Thrombotic thrombocytopenic purpura (TTP) is a kind of thrombotic microangiopathies which has low incidence but highly mortality.The outcome of TTP has been improved significantly since last decade due to the application of plasma exchange,however,early relapse occurs in some patients,and for relapsed/refractory patients the prognosis is still poor.This article reviews the recent progress in treatment and relevant influencing factors of TTP.

Objective To explore the influence of levothyroxine(L-T4) in the treatment of primary hypothyroidism on myocardial enzymes and lipids.Methods 78 patients with primary hypothyroidism were selected and treated with L-T4 for 12 weeks.The fasting serum levels promote thyroid hormone(TSH),free T3 (FT3),free T4 (FT4),total cholesterol (TC),triglycerides (TG),low-density lipoprotein cholesterol (LDL-C),aspartate aminotransferase enzyme (AST),creatine kinase (CK) and its isoenzyme (CK-MB),lactate dehydrogenase(LDH) were monitored and analyzed before and after treatment.Results After L-T4 treatment for 12 weeks,compared with before treatment,TSH,TC,TG,LDL-C,CK,CK-MB,LDH,AST were significantly decreased or restored (t =10.5223,26.8498,22.7699,16.2735,22.9329,13.1910,32.0907,22.9597,all P ＜ 0.01).The FT3 was negatively correlated with TG (r =-0.3782),LDL-C(r =-0.3506),AST(r =-0.2843),LDH(r =-0.2974),CK(r =-0.3726) (all P ＜ 0.01)and CK-MB(r =-0.2559) (P ＜ 0.05).FT4 was negatively correlated with TC (r =-0.2660),TG (r =-0.4661),LDL-C(r=-0.5119),LDH(r=-0.5936),CK(r=-0.4877),CK-MB(r=-0.5463) (all P＜0.01)and AST(r =-0.2328) (P ＜0.05).TSH was positively correlated with TC(r =0.5341),TG(r =0.7567),LDL-C(r =0.8240),AST (r =0.3923),LDH (r =0.8073),CK (r =0.9661),CK-MB (r =0.7336) (all P ＜0.01).TSH had,the best correlationship with CK (r =0.9661).Conclusion L-T4 can significantly improve the thyroid function and reduce the blood lipids,myocardial enzymes levels of patients with primary hypothyroidism.

Blood glucose level responses to different diets were examined in 12 healthy man. The test breakfasts consist of different proportion of protein and carbohydrate. Three kinds of test breakfast were fed respectively for each person. Blood glucose were measured before and 1, 2 and 4 hr after breakfast. The results indicated that high-CHO diet maintained higher level of blood glucose than other diets in 1-2 hr after breakfast. However the blood glucose level began dropping from 2 hr and dropped below fasting level at 4 hr after breakfast. The high-protein diet resulted in decreasing of blood glucose level in 1-2 hr after breakfast. The blood glucose level then increased continuously until 4 hr after breakfast.The results in this experiment showed that adjustment of the proportion of carbohydrate and protein in diets could maintain the blood glucose to a higher and stable level.

Objective To explore the effect of endothelial progenitor cells (EPCs) on the proliferation of co-cultured rat vascular smooth muscle cells(VSMCs). Methods Mononuclear cells were isolated from fresh cord blood by 6% hydroxyethyl starch (HES) and density gradient centrifugation. Isolated mononuclear cells were cultured in EGM-2 medium supplemented with 20% fetal bovine serum (FBS), VEGF,bFGF and other growth factors. Biological features of EPCs were observed at different time points, and EPCs were identified by morphology, fluorescence double-staining and flow cytometry. Indirect immunofluorescence was performed to analyze the expression of α-SM-actin, calponin of VSMCs special antigen. Co-culture system of EPCs and VSMCs was established by transwell permeable support. FBS (20%) was used to stimulate the proliferation of VSMCs. In a VSMCs/EPCs co-culture system, the DNA synthesis ability, total protein level and cell cycle of VSMCs were determined by BrdU marking method,protein quantitation and flow cytometry after co-culture for 6, 12, 24,48 and 72 h. Results After co-culture for 12, 24, 48, and 72 h, the DNA synthesis ability and total protein level of VSMCs significantly decreased compared with the control group(P＜0.05). Flow cytometry showed that the percentage of S phase of VSMCs in VSMCs/EPCs co-cultured group significantly decreased and the percentage of G_1 phase increased markedly compared with the control group(P＜0.05). The maximal inhibitory effect was observed at 48 h. Conclusion Early EPCs could inhibit the proliferation of VSMCs.

