Objective To study the clinical and pathological features of periodic paralysis.Methods The clinical and pathological data of 9 patients with periodic paralysis were analyzed.Results The clinical manifestation of 9 patients were consistent with the general manifestation of periodic paralysis.The typical tubular aggregates in many fibers were observed in 4 cases of 7 patients with skeletal muscle biopsy.A few degenerating fibers were observed,while necrotic fibers and regenerating fibers were not found.Electromicroscope showed Honeycomb appearance in tubular aggregates of cross section,which was filled with glycogen granule.Conclusions According to the clinical manifestation and the laboratory examination,periodic paralysis can be clinical diagnosis.The tubular aggregates in many fibers are important pathological characteristic of periodic paralysis.In electromicroscope analysis,tubular aggregates may be compose of transverse tubular system or sarcoplasmic reticulum expansion.

In order to evaluate the ability of human standing balance scientifically, we in this study proposed a new evaluation method based on the chaos nonlinear analysis theory. In this method, a sinusoidal acceleration stimulus in forward/backward direction was forced under the subjects' feet, which was supplied by a motion platform. In addition, three acceleration sensors, which were fixed to the shoulder, hip and knee of each subject, were applied to capture the balance adjustment dynamic data. Through reconstructing the system phase space, we calculated the largest Lyapunov exponent (LLE) of the dynamic data of subjects' different segments, then used the sum of the squares of the difference between each LLE (SSDLLE) as the balance capabilities evaluation index. Finally, 20 subjects' indexes were calculated, and compared with evaluation results of existing methods. The results showed that the SSDLLE were more in line with the subjects' performance during the experiment, and it could measure the body's balance ability to some extent. Moreover, the results also illustrated that balance level was determined by the coordinate ability of various joints, and there might be more balance control strategy in the process of maintaining balance.
Acceleration ; Biomechanical Phenomena ; Humans ; Models, Biological ; Motion ; Nonlinear Dynamics ; Postural Balance ; physiology

Objective To explore the potential relationship between the mast cells (MCs) in renal interstitium and the renal interstitial fibrosis in lupus nephritis (LN). Methods Renal biopsy specimens from patients with types Ⅲ,Ⅳand Vof LN (n=10, respectively), and with minimal change diseases (n=11,as control) were evaluated. Immunohistochemistry staining and immunofluorescence double-staining were used to detect the amount of MCs, the expression of proteinase-activated receptor-2 (PAR-2), transforming growth factor-?1 (TGF-?1) and collagen type I (Col I ) in the renal tissues. Results The amount of MCs in renal interstitium, the positive areas of PAR-2 and TGF-?1 in the renal tubular epithelial cells (RTECs), the amount of PAR-2-positive cells and TGF-?1-positive cells in renal interstitium, and the positive areas of Col I in the renal inter stitium were all higher in three LN groups compared with those in control. Furthermore, among the three LN groups, the above-mentioned parameters were the highest in type Ⅳ and second in type Ⅲ.There were significant positive correlations between the amount of MCs in renal interstitium and the positive areas of PAR-2, TGF-?1 in RTECs as well as the positive areas of Col I in renal interstitium (r=0.513, 0.508, 0.611, respectively, P

Objective To explore the potential relationship between tryplase-positive mast cells (MCs) infiltration and renal interstitial fibrosis in acute interstitial nephritis (AIN) and chronic interstitial nephritis (CIN). Methods Renal biopsy specimens from patients with AIN (n=11) and CIN (n=16) were studied and 11 patients in minimal change diseases (MM)were as controls. Histochemistry and immunohistochemistry staining assay were applied to delect the expression of tryptase, proteinase-activated receptor-2 (PAR-2), TGF-?1 and collagen type I (Col I )in the renal tissues. Immunofluo-rescence double-staining assay was used to assess the relationship among MCs, PAR-2-positive cells, and TGF-?1-positive cells in the renal interstitium respectively. Results MCs in AIN and CIN were significantly increased compared with those in controls and were mainly scattered in the fibrotic areas of renal interstitium. The relative immunostaining areas for PAR-2, TGF-?1 in the renal tubular epithelial cells (RTECs) and Col I were significantly larger in AIN and CIN than those in controls respectively (P

