Objective To explore the features of clinical manifestations and electrophysiology of spinal muscular atrophy(SMA) in children.Methods The clinical features and laboratory data were analyzed in 32 children with SMA,electromyography tests were carried out in 28 patients and the muscle biopsy were performed in 24 cases.Results The 32 cases were subdivided into 3 clinical groups,15 cases were SMAⅠ,12 cases were SMAⅡ,5 cases were SMAⅢ.They were all characterized by progressive muscle weakness associated with hyptonia and atrophy.The clinical distinction between SMAⅠto SMAⅢ reflected different age of onset and disease severity.All cases of SMAⅠhad symptoms of respiratory disability,only 1 case of SMAⅡ had paradoxical breathing and none of SMAⅢ had similarly symptoms.Electromyographic studies showed a pattern of denervation with no sensory involvement.The rate of spontaneous potential was 87%,with gentle strain,the duration of motor unit was extended(30%-150%) and the amplitude of it was increased(90%-450%),the motor nerve conduction velocity was reduced slightly in 28% patients.The muscle biopsy provided evidence of skeletal muscle denervation with groups of atrophy.Conclusions The clinical features,the changes of electromyography and the muscle biopsy are valuable for diagnosis of SMA.Respiratory management will prolong survival and improve the quality of life for these patients.

ObjectiveTo investigate the effect of nickel-titanium(Ni-Ti) shape memory alloy embracing internal fixer on oblique or spiral fracture.Methods23 cases with spiral or oblique fracture accepted internal fixation with Ni-Ti shape memory alloy embracing.The time of bone healing,articular function,the appearance and function of the wound extremity were evaluated after 9～34 months follow-up.ResultsAll fractures healed in 4～16 weeks after operation.None infection,nonunion,embracing emersion or break had been found. The fine rate was 91.3%.ConclusionThe effect of Ni-Ti shape memory alloy embracing internal fixer on long spiral or long oblique fracture is satisfactory.

Adenocarcinoma, Clear Cell ; pathology ; Adult ; Female ; Humans ; Thyroid Neoplasms ; pathology

0.05).The abrupt onset of the inflammatory arteriopathy group was 10 cases(31.2%),the abrupt onset of the noninflammatory arteriopathy group was 38 cases(58.5%),there was significant difference for the mode of onset between the 2 groups(?2=6.352 P

Objective To summarize the clinical characteristics and causes of ischemic cerebrovascular disease(ICD)in children.Methods A retrospective analysis was conducted on the clinical data of 53 cases with ICD from Feb.2002 to Jun.2008 at the department of neurology in Wuhan Children's Hospital.The self-designed questionnaire of children with ICD was used,whose items included patients' age,gender,personal history,clinical features,cerebrospinal fluid examination,neurological imaging,immunologic examination,metabolic examination,and so on.Results Of 53 children with ICD,30 cases(56.6%)were male,and 23 cases(43.4%)were female.Patients' age varied from 9 months to 12 years old,in which 45 cases(84.9%)were less than 6 years old.Patients from rural area(60.4%)were more than those from city(39.6%).Ratio of limb paralysis was 75.5%(40 cases)in first clinical symptomatology of children with ICD,including hemiplegia in 32 cases(60.4%),alternate hemiplegia in 5 cases(9.4%)and monoplegia in 3 cases(5.7%).Skull CT/MRI scan was performed to reveal 27 cases(50.9%)with basal ganglia region infarction and secondly 15 cases(28.3%)with multi-lobar infarction.Forty cases were found in abnormal cerebrovascular image by means of magnetic resonance angiography/digital subtraction angiography,in which middle cerebral artery and its branches were involved in 21 cases(52.5%).There were 41 cases(77.4%)of patients to be found with clear causes,of which 13 cases(24.5%)were of infections,8 cases(15.1%)of moyamoya disease,5 cases(9.4%)of cerebral vascular malformations,4 cases(7.5%)of head trauma.However,another 12 cases(22.6%)of patients had unknown etiology.Conclusions Children with ICD had characteristics themselves.The limb paralysis was mostly the first symptoms,and the middle cerebral artery and its branches lesions were the most common locations in children with ICD,and next the internal carotid artery involvement,anterior cerebral artery involvement,posterior cerebral artery involvement,cerebral vascular malformations,and so on.Their major cause was infection,followed by Moyamoya disease,cerebrovascular malformations and head trauma,and there were still some unknown causes.

