish NMO and( or) NMOSD from acute disseminating encephalomyelitis and multiple sclerosis in the early stages of the disease. Antibodies to aquapoin-4 (AQP-Ab) testing is very important for differential diagnosis.

Adolescent ; Anti-Inflammatory Agents ; therapeutic use ; Aquaporin 4 ; Autoantibodies ; Brain ; Brain Diseases ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Methylprednisolone ; therapeutic use ; Multiple Sclerosis ; etiology ; Neuromyelitis Optica ; complications ; diagnosis ; drug therapy ; Retrospective Studies

OBJECTIVETo analyze the clinical and PMM2 gene mutation features of congenital disturbance of glycosylation caused by PMM2 gene mutation (PMM2-CDG, previously known as CDG 1a).

METHODThe clinical data of two Chinese patients who were clinically diagnosed as PMM2-CDG at neurology department of Beijing Children's Hospital in 2012 were retrospectively collected. The gene mutations were identified by Sanger sequencing.

RESULTBoth patients were female, aged 1 year and 1 month and 8 months respectively. The main clinical features of the two cases were developmental delay after birth, chronic diarrhea and metabolic acidosis, associated with elevated serum transaminases, and decreased antithrombin III activity. Physical examination showed esotropia, inverted nipples, and abnormal subcutaneous fat pads. The cranial MRI showed cerebellar atrophy. Both cases were treated with occupational therapy, physical therapy and speech therapy. The development was gradually improved but also delayed as compared with normal peers during follow-up for more than 3 years. Genetic analysis showed that patient 1 was compound heterozygous for c. 422G>A(p.Arg141His), which was reported for known pathogenic mutation, and c. 669C>A(p.Asp223Glu), was a new mutation. The patient 2 showed compound heterozygous mutation for c. 634A>G (p.Met212Val)and c. 713G>C(p.Arg238Pro), which were both new mutations.

CONCLUSIONPMM2-CDG is a rare metabolic disease, and the diagnosis should be considered in a child with developmental delay, elevated serum transaminases, decreased antithrombin III activity, inverted nipples, abnormal subcutaneous fat pads, esotropia, and cerebellar atrophy on MRI. It can be confirmed by PMM2 gene analysis.

Asian Continental Ancestry Group ; Congenital Disorders of Glycosylation ; genetics ; DNA Mutational Analysis ; Developmental Disabilities ; Female ; Genetic Testing ; Glycosylation ; Heterozygote ; Humans ; Infant ; Magnetic Resonance Imaging ; Mutation ; Phosphotransferases (Phosphomutases) ; genetics ; Retrospective Studies

OBJECTIVETo investigate the clinical and genetic features of a Chinese girl with Schwartz-Jampel syndrome (SJS).

METHODTo analyze the clinical and genetic data of a girl with Schwartz-Jampel syndrome who was sent to neurology outpatient department of Beijing Children's Hospital in Auguest of 2010. Reports on Schwartz-Jampel syndrome published until July of 2015 were searched and the clinical and genetic characteristics of reported cases were summarized.

RESULTAt 8 months after birth, the girl showed myotonia; at 1 year old when she was walking alone she had myotonia of lower limbs, both feet evaginated, walked slowly and was prone to fall. At 2 years of age, she could not climb up stairs, at 3 years she could not jump continuously. At 3 years and 7 months of age when the girl was taken to neurology outpatient department, on examination, she had a dull facial expression, rigid lips and could not fully open her mouth, a micromandible, low-set and prominent ears, systemic muscle rigidity, there were muscular nodes formation on the limbs and gait stiffness. She had high level of creatine kinase and atlanto-axial joint subluxation on cervical CT reconstruction. She also had spontaneous myotonia-like discharges on needle electromyography (NEMG). X-ray of limbs showed metaphyseal dysplasia. The patient was treated with neurologic rehabilitation and carbamazepine. The myotonia at the last follow-up at her 8 years of age was the same as at the onset. On her HSPG2 gene, two novel heterozygous mutations c.10776delT on exon 78 and c.5702-5G>A on intron 45 were found. c.10776delT resulted in the amino acid change on p.Ala3592fsX6 and c.5702-5G>A maybe changed protein splicing. No reports were found among Chinese journals, while 7 reports were found in English literature. The total 34 mutations were known in reviewed reports, which included eleven deletion or insertion, twelve splice site, eight missense, and three nonsense mutations. Four patients had a single mutation. No definite genotype-phenotype correlation was identified.

CONCLUSIONSchwartz-Jampel syndrome is a rare autosomal-recessive hereditary disease appears to be slowly progressive, in which distinctive clinical features were induced by HSPG2 gene mutation. We reported the c.10776delT on exon 78 and c.5702-5G>A on intron 45 which were not reported previously. This is the first report of Schwartz-Jampel syndrome of which genetic mutations was identified in a Chinese child.

Asian Continental Ancestry Group ; Carbamazepine ; therapeutic use ; Child ; Child, Preschool ; Exons ; Female ; Heterozygote ; Humans ; Infant ; Introns ; Mutation ; Osteochondrodysplasias ; diagnosis ; genetics