Objective To study the changes of neutrophil surface molecule CD64 and inflammatory factor levels in patients with burn infection. Methods 46 cases of patients with burn infection who were treated in the department of burn in our hospital between March 2010 and October 2015 were selected as the observation group. Meanwhile, 46 cases of healthy people who underwent physical examination in our hospital during the same period were included in the control group. The levels of neutrophil surface molecule CD64, C-reactive protein (CRP), white blood cell count (WBC) and inflammatory factor [interleukin IL-6 (IL-6), interleukin (IL-8), tumor necro-sis factor (TNF-α) in the two groups of subjects were determined by flow cytometry and were compared. Results In the observation group, the levels of CD64, CRP, WBC and inflammatory factors were significantly higher than those in the control group (P < 0.05). Conclusion The levels of CD64, CRP, WBC and inflammatory factors in patients with burn infection are significantly higher than those in healthy people , which indicates that neutrophil surface molecule CD64, inflammatory factors and burn infection are closely correlated.

ObjectiveTo observe the effect of VitalStim electrical stimulation on disorder of swallowing function induced by stroke.Methods60 patients with disorder of swallowing function after stroke were randomly divided into the treatment group and control group with 30 cases in each group. The patients in the treatment group were treated by VitalStim electrical stimulation and swallowing exercise and the cases of the control group were treated by swallowing exercise only. All patients were evaluated with the standard of swallowing exercise effect reported by Tengdao-Lang (Japan) before and after treatment.ResultsAfter treatment, the total recovery rate of the treatment group was 93.3%, that of the control group was 66.7%, there was a significant difference between two groups ( P<0.05).ConclusionThe effect of VitalStim electrical stimulation combined with swallowing exercise on disorder of swallowing function induced by stroke is better than swallowing exercise only.

Objective To evaluate the preventive effects of Haishe capsules on the conversion of amnestic mild cognitive impairment (aMCI) to Alzheimer's disease(AD).Methods Patients (n=120) with aMCI from our department were recruited and randomly divided into the treatment group and the control group (n=60 in each group).The treatment group was given 0.9 gram of Haishe capsules three times a day while the control group received no drug treatment.Data on the conversion ratio,memory and cognitive function were comparedbetween the groups in a 24-months follow-up.Results By the end of the study,12 patients in the treatment group and 15 in the control group dropped out.Valid data for 93 patients were available for statistical analysis (48 in the treatment group and 45 in the control group).The number of aMCI patients who converted to AD was 6,with a conversion ratio of 12.5％ (6/48);and the number of patients who went through conversion in the control group was 13,with a conversion ratio of 28.8％ (13/45).The difference in conversion between the two groups was statistically significant (x2 =3.83,P＜0.05).After 24 months,MMSE scores for the treatment group (25.52± 1.07) had no significant change compared with baseline levels,while MMSE scores for the control group decreased significantly(24.75--1.49) and were markedly lower than thosefor the treatment group (t=2.85,P＜0.05).MoCA scores for the treatment group (19.39 ±2.01) did not show decline until the end of the study,while those for the control group started to decrease about half way through the study and were lower than scores for the treatment group (t =2.41,P＜0.05).Compared with baseline levels,ADAS-Cogscores for the treatment group (7.62± 1.06) did not increase significantly during the course of the study.ADAS-Cogscores forthe control group were higher at both half way (7.70±0.75) and the end of the study (8.18±0.80)than base line levels,and there was a statistically significant difference in end-of-study ADAS-Cog scores between the two groups(t =-2.6,P＜ 0.05).Conclusions Haishe capsules not only effectively maintain memory and cognitive function,but also delay the conversion from aMCI to AD.

