Spinal muscular atrophy (SMA) is a common fatal disease in children, which can cause muscle weakness, muscle atrophy, and affect respiratory system, attracting attention from scholars both domestically and internationally. This article elaborates on the concept, application status of family management strategies, and management status for SMA patients, and proposes a concept of family management strategies for SMA patients, providing reference for constructing a suitable family management model for SMA patients.

Objective:To investigate the differences of spontaneous neural activity and functional connectivity between bilateral symmetrical voxels in the local brain regions at resting-state of methamphetamine (MA) dependent patients and healthy controls (HCs).Methods:Forty-six MA-dependent patients, admitted to and received drug rehabilitation treatment for the first time in our hospital from February 2014 to October 2019, and 46 HCs matched with age, gender and education level during the same period were enrolled in this study. The resting state functional magnetic resonance imaging (rs-fMRI) data of these subjects were collected; the static and dynamic regional homogeneity (ReHo, d-ReHo) and static and dynamic voxel-mirrored homotopic connectivity (VMHC, d-VMHC) were used to evaluate MA-related alterations of brain spontaneous activity and interhemispheric functional connectivity. The correlations of brief psychiatric rating scale (BPRS) scores with above values in the brain regions with significant inter-group differences were analyzed.Results:As compared with the HCs, the MA-dependent patients had significantly decreased ReHo in the left medial orbitofrontal cortex (mOFC), and significantly increased d-ReHo in the left mOFC, left middle frontal gyrus, bilateral inferior frontal gyrus, left precentral gyrus and left postcentral gyrus ( P<0.05). As compared with the HCs, the MA-dependent patients had significantly decreased VMHC in the bilateral mOFC, precentral gyrus and postcentral gyrus ( P<0.05). The ReHo, VMHC, d-ReHo, and d-VMHC were not significantly correlated with total scores and each factor scores of BPRS, and total dose of MA (after removing outliers) in MA-dependent patients ( P>0.05). Conclusion:During resting state, MA-dependent patients show obvious abnormalities in the coordination and stability of spontaneous neural activity and the coordination of interhemispheric activity in local brain regions, especially in the mOFC; abnormal ReHo, d-ReHo and VMHC in left mOFC may be important neuroimaging biomarkers for MA-dependence.

Objective:To establish a multi-state Markov model of type 2 diabetes mellitus (T2DM) patients and explore the transition rule between the cumulative number of different chronic complications, estimate the transition probability and intensity between status, and explore the possible factors affecting the transition between status.Methods:A retrospective cohort study of 33 575 patients with T2DM was conducted. According to the baseline and the cumulative number of chronic complications during the follow-up period, the patients were classified based on five status: T2DM, one complication, two complications, three complications, four and above complication, indicated by S0, S1, S2, S3 and S4, respectively. A time-continuous and state-discrete multi-state irreversible Markov model was used for statistical analysis.Results:The study included 33 575 T2DM patients, and their average age was 60 years old, the median of follow-up length was 8 years. In these patients, 32 653 had no baseline complications. At the end of follow-up, the transition probabilities of S0→S1, S1→S2, S2→S3 and S3→S4 were 16.4%, 32.4%, 45.6% and 25.9%, respectively. The results of multivariate analysis showed that being female ( HR=0.919), less than 60 years old ( HR=0.929), higher fasting plasma glucose ( HR=1.601), lower high-density lipoprotein ( HR=1.087), higher total cholesterol ( HR=1.090),weekly exercise ( HR=0.897), vegetarian diet ( HR=0.852) and heavy diet ( HR=1.887) were the risk factors for S0 to S1. And being female ( HR=0.768), less than 60 years old ( HR=0.859) and lower high-density lipoprotein ( HR=1.160) were the risk factors for S1 to S2. Conclusions:The probability of multiple complications in T2DM patients increased over time, the transition intensity of S2→S3 was largest, followed by S1→S2. Therefore, we need to conduct both early and long-term indicators monitoring and disease prevention, strengthen the health education to improve patients' daily living habits at early stage of the illness, encourage patients to have moderate exercise and balanced diet, strengthen the monitoring of fasting blood- glucose, cholesterol and high-density lipoprotein levels to prevent the deterioration of the illness.

