Objective To explore the features of clinical manifestations and electrophysiology of spinal muscular atrophy(SMA) in children.Methods The clinical features and laboratory data were analyzed in 32 children with SMA,electromyography tests were carried out in 28 patients and the muscle biopsy were performed in 24 cases.Results The 32 cases were subdivided into 3 clinical groups,15 cases were SMAⅠ,12 cases were SMAⅡ,5 cases were SMAⅢ.They were all characterized by progressive muscle weakness associated with hyptonia and atrophy.The clinical distinction between SMAⅠto SMAⅢ reflected different age of onset and disease severity.All cases of SMAⅠhad symptoms of respiratory disability,only 1 case of SMAⅡ had paradoxical breathing and none of SMAⅢ had similarly symptoms.Electromyographic studies showed a pattern of denervation with no sensory involvement.The rate of spontaneous potential was 87%,with gentle strain,the duration of motor unit was extended(30%-150%) and the amplitude of it was increased(90%-450%),the motor nerve conduction velocity was reduced slightly in 28% patients.The muscle biopsy provided evidence of skeletal muscle denervation with groups of atrophy.Conclusions The clinical features,the changes of electromyography and the muscle biopsy are valuable for diagnosis of SMA.Respiratory management will prolong survival and improve the quality of life for these patients.

ObjectiveTo investigate the effect of nickel-titanium(Ni-Ti) shape memory alloy embracing internal fixer on oblique or spiral fracture.Methods23 cases with spiral or oblique fracture accepted internal fixation with Ni-Ti shape memory alloy embracing.The time of bone healing,articular function,the appearance and function of the wound extremity were evaluated after 9～34 months follow-up.ResultsAll fractures healed in 4～16 weeks after operation.None infection,nonunion,embracing emersion or break had been found. The fine rate was 91.3%.ConclusionThe effect of Ni-Ti shape memory alloy embracing internal fixer on long spiral or long oblique fracture is satisfactory.

Adenocarcinoma, Clear Cell ; pathology ; Adult ; Female ; Humans ; Thyroid Neoplasms ; pathology

Animals ; Blood Coagulation ; drug effects ; Blood Platelets ; drug effects ; Female ; Haplorhini ; Humans ; Interleukin-11 ; pharmacology ; Male ; Platelet Count ; Thrombopoietin ; pharmacology

Adolescent ; CD3 Complex ; blood ; CD4 Antigens ; blood ; CD8 Antigens ; blood ; Child ; Fetal Blood ; immunology ; Humans ; Infant, Newborn ; Interleukin-6 ; blood ; Interleukin-8 ; blood ; Male ; Nitric Oxide ; blood ; T-Lymphocytes ; immunology ; Tumor Necrosis Factor-alpha ; analysis

The levels of triglyceride(TG)and free fatty acid(FFA)in serum,liver,skeletal muscle,and pancreas of lipoprotein lipase gene knockout heterozygous(LPL+/-)mice and C57 mice were determined.Intraperitoneal glucose tolerance test(IPGTT)was performed to evaluate insulin sensitivity and β-cell function.The results showed that the Iipid content in 16 weeks LPL+/- group did not increase significantly.The TG and FFA contents in 28 weeks LPL+/- group were significantly higher than those in control and 16 weeks LPL+/-group(all P<0.05).In 50 weeks LPL+/- group,FFA levels in serum and pancreas,and TG content in pancreas increased significantly compared with other three groups(all P<0.05).The IPGTT result showed that the blood glucose levels increased from 15 to 120 min,not at 0 and 5 min.The blood glucose levels during 30-120 min increased significantly in 50 weeks LPL+/- group compared with other three groups(P<0.05).Fasting insulin(FINS),homoestasis assessment of insulin resistance(HOMA-IR)and pancreatic β cell function also increased gradually with age.FINS and HOMA-IR in 28 weeks LPL+/- group were higher than those in control and 16 weeks LPL+/- group.These results suggest that LPL is a key enzyme in lipid metabolism and plays a crucial role in the development of insulin resistance and diabetes.

