ABSTRACT
ObjectiveUsing the liquid chromatography-tandem mass spectrometry (LC-MS/MS) to determine the plasma level of Lyso-GL3 in patients with Fabry disease and to analyze the clinical application of the method.MethodsThirty-nine patients with a genetic diagnosis of Fabry disease were included, and plasma levels of Lyso-GL3 were measured by LC-MS/MS analysis, and detailed clinical information of the patients was obtained including: α-galactosidase A activity, genetic variants, quantification of urine protein, mean arterial pressure, and estimation of glomerular filtration rate, and the differences in the levels of Lyso-GL3 in different clinical phenotypes and genotypes were statistically analyzed, as well as the association with clinical indicators.ResultsLyso-GL3 showed good linearity within 0.7856-400 ng/mL(r=0.9992).Further analysis of 39 Fabry disease patients diagnosed in Ruijin Hospital, Shanghai Jiao Tong University School of Medicine showed a median Lyso-GL3 concentration of 23.6 ng/mL(4.3-92.9 ng/mL); Lyso-GL3 levels were significantly higher in patients with both the frameshift and the splicing mutations, as well as in patients with the nonsense mutations, than in patients with the missense mutations (median value 119.7 ng/mL vs. 11.9 ng/mL, P=0.006, and median value 97.0 ng/mL vs. 11.9 ng/mL, P=0.015, respectively). Whereas, association analysis revealed that Lyso-GL3 was not significantly associated with urinary protein, mean arterial pressure and estimated glomerular filtration rate.ConclusionsThe using of LC-MS/MS to quantify plasma Lyso-GL showed significant differences in Lyso-GL3 concentrations between classical and atypical phenotypes, suggesting that plasma Lyso-GL3 may help with clinical phenotypes. However, Lyso-GL3 levels is found to be overlapped between genotypes. No significant linear correlation was found between Lyso-GL3 and renal clinical indicators, suggesting the urgent need in finding a more accurate tool to assess renal involvement and prognosis in patients with Fabry disease.
ABSTRACT
ObjectiveUsing the liquid chromatography-tandem mass spectrometry (LC-MS/MS) to determine the plasma level of Lyso-GL3 in patients with Fabry disease and to analyze the clinical application of the method.MethodsThirty-nine patients with a genetic diagnosis of Fabry disease were included, and plasma levels of Lyso-GL3 were measured by LC-MS/MS analysis, and detailed clinical information of the patients was obtained including: α-galactosidase A activity, genetic variants, quantification of urine protein, mean arterial pressure, and estimation of glomerular filtration rate, and the differences in the levels of Lyso-GL3 in different clinical phenotypes and genotypes were statistically analyzed, as well as the association with clinical indicators.ResultsLyso-GL3 showed good linearity within 0.7856-400 ng/mL(r=0.9992).Further analysis of 39 Fabry disease patients diagnosed in Ruijin Hospital, Shanghai Jiao Tong University School of Medicine showed a median Lyso-GL3 concentration of 23.6 ng/mL(4.3-92.9 ng/mL); Lyso-GL3 levels were significantly higher in patients with both the frameshift and the splicing mutations, as well as in patients with the nonsense mutations, than in patients with the missense mutations (median value 119.7 ng/mL vs. 11.9 ng/mL, P=0.006, and median value 97.0 ng/mL vs. 11.9 ng/mL, P=0.015, respectively). Whereas, association analysis revealed that Lyso-GL3 was not significantly associated with urinary protein, mean arterial pressure and estimated glomerular filtration rate.ConclusionsThe using of LC-MS/MS to quantify plasma Lyso-GL showed significant differences in Lyso-GL3 concentrations between classical and atypical phenotypes, suggesting that plasma Lyso-GL3 may help with clinical phenotypes. However, Lyso-GL3 levels is found to be overlapped between genotypes. No significant linear correlation was found between Lyso-GL3 and renal clinical indicators, suggesting the urgent need in finding a more accurate tool to assess renal involvement and prognosis in patients with Fabry disease.
