ObjectiveTo discuss the clinical features, diagnosis and treatment of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome in children.Methods The clinical features and treatment process of two children with MELAS were retrospectively analyzed.ResultsThe main clinical features of MELAS were stroke-like epi-sodes, seizure, visual anomaly and lactic acidosis. Cephalic MRI ifndings performed during episode periods were in accord with the typical radiographic features of MELAS. Gene testing on the two children and their mothers showed the point mutation of A3243G in mitochondrial genome. The symptoms were improved signiifcantly after energy supply and corticosteroid treatment. Conclusions MELAS syndrome is easy to be misdiagnosed due to the varied clinical features. The diagnosis depends on the musclebiopsy and gene testing. Corticosteroid therapy is effective for MELAS syndrome.

Adult ; Humans ; Otitis Media with Effusion

Case-Control Studies ; Child ; Female ; Humans ; Male ; Otitis Media with Effusion ; etiology ; Risk Factors

Objective To study the clinical results of phacoemulsification with foldable posterior chamber intraocular lens implantation(PC-IOL) in the management of angle-closure glaucoma with cataract.Methods Phacoemulsification with PC-IOL implantation was performed on 58 eyes(52 cases),after a mean postoperative follow-up of (16.50?6.20) months.Results The best corrected visual acuity was improved in 53 of 58 eyes.The intraocular pressure was reduced from a preoperative mean of (19.56?3.36)mmHg to a postoperative mean of (14.26?3.20)mmHg(P

Objective To compare the clinical performance of the self-etching and the phosphoric acid etching adhesive system for restoration of non-carious Class V restorations for a period of two years.Methods There were 37 patients with at least one pairs of similar-sized non-carious cervical lesions participated in the study.120 restorations were placed by one operator,of which 60 with phosphoric acid etching adhesive system(Spectrum TPH,Prime& Bond)and 60 with self-etch(Clearfil AP-X,SE Bond).The restorations were evaluated by ?2 statistics at the baseline,6th,12th and 24th month according to the modified Ryge"s USPHS criteria.Results At two years,106 teeth were reviewed in 33 patients.The retention rates for self-etching adhesive system were 89% and for phosphoric acid etching adhesive system 77%.The percentage of the retention rates of both adhesive systems was not found to be different when calculating the failure rates.No cases in both adhesive systems showed slight marginal discoloration problems.Some cases for each adhesive system had slight color change after the same period.Conclusions The performance of both adhesive systems is excellent during this two-year clinical trial.The self-etch adhesive system exhibites slightly better coloar match than the phosphoric acid etching system.

Objective To investigate the effects of omega-3 polyunsaturated fatty acids (ω-3 PUFA) supplementation on neuron apoptosis,brain edema,activation of microglia,inflammatory response and neural function after traumatic brain injury (TBI) in rats,so as to understand the protection of ω-3 PUFA in rats following TBI and its mechanism.Methods TBI model was established using Feeney's method.Ninety SD rats were randomly divided into 5 groups:sham operation group (sham group),TBI group,TBI + selective activator of c-Jun N-terminal kinase (JNK) anisomycin group (TBI + Aniso group),TBI + ω-3 PUFA supplementation group (TBI + ω-3 group),and TBI + ω-3 PUFA supplementation + JNK activation group (TBI + ω-3 + Aniso group).We measured rat behavioral outcomes by modified neurological severity score (mNSS) on day 1,3,and 7 after TBI.Brain water content was measured with wet-dry weight method.The neuron apoptosis and microglial activation (identified by specific marker IBA-1) in TBI cerebral cortex were determined by TUNEL staining and immunofluorescence.Inflammatory cytokines [tumor necrosis factor-α (TNF-α),interleukin (IL)-1α,IL-1β,and IL-6] and the JNK signaling pathway (JNK,pJNK) were tested with reverse transcription-polymerase chain reaction and Western blot,respectively.Results Compared with the sham group,the levels of brain cell apoptosis,brain edema,neuron apoptosis,and inflammatory-relatived factors (TNF-α,IL-1 α,IL-1β,and IL-6) were significantly increased in the other four groups (P ＜ 0.05).Compared with the TBl group,ω-3 PUFA supplementation reduced brain water content following TBI,especially on day 3 after TBI [(78.14 ± 0.57) ％ vs.(82.31 ± 0.81) ％,P ＜ 0.01],and improved neurological function score (P ＜ 0.05).Meanwhile,ω-3 PUFA supplementation suppressed neuron apoptosis,the activation of microglia,and the mRNA and protein expressions of inflammatory cytokines (TNF-α,IL-1α,IL-1 β,IL-6).The activation of JNK signaling pathway was also inhibited by ω-3 PUFA.Conclusion ω-3 PUFA supplementation may markedly reduce brain edema,suppress neuron apoptosis,and improve neurological outcomes after TBI in rats,possibly mediated by inhibiting JNK signaling pathway and microglial activation,reducing microglia-induced cerebral inflammatory responses,demonstrated as down-regulated expression of TNF-α,IL-1α,IL-1β,and IL-6.

