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Objective:To compare the differences in sleep structure between healthy children and children with cerebral palsy (CP) using polysomnography (PSG).Methods:Fifty-six children aged 1-15 hospitalized for cerebral palsy formed the experimental group, while 30 healthy children served as controls. Both groups were given 24-hour PSG, and their sleep structures were compared and analyzed.Results:The incidence of sleep disorders in the children with cerebral palsy (55.4%) was significantly higher than among the healthy children (20.0%). The average sleep latency was significantly higher than among the healthy children, while the duration and the percentage of the rapid eye movement (REM) stage were significantly lower than among the healthy children. Total sleep time [(458.47±95.62)min], sleep efficiency [(74.26±13.63)%], duration of REM [(68.90±42.70)min] and REM percentage [(13.87±7.12)%] were all significantly lower for the children with severe cerebral palsy than for those with mild or moderate disorder. Their time to wake up after falling asleep was significantly longer. Moreover, the duration of REM and the REM percentage of children with dyskinetic cerebral palsy were significantly lower than for those with spastic cerebral palsy.Conclusions:The incidence of sleep disorders among children with cerebral palsy is higher than among healthy children. They have more difficulty in falling asleep and have a shorter REM stage. Children with severe cerebral palsy and involuntary cerebral palsy have more sleep problems.
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Objective:To investigate the clinical features and treatment status of immune-related myositis (IRM) caused by immune checkpoint inhibitors (ICIs) in order to improve the diagnosis and treatment rate of the disease.Methods:Two cases of IRM combined with the diagnosis and treatment were described and the literature about IRM in the past 10 years was reviewed, and the clinical data of 59 patients were analyzed.Results:IRM was more common in males, with a total of 47 (79.7%). IRM usually occurred after 45 days of medication or after two doses. The clinical manifestations were mainly myalgia and muscle weakness, which were more common in the limbs. The initial symptoms were ptosis and diplopia. Fifty patients (84.7%) had serum creatine kinase (CK) levels higher than twice the upper limit of normal (UNL). In immunological examinations, 18 patients were found to be positive for anti-rhabdoid muscle antibody (AsM-Ab), while most of the myositisspecific antibodies (MSAs) and myositis-associated antibodies (MAAs) were negative. Thirty-four patients (75.6%) had abnormal EMG, and most patients showed myogenic injury. Muscle magnetic resonance imaging (MRI) showed muscle edema and inflammation in 8 patients. Muscle biopsies from 18 (45.0%) patients showed varying degrees of necrotic myofibers. Fifty-seven patients (96.6%) discontinued ICIs after developing IRM, 54(91.5%) received cortico-steroids, and 20(33.9%) received other treatments including intravenous immuno-globulin (IVIG), plasma exchange.Conclusion:IRM can occur in the early stage of ICIs treatment. Electro-myography, muscle MRI and muscle biopsy in suspicious cases can improve the diagnosis rate of the disease. Early use of corticoteroid, IVIG and other immunotherapy can effectively alleviate the disease.
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Objective:To document the clinical features of children with cerebral palsy (CP) using magnetic resonance imaging (MRI).Methods:The gross motor functioning of 325 children diagnosed as having CP was graded using the gross motor function classification system (GMFCS). The GMFCS grades were correlated with MRI results in univariate and multivariate logistic regression analyses. The significance of any relationship between the MRI results and co-morbidities was tested using chi-squared tests.Results:Cerebral dysplasia, cerebroventricular enlargement, periventricular leukomalacia (PVL), abnormal signals in the thalami, and morphological changes after hypoxic ischemic encephalopathy were all found to be significantly correlated with GMFCS grading. Moreover, the chi-squared tests indicated that PVL children, children with thinning of the corpus callosum and/or abnormal signals in the thalami were significantly more likely to have visual, auditory or speech impairment complications and/or mental retardation.Conclusions:The findings from MRI correlate well with types of CP, GMFCS grades and co-morbidities among CP children. MRI can be an effective tool for early diagnosis and prognosis of CP in children, indicating needs for clinical rehabilitation.
