Objective To investigate the clinical and pathological features of riboflavin-reactive lipid storage myopathy.Methods Clinical material of 4 patients with riboflavin-reactive lipid storage myopathy were analyzed retrospectively.Results All the patients were subacute onset and presented proximal and axial muscle weakness accompanied by intolerance to excise.Amyotrophy and weakness involved in neck and paraspinal muscle were found in 3 cases,and chewing muscle weakness in 2 cases.Electromyogram showed myogenic changes in 2 cases and reduced conductive velocity of tibial nerves in 1 case.Muscle Biopsy study showed markedly increased lipid droplets in muscle fibers.The ragged red fibers,succinate dehydrogenase strongly reactive vessels and COX deficiency fibers which supported mitochondrial myopathy were not detected.Electromicroscope observation revealed that the structure and quantity of mitochondria were normal.All the patients had a dramatic response to riboflavin treatment.Two cases were cured and the other two were improved significantly.Two cases relapsed 1 year and 5 years later,respectively,and riboflavin was still effective for them.Conclusions Riboflavin-reactive lipid storage myopathy is a myopathy characterized by preferential involvement of neck,paraspinal and chewing muscles.The distinct pathological features are lipid accumulation in muscle fibers without any abnormalities of mitochondrial structure and quantity.Riboflavin alone is effective for this myopathy and this is distinguished from other myopathies.

Objective To investigate the clinical and pathological features of adult form nemaline myopathy. Methods Biopsied muscle specimens from two patients were observed using enzymatic histochemical method and electron microscope. Results These two patients were found with an initial onset from neck muscle weakness at the age of 52 and 36 years respectively, followed by trunk and limb muscle involvement to a variant degree. Type Ⅰ fiber atrophy and numerous granules (rod) were found in both patients in light microscopy. In case 1, the centralized nuclei were seen almost in all normal sized muscle fibers. Electron microscopic observation showed marked myofibril disorganization and numerous rod-like formations. Two inclusion-carrying nuclei were detected in case 1. The intranuclear inclusion showed a lattice pattern of Z-disc line or sarcoplasmic rod. Conclusions Adult form nemaline myopathy is clinically nonspecific. Rod-like structures might be found either in sarcoplasma or in nucleus. Centralized nuclei might coexist with rods in adult form nemaline myopathy. As compared to the child form, the adult one might often show a marked muscle fiber atrophy.

Objective: To study the influence of anterior crossbite on the position and morphology of teeth. Methods: 81 teenagers with permanent dentition and anterior crossbite were in volved in group 1 and 95 teenagers in permanent dentition without anterior crossbite in group 2. The teeth in both groups were compared on their position and morphology. Results:① The percentage of ill-positioned teeth in maxilla in group 1 and in group 2 was 14.0% and 8.8% respectively (P

Objective The oligomeric and polymeric proanthocyanidins in different fractions from Hippophae rhamnoides seeds were investigated. Methods The monomeric and oligomeric fractions were analyzed by HPLC/ESI-MASS detection. The polymeric proanthocyanidins were fractionated by Sephadex LH-20 column chromatography and their chemical constructures were studied by acid-catalysed degradation in the presence of benzyl mercaptan. Results Four monomers were identified as catechin, epicatechin, gallocatechin, and epigallocatechin. Eight dimers including three dimeric procyanidins and one dimeric prodelphinidin, and four mixed dimers were detected and their structures were partly elucidated by the detection of the pseudomolecular ion and main ion fragmentations. The results also showed that polymers to be highly heterogenous with catechin, epicatechin, gallocatechin, and epigallocatechin all being components of both the extension and the terminal units. The extension units were mainly gallocatechin, whereas the terminal units were mainly gallocatechin and epigallocatechin for all fractions. The proanthocyanidins with a mean degree of polymerization (mDP) of 9.1, 13.2 and 17.0 represented the three major polymeric fractions. Conclusion Proanthocyanidins of H. rhamnoides seeds, predominantly being of prodelphinidin-type, are remarkably different from that of grape seeds.

Objective To investigate the clinical,genetic and pathological features of familial chronic progressive external ophthalmoplegia (CPEO) type of mitochondrial myopathy.Methods Clinical manifestations, family histories and pathological findings of 21 patients with CPEO type of mitochondrial myopathy from 3 families constellations were analyzed retrospectively.Results All the patients had ptosis and movement disorder of eyeball, with or without myasthenia. An autosomal dominant pattern of transmission was deduced from one family and a maternal transmission appeared most likely in the other two families. The striking and common pathologic findings were presence of ragged red fibers and cytochrome C oxidase (COX) deficiency fibers under microscope. Ultrastructural alterations included subsarcolemmal accumulation of mitochondria, increase of mitochondria with abnormal shape, disarrangement of cristae and paracrystaline inclusion bodies.Conclusions The clinical and pathological features between generations and families seem to be similar. It is suggested that different genetic mode of CPEO may lead to similar clinical and pathological features.

Objective To analyze the current status of the drug resistance of enterococci and monitor the prevalence of drug-resistant strains of enterococci in hospital infection. Methods 318 strains of enterococci separated from infectious samples in nine general hospitals in Hubei were assayed and tested with regard to drug sensitivity and the software of WHONET4 was used to analyze the data. Results The separation ratio of ARE, HLARE, VRE and ARE complicated with HLARE was respectively 16.98% , 37.76% , 0 and 5.66% . Conclusion The drug resistance of enterococci shows the tendency of increasing and consequent hospital infection shows the tendency of scattered prevalence. Laboratories of clinical microorganisms ought to closely watch the detection and spread of VRE.