Objective To examine the influencing factors related to clinical efficacy and outcomes of adult primary immune thrombocytopenia (ITP).Methods The clinical data of 161 cases of ITP admitted in the Second Hospital of Shanxi Medical University from June 2013 to March 2017 were collected.The influencing factors related to clinical efficacy and prognosis of adult ITP patients were analyzed.Results There were 60 males and 101 females with a M/F ratio of 0.59∶1 and a median age of 45 years (18-84 years).There were 109 newly diagnosed ITP cases,14 persistent ITP cases and 38 chronic ITP cases in this series.Seventy nine patients received intravenous immunoglobulin g (IVIg) treatment and 82 patients received high dose-dexamethasone treatment.There were no significant differences in clinical efficacy [91.13％(72/79) vs.87.80％(72/82),x2=0.181,P=0.914] and relapse rate [36.11％(26/72) vs.30.55％(22/72),x2=0.189,P=0.910] between IVIg and high dose-dexamethosone groups.Multivariate regression analysis showed that bleeding score ≥2 was the independent risk factor for the lower clinical efficacy (RR=1.415,95％CI:1.008-1.986,P＜0.05).Patients were followed up for a median of 9.0 months (0.5-55.0 months),48 patients relapsed with a relapse rate of 33.33％ and a median relapse time of 1.8 months (0.5-24.0 months).Conclusions IVIg and high dose-dexamethasone have the similar clinical efficacy and relapse rate for treatment of adult ITP.The patients with the bleeding score ≥2 are more likely to get lower remission rate.

Objective: To analyze the clinical characteristics and gene mutations of early-onset epileptic encephalopathy(EOEE) caused by ion channel gene mutation, to identify the etiology, to guide the treatment and to provide the basis for genetic counseling. Methods: The clinical data from 17 children with EOEE caused by ion channel gene mutation and the peripheral blood of the children and their parents were collected from June 2014 to May 2018 at the Department of Neurology, Tianjin Children′s Hospital.Epilepsy gene sequencing was performed by using disease gene targeting second generation sequencing technology.The mutation of pathogenic ion channel gene was found.The confirmed mutations were verified by Sanger sequencing and the source of the mutation was identified. Results: Among 17 case with EOEE, 3 cases had genetic mutation, and 14 cases had denovo mutations.Dravet syndrome was found in 8 cases (47.1%), there were SCN1A gene missense mutation in 5 cases, SCN1A gene nonsense mutation in 3 cases, KCNQ2 gene missense mutation in 1 case (5.9%) and non-specific epileptic encephalopathy in 8 cases (47.1%). SCN2A gene missense mutation, SCN4A gene missense mutation, SCN8A gene missense mutation, KCNQ2 gene missense mutation and KCNH gene missense mutation were found in suspected pathogenic mutations.There were 1 missense mutation out of 5 genes, 1 missense mutation of CACNA1A gene, 1 missense mutation of GRIN2A gene and 1 missense mutation of GRIN3A gene.Seventeen patients were treated with 2 or more antiepileptic drugs, 4 with ketogenic diet and 1 with vitamin B₆ supplementation.During 11 to 96 months of follow-up, seizures were completely controlled in 3 cases (17.6%), decreased in 7 cases (41.2%) by more than 50%, and decreased in 7 cases (41.2%) by less than 50%. Conclusions The clinical phenotypes for children with unexplained EOEE are varied, and gene mutations of ion cha-nnel are most common.Some gene sites are denovo mutations which have not been reported such as missense mutation for 3 case SCN1A gene, 1 case SCN2A gene, 1 case CACNA1A gene, 1 case KCNH5 gene, and nonsense mutation for 2 case SCN1A gene, which have enriched the mutation spectrum of EOEE.

Objective: To analyze the epidemiological characteristics of varicella epidemic situation at schools and kindergartens in Fengxian district of Shanghai during 2012-2018, and to provide evidence for developing control and prevention strategies. Methods: Data of varicella epidemic situation at schools and kindergartens and varicella cases were analyzed by descriptive methods. The rates were compared with chi-square test. Results: A total of 6 727 varicella cases were reported in Fengxian during 2012-2018, for an incidence of 85.34 per 100 000 population. Totally 66 varicella epidemic outbreaks and public health emergencies caused by varicella were reported, which involved 741 varicella cases. Among them, 426 were breakthrough cases (57.49%). The average interval time of breakthrough cases were (6.78±2.79)years, and the incidence was more concentrated in the 3-9 years after vaccination with varicella vaccine. Most breakthrough cases appeared 5 years past vaccination. The peak time of varicella was from April to June and from October to December. Most of cases were in the 5-11 year-old group and 15-17 year-old group. The cases in males was higher than in females. Kindergarten childcare and primary school children were at high risk of varicella. Timeliness of reporting was positively correlated with the duration of the varicella epidemic situation( r =0.52， P <0.05). Conclusion Most varicella epidemic situation in Fengxian district of Shanghai occued in primary schools during autumn and winter, and most of the varicella cases were breakthrough cases. Emergency vaccination of varicella vaccine can effectively control the spread of the epidemic. It is suggested to increase the publicity of the immunization program of 2 doses of varicella vaccine and raise the awareness of varicella vaccination of children’s parents.