Objective:The clinical characteristics of patients with primary central nervous system lymphoma-diffuse large B-cell lymphoma (PCNSL-DLBCL) and the effects of different treatment schemes on their survival and prognosis were analyzed retrospectively.Methods:A total of 49 patients with PCNSL-DLBCL who presented at the Tianjin Medical University General Hospital from July 2014 to December 2020 were included, and their clinical data were retrospectively analyzed. They were divided into four groups: the MTX group, the R-CDOP group, the BTKi-R-MTX group, and the RLZT group. The median overall survival (OS) and progression-free survival (PFS) were calculated, and the survival prognosis was compared by univariate and multivariate prognostic analysis.Results:The median OS time of the MTX group, the R-CDOP group, the BTKi-R-MTX group, and the RLZT group was 16.5 months, 4.5 months, 42 months, and not reached, respectively ( P<0.001) . The median PFS time of the MTX group, the R-CDOP group, the BTKi-R-MTX group, and the RLZT group was 7 months, 1.5 months, 20 months, and 5 months, respectively ( P=0.005) . Multivariate prognostic analysis showed that double expressor lymphoma, IESLG risk grade, and different treatment methods were the prognostic factors of PCNSL-DLBCL. Conclusion:The survival and prognosis of PCNSL-DLBCL are affected by different treatment schemes. The role of CD20 monoclonal antibody in the treatment of PCNSL-DLBCL is still controversial. The treatment scheme containing BTKi has great potential for PCNSL-DLBCL. RLZT scheme has a good prospect for elderly patients who cannot tolerate high-dose chemotherapy and radiotherapy.

Objective To observe the effects of Soyasaponins on inflammatory factors, antioxidant activity and exercise ability in rats with severe heat stroke. Methods Eighty male Sprague-Dawley (SD) rats were randomly divided into normal control group, heat shock model group, saline control group and Soyasaponin group, The rats that died during the experiment or with a low rectal temperature (< 41℃) were excluded, and finally 54 rats were included, 18 rats remaining in each group. The rats in the heat shock model group were placed in the simulated hot climate animal cabin at 30 ℃, and the temperature within 30 minutes was raised to 39 ℃ in the cabin with 65% humidity; in the mean time, the rat models of heat shock were replicated under the following situations: let the rats exercise on a treadmill with running speed set at 15 m/min, slope degree 0°, once running for 8 minutes, interval 2 minutes and the heat shock time was 90 minutes, the rats in the normal control group were fed in an environment with temperature ranging from 23-25 ℃ and relative humidity ranging from 50%-70%. After the establishment of models, the saline control group and Soyasaponin group were given daily saline and Soyasaponin (10 mg/kg) respectively by gavage for 3 consecutive months, while the heat shock model group was not given any treatment. The femoral artery blood was collected 24 hours after the rats left the cabin. The serum levels of interleukins (IL-6, IL-1β), tumor necrosis factor-α (TNF-α), interferon-γ (IFN-γ), malonaldehyde (MDA), superoxide dismutase (SOD), glutathione peroxidase (GSH-Px) were measured by enzyme-linked immunosorbent (ELISA) and the contents of serum hemoglobin (Hb), serum urea (BUN), lactate dehydrogenase (LDH) and blood lactic acid (Lac) were measured by automatie biochemical analyzer. Results The levels of IL-6, IL-1β, TNF-α, IFN-γ, MDA, Hb, BUN, LDH, Lac in heat shock model group were significantly higher than those of the normal control group [IL-6 (ng/L): 86.17±4.82 vs. 12.60±3.49, IL-1β (ng/L): 83.00±5.98 vs. 15.70±3.64, TNF-α (ng/L): 72.22±6.93 vs. 13.75±2.69, IFN-γ (ng/L): 36.22±3.02 vs. 7.35±1.60, MDA (nmol/mg): 19.78±4.56 vs. 6.40±1.35, Hb (g/L): 136.22±1.93 vs. 126.75±5.84, BUN (mmol/L):21.06±3.44 vs. 5.65±1.35, LDH (μmoL·s-1·L-1): 9.65±0.83 vs. 2.12±0.17, Lac (mmol/L): 552.56±78.33 vs. 1.32±0.18, all P ＜ 0.05], SOD and GSH-Px were significantly lower than those in normal control group [SOD (kU/L):97.89±10.57 vs. 126.65±11.35, GSH-Px (kU/L): 19.22±2.58 vs. 43.45±4.02]; however, the levels of IL-6, IL-1β, TNF-α, IFN-γ, MDA, BUN, LDH and Lac in Soyasaponin group were significantly lower than those in heat shock model group [IL-6 (ng/L): 45.28±3.54 vs. 86.17±4.82, IL-1β (ng/L): 41.61±2.93 vs. 83.00±5.98, TNF-α (ng/L):37.22±2.46 vs. 72.22±6.93, IFN-γ (ng/L): 19.22±2.60 vs. 36.22±3.02, MDA (nmol/mg): 11.28±1.74 vs. 19.78±4.56, BUN (mmol/L): 11.78±2.13 vs. 21.06±3.44, LDH (μmoL·s-1·L-1): 3.70±0.26 vs. 9.65±0.83, Lac (mmol/L): 274.56±59.08 vs. 552.56±78.33, all P < 0.01], SOD, GSH-Px and Hb were significantly higher than those of heat shock model group [SOD (kU/L): 116.11±11.28 vs. 97.89±10.57, GSH-Px (kU/L): 31.17±2.90 vs. 19.22±2.58, Hb (g/L): 141.33±3.79 vs. 136.22±1.93, all P < 0.01]; there were no significant statistical differences in above indexes between heat shock model group and saline control group (all P > 0.05). Conclusion After heat shock and exercise management, the production and release of inflammatory factors are increased, and the level of lipid peroxidation was elevated in rats. The Soyasaponin can improve the ability to withstand heat shock and strong exercise by reducing the production and release of inflammatory factors and lipid peroxidation in the rats with severe heatstroke.

