OBJECTIVE: This article evaluates the auditory and speech perception outcomes of sensorneural hearing loss with cerebral white matter abnormalities after cochlear implantation. METHOD: A retrospective analysis was performed on the cochlear implantatees with cerebral white matter abnormalities by using the categories of auditory performance and speech intelligibility rating, and compared with the non-syndrome patients with sensorineural hearing loss. Paired t-test was used for statistical analysis. RESULT: There was statistical difference between normal group and white matter changes group with sensorineural hearing loss after 6 month. No statistical difference was found after 12 month and 24 month. CONCLUSION In the short term, the cochlear implant can be performed safely in patients with white matter changes. After a formal rehabilitation training,no significant difference in auditory or language ability was found between normal group and white matter changes group with sensorineural hearing loss.
Child, Preschool ; Cochlear Implantation ; rehabilitation ; Female ; Follow-Up Studies ; Hearing Loss, Sensorineural ; etiology ; surgery ; Humans ; Leukoencephalopathies ; complications ; Male

OBJECTIVESince glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary hemolytic erythrocyte enzyme deficiency, most cases have single nucleotide mutations in the coding region, and current test methods for gene mutation have some missed detections, this study aimed to investigate the feasibility of RT-PCR sequencing in the detection of gene mutation in G6PD deficiency.

METHODSAccording to the G6PD/6GPD ratio, 195 children with anemia of unknown cause or who underwent physical examination between August 2013 and July 2014 were classified into G6PD-deficiency group with 130 children (G6PD/6GPD ratio <1.00) and control group with 65 children (G6PD/6GPD ratio≥1.00). The primer design and PCR amplification conditions were optimized, and RT-PCR sequencing was used to analyze the complete coding sequence and verify the genomic DNA sequence in the two groups.

RESULTSIn the G6PD-deficiency group, the detection rate of gene mutation was 100% and 13 missense mutations were detected, including one new mutation. In the control group, no missense mutation was detected in 28 boys; 13 heterozygous missense mutations, 1 homozygous same-sense mutation (C1191T) which had not been reported in China and abroad, and 14 single nucleotide polymorphisms of C1311T were detected in 37 girls. The control group showed a high rate of missed detection of G6PD deficiency (carriers) in the specimens from girls (35%, 13/37).

CONCLUSIONSRT-PCR sequencing has a high detection rate of G6PD gene mutation and a certain value in clinical diagnosis of G6PD deficiency.

Adolescent ; Child ; Child, Preschool ; Female ; Glucosephosphate Dehydrogenase ; genetics ; Glucosephosphate Dehydrogenase Deficiency ; diagnosis ; genetics ; Humans ; Infant ; Male ; Mutation ; Reverse Transcriptase Polymerase Chain Reaction ; methods ; Sequence Analysis, DNA

Induced pluripotent stem cells (iPSCs) can be propagated indefinitely, while maintaining the capacity to differentiate into all cell types in the body except for the extra-embryonic tissues. This iPSC technology not only represents a new way to use individual-specific stem cells for regenerative medicine but also constitutes a novel method to obtain large numbers of disease-specific cells for biomedical research. However, the low efficiency of reprogramming and genomic integration of oncogenes and viral vectors limit the potential application of iPSCs. Chemical-induced reprogramming offers a novel approach to generating iPSCs. In this study, a new combination of small-molecule compounds (SMs) (sodium butyrate, A-83-01, CHIR99021, Y-27632) under conditions of transient folate deprivation was used to generate iPSC. It was found that transient folate deprivation combined with SMs was sufficient to permit reprogramming from mouse embryonic fibroblasts (MEFs) in the presence of transcription factors, Oct4 and Klf4, within 25 days, replacing Sox2 and c-Myc, and accelerated the generation of mouse iPSCs. The resulting cell lines resembled mouse embryonic stem (ES) cells with respect to proliferation rate, morphology, pluripotency-associated markers and gene expressions. Deprivation of folic acid, combined with treating MEFs with SMs, can improve the inducing efficiency of iPSCs and reduce their carcinogenicity and the use of exogenous reprogramming factors.

