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Objective:To analyze the diagnosis and treatment process, treatment methods and clinical efficacy of children with refractory Tourette′s syndrome (TS), thus providing a basis for comprehensive prevention and treatment of refractory TS.Methods:A total of 90 children with refractory TS treated in the Child Mental Health Research Center of Nanjing Brain Hospital Affiliated to Nanjing Medical University from May 2012 to July 2019 were recruited.Their baseline characteristics, diagnosis of comorbidities before and after hospitalization, combined non-drug therapy during hospitalization, the drug types used before and after admission, the dosages of main anti-tic drugs used before admission and at discharge, and the treatment outcomes of comorbidities after admission were retrospectively analyzed.The Yale global tic severity scales (YGTSS) scores and the reduction rate were used to assess the severity of tic disorder and therapeutic effect, and the clinical global impression-efficacy index (CGI-EI) scores were graded for assessing the final therapeutic efficacy.Results:Among the 90 children with refractory TS, 82 children were males and 8 children were females.There was a significant difference in the YGTSS scores at admission and discharge (25.04±12.77 vs.67.64±12.46) ( t=27.55, P<0.05). The proportion of all recruited children diagnosed with comorbidities at discharge was significantly higher than that of admission (85.56% vs.47.78%, χ2=28.90, P<0.05). Combined non-drug therapies after admission mainly included psycho-education and supportive therapy (90 cases), comprehensive behavioral intervention for tics (47 cases) and relaxation therapy (19 cases). The distribution of drugs used before and after admission was the same, and there was no significant difference in the dosages of the five major anti-tic drugs before admission and at discharge (all P>0.05). There were no significant differences in YGTSS scores and reduction rate, and CGI-EI scores of children with or without comorbidities before and after admission (all P>0.05), suggesting the similar therapeutic outcomes. Conclusions:There is no difference in efficacy between outpatient treatment and anti-tic medication of children with refractory TS, and a comprehensive hospitalized intervention can significantly improve their clinical symptoms.Diagnosis and treatment of comorbidities and combined non-drug treatments like comprehensive psychological and behavioral interventions are the key events to improve the prognosis of children with refractory TS.
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Objective:To explore the significance of empathy and executive function indexes in the diagnosis and classification of autism spectrum disorder (ASD) and attention deficit hyperactivity disorder (ADHD).Methods:According to DSM-Ⅴ diagnostic criteria, 33 children with ASD, 30 children with ADHD and 39 typical development (TD) children and adolescents were enrolled as the research subjects.The empathy and executive function characteristics were compared and analyzed in the subjects.Based on empathy and executive function indicators, the three groups of subjects were diagnosed and classified by machine learning method.Results:The total score of Griffith empathy measure parent ratings(GEM-PR)(ASD: (0.67±0.64), ADHD: (1.00±0.79), TD: (0.98±0.73)) and each factor score ( F=3.595-10.363, all P<0.05) and the total score of behavior rating inventory of executive function(BRIEF)(ASD: (62.79±7.45), ADHD: (59.47±8.77), TD: (49.08±7.91)) and each factor score of the three groups were different ( F=6.557-33.205, all P<0.01). Among them, the scores of empathy and executive function in ASD and ADHD groups were generally higher than those in TD children (all P<0.05). When combined with BRIEF scale and GEM scale, the classification accuracy of the three groups reached 62.75%. Conclusion:Both ASD and ADHD children have damages in empathy and executive function.Combining empathy and executive function indexes are more helpful for diagnosis and classification than single index.