Objective To analyze the clinical and laboratory phenotypes of Duchenne muscular dystrophy(DMD)in a retrospective study of 96 cases and to evaluate therapeutic efficacy of glucocorticosteroid.Methods The clinical phenotype,laboratory examinations resuhs and the records in the follow-up in 96 patients with DMD were collected.The level of serum creatine kinase(CK)and motor ability before and after glucocorticosteroid therapy were analyzed by statistical analysis.Their myocardium impairments and intelligence conditions were also assessed.Results(1)The level of serum CK (mmol/L)had three peaks at the age of ≤ 3 years old(16 547.9 ±770.9),5 years old(14 371.9 ± 696.7)and 8 years old(13 089.8 ± 877.6).The CK level significantly decreased after dexamethasone (5-10 mg,iv)treatment for 10-15 days,but increased again after prednisone acetate(0.50-0.75 mg · kg-1 · d-1,oral)administration for one month(F =6.758,P =0.003).(2)The motor ability improved in 51 DMD cases with long-term oral admission of prednisone,including 24 cases receiving repeated dexamethasone,iv.(3)The myocardial perfusion imaging in 37 DMD cases showed remarkable uneven “spotted like” radionuclide distribution in ventricle.(4)The intelligence quotients in 24 DMD were lower than normal population.Conclusions There are high CK hyperlipidemia and myocardial damage in the sub-clinical stage of DMD and myocardium impairment is positively correlated with age.Glucocorticosteroid therapy has an important effect on the protection of motor and cardiac functions,with recommendation of using in early stage of disease.

Objective To explore the Hobiletin improving cognitive function and the possible mechanism to protect the hippocampal neurons.Methods Eight of Wistar rats,SPF,12 weeks of age,male,were set to the normal group;Eight of GK male rats,SPF,12 weeks of age,only for diabetes group;Both of the two groups were given the normal feed.6 of GK male rats,12 weeks of age,to feed Hobiletin of orange peel 10 mg/kg and lavage for 8 weeks,were set to the NOB group.After 22 weeks the rats were tested by Morris water maze behavior experiment for test the ability of learning and memory;the expression of Nrf-2 was detected by immunohistochemistry and Western Blot.Results Compared with the normal group,the incubation period of diabetes group was longer (P＜0.05),and theescape latency of NOB group was shorter than that of diabetes group (P＜0.05).Compared with the normal group,the residence time in the target quadrant was shorter in the diabetes group (P＜0.05),and prolonged in the NOB group than in the diabetes model group (P＜0.05).The expression of Nrf-2 in diabetes group was significantly higher than that in diabetes group (P＜0.05).The expression of NH-2 in NOB group was significantly higher than that in diabetes group (P＜0.05).Conclusion NOB can increase the expression of Nrf-2 in hippocampus and improve the cognitive function of diabetic rats,which may be related to the enhancement of Nrf-2 activity and the activation of anti-oxidative stress pathway.

Objective To analyze retrospectively the clinical manifestations,features of the biopsy of skeletal muscle with histochemistry and immunohistochemistry staining of 40 patients with dysferlinopathy and investigate its clinical,pathological diagnostic value.Methods The clinical data,features of the biopsy of skeletal muscle with histochemistry,immunohistochemistry staining of 40 patients with dysferlinopathy were analyzed.Results Chronic progressive weakness and wasting were the general clinical manifestations.In our study,it was divided into three phenotypes according to the involved muscles of dysferlinopathy:27 cases with proximal muscle,12 cases with the gastrocenemius,1 case with the tibialis anterior muscle.The serum creatine kinase levels all had a rise in different degree (134-19 795 U/L).All the patients showed myogenic lesions in electrophysiologic study.12 patients underwent skeletal muscle MRI.Proximal muscle was involved in 4 cases ; gastrocnemius muscle was mainly involved in 7 cases ; and anterior tibial muscle initially was involved in 1 case.All 40 cases showed active muscle fiber degeneration,necrosis and regeneration on muscle pathology.Connective tissues were proliferated and inflammatory cells infiltrated in endomysium,perimysium and perivascular sites of 16 patients.Immunohistochemical staining with anti-dysferlin monoclonal antibody identified the deficiency of dvsferlin in the sarcolemma of 30 cases with dysferlinopathy,and dysferlin was severely reduced in 10 cases.Conclusion Progressive weakness and wasting of skeletal muscle are the clinical manifestations of dysferlinopathy.The early involved muscles determine the clinical phenotype of dysferlinopathy.High serum creatine kinase levels show that dysferlinopathy is a membrane protein null disease.Muscle MRI of lower limbs may reflect the involved muscles,which is essential for clinical phenotypes and selecting muscle biopsy.The pathological characters of dysferlinopathy are changes of muscular dystrophy.Inflammatory cellular infiltration is relatively common in biopsied muscles of many dysferlinopathy patients,and dysferlinopathy needs to be differentiated from inflammatory myopathies.The deficiency or severely decreased dysferlin on the sarcolemma in immunohistochemical staining with anti-dysferlin monoclonal antibody is an important information for diagnosing dysferlinoapthy.