ABSTRACT
Objective:To investigate the clinical characteristics and treatment of patients with refractory spasm tetanus.Methods:Tetanus patients admitted to the Affiliated Hospital of Zunyi Medical University from January 2011 to April 2021 were collected and divided into the refractory spasm group and general group according to whether they were refractory spasm. The general demographic characteristics, clinical characteristics, treatment and prognosis of the two groups were compared. The treatment and risk predictors of patients with refractory spasm tetanus were explored.Results:Among the 59 tetanus patients, 35 patients (59.32%) were in the refractory spasm group and 24 patients (40.68%) were in the general group. There were no significant differences in sex, age, latency and trauma site between the two groups ( P>0.05). All patients with refractory spasmodic tetanus were treated with mechanical ventilation, the length of hospital stay was significantly prolonged, and the rate of pulmonary infection was significantly increased ( P<0.05). The incidence of multiple sites muscular rigidity (spasm/limb stiffness, neck stiffness, and angular pedicle tension) in patients with refractory spasmodic on the first day of admission was higher than that in the general group ( P<0.05). The patients' initial symptoms (within 24 h after admission) were muscle spasm/limb stiffness + neck stiffness + angular pedicle inversion, which had the highest specificity for predicting the occurrence of refractory spasm. The proportion of Ablett grade Ⅳ in patients with refractory spasm was higher than that in the general group, and the difference was statistically significant ( P<0.05). The 48.75% patients with refractory spasm tetanus were treated with more than 3 sedatives combined with muscle relaxants, and the duration of sedative use was significantly prolonged ( P<0.05). Conclusions:The mechanical ventilation time and hospitalization time in tetanus patients with refractory spasm are significantly prolonged, and the incidence of pulmonary infection is significantly increased, which requires the combined application of a large number of sedative and muscle relaxants for a long time, and the incidence of refractory spasm is higher in patients with multi-site muscular rigidity at the early stage of the disease.
ABSTRACT
Ischemic stroke has the characteristics of high morbidity, high disability rate, and high fatality rate. There is currently no effective rehabilitation treatment method. With the advancement of basic research and preparation technology of stem cells and the extensive development of animal experiments, stem cell transplantation has shown great potential for application in the treatment of ischemic stroke. This article elaborated on the mechanism, types and sources of stem cell transplantation and transplantation methods. By reviewing the research process of stem cells in ischemic stroke model, we can provide reference for clinical research of stem cell transplantation in the treatment of ischemic stroke.
ABSTRACT
Objective To discuss the clinical characteristics of patients withclinical features of patients with neuromyelytis opica and spectrum of neuromyelytis opica(NMOSD) and neuromyelitis optica (NMO).Methods With a retrospective study,From February 2013 to September 2016,a total of 72 NMO patients in Navy General Hospital for diagnosis and treatment were selected as the NMO the NMO group and the other 72 patients of NMOSD patients were selected as the NMOSD group.The results of two groups of patients with general demographic data,ocular symptoms,spinal cord and brain MRI,influence NMO-IgG were recorded.Results There were no significant differences in gender and age compared between the two groups (P ＞ 0.05).The clinical characteristics,frequency and duration in the NMOSD group compared to the NMO group were significantly different (P ＜ 0.05).In the NMOSD group,there were 8 patients who were decreased vision,4 patients were visual field defect,3 patients were discoloration,5 patients were diplopia.While in the NMO group,32 patients were decreased visual acuity,14 patients were visual field defect,12 patients were color vision,21 patients were diplopia.There were statistically significant difference between the two groups (P ＜ 0.05).In the NMOSD group,there were 8 patients were MRI of the spinal cord were normal,64 patients were abnormal and 32 patients of brain lesions in the head MRI.In the NMO group,MRI of the spinal cord were all abnormal,and there were 28 patients were brain lesions in the head MRI.The serum positive rate of NMO group was 41.7％,and the serum positive rate of NMOSD group was 59.7％.The sensitivity of AQP4-Ab antibody to NMO was 44.4％,the specificity was 75％,the sensitivity of AQP4-Ab was 61.1％,and the specificity of NMOSD was 75％.Conclusion The NMOSD is more with female patients,the first symptom is more with the spinal cord that the gray matter involvement in the spinal cord,and the performance of complex,NMO-IgG antibody can be used as a support for NMOSD diagnosis.
