Objective To explore the clinical and electroencephalographic characteristics of frontal lobe ep- ilepsy(FLE).Methods The clinical and electroencephalographic data of 80 patients with FLE were analyzed.Re- suits The seizure types of frontal lobe epilepsy were simple focal seizure-complex focal seizure and secondary gen- eral tonic.clonic seizure.Seizures were often exhibited in relatively short duration,hyper motor,tonic or postural.Fre- quent nocturnal attacks and slight postictal mental confusion was increased.Interictal EEG manifested sharps or spikes or slow wave in the frontal area.Ictal EEG showed paroxysm rhythms.Conclusion FLE is a distinct epilepsy syndrome.

Objective To investigate the clinical characteristics,treatment and prognosis of senile epilepsy. Methods The clinical characteristics of 180 elderly patients with epilepsy were retrospectively studied. Results The common causes of senile epilepsy were cerebrovascular disease (8 cases,4.4％),brain tumor(24 cases,13.3％),brain trauma and operation (18 cases,10.0％),brain atrophy (6 cases,3.3 ％ ),The most type of seizures were focal seizures (106 cases,58.9 ％) or focal seizures preceding a generalized (62 cases,34.4％). Electroencephalogram (EEG) usually showed focal slow wave activity or rhythm.Most of seizures were responsive well to single antiepilepsy drug. Conclusions The causes of elderly epilepsy are cerebrovascular disease,brain tumor,brain trauma, brain operation, brain atrophy. Senile epilepsy is more likely to have symptomatic and focal seizures,and can be controlled by anti-epilepsy drugs.

Objective To present the clinical effect of dorsal carpometacarpal island flap with fascia, vessel and nerve to repair soft tissue defected. Methods The island flap was designed with root of fascia, dorsal metalcarpals artery and nerve.The flap to soft tissue defected in the nearby area was applied, including 30 cases in the last segment of thumb, 4 cases in the first segment of thumb, 1 case in the IP articulation of thumb, 1 case in the first segment of middle finger, 1 case in the carpometacarpal dorsal area and 1 case in the back of index finger. Results Thirty-eight cases were survived. All cases obtained good appearance and sensory recovery approach normally with little affection in supply area. Conclusions This method has advantage of simple, practical, high survive rate, low impairment, sensible and good appearance.

ObjectiveTo explore linkage relationship between polymorphisms of (AC)n (AT)xTy and mutations in the β-globin gene in patients with mild β-thalassemia.MethodsThe subjects were 89 mild β-thalassemia patients with known mutations and 110 healthy subjects from People's Hospital of Baoan District of Shenzhen from February 2009 to July 2010.Genomic DNA was extracted from peripheral leukocytes.Sequence of the BP1 binding site upstream of the β-globin gene was amplified by polymerase chain reaction,polymorphisms of (AC)n (AT)xTy were determined by DNA sequencing.Allelic frequencies of (AC)n (AT)xTy between mild β-thalassemia patients and healthy subjects were compared using x2 test.Mutation rates between two groups were also compared using x2 test for subjects carrying same haplotype. Linkage relationship was conducted according to allelic frequencies and mutations. Results Analysis of the (AC)n(AT) xTy polymorphisms of the BP1 binding site upstream of the β-globin gene showed 9 different genotypes: (AC)2( AT)7T7,( AC)2( AT)8T5,( AC)3( AT)7T5,( AC)2( AT)9T5,( AC)2(AT)8T9,(AC)3(AT)8T5,(AC)2(AT)10T3,(AC)2(AT)7T5 and (AC)2(AT)11T3.The (AC)2(AT)7T7 and (AC)2 (AT)8T5 genotypes were common for patients with mild β-thalassemia.Allele frequencies of (AC)2(AT)7T7,(AC)3 ( AT)7T5 and ( AC)2( AT)8T9 were 38.8％ (69/178),11.8％(21/178),9.0％ ( 16/178 ) for mild β-thalassemia patients,and 24.1％ ( 53/220),5.4％ ( 12/220),3.2％(7/220)for healthy subjects, respectively, there were significant differences between mild β-thalassemia patients and healthy subjects (x2 =9.966,4.371,6.093,P ＜ 0.05 ).Allele frequency of (AC)2(AT)9T5 was 10.1％ (18/178) and 33.2％ (73/220) for mild β-thalassemia patients and healthy subjects,frequency of (AC)2 (AT)9T5 was significandy lower in mild β-thalassemia patients than in healthy subjects (x2 =29.691,P ＜0.01 ).Allele frequency of (AC)2(AT)8T5 was 25.3％ (45/178) and 29.1％(64/220) for mild β-thalassemia patients and healthy subjects,there wasn't significant difference between patients and healthy subjects (x2 =0.718,P ＞0.05).The mutation rates of codon41/42(-TTCT) and IVSⅡ-654(C→T) were 59％ (10/17) and 29％ (5/17) for mild β-thalassemia patients carrying (AC)2(AT)7T7 allele,and 29％ (4/14) and 57％ (8/14) for patients carrying ( AC)2 (AT)8T5 allele.There were not significant differences between codon41/42(-TTCT) mutation rate and IVS-Ⅱ-654(C→T) mutation rate (x2 =2.982,2.333,P ＞ 0.05 ) for mild β-thalassemia patients carrying ( AC)2 ( AT)7T7 and ( AC)2(AT)8T5 allele.ConclusionsAllele of (AC)2(AT)7T7,(AC)3(AT)7T5 and (AC)2(AT)8T9 are in linkage disequilibrium with β-thalassemia.Most mild β-thalassemia patients carrying (AC)2 (AT)7T7 allele are caused by codon41/42 (-TTCT) mutation in the β-globin gene,and IVS-Ⅱ-654 (C→T) is a major mutation for patients carrying (AC)2(AT)8T5 allele.