AIM:To explore the involvement of oxidative stress and apoptosis in the pathogenesis of diabetic cataract induced by Streptozotocin ( STZ) and the interventions of puerarin in order to supply references for clinical treatment.
METHODS:Male SD rats were divided into four groups randomly, control group, diabetic group, apocynin group and puerarin group. The diabetic group were replicated by single injection of STZ (65mg/kg, ip). The expression of p22, p47, p67, Bax/Bcl2, Caspase 3 and P53 proteins were detected by Western Blotting.
RESULTS:The diabetic rats were replicated successfully and the expression of Bcl2 was downregulated while the expression of p22, p47, p67, Bax, Caspase 3 and P53 were upregulated in diabetic group with a significant statistical differences when compared with control group (P<0. 05). Apocynin and prerarin can reverse the abnormal expression of the aforementioned proteins dramatically (P<0. 05).
CONCLUSION: NADPH oxidase mediated oxidative stress and P53, Bax/Bcl2 mediated apoptosis are involved in the pathogenesis of diabetic cataract and puerarin can alleviate cataract greatly by inhibiting the aforementioned signal pathway.

Spinal biomechanics, especially the range of spine motion,has close connection with spinal surgery. The change of the range of motion (ROM) is an important indicator of diseases and injuries of spine, and the essential evaluating standards of effect of surgeries and therapies to spine. The analysis of ROM can be dated to the time of the invention of X-ray and even that before it. With the development of science and technology as well as the optimization of various types of calculation methods, diverse measuring methods have emerged, from imaging methods to non-imaging methods, from two-dimensional to three-dimensional, from measuring directly on the X-ray films to calculating automatically by computer. Analysis of ROM has made great progress, but there are some older methods cannot meet the needs of the times and disappear, some classical methods such as X-ray still have vitality. Combining different methods, three dimensions and more vivo spine research are the trend of analysis of ROM. And more and more researchers began to focus on vivo spine research. In this paper, the advantages and disadvantages of the methods utilized recently are presented through viewing recent literatures, providing reference and help for the movement analysis of spine.
Animals ; Humans ; Imaging, Three-Dimensional ; instrumentation ; methods ; trends ; Radiography ; Spine ; diagnostic imaging

Objective The aim of this phase Ⅱ study was to determine the efficacy and safety of combined bortezomib and thalidomide (VT) regime as initial treatment for newly diagnosed multiple myeloma (MM) in China. Methods Thirty-four patients were enrolled in this study and received VT regime up to 21-day cycles. Bortezomib (1.3 mg/m2) was administered intravenously on days 1, 4, 8, and 11, while oral thalidomide ( 100 mg/day) was given from days 1 to 21. The primary end point was clinical response.The secondary end point was safety. Results Among the 34 patients, 20 were male, 14 were female, with a median age of 59 years, and 15 in international stage system (ISS) Ⅲ ,18 in ISS Ⅱ , 1 in ISS Ⅰ . Among them, 28 completed 2 cycles' treatment and achieved an overall response rate (ORR) of 92.9%; 26 were able to complete the planned 8 cycles of therapy. After 8 cycles, the ORR was 100% ( complete response 30. 8%, near-complete response 23.1%, partial response 42. 3%, minimal response 3.8% ). After followed up with a median time of 12 months, the estimated rate without progress of disease was 62%, and the estimated continous remission rate of 12 months was 62%. The median survival time was not achieved. The most common adverse events were mild to moderate ( grades 1, 2). The main toxicities were hematologic (53. 3% ), gastrointestinal ( 40. 0% ), peripheral neuropathy ( 38.0% ), fatigue ( 36. 6% ) and fever (32. 0% ). Conclusions VT regime provides a very high ORR and complete response rate in the treatment of newly diagnosed MM patients. No patients experienced deep venous thrombosis. In conclusion,bortezomib in combination with thalidomide is a very effective regimen for newly diagnosed MM patients and the toxicities are manageable.