ABSTRACT
Objective To investigate the effect of sulforaphane synergistic with lithium chloride on neuroprotective of rats with traumatic brain injury. Methods According to feeney method free fall production models of traumatic brain injury induced injury models, and rats were divided into control group, model group, SFN group and collaborative group, each group 15 rats, control group without impact experiment, after operation the SFN group received daily intraperitoneal injection of sulforaphane, collaborative group were given sulforaphane and chloride lithium intraperitoneal injection, the model group received daily intraperitoneal injection of saline, the degree of nerve function impairment score (mNSS) in rats of each group postoperative 1 d, 3 d, 5 d and 7 d, and brain tissue of rats, the water content of brain tissue, HE staining of brain tissue and activity of SOD, IL-6, TNF-alpha, the level of MDA in each group postoperative 7 d were compared. Results Compared with the control group, mNSS scores in the model group, SFN group, collaborative group at each time point were significantly increased (P<0.05); After 5 d, 7 d of operation, synergy scores in SFN group and mNSS group rats were significantly lower than those in the model group (P<0.05), and mNSS score in collaborative group was significantly lower than that of SFN group(P<0.05); After 7 d of operation, the brain tissue water content in control group of rats was (69.29±2.06)%, the model group was(75.40±1.73)%, SFN group was (73.08±1.06)%, collaborative group was (71.27±1.52)%; Brain tissue HE staining results showed that the nerve cell injury of rats in SFN group and cooperative group was obviously alleviated than those in the model group, necrotic cells decreased, and the collaborative group relieve neuronal injury was the most remarkable; The SOD in model group, SFN group, collaborative group decreased significantly compared with the control group (P<0.05), IL-6, TNF-alpha, MDA levels increased significantly(P<0.05);The activity of SOD in SFN group and collaborative group increased significantly compared with the model group(P<0.05), IL-6, TNF-alpha, MDA levels decreased significantly (P<0.05), and collaborative TNF-alpha and IL-6 levels were significantly lower than that of SFN group (P<0.05). Conclusion Sulforaphane in combination with lithium chloride have remarkable neuroprotection on traumatic brain injury in rats, its mechanism may be related with the reduction of brain edema in rats, nerve cell damage, inhibition of oxidative stress and related to the release of inflammatory factors.
ABSTRACT
Objective To explore the treatment effect and application value of proximal humeral locking plate combined with injectable bone graft in the treatment of proximal humeral fractures.Methods 86 patients with proximal humeral fractures were selected,using the random number table method they were randomly divided into the observation group and control group,each group had 43 cases.The control group was treated with locking plate method,and the observation group was treated by the combination of artificial bone and locking plate.The therapeutic effect of the two groups was observed.Results The excellent and good rate of the observation group was 93.03％,that of the control group was 74.42％,the difference was statistically significant between the two groups(x2 =5.460 3,P ＜0.05).In observation group,the fracture healing time was (11.78 ± 3.64) weeks,the shoulder joint function score was (96.87 ± 4.23) ; those of the control group were (19.65 ± 6.27) weeks,(78.73 ± 8.28),the differences were statistically significant between the two groups (t =7.118 2,12.793 4,all P ＜ 0.05).The incidence rate of complications of the observation group was 6.98％,which was significantly lower than 30.23％ of the control group (x2 =7.678 6,P ＜ 0.05).Conclusion Proximal humeral locking plate combined with injectable bone graft in the treatment of proximal humeral fracture has reliable curative effect,short fracture healing time and mild surgical complications,which is worth popularizing in clinical.
ABSTRACT
A novel automatic manufacture device for tissue micro-array is introduced in this paper. Based on the analyses of task and process, the new device prototype is researched and developed. The device consists of a paraffin positioning module and a three-manipulator module. The control system is composed of accurate navigation sub-system, digital image recognition sub-system and punching-filling operating sub-system. The results of experiment demonstrate that the device can accomplish the operations such as image automatic recognition, accurate position, auto-punching and filling. It fulfills the requirements to automatic manufacture of tissue micro-array.