ObjectiveTo study the clinical characteristics of the temperature instability in Prader-Willi syndrome (PWS). MethodsThe clinical characteristics of one case of PWS with recurrent fever were retrospectively analyzed.ResultsA 5-month-old boy who had such clinical characteristics as low central muscle tension, sucking weakness, feeding dififculties, cryptorchidism and white skin was diagnosed as PWS by chromosomal microarray analysis and methylation-speciifc PCR. In the course of the disease, the infant presented recurrent fever. The pathogenic bacteria culture and virus antibody detection showed negative, and the treatment of reducing fever, anti-infection and immune support were ineffective.ConclusionsThe cause of the recurrent fever is unknown and central fever is suspected. Whether the temperature instability is the clinical feature of PWS still needs a lot of clinical researches.

Objective To analysis the long-term retention rate of Levetiraceram (LEV) monotherapy or combination therapy of infant epilepsy. Methods The clinical data of patients with infant epilepsy treated by LEV had been retrospectively analyzed from July 2006 to June 2007. Results Sixty patients with infant epilepsy treated by LEV had been recruited, 20 cases with partial seizures, 19 cases with generalized seizures, 21 cases with epilepsy syndrome. Among them 21 cases was intractable epilepsy. The retention rates of LEV in 6-month, 1-year, 2-year, 3-year and 4-year were 95.5%, 75.0%, 60.0%, 51.7%, and 38.3%. The most common reason for withdrawal was lack of effect (43.2%). COX regression model suggested that duration>1 month (RR=2.91, 95%CI:1.16~7.30) and refractory epilepsy (RR=2.30, 95%CI:1.22~4.32) were risk factors of withdrawal (all P<0.05). After treatment, the seizure frequency signiifcantly reduced compared with baseline (P<0.01). To the end of the follow-up, the efifciency was 100%and the complete remission rate was 69.57%in 23 cases continued treatment. The main side effect were fatigue (56.0%), and sleep increased, irritability, and so on. Conclusions LEV monotherapy or combination therapy has well long-term retention rate, maintains well efifcacy and tolerability in infant epilepsy.

Objective To study the serum immunoglobulin IgG,IgM,IgA and complements C3,C4 level changes in hand foot and mouth disease (HFMD) combined with acute flaccid paralysis (AFP).Methods The cases were divided into three groups in this study,including 30 cases of HFMD,30 cases of HFMD combined with AFP,and 30 cases of healthy(normal control group).Immunoturbidimetric assay was used to test the level changes of IgG,IgM,IgA,and complements C3,C4.Results The IgG,IgA,C3 and C4 in HFMD combined with AFP group were (5.49±1.04) g/L,(0.39±0.27) g/L,(0.65.±0.19) g/L and (0.16.±0.11) g/L respectively,lower than those in HFMD group((7.07± 1.63) g/L,(0.55±0.32) g/L,(0.97.±0.18) g/L,(0.23.±0.09) g/L) and normal control group((9.58±1.42) g/L,(0.81±0.33) g/L,(1.28.±0.25) g/L,(0.34.±0.16) g/L),there were statistically significant differences among groups(F=12.04,1.84,1.65,1.29;P=0.031,0.020,0.018,0.025).However,the expression of IgM in HFMD combined with AFP group was (1.34±0.26) g/L,higher than that in HFMD group((1.02±0.29) g/L) and normal control group ((0.76±0.28) g/L),the difference was statistically significant(F=3.62,P=0.014).Conclusion HFMD combined with AFP exists severe humoral immune dysfunction,which provides a theoretical evidence for the prevention and treatment of HFMD combined with AFP.

Objective To detect kinematics and surface electromyography (sEMG) of upper limbs in normal children and motor-delayed children for clinical assessment. Methods From December, 2015 to June, 2016, twelve healthy children and thirteen children with motor de-velopmental delay less than two years old were analyzed kinematics with Motion Analysis system and sEMG. Results The angle of motion of right shoulder was more in the motor-delayed children than in the normal children (t=2.576, P<0.05). The difference of root mean square values of bilateral triceps brachii was more in the motor-delayed children than in the normal ones (t=2.448, P<0.05). Conclusion Detecting kinematics and sEMG may supply information for early personalized treatment strategy.

Objective To explore the therapeutic effects of 660 nm red light on sciatic nerve injury in adult rats.Methods Forty-five adult,male rats were divided into a control group and treatment groups 1,2,3 and 4.Sciatic nerve injury was modeled by crushing the nerve.The treatment groups received irradiation with red light once daily for 21 consecutive days.The power density of red light and irradiation time varied among the groups.The latency and amplitude of compound muscle action potentials (CMAPs) and nerve conductive velocity were examined at different time points.The Sciatic Function Index (SFI) was used to evaluate walking function.Results After 21 days of red light therapy no statistically significant differences were observed between the control group and treatment groups 1 to 4 with regard to the latency or the amplitude of the CMAPs.There was a significant difference between the control group and treatment group 3 in terms of sciatic nerve conduction velocity.The average Sciatic Function Indexes of treatment groups 2,3 were significantly different from that of the control group.Conclusion Red light irradiation can promote recovery after sciatic nerve injury,at least in rats,thereby improving walking function.