A tumor-targeting recombinant fusion immunotoxin B-L-SEC2 was constructed by fusing staphylococcal enterotoxin C2 (SEC2) and an anti-HER-2 single-chain Fv B1 through a peptide linker, and expressed in E. coli strain BL21 (DE3) with an improved expression vector pASK75-EX as inclusion body. The denatured inclusion body was purified with Ni-NTA chelate agarose, and then re-natured by dialysis. FACS and MTT assays indicated that the re-natured fusion immunotoxin B-L-SEC2 could target the HER-2 over-expressing breast tumor cell SK-Br-3 in vitro, and inhibit the growth of SK-Br-3.

Objective To investigate the acid α glucosidase(GAA)gene mutations and clinical features of a Chinese patient exhibiting signs and symptoms of infantile glycogen storage disease type Ⅱ(GSD Ⅱ). Methods Clinical features of the patient were reviewed,and GAA activity in the patient＇s and her parents＇ whole leukocytes were measured. GAA coding regions were amplified by polymerase chain reaction(PCR),and analyzed by direct DNA sequencing. Results The patient showed feeding difficulties,generalized hypotonia and weakness starting at 2 months of age. Cardiomegaly and cardiomyopathy were found at 4 months. She died of cardiorespiratory failure at the age of 6 months. GAA activity in leukocytes was low in the patient(17.3% of the median normal range). Genotyping revealed the patient was a heterozygote for a novel nonsense mutation p.W738X and a previously reported nonsense mutation p.E888X. The reported pseudodeficiency allele c.1726G ＞ A;2065G ＞ Awas found in the patient and her mother. Conclusions Correct diagnosis was made for this patient by combination of GAA activity assay and genetic analysis. From the clinical course,this patient should be classified as infantile type of GSD Ⅱ,suggesting that the novel mutation p.W738X may have a damaging effect on the function of GAA. Pseudodeficiency allele found in this family highlights the importance of genetic analysis of GAA when performing diagnosis and prenatal diagnosis for the affected families,as this allele causes low GAA activity in normal individuals.

Patient satisfaction is an important index of hospital quality evaluation and performance evaluation.This study established multi-attribute patient satisfaction index system based on the theory of customer satisfaction index,determined the index weight by combination weighting approach,and graded the hospitals and adjusted the weights by the RSR method.A set of comprehensive evaluation scheme is initially formed,which is suitable for patient satisfaction evaluation and performance evaluation.

Objective To investigate the clinical, laboratory and genetic features of glycogen storage disease(GSD)IXc. Methods Five patients suspected as liver GSD were included in our study. DNA was extracted from peripheral blood of all the patients and diagnoses were made after target sequencing to nearly 2700 disease causing genes. All detected mutations were confirmed in the probands and their parents. Further analysis was based on clinical features, routine laboratory examinations and treatment. Results All the 5 patients manifested with severe hepatomegaly, hypoglycemia, moderately to severely elevated liver enzyme levels, hypertriglyceridemia and growth retardation. Four cases showed poor exercise tolerance but with normal creatine kinase (CK) levels. None of the patients showed liver cirrhosis. Growth velocity and hepatomegaly was improved after the uncooked corn starch treatment was initiated. In the 5 patients, 6 different pathogenic or likely pathogenic mutations in the PHKG2 gene were identified, including one reported mutation (p.E157K) and five novel mutations (p.E56X, p.R185X, c.79_88delinsTCTGGTCG, c.761delC,p.R279C). The p.E157K was the most frequently mutation identified (6/12, 50%). Conclusions The p.E157K mutation is the hot mutation in our small cohort. Main clinical features of our patients include fasting hypoglycemia, impaired liver function,short statures and poor exercise tolerance, without developing liver cirrhosis.

BACKGROUND: It remains poorly understood whether anterior column quadrilateral wing plate exists to solve intraoperative multiple plastic and quadrilateral in vivo shift for treating acetabular anterior column and acetabular quadrilateral body fractures.OBJECTIVE: To figure out the promising application on measurement of anatomical character parameters when designing acetabular anterior column and acetabular quadrilateral body using Mimics software. METHODS: 60 pelvic CT scan data were collected and three-dimensionally reconstructed by Mimics software. The following anatomical character parameters were measured, including the angle between plane of arcuate line of true pelvis and plane of quadrilateral surface, the four boundary lines of quadrilateral body, and the thickness of substance of bone in quadrilateral region. The projection curve on quadrilateral surface of acetabular margin and dangerous zone for screw placement were both drew. Above all, the study attempted to find out the proper safe entry point of quadrilateral screw and to measure their leaning inside angles.RESULTS AND CONCLUSION: (1) The angle between plane of arcuate line of true pelvis and plane of quadrilateral surface was not significantly different between males and females. (2) The minimum thickness of quadrilateral body in males was larger than that in females. (3) The maximum leaning angle flapper plate screw P1 and P2 for quadrilateral body was significantly smaller in males than in females, but that of screw P3 was not significantly different between males and females. (4) The application of Mimics software made it easier, more intuitive and more practical for the design or development of new plate for acetabular anterior column and acetabular quadrilateral body. The common points and difference between acetabular anterior column and acetabular quadrilateral body could be specifically described by the new anatomical character parameters, which are defined by bone surface features of pelvis.

Objective Examination and evaluation of employee satisfaction index in the hospital performance evaluation.Methods Stratified sampling,field survey and telephone survey were used in customizing a questionnaire for two surveys in July and December 201 5 respectively.Results The standardized score of employee satisfaction was 86.252±1 5.1 53,and the lowest score was found in the canteen environment and food quality.Conclusion Employee satisfaction is found as good overall,and targeted measures are recommended to improve insufficiencies for better employee satisfaction.