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Objective To evaluate the value of indirect immunofluorescence (IIF) on three different substrates including normal human skin (NS),monkey esophagus (ME) and salt-split human skin (SS) in the diagnosis of autoimmune subepidermal bullous diseases.Methods A total of 56 patients with autoimmune subepidermal bullous diseases,including 47 with bullous pemphigoid (BP),6 with epidermolysis bullosa acquisita (EBA),2 with linear IgA bullous dermatosis,and 1 with anti-P200 pemphigoid,were diagnosed in and enrolled from Department of Dermatology,Institute of Dermatology,Chinese Academy of Medical Sciences between January 2015 and December 2016.Seventy patients with pemphigus,15 patients with chronic eczema and 15 healthy adults served as controls.Blood samples collected from these patients and controls were subjected to IIF on three different substrates including NS,ME and SS,and the fluorescence deposition was observed.The sensitivities and specificities of IIF in the diagnosis of different subepidermal bullous diseases were compared.Statistical analysis was carried out with SPSS 13.0 software by using chi-square test for the comparison of enumeration data.Results IIF on NS or ME in the serum of patients with BP showed linear deposition of fluorescent material along the basement membrane zone.IIF on SS showed linear deposition of fluorescent material in the epidermis in the patients with BP,but in the dermis in the patients with EBA and anti-P200 pemphigoid.The sensitivities of IIF on NS,ME or SS in the diagnosis of subepidermal bullous diseases were 73.2％,60.7％ and 94.6％ respectively,and the specificities were 98.0％,100％ and 97.1％ respectively.There were significant differences among the sensitivities (x2 =18.2,P ＜ 0.05),but no significant difference was observed among the specificities (P ＞ 0.05).The diagnostic sensitivity of IIF on SS was significantly higher than that of IIF on NS or ME(x2 =8.0,16.7,both P ＜ 0.05).Conclusion In the diagnosis of autoimmune subepidermal bullous diseases,IIF on SS is superior to IIF on ME or NS.
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A 55-year-old male patient presented with tense bullae on the extremities and trunk.Histological examination revealed subepidermal vesicles and superficial dermal infiltration of eosinophils and lymphocytes.The patient was primarily diagnosed with bullous pemphigoid.However,serum autoantibodies of the patient bound to the dermal side of salt-split skin,and no serum antibodies against BP180,BP230 or type Ⅶ collagen were detected by enzyme-linked immunosorbent assay.Hence,the diagnoses of bullous pemphigoid and epidermolysis bullosa acquisita were excluded.As Western blot and immunoprecipitation analysis showed,there existed antibodies capable of binding to a dermal antigen with a relative molecular mass of 200 000 in the serum of the patient.Based on the above findings,the patient was diagnosed as anti-laminin γ1 (p200) pemphigoid.
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Objective To detect the mRNA and protein expressions of desmoglein 1 (DSG1) and DSG3 in different types of keratinocytes (KCs).Methods Two keratinocyte cell lines HaCaT and A431,as well as primary keratinocytes from human abdomenal skin served as the object of this study.Direct immunofluorescence assay was performed to observe and quantify the expressions of DSG1 and DSG3,and quantitative PCR (qPCR) to determine the mRNA expressions of DSG1 and DSG3,in these cells.Results Both DSG1 and DSG3 were expressed in all the three types of keratinocytes,and the fluorescence intensity of DSG1 and DSG3 in HaCaT cells was higher than that in primary keratinocytes but lower than that in A431 cells.Similarly,all the keratinocytes expressed DSG1 and DSG3 mRNA,with the relative expression levels of DSG1 and DSG3 mRNA in primary keratinocytes being 291.7％ and 237.4％ of those in HaCaT cells respectively (both P ＜ 0.01),and those in A431 cells being 0.1％ and 18.8％ of those in HaCaT cells respectively (both P ＜ 0.05).Conclusions HaCaT cells,A431 cells and primary keratinocytes all can be used for the study of DSG1 and DSG3,of which,A431 cells show the strongest expressions of DSG1 and DSG3,and primary keratinocytes display the highest expressions of DSG1 and DSG3 mRNAs.
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Objective To investigate the relationship between disease severity and enzyme-linked immunosorbent assay (ELISA) index values of anti-desmoglein (Dsg) 1 and-Dsg3 antibodies in 56 patients with pemphigus,and to characterize fluctuations in anti-Dsg1 and-Dsg3 antibodies in different forms of pemphigus.Methods Fifty-six patients with pemphigus (including 36 cases of pemphigus vulgaris (PV) and 20 cases of pemphigus foliaceus (PF)) were enrolled in this study.Blood samples were obtained from these patients before treatment and at the following four time points:when the condition was relieved and the taper of glucocorticoids began,the dose of glucocorticoids was tapered to half of their initial dose,the maintenance treatment started,and when the duration of maintenance treatment reached two years.ELISA was performed to determine the levels of anti-Dsg1 and-Dsg3 antibodies in these serum samples.Spearman correlation analysis was carried out to assess the relationship between disease severity and ELISA index values,and independent sample's t test to compare the levels of anti-Dsg antibodies among these time points.Results The severity of pemphigus was correlated with anti-Dsg antibody ELISA index values.Both anti-Dsg1 and anti-Dsg3 ELISA index values were significantly reduced at the remission of pemphigus compared with those before treatment (all P ＜ 0.01).At the end of the two-year maintenance treatment,10 (50％) patients with PF and 7 (19.4％) patients with PV became negative for anti-Dsg1 ELISA,whereas only 1 (2.7％) patient with PV became negative for anti-Dsg3 ELISA.Conclusions Anti-Dsg antibody ELISA index value is correlated with disease severity in patients with pemphigus,which may serve as a useful marker for assessing disease severity and activity as well as evaluating therapeutic efficacy.