AIM: To evaluate the repeatability and reproducibility of thickness profile measurement of intra-retinal layers determined by an automated algorithm applied to OCT images from Cirrus optical coherence tomography (OCT) instrument.METHODS: In this prospective cross-sectional study,retinal thickness images at 6mm×6mm around fovea were obtained from 86 eyes of 43 normal subjects with Cirrus HD-OCT instrument.The retinal images from patients were analysis by Cirrus automated algorithm GCA software,including ganglion cell layer and inner plexiform layer(GCIPL).During this study,operator A would make 2 times measurements to all patients,after that operator B would make another 2 times measurements by Repeat scan model.All the data,including GCL average thickness,min thickness,12 clock average thickness,2 clock average thickness,4 clock average thickness,6 clock average thickness,8 clock average thickness and 10 clock average thickness were measured by SPSS 15.0 software.Standard deviation (SD),coefficient of variation (CV) and interclass correlation coefficient (ICC) were calculated from the results of three-times tests by different examiner to evaluate the repeatability and from the results of two different examiners to assess the reproducibility.Written informed consent was obtained from each subject prior to any medical procedure.RESULTS: The average GCIPL thickness of OD was 85.12±3.95μm;the minimum average GCIPL thickness was 83.21±4.41μm;the standard deviation of OD in clock map was from 3.79μm to 4.84μm;CV(95%CI) was between 0.26 and 0.93;ICC was from 0.939 to 0.989.The average GCL+IPL thickness of OS was 84.65±8.73μm;the minimum average GCPL thickness was 81±13.08μm;the standard deviation of OS in clock map was from 8.24μm to 10.56μm;CV(95%CI) was between 0.92 and 4.94;ICC was from 0.264 to 0.968.CONCLUSION: Cirrus HD-OCT GCA software with automated algorithm can offer accurate and repeatable thickness profile of OCT retinal image.GCA is a useful and reliable approach to the measurement of intra-retinal layer thickness,which may improve the diagnosis and monitoring of retinal diseases.

Adolescent ; CD3 Complex ; blood ; CD4 Antigens ; blood ; CD8 Antigens ; blood ; Child ; Fetal Blood ; immunology ; Humans ; Infant, Newborn ; Interleukin-6 ; blood ; Interleukin-8 ; blood ; Male ; Nitric Oxide ; blood ; T-Lymphocytes ; immunology ; Tumor Necrosis Factor-alpha ; analysis

Animals ; Blood Coagulation ; drug effects ; Blood Platelets ; drug effects ; Female ; Haplorhini ; Humans ; Interleukin-11 ; pharmacology ; Male ; Platelet Count ; Thrombopoietin ; pharmacology