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Objective:To explore the clinical application and long-term safety of hydroxychloroquine sulfate (HCQ) in the treatment of rheumatic diseases.Methods:A multi-center cross-sectional study was conducted between August 2017 and August 2018 in a random sample of eleven medical institutions of rheumatology and immunology in China. Patients who took HCQ for more than 3 months were enrolled into this study. The cumulative dose and long-term side effects of HCQ were recorded. The changes of laboratory indexes before and after treatment with HCQ were analyzed. Categorical variables were presented with counts and proportions, and evaluated by Chi-square test. Continuous parametric data were presented as Mean±standard deviation, and evaluated by Student's t test or Mann-Whitney U test. P-values less than 0.05 were considered statistically significant. Results:A total of 886 patients with rheumatic diseases were enrolled into this study, including 505 cases with systemic lupus erythematosus (57.0%), 210 cases with rheumatoid arthritis (23.7%), 80 cases with Sj?gren's syndrome (9.0%), 57 cases with undifferentiated connective tissue disease (6.4%), 12 cases of systemic vasculitis (1.4%), 10 cases of mixed connective tissue disease (1.1%), 7 cases of myositis (0.8%) and 5 cases with systemic sclerosis (0.6%). The most common long-term side effects of HCQ was skin or mucous lesions (12.4%) and vision problems (8.0%). Other adverse reactions included problems of digestive system (3.0%), nervous system (2.1%), musculoskeletal system (1.1%) and cardiovascular system (0.9%). 140 cases (15.8%) had stopped taking HCQ during the treatment. More than half of them decided to stop taking medicine by themselves. Fifty-four patients (6.1%) stopped using HCQ due to side effects while 24 of them took it again, and another 12 patients (1.4%) stopped the drug due to remission of illness. Patients were divided into three groups according to the cumulative dose of HCQ: less than 500 g, 500-1 000 g and more than 1 000 g respectively. There was significant difference in the incidence of long-term side effects among the three groups ( χ2=6.382, P=0.041). The last group (more than 1 000 g) suffered the highest incidence of long-term adverse reactions (37.1%). No severe adverse drug reactions were observed in this study. Conclusion:Hydroxychloroquine is widely used in the treatment of rheumatic diseases. The incidence of long-term side effects is 20.4%, is 6.1% lead to drug withdrawal, which are especially related to the cumulative doses. It should be adjusted properly according to the clinical application.
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Objective To evaluate the value and the expression of Axl receptor tyrosine kinase (AxlTK) in renal pathology of lupus nephritis (LN) patients. Methods The expression of AxlTK were detected in 29 LN and 10 primary nephritic syndrome (NS) patients using immunohistochemistry in renal tissue. The correlations between the levels of AxlTK on glomeruli and clinical manifestations, laboratory parameters, disease activity, and renal pathology in LN patients were investigated. T test and single factor analysis of variance were used tp compare between groups, and Pearson or Spearman test was used for correlation analysis. Results AxlTK was extensively expressed on surface of mesangial cells, endothelial cells in glomeruli. There was more expression of AxlTK on mesangial cells in LN patients [(7.0 ±2.2)×10-2] than that in primary NS patients [(3.1 ±1.2) ×10-2] (t=5.382, P0.05). Conclusion The expression of Axl TK in glomerular is significantly increased in glomeruli in LN patients. The expression level of AxlTK on glomeruli is correlated with the occurrence of oral ulcers and AnuA. The level of AxlTK expression is related to pathological categories of kidney in LN patients. The results of this study suggest that AxlTK might participate in the pathogenesis of LN.
