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 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 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 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,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.

In current health care management of floating population pregnant women, health care utilization rate does not reach the ideal level.Push service has been used as a key business in development and utilization of information resources for knowledge service institution, with initiative, customized, personalized, intelligent, dynamic and integrated characteristics.On the basis of analysis of cur-rent situation about the health care management of floating population pregnant women, the paper discussed the applications of push serv-ice.New thinking of application of push service to the health care management is put forward, The right aged women can be early linked through integrated mobile phone SMS, micro message interaction, email, channel type push and other means, and broad cover and whole course service management can be achieved, leading to the enhanced care utilization rate of floating population pregnant women.

Objective To evaluate the value of echocardiography in the diagnosis of truncus arteriosus communis(TAC)and associated intracardiac malformations. Methods We retrospectively reviewed 30 patients with TAC, their datum of clinical and echocardiographic diagnosis were studied during the period of January 2006 to May 2014 in Shengjing Hospital Affiliated to China Medical University. Results Based on Collet Edwards method, these 30 TAC patients included 10 cases of typeⅠ, 7 cases of typeⅡ, 5 cases of typeⅢ, 8 cases of typeⅣ. The most common associated intracardiac malformation was ventricular septal defect in 24 cases, 16 cases had arterial septal defect, 7 cases had patent ductus arteriosus, 6 cases had single ventricle, 3 cases had right aortic arch, etc. Among them, 8 cases were confirmed by surgery, 5 cases of typeⅠ, 3 cases of typeⅡ, and 1 case of typeⅠdied after operation;16 cases were confirmed by CT angiography (CTA) examinations, all of them died without operation within 6 months. Conclusion Echocardiography is a preferred and non-invasive method for TAC and the intracardiac malformations.

ObjectiveTo analyze the craniocerebrum ultrasonographic images of the infants born by women with hypertension in pregnancy and investigate the effect of hypertension in pregnancy on neonate brain injury and brain development.MethodsTo assess the brain injury and brain development, 106 infants born by women with hypertension in pregnancy (study group) and 200 infants born by women without high risk factors during perinatal period (control group) in the neonatal intensive care unit of First Affiliated Hospital of Harbin Medical University between 2013 and 2014 underwent brain ultrasonography. The abnormal ultrasound images and the constitution of abnormal ultrasound images distribution in two groups were compared. The relationship between the abnormal ultrasound images and the degree of hypertension in pregnancy were analyzed.ResultsThe brain ultrasonographic result of the infants : (1) In the group with hypertension in pregnancy, the abnormal ultrasound images were found in 78 cases (73.6%). For the preterm infants, the solitary abnormality was found in 41 cases (25 cases of cerebral white matter injury, 14 cases of peri-intraventricular hemorrhage and 2 case of brain hypoevolutism) and multiple abnormalities were found in 15 cases; for the full-term infants, the solitary abnormality was found in 17 cases (12 cases of peri-intraventricular hemorrhage, 3 cases of hypoxic-ischemic encephalopathy and 2 case of brain hypoevolutism) and multiple abnormalities were found in 5 cases. (2) In the group without high risk factors, abnormalities were found in 73 cases (36.5 %). For the preterm infants, abnormal ultrasound images were found in 45 cases, including solitary abnormality found in 30 cases (24 cases of cerebral white matter injury, 4 cases of peri-intraventricular hemorrhage and 2 case of brain hypoevolutism) and multiple abnormalities found in 15 cases; for the full-term infants, the abnormal ultrasound images were found in 30 cases, including solitary abnormality found in 24 cases (10 cases of peri-intraventricular hemorrhage and 14 cases of hypoxic-ischemic encephalopathy) and multiple abnormalities found in 6 cases. (3) There were obvious statistical significances between the comparativeness of two groups (χ2=38.119,P<0.001), but there were no statistical significances in the constitution of the abnormal ultrasound images distribution between the two groups (χ2=0.552,P=0.759). (4) There were statistical significances between the comparativeness of the abnormal ultrasound images and the degree of hypertensive disorder complicating pregnancy (P<0.001). ConclusionCraniocerebrum ultrasonography is the first choice to detect and monitor brain abnormal and can provide basis for clinical diagnosis, treatment and intervention at early stage for the infants born by women with hypertension in pregnancy.

Seat lumbar rotation technique is a commonly used technique,and the clinician is also a kind of clinical curative effect more apparent bone-setting reduction technique.However,the power of spinning technique,direction and position selection is based on the personal experience of the clinician,the lack of a unified,standardized scientific basis for support.Now the article reviews the studies of the rotation of the seat,including lumbar anatomy,biomechanical characteristics,clicks,mechanism of action,and safety.It will provide new ideas for standardizing operations research techniques.