Objective To study the clinical characteristics and treatment of neurofibromatosis type Ⅱ (neuro-fibromatosis type 2 ,NF2 ,bilateral acoustic neuroma) ,and the effects of auditory brainstem implant for treating to-tal deafness after bilateral acoustic neuroma resection .Methods One case of bilateral acoustic neuroma and all clini-cal data in terms of diagnosis ,treatment and hearing -speech rehabilitation after surgery were retrospectively stud-ied .Results The patient was a thirteen years old boy .His clinical symptoms were hearing loss on the right ear ,tin-nitus ,hoarseness and gait instability three years .MRI showed space occupying lesion in the cerebellopontine angle . The postoperative pathological diagnosis was bilateral acoustic neuroma .The initial switch -on was peformed six weeks after the surgery ,and confirmed that all electrodes generated listening responses .As the extension of recov-ery time ,the correct recognition rate of patients on the natural environment sound ,vowel ,monosyllabic were on the rise and the pure tone hearing threshold gradually decreased .The vowel correct recognition rate of postoperative 6 , 9 ,12 ,24 ,and 36 months were 14% ,18% ,20% ,24% ,and 20% ,respectively .The recognition rate of monosyl-labic and open words at each postoperative rehabilitation stage were 0 .Conclusion The clinical characteristics and treatment of bilateral acoustic neuroma were different from the unilateral acoustic neuroma .The individualized treat-ment should be followed .Auditory brainstem implant could be performed in patients with post - bilateral acoustic neuroma resection .The accurate location of the cochlear complex during the surgery was the crucial point for the success of ABI .

Objective To evaluate the radiation dose from enhanced dual-source computed Tomography(DSCT) scan on children with congenital heart disease(CHD). Methods Seventy children with CHD, age from 1 month to 8 years old, were scanned with enhanced DSCT. Children were divided by age into ＜ 1 year old group, 1-5 years old group, ＞ 5 years old group. The differences among three groups were tested by F test. Then, the SNK test was used to compare the difference between each group. Multiple linear regression analysis was used to test the relationship of dose length product (DLP) with the age,weight, voltage, current, pitch and scan sheet. Results The average value of DLP was (144.46 ±test showed statistical differences of DLP between ＜ 1 year old group and 1-5 years old group, and between ＜1 year old group and ＞5 years old group(q =5.21,6.52,P =0.009,0.004). The difference of DLP between 1-5 years old group and ＞ 5 years old group did not reach significant ( q = 0.28, P = 0.48 ). The differences of ED was not statistically significant among the three groups [＜ 1 year 3.20 mSv, 1-5 years (5.17 ± 1.98 ) mSv, ＞ 5 years ( 3.74 ± 1.33 ) mSv, F = 0.54, P = 0.59]. DLP was positively correlated with age (4.3 years,r = 0.541 86, P = 0.0008 ), weight ( 12.1 kg, r = 0.563 71, P = 0.0004 ), voltage [(95.48±6.99) kV,r =0.632 69, P＜0.01], current [(138.55±40.67) mA,r =0.796 08, P＜0.0001] and scan sheet (236.10 ±46.51 ,r = 0.721 92, P ＜ 0.01 ). DLP was negative correlated with pitch (0.48 ± 0.03,r = - 0.466 93, P = 0.0047 ). Conclusion Higher DLP was observed in children over 1 year old under enhanced DSCT scan, but ED was not statistically significant among the three groups due to the higher K value in the children under 1 year old.