Objective To investigate the expressions and relationship between lysine-specific demethylase 1 (LSD1) and E-cadherin protein in gastric cancer tissues and adjacent normal tissues,and the correlation with the clinicopathological features and prognosis of patients with gastric cancer.Methods The case-control study was adopted.The gastric cancer tissues and adjacent normal tissues were collected by surgical resection from 80 patients with gastric cancer who were admitted to the Xiangya Hospital of Central South University from June 2008 to June 2009.Expressions of LSD1 and E-cadherin protein were detected by immunohistochemistry (IHC).The follow-up of telephone interview was performed to detect survival of patients till June 2015.Relationships between the expressions of LSD1 and E-cadherin protein and clinicopathological features or prognosis of patients were analyzed.Comparison of count data and correlation were analyzed by the chi-square test and Spearman rank correlation analysis.Survival curve was drawn using the Kaplan-Meier method,and survival analysis was done using the Log-rank test.Results Expressions of LSD1 in cancer tissues and adjacent normal tissues were located at the cell nucleus.The positive expression rate of LSD1 was 67.5％ (54/80) and 43.8％ (35/80) in cancer tissues and adjacent normal tissues,respectively,with a significant difference (x2=9.141,P ＜ 0.05).Expressions of E-cadherin protein in cancer tissues and adjacent normal tissues were located at the cell membrane and cytoplasm.The positive expression rate of E-cadherin was 63.8％ (51/80) and 81.3％ (65/80) in cancer tissues and adjacent normal tissues,respectively,with a significant difference (x2 =6.140,P ＜ 0.05).The positive expression rate of LSD1 was 83.3％ (25/30),76.0％ (19/25) and 40.0％ (10/25) in the low-,moderate-and high-differentiated tumors,37.5％ (6/16),72.7％ (16/22),71.9％ (23/32) and 90.0％ (9/10) in the Ⅰ,Ⅱ,Ⅲ and Ⅳ stages of TNM stage,36.4％ (4/11) and 72.5 ％ (50/69) in patients without and with lymph node metastasis,respectively,showing significant differences (x2=12.870,9.425,4.111,P ＜ 0.05).The positive expression rate of E-cadherin protein was 53.3％ (16/30),56.0％ (14/25) and 84.0％ (21/25) in the low-,moderate-and high-differentiated tumors,75.0％ (12/16),63.6％ (14/22),71.9％ (23/32) and 20.0％ (2/10) in the Ⅰ,Ⅱ,Ⅲ and Ⅳ stages of TNM stage,100.0％ (11/11) and 58.0％ (40/69) in patients without and with lymph node metastasis,70.0％ (49/70) and 20.0％ (2/10) in patients without and with distant metastasis,respectively,showing significant differences (/x2 =6.494,10.073,5.547,7.426,P ＜ 0.05).There was a negative correlation in expressions between LSD1 and E-cadherin protein (r =-0.355,P ＜ 0.05).The survival time and 5-year overall survival rate of patients with positive and negative expressions of LSD1 were (36.9 ± 2.5) months and 31.1％,(47.4 ± 3.4) months and 56.0％,respectively,showing a significant difference (x2=4.550,P ＜0.05).The survival time and 5-year overall survival rate of the patients with positive and negative expressions of E-cadherin protein were (44.0 ± 2.5) months and 46.7％,(32.6 ± 3.5) months and 24.9％,respectively,showing a significant difference (x2 =7.306,P ＜ 0.05).Conclusions Positive expression of LSD1 in gastric cancer tissues is higher than that in adjacent normal tissues.There are increased expression of LSD1 and reduced expression of E-cadherin protein in low-differentiated gastric cancer tissues and high TNM stage,showing a negative correlation between them.Positive expression of LSD1 and negative expression of E-cadherin protein may indicate a poor prognosis.

ObjectiveTo investigate the current situation of intensive care unit (ICU) inpatients in Infectious Disease Hospital of Nanning to provide a reference for the prevention and control of infectious diseases and construction and development of ICU in such a hospital.Methods The clinical data of inpatients from January 2011 to December 2015 in the Infectious Disease Hospital Affiliated to Guangxi Medical University (that is the Fourth People's Hospital of Nanning City) were retrospectively analyzed, and the distributions of patients with various diseases, ages, of the characteristics of children severe infectious diseases in ICU, the numbers of patients with infectious diseases in general ward and ICU, the average time and daily cost of hospitalization and mortality were conducted.Results During 2011 to 2015, 855 cases in ICU were treated, including 709 cases of infectious diseases, 146 cases of non-infectious disease. The top 5 infectious diseases were ranked as follows: tuberculosis, human acquired immune deficiency syndrome (AIDS), hand-foot-mouth disease (HFMD), viral hepatitis, tetanus, accounting for 96.47% of the total infectious disease inpatients in ICU; there were 25 cases of other legal infectious diseases, accounting for 3.53%, including dengue fever, measles, malaria, chicken pox, mumps, rabies, typhoid fever, paratyphoid fever, scrub typhus, bacillary dysentery, leptospirosis, human infections with highly pathogenic H7N9 avian influenza, epidemic hemorrhagic fever, A H1N1 influenza, and besides, critically ill patients carrying virus (viral hepatitis B and AIDS) in 7 pregnant women, 22 surgical patients, and 117 patients with internal medicine diseases were also admitted and treated. The top 4 infectious diseases causing deaths in ICU were tuberculosis, AIDS, viral hepatitis and HFMD: The infectious diseases with top 6 high mortalities in ICU were as follows: rabies, viral hepatitis, AIDS, tuberculosis and tetanus. The age range was wide in this study, from 73 days to 97 years, among which there were 167 cases of pediatric infectious diseases from pediatric department, accounting for 23.55% of ICU inpatients, being mainly severe HFMD, tuberculosis and severe varicella. From 2011 to 2015, the number of hospitalized patients showed a rising trend, and the average length of stay of patients in general wards presented a downward tendency, but the average time of hospitalization of inpatients with infectious disease in ICU, mortality and daily cost of hospitalization revealed a gradually increasing trend year by year.Conclusions It is of far-reaching significance to master the spectrum of dangerous severe infectious diseases to deal with the infectious disease epidemic situation and outburst with public health events.