Background The neovascular form of the disease usually causes severe vision loss in a number of eye diseases.Special-domain optical coherence tomography (SD-OCT) provides high-quality in retinal imaging and the possibility of the measurement in vivo.Objective This study aimed to investigate the feasibility of SD-OCT dynamically measuring choroidal neovascularization (CNV).Methods CNV was induced in 30 left eyes of 30 clean Brown Norway(BN)rats by retinal photocoagulation with the laser parameter as follows: wavelength 532 nm,exciting power 200 mW,spot diameter 100 μm and irradiating time 50 ms.Bubble or less retinal bleeding was thought as Brunch membrane breakage and CNV model establishment.Fundus fluorescein angiography(FFA) was performed to determine the establishment of CNV model and scored based on the fluorescein leakage on 3,7,14,21 days after photocoagulation.Meanwhile,CNV memberane thickness (CMT) was dynamically measured in vivo as the maxiume value from retinal inner limiting membrane through choroidal vessel layer in various time points.Histopathologic examination was used in the 14th day to evaluate and verify the result of SD-OCT.The right eyes were as controls.Results FFA examination showed that disc-like leakage of fluorescein appeared in 7 days and extended in 14 days after photocoagulation with the scores of 1.6±0.4,2.5±0.6 and 2.4±0.5 in 7,14 and 21 days,showing a significant difference among them(F=13.11,P＜0.01).The fluorescein leakage score was significantly higher in 14 and 21 days than that of 7 days(both P＜0.05).CMT measured by SD-OCT was(76.33±10.09),(102.03±14.21)and(98.03±13.76) μm in 7,14 and 21 days after photocoagulation respectively,with a significant difference among 3 time points (F=23.25,P＜0.01),and that in 14 and 21 days was significantly declined in comparison with 7 days(both P＜0.05).The results of SD-OCT showed a consistent tendency with that of FFA.Histopathological examination showed CNV formation in 14 days after photocoagulation.Conclusions Experimental CNV model was successfully induced by laser photography.SD-OCT technology allows excellent visualization of CNV in vivo.

A two-step method for the purification of blocking-type anti-human CD80 monoclonal antibody 4E5 from mouse ascites was developed using anion exchange and gel filtration in combination. The ascites was first purified by anion exchange after centrifugation and filtration. The experimental parameters of sample loading and elution were optimized. The optimized loading condition was pH 8.0,50 mmol/L Tris-HCl and satisfactory results were obtained using a 0~0.5mol/L NaCl step elution. The fraction containing the protein of interest was directly loaded on gel filtration column and eluted using a 20 mmol/L phosphate buffer at pH 7.2. The purity of the obtained monoclonal antibody was up to 95% with a recovery of 61%. The purity of mAb could efficiently inhibit the growth of Daudi cells. The amplification of the method was also studied using a Bio-Scale Q5 column and the result was satisfied.

Objective To investigate the risk factors of intrahepatic recurrence of hepatocellular carcinoma(HCC) after radiofrequency ablation(RFA).Methods Forty-seven patients with total of 55 HCC mass were treated with RFA between March 2001 to August 2006.The patients were either Child- Pugh class A or B with total number of mass

PurposeThe bone flare phenomena has been well described on bone scintigraphy for efficacy monitoring up to now, but our knowledge has been rarely described on MSCT, the phenomena may be erroneously classiifed as disease progression. This article intends to evaluate the existence and CT features of bone flare phenomena of metastatic bone disease in lung cancer patients treated with ibandronate, to raise awareness of this phenomenon.Materials and Methods The clinical and image data of 45 patients with bone metastases of lung cancer were retrospectively analyzed prior to treatment and 3, 6 months after treatment, the change of CT value and CT features 3 months after treatment between bone flare phenomena group, progressive disease group 1 and progressive disease group 2 were compared.Results The incidence of bone flare phenomena was 6.7% (3/45). 3 months after treatment, CT value of the bone flare phenomena group and progressive disease group 1 changed when compared with before treatment, the differences were statistically signiifcant (t=-5.787 and-2.788,P<0.05) and there was no statistically signiifcant difference (t=1.691,P>0.05) of CT value in the progressive disease group 2 after 3 months' treatment. After 3 months' treatment, the bone flare phenomena group mostly appeared as osteogenic sclerosis of osteolytic lesions, while the cases of progressive disease group mostly appeared as new periosteal reaction of the lesion, or osteogenic/mixed lesion combined with osteolytic damages, the difference between the two groups was statistically signiifcant (χ2=10.139, 8.041 and 4.154,P<0.01,P<0.05). There was no statistically signiifcant difference in increase of density in osteosclerotic lesions (χ2=0.059, P>0.05).Conclusion In patients treated with ibandronate, when there is therapeutic effect evaluation standard of bone metastases (disease progression) and clinical comprehensive curative effect evaluation standard (effective) discordance at 3 months after treatment, it can be interpreted as bone flare phenomena, and the change of CT features contributes to the differential diagnosis of bone flare phenomena with progressive disease.