OBJECTIVETo compare the effect difference for stroke between meridian-collateral diagnosis and therapy byand conventional acupuncture with syndrome differentiation.

METHODSTotally 148 patients were assigned into an observation group(72 cases) and a control group(76 cases) by random number table,with 10 cases dropping out in the observation group. In the observation group,meridians were examined and differentiated and then the treating meridians and acupoints were defined. Corresponding acupuncture was used according to them. In the control group,acupuncture was applied at acupoints by internal differentiation and experience. Treatment was given once a day and five times a week,with total 20 times. The motion function of limbs and coloboma degree of nerve function were assessed by Fugl-Meyer score and National Institutes of Health Stroke Score(NIHSS) before and after treatment as well as at three-month follow-up.

RESULTSAfter treatment,the Fugl-Meyer scores increased and the NIHSS scores decreased in the two groups compared with those before treatment(all<0.05). At follow-up three months after treatment,Fugl-Meyer score upgraded in the observation group (<0.05) and NIHSS score declined in the two groups (both<0.01) than those before treatment,and NIHSS scores were statistically different between the two groups(<0.05).

CONCLUSIONSMeridian-collateral diagnosis and therapy byhas better long-term efficacy when it is compared with conventional acupuncture with syndrome differentiation for motion function of limbs and nerve function of stroke.

Objective To evaluate the effectiveness of professional nurses in atrial fibrillation (AF) anticoagulation management system.Methods 217 consecutive patients with nonvalvular AF were enrolled.All patients received warfarin therapy for 3-6 months after catheter ablation for AF,who were divided into experimental group (n=102) and control group (n=115) by random digits table.The patients of control group adjusted their warfarin doses by following doctor's advice when discharged and by visiting the outpatient clinic regularly after discharged.The patients of treatment group adjusted their warfarin doses under the guidance of one well-trained nurse.Days of the international normalized ratio (INR) value achieved therapeutic anticoagulation range (2.0-3.0) for the first time,the effective anticoagulation rate that defined as more than 70％ of INR values between 2.0-3.0 after titration period,the ratio of times of INR value 2.0-3.0 to total times after titrating and INR monitoring frequency were compared between the 2 groups.Results In treatment group,days of the INR value achieved therapeutic 2.0-3.0 for the first time were less than that in control group (8 d vs.15 d,P＜0.01),the effective anticoagulation rate [45.1％(46/102)] and the ratio of times of INR value 2.0-3.0 to total times after titrating (67.6％±18.5％) was significantly higher than that in control group [31.3％(36/115) and 62.0％±23.1％ respectively,P＜0.05].INR monitoring during the whole period and after titrating were more frequently in treatment group [(9.4±2.2)times vs.(8.4±2.7) times,P＜0.05;(7.9±2.4) times vs.(6.3±2.8) times,P＜0.01].The number of patients in treatment group who monitor INR less than 3 times after titrating was larger than that in control group (18 vs.1,P＜0.01).Conclusions The participation of professional nurses in atrial fibrillation anticoagulation management system was helpful not only in achieving INR 2.0-3.0 more quickly but also in improving the effective anticoagulation rate.