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Objective To explore the status of glucocorticoid application in patients with systemic lupus erythematosus (SLE) in China.Methods Epidemiological survey was used.The SLE patients who met the 1997 classification criteria of American College of Rheumatology were enrolled.The usage of glucocorticoid and related adverse reactions were recorded and analyzed.Results A total of 400 SLE patients were enrolled,including 35 men and 365 women.The average age was (37.4 ± 14.0) years old,and the average duration of disease was (6.7 ± 5.8) years.There were 310 patients using glucocorticoid as maintenance.Sixty-one percent (n =244) patients started using medium dose (prednisone 30-＜ 60 mg/d) as the initial treatment of glucocorticoid,which lasted for (37 ± 11) days.The time of drug duration in patients with low dose prednisone (7.5-＜30 mg/d)and high dose (60-100 mg/d) was(92 ± 20)and (17 ± 3) days respectively (P ＜ 0.05 between 3 groups).However,patients receiving different initial dosage were of no discrepancy in the maintenance therapy.During maintenance,even though 51.0％ (n =158) patients were on prednisone 2.5-5 mg/d,the duration of drug use in ＞ 5-10 mg/d groupwas longer [(29.9 ± 3.3) months].Patients with involvement of internal organs had a higher tendency to use 60-100 mg/d prednisone or pulse-dose therapy in the initial treatment,nevertheless these two groups had no difference of maintenance dosage.Among all 400 patients,62 patients withdrew glucocorticoid,including 17 patients with disease remission (4.3％),44 by self-withdrawal and one with adverse reaction.Conclusion In China,the medium dosage of glucocorticoid is the most common initial treatment in patients with SLE.Prednisone 2.5-5 mg/d was the most common choicefor maintenance therapy.Currently,the proportion of glucocorticoid withdrawal remains low in SLE patients achieving remission.
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Abstrac:Aim To study the effect of hydroxysafflor yellow A ( HYSA ) on the proliferation of vascular smooth muscle cells ( VSMCs) and the related molecu-lar mechanism. Methods The inhibitory effects of hydroxysafflor yellow A on VSMC proliferation was de-tected using cell culture, MTT assay, Western blot and immunohistochemical staining. Results The results showed that HYSA inhibited cell proliferation induced by PDGF in a dose-dependent (5,10,20,40 μmol· L-1 ) manner, reduced proliferating cell nuclear anti-gen ( PCNA ) expression and blocked PDGFR-MEK-ERK1/2 signaling pathway activated by PDGF in VSMCs. Conclusion HYSA inhibits VSMCs prolifer-ation via reducing the expression of PCNA and blocking signal transduction of MEK-ERK1/2 in VSMCs.
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Objective To explore the factors that could affect plasma level of D-dimmer test in acute aortic syn-drome. Methods Blood samples (2 mL) from acute aortic syndrome patients (n=76) obtained immediately after admission to detect D-dimmer using ELISA. Blood routine test and biochemical indicators tests including creatinine were also performed. White blood cell (WBC), serum value of creatinine, aortic contrast-enhanced CT, incidence of Shock and death were all re-corded. The receiver-operating characteristic curve (ROC) was established to assess the potency of D-dimmer to predict hospital mortality. Results According to ROC analysis, the optimal cut-off value of D-dimmer to predict hospital mortality was >2 988.6 μg/L (FEU), with 86.7% sensitivity and 70.5% specificity. The patients were divided into group A (D-dim-mer<2 988.6μg/L FEU, n=45) and group B (D-dimmer≥2 988.6μg/L FEU,n=31). Onset timing was longer in group A than that in group B(P<0.01). Involvement of ascending aorta was less common in group A than in group B(P<0.05). Aortic intramural hematoma was less common in group A than in group B(P<0.05). Logistic analysis demonstrated that short time of onset, involvement of ascending aorta, non-aortic intramural hematoma were all independent factors of higher D-dimmer (≥2 988.6μg/L FEU). Conclusion Patients with long time of onset, without involvement of ascending aorta, with intramural hematoma are liable to have lower values of plasma D-dimmer.