Objective To estimate the consistency between the diagnostic criteria for dermatomyositis (DM) and polymyositis (PM) developed by Bohan and Peter criteria (B/P criteria) and ENMC criteria.Methods The clinical,laboratory and pathological data from 86 patients who were initially diagnosed with idiopathic inflammatory myopathy were collected retrospectively.These patients were diagnosed according to B/P criteria and ENMC criteria,and the similarities and differences between these two criteria were compared.The data were analyzed with Mann Whitney U test and Kappa test by SPSS 13.0 software.Results Thirtyseven DM and 49 PM were diagnosed using B/P criteria.Forty-six DM and 14 PM were diagnosed using ENMC criteria,and 1 was diagnosed as eosinophilic myositis,9 were diagnosed as sporadic inclusion body myositis (sIBM),11 cases were diagnosed as limb-girdle muscular dystrophy type 2B,and the diagnosis of 5 patients could not be clarified.Agreement for DM between these two sets of criteria was very good by Kappa test (κ=0.79),but the corresponding between the two tests for PM was poor (κ=0.26).Conclusion Our study has demonstrated that B/P criteria may cause over-diagnosis and misdiagnosing for PM.ENMC criteria involves immunohistochemical pathology,stratified clinical and pathological exclusion criteria.The diagnostic accuracy of ENMC criteria is much improved.

Objective: To systematically evaluate the incidence and influencing factors of depression in family caregivers of Alzheimer's disease (AD) patients, so as to provide the basis for the prevention and treatment of depression among the family caregivers of AD patients. Methods: Publications pertaining to depression in family caregivers of AD patients were retrieved from CNKI, Wanfang Data, PubMed and other databases from the time of their establishment to June 15, 2023. The evaluation criteria recommended by the Agency for Healthcare Research and Quality (AHRQ) and the Newcastle-Ottawa Scale were used to assess the quality of cross-sectional and cohort studies, respectively. Stata 16.0 and Revman 5.4 softwares were used to conduct a meta-analysis on the incidence and influencing factors of depression in family caregivers of AD patients. Sensitivity analysis and publication bias assessment were also performed on the results. Results: A total of 2 324 articles were retrieved, and ultimately 14 articles were included, with a total sample size of 8 313 individuals. There were 6 high-quality articles and 8 moderate-quality articles. Meta-analysis showed that the incidence of depression in family caregivers of AD patients was 37.5% (95%CI: 30.2%-45.1%). Factors associated with depression included patients' high degree of dementia (OR=1.718, 95%CI: 1.059-2.789), patients' low scores on Activities of Daily Living Scale (OR=1.344, 95%CI: 1.059-1.706), patients' psychobehavioral abnormalities (OR=1.248, 95%CI: 1.155-1.348), long duration of caregiving (OR=1.998, 95%CI: 1.637-2.437), less involvement of other family members in caregiving (OR=1.597, 95%CI: 1.237-2.061), low educational level (OR=1.191, 95%CI: 1.044-1.359), poor caregiving skills (OR=3.060, 95%CI: 2.257-4.149), poor self-rated health (OR=2.536, 95%CI: 1.114-5.771) and social support (OR=0.424, 95%CI: 0.232-0.774). The results of depression incidence demonstrated good stability with no significant publication bias. However, publication bias was observed in the influencing factors for depression, which were patients' high degree of dementia and patients' low scores on Activities of Daily Living Scale. Conclusions The incidence of depression in family caregivers of AD patients ranges from 30.2% to 45.1%. It is primarily influenced by the severity of patients' symptoms and ability to perform daily activities, and caregivers' educational level, caregiving skills, health status, caregiving duration and social support.

OBJECTIVEThe aim of this study was to explore the influencing factors of prognosis for recurrent and metastatic esophageal carcinoma, and to provide reference for clinical treatment for these patients.

METHODSThe clinicopathological and follow-up data of 247 patients with recurrent and metastatic esophageal squamous cell carcinoma after radical resection were retrospectively reviewed, combined with analysis of prognostic factors in these patients. Kaplan-Meier method was used to analyze the survival, difference between groups was compared by Log rank test, and Cox model was used for multivariate analysis.

RESULTSAmong the 247 recurrent and metastatic patients, locoregional recurrence was in 139 patients (56.3%), distant metastasis in 60 patients (24.3%), and combined recurrence in 48 patients (19.4%). The survival time was 1 to 42 months in the 247 patients, and the median survival time was 10 months. The 1-, 3- and 5-year survival rate after recurrence and metastasis was 26.4%, 6.3% and 2.4%, respectively. Univariate analysis indicated that regional lymph node metastasis of the primary tumor, distant lymph node metastasis, clinical staging, interval between operation and recurrence, recurrent and metastatic patterns, and treatment methods after recurrence and metastasis were influencing factors of prognosis (all P<0.05). Cox multivariate analysis indicated that clinical staging of the primary tumor, interval between operation and recurrence, recurrent and metastatic patterns, and treatment methods after recurrence and metastasis were independent factors influencing prognosis (all P<0.05).

CONCLUSIONSThe prognosis of patients with recurrent and metastatic esophageal carcinoma is poor, and it is affected by many factors. Comprehensive treatment is effective in prolonging the survival time of the patients.

Carcinoma ; Carcinoma, Squamous Cell ; diagnosis ; Esophageal Neoplasms ; diagnosis ; Esophagectomy ; Humans ; Lymphatic Metastasis ; Neoplasm Recurrence, Local ; diagnosis ; Neoplasms, Second Primary ; Prognosis ; Retrospective Studies ; Survival Rate