Objective To study the clinical value of Na+/I-symporter(NIS)expression on thyroid carcinoma diagnosis and 131I therapeutic effects prediction.Methods Thirty-one cases of thyroid carcinomas enrolled in this hospital from 1998 to 2006 were included.Using immunohistochemical method,NIS expression location,positive cell staining and expression intensity were observed,which was calculated by immunohistochemical scores(IHS)and NIS expression level was compared between primary and metastatic carcinoma.Results NIS was over-expressed on the basolateral membrane in positive control——Grave disease tissue,and showed no staining in negative control.NIS was expressed in cytoplasm in all 31 primary carcinomas,and IHS was over or equaled to 4 in 80.65% of them.Except for 2 no staining,NIS was expressed in cytoplasm in the rest 28 metastatic carcinomas.NIS expression was related to the pathological type of thyroid carcinoma,the strongest in PTC,then FTC,and the weakest in fvPTC.NIS expression in metastatic carcinoma was related to that in primary carcinoma.Conclusion NIS is over-expressed in cytoplasm in most thyroid carcinoma,and the iodide uptaking defect is mainly due to its wrong location.It has great potential to be applied in clinic by that it can help with the differential diagnosis of benign and malignant thyroid diseases,especially between FTA and FTC,and that it can help predict the therapeutic effects of 131I therapy following thyroid operation.

Objective:To evaluate the treatment effects of the half-columnar shaped mandibular block bone onlay grafting technique for augmentation of the resorbed maxillary anterior alveolar ridge after single tooth missing.Methods: A total of 15 sites of 14 patients received ridge augmentation surgeries.The recipient sites were prepared with trephines,the half-columnar shaped bone blocks were harvested from the ramus and external oblique ridges with trephines according to diameters of the recipient sites.The bone blocks were placed as lateral onlay grafts on recipient beds and secured by means of titanium screws.Particulate bone was added and absorbable membranes were used to stabilize and protect the grafts.After a mean interval of 4.5 months of healing the flaps were re-opened,the screws were removed and non-submerged implants were placed.The width and height of the alveolar ridges were recorded.After 3 months,implant-supported crowns were provided to the patients.One year later,the peri-impant condition and the marginal bone resorption on the proximal sites were observed.Results: Mean lateral augmentation obtained at the time of bone grafting was(3.8?0.8) mm(x?s),5 out of 15 sites exhibited a mean of 3 mm of vertical augmentation.The mean healing time was 4.5 months,the mean percentage of horizontal and vertical bone resorption in the mean time were 8% and 7% respectively.No major complications were recorded at donor sites.No implant was lost during the study period.Clinical parameters and probing depth(≤4 mm) demonstrated the presence of a healthy peri-implant mucosa after 1 year of prosthetic reconstruction.The clinical and radiographic bone observations showed no more than 1.2 mm of resorption after bone graft and implant placement.Conclusion: The half-columnar shaped mandibular bone graft(from the ramus and external oblique ridge) is a promising technique for bone augmentation in localized alveolar ridge defects after single tooth missing.This procedure offers easy access,good bone quantity for localized repair,low morbidity,decreased complaints of postoperative sensory disturbances or discomfort,minimal graft resorption,and a shorter healing time as compared with other methods for bone repair.