0.05).The abrupt onset of the inflammatory arteriopathy group was 10 cases(31.2%),the abrupt onset of the noninflammatory arteriopathy group was 38 cases(58.5%),there was significant difference for the mode of onset between the 2 groups(?2=6.352 P

Objective To summarize the clinical characteristics and causes of ischemic cerebrovascular disease(ICD)in children.Methods A retrospective analysis was conducted on the clinical data of 53 cases with ICD from Feb.2002 to Jun.2008 at the department of neurology in Wuhan Children's Hospital.The self-designed questionnaire of children with ICD was used,whose items included patients' age,gender,personal history,clinical features,cerebrospinal fluid examination,neurological imaging,immunologic examination,metabolic examination,and so on.Results Of 53 children with ICD,30 cases(56.6%)were male,and 23 cases(43.4%)were female.Patients' age varied from 9 months to 12 years old,in which 45 cases(84.9%)were less than 6 years old.Patients from rural area(60.4%)were more than those from city(39.6%).Ratio of limb paralysis was 75.5%(40 cases)in first clinical symptomatology of children with ICD,including hemiplegia in 32 cases(60.4%),alternate hemiplegia in 5 cases(9.4%)and monoplegia in 3 cases(5.7%).Skull CT/MRI scan was performed to reveal 27 cases(50.9%)with basal ganglia region infarction and secondly 15 cases(28.3%)with multi-lobar infarction.Forty cases were found in abnormal cerebrovascular image by means of magnetic resonance angiography/digital subtraction angiography,in which middle cerebral artery and its branches were involved in 21 cases(52.5%).There were 41 cases(77.4%)of patients to be found with clear causes,of which 13 cases(24.5%)were of infections,8 cases(15.1%)of moyamoya disease,5 cases(9.4%)of cerebral vascular malformations,4 cases(7.5%)of head trauma.However,another 12 cases(22.6%)of patients had unknown etiology.Conclusions Children with ICD had characteristics themselves.The limb paralysis was mostly the first symptoms,and the middle cerebral artery and its branches lesions were the most common locations in children with ICD,and next the internal carotid artery involvement,anterior cerebral artery involvement,posterior cerebral artery involvement,cerebral vascular malformations,and so on.Their major cause was infection,followed by Moyamoya disease,cerebrovascular malformations and head trauma,and there were still some unknown causes.

AIM: To evaluate the repeatability and reproducibility of thickness profile measurement of intra-retinal layers determined by an automated algorithm applied to OCT images from Cirrus optical coherence tomography (OCT) instrument.METHODS: In this prospective cross-sectional study,retinal thickness images at 6mm×6mm around fovea were obtained from 86 eyes of 43 normal subjects with Cirrus HD-OCT instrument.The retinal images from patients were analysis by Cirrus automated algorithm GCA software,including ganglion cell layer and inner plexiform layer(GCIPL).During this study,operator A would make 2 times measurements to all patients,after that operator B would make another 2 times measurements by Repeat scan model.All the data,including GCL average thickness,min thickness,12 clock average thickness,2 clock average thickness,4 clock average thickness,6 clock average thickness,8 clock average thickness and 10 clock average thickness were measured by SPSS 15.0 software.Standard deviation (SD),coefficient of variation (CV) and interclass correlation coefficient (ICC) were calculated from the results of three-times tests by different examiner to evaluate the repeatability and from the results of two different examiners to assess the reproducibility.Written informed consent was obtained from each subject prior to any medical procedure.RESULTS: The average GCIPL thickness of OD was 85.12±3.95μm;the minimum average GCIPL thickness was 83.21±4.41μm;the standard deviation of OD in clock map was from 3.79μm to 4.84μm;CV(95%CI) was between 0.26 and 0.93;ICC was from 0.939 to 0.989.The average GCL+IPL thickness of OS was 84.65±8.73μm;the minimum average GCPL thickness was 81±13.08μm;the standard deviation of OS in clock map was from 8.24μm to 10.56μm;CV(95%CI) was between 0.92 and 4.94;ICC was from 0.264 to 0.968.CONCLUSION: Cirrus HD-OCT GCA software with automated algorithm can offer accurate and repeatable thickness profile of OCT retinal image.GCA is a useful and reliable approach to the measurement of intra-retinal layer thickness,which may improve the diagnosis and monitoring of retinal diseases.

PEI Xue-yi has 60 years,experience in treating pediatric diseases with traditional Chinese medicine,especially obtains good effect in children's nephrapiathy.Based on the regulation and characteristics of children's zang-fu,yin-yang,blood-qi and children's nephrapiathy,he makes different therapeutic plans in different phases.In clinical treatment,he emphasizes the lung,the spleen and the kidney and combines syndrome differentiation with disease diagnosis.Eliminaing pathogens,excessive damp-heat is used in acute phase and strengthening healthy qi,securing the spleen and kidney is used in recovery phase.Children's nephrapiathy is treated by three phases as the edema,protein urine and rescovery stage.He adopts the methods of dispersing lung qi and invigorating spleen for diuresis,clearing heat-damp,nourishing spleen and kidney,nourishing yin for protecting lower jiao.