Objective To explore the changes of left atrial systolic function in patients with coronary heart disease after coronary artery bypass grafting (CABG). Methods Strain rate imaging (SRI) was performed on 23 patients with coronary heart disease before CABG, 1 week, 1 and 3 months after CABG to evaluate left atrial systolic function quantitatively. Results No significant change of left atrial systolic function was detected 1 week after CABG (P＞0.05 ). E/A and LVEF increased, LAFS, AEF and SRa decreased 1 month after CABG compared with those before CABG (P＜0.05). Three months after CABG, changes turned more significantly (P＜0.01). Left ventricular ejection fraction (LVEF) increased 1 and 3 months after CABG, and its changing rate negatively correlated with those of Sra (r=-0.751,-0.783; all P＜0.01). Conclusion Left atrial systolic function is affected by CABG, presenting as decrease of pump function. SRI can be used to evaluate the atrial systolic function quantitatively and monitor the changing of left atrial systolic function dynamically after CABG.

Objective:To investigate the clinical and diagnostic characteristics of audiometric findings and vestibular-evoked myogenic potentials in patients with large vestibular aqueduct syndrome (LVAS).Method:Thirty LVAS subjects(60 ears) recruited received pure tone audiometry, acoustic immittance, auditory brain stem responses (ABRs), distortion-product otoacoustic emission(DPOAE), Vestibular evoked myogenic potentials(VEMP) and caloric test, and the diagnostic significance of the results was analyzed.Result:All 30 cases(60 ears) showed progressive and fluctuating hearing loss, while 16 cases experienced dizziness when hearing fluctuated. Most of our cases showed sensorineural hearing loss, and 47 ears(94.0%) showed air-bone gap in the low frequencies, with mean gaps of (43±17)dB HL at 250 Hz, (33±18 )dB HL at 500 Hz, in which the middle ear function showed normal. The acoustically evoked short latency negative response(ASNR) with medium latency(3.06±0.52)ms was elicited from 18 ears(64.3%). The mean amplitude of vestibular evoked myogenic potentials(VEMP) of 42 ears was (147.10± 107.55)μv,and the threshold of VEMP of 19 ears was 75 dB nHL,of 7 ears was 65 dB nHL.Conclusion:Characteristics of hearing performance, such as progressive and fluctuating hearing loss, air-bone gap at the low frequencies with normal middle ears, the ASNR, and increased amplitude and decreased threshold of the VEMPs, will help clinicians make initial diagnosis of LVAS ,and provide a reference for further imaging examination.

ObjectiveTo evaluate left atrial function in patients with dilated cardiomyopathy (DCM)by echocardiography.MethodsThere were 31 patients with DCM and 30 controls in this study.Left atrial diameter (LAD),left ventricular diameter (LVD) were measured by M-mode in parasternal long-axis view.Mitral flow parameters including peak velocities during early diastole (E) and late diastole (A) were measured by pulsed-wave Doppler echocardiography,and the E/A ratio was calculated.Left ventricular ejection fraction (LVEF) was measured with Simpson′s method on the 2-chamber (Ap2c) and 4-chamber (Ap4c) views.Strain rate (SR) imaging was performed on all cases.Peak SR was measured at each segment (septal,lateral,posterior,anterior,and inferior walls) and mean peak systolic left atrial SR (mLASRs),mean peak early diastolic left atrial SR (mLASRe) and mean peak atrial systolic left atrial SR (mLASRa) were calculated by averaging the data in all segments.ResultsCompared with controls,LAD and LVD were significantly increased ( P ＜0.01 ),the E/A ratio had no significant changes ( P ＞0.05).LVEF and left atrial fractional shortening (LAFS) turned lower,mLASRs,mLASRe,and mLASRa of DCM decreased significantly ( P ＜ 0.05).mLASRa of DCM correlated positively with LAFS ( r =0.79,P ＜ 0.01 ).ConclusionsThe left atrial function of DCM decreased,including the reservoir,conduit and pump function.Echocardiography could evaluate the left atrial function of DCM quantitatively.

Objective To detect cardiac function in rabbits models with isehemic reperfusion and to evaluate the short-term protective effects of ischemic postconditioning on regional systolic function of left ventricle by echocardiography. Methods Twenty-eight rabbits were divided into 2 groups:control group and ischemic postconditioning(IP) group. Echocardiography were performed before tests and 2 weeks after tests. The conventional echocardiography indices included left ventricular end-diastolic diameter(LVDd) ,left ventricular anterior wall end-diastolic thickness (AW) and left ventricular ejection fraction (LVEF). Quantitative tissue velocity imaging and strain rate imaging indices included the peak velocity in systole(Vs) and radial peak strain rate in systole(SRs). Results Before tests, there were no difference in conventional echocardiography indices between the two groups; compared with before test, the two groups had significantly larger LVDd,thiner AW and lower LVEF(P <0.05) ; compared with the controls, the group IP had significantly smaller LVDd, thicker AW and higher LVEF 2 weeks after tests(P<0.05) ; compared with controls, the number of segments with abnormal wall motion in group IP significantly decreased. Before tests,there were no differences in Vs and radial SRs between the two groups; compared with before test,the two groups had significantly lower Vs and radial SRs(P<0.05) ; compared with the controls,Vs and radial SRs in group IP significantly increased 2 weeks after tests (P<0.05). Conclusions Ischemic postconditioning can protect regional systolic function of rabbits models with ischemic reperfusionin in short-term,which can be effectively evaluated by strain rate imaging.