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ABSTRACT:Recently ,prion‐like transmission has been found in various amyloidosis .AApoAII amyloid fibrils in mouse senile amyloidosis have exhibited transmissibility .AApoAII amyloid fibrils ,which were excreted from mice and contained in fe‐ces or milk ,cause mouse senile amyloidosis .However ,transmissibility of AApoAII amyloid fibrils through other pathways has not yet been established .In this study ,we injected AApoAII amyloid fibrils into R1 .P1‐A poa2c mice to induce AApoAII sys‐temic amyloidosis .Two months later ,AApoAII amyloid fibrils ,which deposited in the skeletal muscles of amyloid‐affected mice ,were used to induce AApoAII systemic amyloidosis .Mouse senile amyloidosis which deposited in skeletal muscles could induce secondary transmission of AApoAII amyloidosis .The evidence of transmission through skeletal muscles in non‐prion systemic amyloidosis is found in our study .This pathway of transmission provides new insight into the potential for food‐borne pathogenesis and etiology of systemic amyloidosis .
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Objective To investigate the expression of tissue factor and explore its clinical significances in pulmonary artery after acute pulmonary thromboembolism.Methods Thirty-four Japanese white rabbits (Level Ⅱ animals) were randomly (random number) assigned into four groups:group A (specimen of pulmonary artery was taken 3 hours after pulmonary embolism,n =8),group B (specimen of pulmonary artery was taken 8 hours after pulmonary embolism,n =8),group C (specimen of pulmonary artery was taken 24 hours after pulmonary embolism,n =8) and control group (pseudo-operations were carried out without injecting autologous blood clots,n =10).The animal model of pulmonary thromboembolism was established by injecting autologous blood clots into jugular vein through a 5F catheter and confirmed by digital subtraction angiography.The mRNA expression of TF in different parts of pulmonary artery was assayed by RT-PCR.The q test was utilized if there was a significant difference in a given continuous variable among three groups analyzed by ANOVA.Results The TF expression in the specimen adjacentto emboli was stable at 3 h,8 h or 24 hours after embolism.The mRNA expression of TF at 3 h and 8 h after embolism was lower in specimen taken from distal-end of morbid pulmonary artery than those adjacent to emboli.While at 24 hours after embolism,there were similar mRNA expressions in specimen either adjacent or distal to emboli.Conclusions The high expression of tissue factor in pulmonary artery tissue adjacent to emboli could lead to locally increased coagulation activity,indicating the necessity of initiating anti-coagulation treatment as soon as possible after acute pulmonary embolism.
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Objective To investigate the effects of Matrine (Mat) on the proliferation and cell cycle of the peripheral blood lymphocytes of patients with rheumatoid arthritis.Furthermore,its mechanism of treatment for rheumatoid arthritis (RA) is explored by comparing with methotrexate (MTX).Methods MTr method was used to measure the light-absorption value of the cells treated with different doses of Mat and MTX at different time,and the inhibition rate(IR% ) was calculated.Flow cytometry was used to measure cell cycle of the cells treated with different doses of Mat and MTX for 48 h.Results ① Both MTX and Mat could inhibit the proliferation of PBL in patients with RA and the effect was dose-dependent.The IR%was increased (P＜0.05).IR% at 48 h was higher than IR% at 24 h (P＜0.01).The two medications had no significant difference (P＞0.05).② Both MTX and Mat could change the cell cycle and acted on G1/S point.They both increased the number of cells in G0/G1 phase and decreased cells in S and G2/M phase (P＜0.05).The two medications had no significant difference (P＞0.05).Conclusion Mat inhibits the proliferation and proliferation-dependent processes of PBL in patients with RA.Its effect is comparable to MTX.Mat may have the potential in the trea-tment of RA.
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Objective To better understand the age related changes of cardiac structure and function and their relationship with gender, body weight and blood pressure. Methods M mode, 2 dimensional, and Doppler echocardiographic studies were performed on 306 healthy intellectuals, including 165 males and 141 females, ranging in age from 30 to 85 years. Results Parameters in both male and female including the ratio of peak E wave to peak A wave velocity(E/A), the ratio of the right ventricular peak E wave to peak A wave velocity(E/Ar), the amplitude of aortic wall (Aao) and the angle between septum and the root of aorta (?) were all decreased with the aging significantly( P