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.

Dengue virus (DENV) envelope [E] protein is the major surface protein of the virions that indued neutralizing antibodies. The domain III of envelope protein (EDIII) is an immunogenic region that holds potential for the development of vaccines; however, the epitopes of DENV EDIII, especially neutralizing B-cell linear epitopes, have not been comprehensively mapped. We mapped neutralizing B-cell linear epitopes on DENV-1 EDIII using 27 monoclonal antibodies against DENV-1 EDIII proteins from mice immunized with the DENV-1 EDIII. Epitope recognition analysis was performed using two set of sequential overlapping peptides (16m and 12m) that spanned the entire EDIII protein from DENV-1, respectively. This strategy identified a DENV-1 type- specific and a group-specific neutralizing epitope, which were highly conserved among isolates of DENV-1 and the four DENV serotypes and located at two regions from DENV-1 E, namely amino acid residues 309-320 and 381-392(aa 309-320 and 381-392), respectively. aa310 -319(310KEVAETQHGT319)was similar among the four DENV serotypes and contact residues on aa 309 -320 from E protein were defined and found that substitution of residues E309 , V312, A313 and V320 in DENV-2, -3, -4 isolates were antigenically silent. We also identified a DENV-1 type-specific strain-restricted neutralizing epitope, which was located at the region from DENV-1 E, namely amino acid residues 329-348 . These novel type- and group-specific B-cell epitopes of DENV EDIII may aid help us elucidate the dengue pathogenesis and accelerate vaccine design.
Amino Acid Sequence ; Animals ; Antibodies, Neutralizing ; immunology ; Dengue ; virology ; Dengue Virus ; chemistry ; genetics ; immunology ; Epitope Mapping ; Epitopes, B-Lymphocyte ; chemistry ; genetics ; immunology ; Humans ; Mice ; Molecular Sequence Data ; Viral Envelope Proteins ; chemistry ; genetics ; immunology

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.

AlM: To observe retinal hemodynamic influence of compound xueshuantong capsule on nonproliferative diabetic retinopathy ( NPDR) after laser photocoagulation.METHODS:A total of 41 patients (72 eyes) with NPDR after laser photocoagulation were enrolled in this study. They were all given compound xueshuantong capsule, and used color Doppler flow imaging for detection of retinal hemodynamics.
RESULTS: After treatment, patients with retinal blood perfusion significantly improved; central retinal arterial peak systolic velocity ( PSV ) , end - diastolic velocity (EDV) and medial velocity (Vm) were increased, while the resistance index ( Rl) decreased. The difference have statistical significance (P<0. 05). The visual acuity of 61 eyes improved, efficiency was 85%. Visual acuity was related with PSV, Vm and Rl.
CONCLUSlON: Compound xueshuantong capsule can improve retinal blood perfusion for nonproliferative diabetic retinopathy after laser photocoagulation, which is related to improvement of visual prognosis.

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.

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.

Professor LI Zhi-dao's acupuncture technique at muscle belly and its clinical experiences for the treatment of arthralgia are introduced. From aspects of TCM, anatomical physiology and sports biomechanics, the theory basis of this technique is explained, while its method is elaborated in details from acupoint selection and manipulation combined with classic medical cases. The acupuncture technique at muscle belly is applied at the muscles which already have pathological changes, avoiding re-stimulation on the pain area. It provides new thinking methods and references for acupuncture to treat arthralgia.
Acupuncture Points ; Acupuncture Therapy ; methods ; Adult ; Aged ; Arthralgia ; therapy ; Female ; Humans ; Male ; Middle Aged