Objective To describe the temperature variation within 24 h of the children with congenital heart disease after open heart surgery , the incidence of fever and the relation between fever and clinical outcome, aiming to provide basis for clinical nursing .Methods The convenience sampling method was used to select 200 cases of children with congenital heart disease after open heart operation , collecting body temperature within postoperative 24 hours and clinical outcomes .Results The body temperature of children reached the peak after three hours of open heart operation , then it began to slow down .The fever rate of early postoperative period (within 24 h) was 63.5%.The demographic data of fever group (≥38.0 ℃) and no fever group (<38.0℃) had no significant difference(P>0.05), except for the aspect of clinical diagnosis (χ2 =10.641, P=0.001).The median of ICU stay length, the ventilation time and the total length of hospital stay in fever group and no fever group were 68.50 h and 46.00 h, 20.00 h and 16.00 h, 16.00 d and 12.00 d, which were significantly different (Z =-1.971,-1.998,-3.700, respectively;P<0.05).Conclusions The body temperatures of children with congenital heart disease after open heart operation reach the peak after three hours . More than half of postoperative children will appear postoperative fever , which could affect the clinical outcome .

OBJECTIVETo evaluate the biomechanical effects of intracellular changes on the voltage-gated sodium channels (VGSCs) on trigeminal ganglion neuron (TRGN).

METHODSTRGN cells were acutely isolated from the neonatal SD rats. The voltage-dependent currents of the VGSCs on these neurons were elicited and analyzed by whole-cell patch-clamp recordings and the intracellular anisotonicity stimuli was established by adjusting the content of pipette solution. The effects of hypo-(260 mOsm) and hypertonic (350 mOsm) osmolarity on the activation and inactivation kinetics of VGSCs on TRGN were evaluated, compared with the normal intracellular environment.

RESULTSThe results demonstrated that intracellular hypotonic stimuli could influence both the activation and inactivation characteristics of VGSCs currents, including the membrane potential at half inactivation (V0.5) of the G-V and inactivation curves had obvious statistics significance (P<0.05) between hypotonicity (260mOsm) and isotonicity (306mOsm). However, only inactivation properties changed under intracellular hypertonic effects, including inactivation rate and k value.

CONCLUSIONIt suggests that the kinetics of VGSCs on TRGN can be modulated both by intracellular hypo- and hypertonic with different characteristics.

Animals ; Cells, Cultured ; Membrane Potentials ; Neurons ; Osmolar Concentration ; Patch-Clamp Techniques ; Rats ; Rats, Sprague-Dawley ; Sodium Channels ; Trigeminal Ganglion

Objective To analyze and explore the operative effect of coraccid process basilar part fractures combined injury of ligamental structure. Methods Use ilium-fascia lata complex tissue to reconstruct coronoid process of ulna and medial collateral ligament, recoverd anterior and wall horn anatomic structure of elbow joint. Total 9 cases of ulna coracoid process fractures concomitancy with elbow posterior dislocation . Among these one with olec-ranon fracture,five patients with head of radius fracture. Anterior trance joint approach was used,removal bone chips, restore defect with ilium-fascia lata complex tissue after cuting and trimming. Once reduction was achieved,fixed with screw. Repaird the collateral ligament and anterior joint capsule use the fascia lata, If there were combined fracture of the radius head or olecranon fracture,fixed through lateral approach,All injured extremities were treated with a posterior plaster splint with functional position for 3 weeks followed by elbow rehabilitation training. Results According to Moneys evaluation method,2 patients were classified as excellent ,5 as good,l as fair and 1 as poor. The excellent and good rate was 77. 8%. Two patients with gently ossifying myositis but no patients with wound infection,internal fixation loosening or break,elbow joint destabilizing orcorpus libemm. Conclusions Coracoid process basilar part fractures was simultaneous with the instability of osteal frame and soft tissue. To attain the favourable anatomical foundation for elbow joint functional recovery, it should rebuild the height and shape of coronoid process first and think highly of repair or rebuild medial collateral ligament and joint capsule. It reconstructed the coronoid process osteal frame and soft tissue instability at the same time to use ilium-fascia lata complex tissue. The operative procedure is simplify with reliability effect and fine functional rehabilitation.