Objective To investigate the effect of red light on the expression of caspase-8 and caspase-3 mRNA and protein during the acute stage of hypoxic-ischemic brain damage (HIBD) in neonatal rats.Methods Forty-five seven-day-old Sprague-Dawley rats were randomly divided into a sham group,an HIBD model group (model group) and an irradiation group.The rats in the model and irradiation groups were subject to HIBD induced using the Rice-Vannucci method.The irradiation group was treated by irradiation with red light on the forehead immediately after the establishment of the HIBD model for 30 min/d on 3 consecutive days,while the sham group and the model group received no treatment.On the 3rd day after the operation,10 rats from each group were sacrificed by cervical dislocation and the left hippocampus was rapidly isolated and snap-frozen in liquid nitrogen for mRNA and protein examination.Another 5 rats in each group were used for immunofluorescence testing to localize and semi-quantify the expression of caspase-8 and caspase-3.Results In the model group,expression of caspase-8 and caspase-3 mRNA and protein in the left hippocampus was higher than in the sham group on the 3rd day.After red light irradiation,expression of caspase-8 and caspase-3 mRNA and protein decreased significantly in the irradiation group compared with the HIBD group.In the CA 1 region of the hippocampus,the levels of caspase-8 and caspase-3 in the irradiation group were significantly lower than in the model group.Conclusion Red light irradiation can decrease the expression of such apoptosis factors as caspase-8 and caspase-3 in hypoxic-ischemic brain damage at the acute stage and inhibit neural cell apoptosis so as to exert therapeutic effects for hypoxic-ischemic brain damage.

Objective To study the effectiveness of personalized rehabilitation treatments based on threedimensional gait analysis (3DGA) for improving the walking function of children with cerebral palsy (CP).MethodsA total of 21 spastic CP children with diplegia or hemiplegia,IQ scores ＞60,and an average age of 8.5 years received 3DGA.They then received personalized rehabilitation treatment designed according to the 3DGA results.After four weeks of treatment the children accepted 3DGA again.Their gait descriptors before and after treatment were compared. ResultsAfter the personalized rehabilitation the subjects'clinical foot dorsiflexion angle,clinical popliteal fossa angle,walking velocity,stride length,step length,peak ankle dorsiflexion in prime stance,peaking ankle plantar flexion in last stance,peak back ground reaction forces (GRFs) and peak vertical GRF in stance all had improved significantly.The cadence,total support time,swing phase,initial double support time,peak knee extension in stance and the peak forward GRF were not,however,significantly different compared with before the personalized rehabilitation treatment.Conclusion Under the guidance of 3DGA,the walking function of spastic CP children improved significantly after 4 weeks of personalized rehabilitation treatment.3DGA can play an important role in formulating personalized rehabilitation protocols and guiding rehabilitation treatment for CP children.

BACKGROUND:Abnormal gaits are very common in children with cerebral palsy, and how to improve the gait is the focus of rehabilitation therapy. OBJECTIVE:To analyze the biomechanical parameters of gaits in cerebral palsy children and to observe the effects of brace control on the exercise capacity of dyskinetic cerebral palsy children. METHODS:In this paper, we compared the biomechanical parameters of children with cerebral palsy and normal children in the initial process of walking and during walking. Biomechanical parameters in the initial process of walking include spatial and temporal parameters, kinematic and kinetic parameters of the range of motion of the knee and ankle. Biomechanical parameters during walking include spatial and temporal parameters. With the aid of hand brace, dyskinetic cerebral palsy received postural control, gait training, and sling exercise therapy for 3 months. The Gross Motor Function Measure (GMFM-88) assessment and video control were conducted for evaluation of therapeutic efficiency. RESULTS AND CONCLUSION:Biomechanical parameters in the initial process of walking showed significant differences between children with cerebral palsy and normal children except for the peak of ground reaction force at frontal axis with the right foot to start walking. Biomechanical parameters during walking showed that the stance phase and bipedal stance phase were prolonged, while the step length and stride length were shortened in the children with cerebral palsy compared with the normal children. For cerebral palsy children with brace control, the GMFM-88 scores were significantly decreased after treatment, involuntary movements were reduced shown on the video, the neck and body were stable, and life skil s were improved. Children with epilepsy and cognitive impairment or who had imaging changes in the basal ganglia showed a little improvement, and children with dance-athetoid type and dystonia type had a better progress than those with athetoid spasm.

In this study, plate transparent circle by Davis method was introduce firstly screening ?-Rhamnosidase high-yield strain. The spore-sprouted Aspergillus niger 8-hour were mutagenized by ethyl methane sulphonate and pre-screened via transparent circle. 11% mutants yield 40% higher of ?-rhamnosidase than the original strain. A high-yield strain, T-226 with the highest ?-rhamnosidase activity of 373.4 U/mL was finally selected from these potential high-yield mutants after rescreened by shake flask fermentation twice. When the T-226 strain was fermented in 5 L bioreactor, the enzyme activity could reach to 631.9 U/mL after 84 h. Thus, the established screening method is highly efficient to isolate ?-rhamnosidase high-yield mutant of A. niger.

Objective To evaluate the precision of BREEZE~(TM) glucose monitor and correlation of glucose measurements between fingertip capillary whole blood glucose(CBG)using BREEZE~(TM) glucose monitor and venous plasma glucose(VPG)using autoanalyzer.Methods All samples of venous plasma and fingertip blood from 188 diabetes or non-diabetes patients were detected for glucose level at fasting,30,60 and 120 min postprandial.BREEZE~(TM) glucose monitor and autoanalyzer measured CBG and VPG respectively.Intra-and inter- coefficients of variations were determined using 10 BREEZE~(TM) glucose monitors with normal,slight high and high glucose levels for the three lots of strips.Results The correlation coefficients between CBG and VPG were all higher than 0.950 at fasting and different postprandial time.98.94% of all measurements were in the A zone when using error-grid analysis.The relevant differences between CBG and VPG were less than 5% at different blood glucose concentrations.The intra-and inter-coefficients of variations of blood glucose values at different blood glucose concentrations using different lots of strips were within 5%.Conclusion BREEZE~(TM) glucose monitor provides high accurate and precise glucose readings on fasting and different postprandial time points over a variety of blood glucose concentrations.