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Objective To explore the expression of neutrophil adhesion molecule CD11b,and plasma level of elastase in patients with anaphylactoid purpura during different clinical phases and their correlation with disease activity.Methods A total of 20 patients with anaphylactoid purpura were recruited into this study,along with 20 normal human controls.Two blood samples were collected from each patients at the first visit(active phase)and after 3～5 weeks of treatment(remission phase).The expression of CD11b was measured by flow cytometry in 12 patients and normal controls,and plasma levels of elastase by ELISA in 20 patients and normal controls.Results Increased CD11b expression and elastase level were noted in patients in active phase compared with those in patients in remission phase(3367.25±434.57 vs 2569.33±411.06.13.98±2.05 vs 4.29±0.80.both P＜0.01).No significant difference was found in CD11b expression between patients in remission phase and normal controls(P＞0.05).while the elastase level was higher in patients in remission phase than in normal controls(4.29±0.80 vs 3.67±0.54.P＜0.05).In active phase of anaphylactoid purpura,the expression of CD11b was positively correlated with the plasma level of elastase(r=0.73,P＜0.01),while no correlation was noticed between them in remission phase(r=0.20,P=0.54).Conclusion Peripheral neutrophils are activated in anaphylactoid purpura,which seems to be more obvious in active phase than in remission phase.
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Objective To investigate the mRNA expression of Fc gamma RⅡA(FcγR ⅡA)on polymorphonuclear neutrophils(PMN)from patients with Beh(c)et's disease(BD).Methods Twenty-five patients with active BD and 20 healthy human controls were included in this study.Blood samples were obtained from all patients with active BD before treatment,from 15 patients with inactive BD after treatment and from healthy controls.PMN were isolated.The FcγR Ⅱ A mRNA expression on PMNs was detected by RT-PCR,and plasma myeloperoxidase (MPO)activity which represented neutrophil activation,was measured spectrophotometricaily.Results The relative expression level of FcγR Ⅱ A on PMN and plasma MPO aetivity were 1.80 1±0.829 and 32±5 U/L.respectively,in patients with active BD,0.820±0.625 and 27±4 U/L,respectively,in those with inactive BD,and 0.745 ±0.931 and 29±5 U/L,respectively,in normal controls;the differences were significant in the two parameters between the patients with active and inactive BD (both P＜0.01),while no statistical difference was observed between inactive patients and normal human controls(P＞0.05).There was a positive eorrelation between the expression level of FcγR Ⅱ A on PMN and plasma MPO activity in patients with BD(r=0.39,P＜0.0 1).Conclusions The mRNA expression of FcγR ⅡA on neutrophils is up-regulated in patients with active BD.It is likely that FcγR Ⅱ A is involved in the activation of neutrophils in BD.
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ObjectiveTo study the clinical effect of using traditional Chinese medicinal therapy and interventional therapy for patients with acute necrotizing pancreatitis, and summarize the nursing points. Methods Divided 76 ANP patients into experimental group and control group randomly, there were 38 cases in these 2 groups respectively. Using traditional Chinese medicinal therapy and interventional therapy in experimental group, using interventional therapy in control group. Compared the clinical values in 2 groups. [WTHZ]Results All the clinical values were significant better in experimental group than those in control group,P
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Objective To investigate the predisposing alleles of HLA-DR genes in pemphigus. Methods Polymerase chain reaction specific sequence primers (PCR-SSP) method was applied to type HLA-DR subregion in the patients with pemphigus vulgaris (PV), pemphigus erythematosus (PE) and matched control subjects of Han nationality from Jiangsu and Anhui provinces. Results The results demonstrated that DR4, DRB1*14 (*1401, *1404,*1405)gene frequencies were significantly higher in both PV(Pc