Acute Disease ; Adult ; Carbon Tetrachloride Poisoning ; diagnosis ; drug therapy ; Humans ; Male

Objective To report the clinical,electrophysiological and genetic features in a family with Charcot-Marie-Tooth disease type 1D (CMT1 D).Methods The proband,a 53-year-old man who was found with pes cavus when he was 15 years old,presented with weakness in both lower limbs at the age of 37,aggravated and numbness in legs at the age of 50.His daughter was confirmed pes cavus in her teens and weakness in both lower limbs at the age of 18.Electrophysiology and next generation sequencing were performed in the proband.Results Electrophysiological results of the proband showed demyelinating change in motor and sensory nerves.Latency prolongation was found in bilateral waves Ⅲ,V and abnormal differentiation in bilateral waves Ⅰ of brainstem auditory evoked potential,while both interpeak latencics of Ⅲ-Ⅴ were normal.DNA analysis revealed a heterozygous 1141C ＞ T mutation in exon 1 of early growth response 2 (EGR2) gene in both of the proband and his daughter.Conclusions The onset age of Arg381Cys mutation in EGR2 gene could be at juvenile with weakness in both lower limbs.The phenotype of CMT1D is mild and progressive slowly.

Objective To investigate the influence of thiamine deficiency(TD)at early pre- pathological lesion stage on cognitive function and the correlation between cognitive dysfunction and hippocampal neurogenesis.Methods TD mouse model was prepared by feeding a thiamine-depleted diet. Learning and memory functions of TD mice were tested with Y-maze.Hippoeampal neurogenesis was studied with bromodeoxyuridine(BrdU),proliferative cell nuclear antigen(PCNA),and Doublecortin(Dcx) immunohistochemical staining on the 7th(TD7),9th,14th and TD25th day.Results TD9 mice without pathological impairment and cholinergic nerve degeneration needed more times of training(22.3?2.2)in the learning test of Y maze compared with the controls(13.5?3.5).Correspondingly,the numbers of BrdU-positive ceils and the immunoreactivity of Dcx decreased significantly in the TD9 mice(19.8?0.4, 1537.2?50.2 vs 23.9?0.3,2688.9?127.9 pixels/mm~2).Thiamine re-administration reversed the declined hippocampal neurogenesis:the number of BrdU-positive cells was 23.6?1.9 and Dcx immunoreactivity was 2052.3?269.6 pixels/mm~2:the impaired learning ability was simultaneously restored,with the number of total training trial being 16.8?0.5.Conclusion The decreased hippocampal neurogenesis contributes to retarded learning ability at early pre-pathological lesion stage of TD.

To investigate whether accelerated blood clearance (ABC) phenomenon could be induced after repeated injection of mitoxantrone thermosensitive liposomes, LC-MS/MS and enzyme linked immunosorbent assay (ELISA) were used to measure the concentration of mitoxantrone and the anti-polyethylene glycol (PEG) IgM levels in rat plasma, separately. The drug was rapidly cleared away after the second administration. The anti-PEG IgM was detected after the first dose which was neutralized quickly after the second dose. It is proved that repeated administration of mitoxantrone thermosensitive liposomes in rat caused the ABC phenomenon.
Animals ; Antineoplastic Agents ; administration & dosage ; blood ; pharmacokinetics ; Chromatography, High Pressure Liquid ; Immunoglobulin M ; blood ; Liposomes ; administration & dosage ; blood ; pharmacokinetics ; Male ; Metabolic Clearance Rate ; Mitoxantrone ; administration & dosage ; blood ; pharmacokinetics ; Polyethylene Glycols ; administration & dosage ; chemistry ; pharmacokinetics ; Rats ; Rats, Wistar ; Spectrometry, Mass, Electrospray Ionization ; Tandem Mass Spectrometry

Objective To evaluate the effects of a health education and behavior intervention project,and to provide.a scientific basis for brucellosis control.Methods By stratified cluster sampling method,target population from 6 township in Meilisi District were randomly selected to carry out baseline survey.Of these 6 townships 4 were selected as intervention townships,and two as control townships.A health education and behavior intervention program was carried out in intervention townships,and after intervention target population from 6 township were participated in the questionnaire survey.Results Awareness rate of common sense knowledge of farmers in intervention townships was 84.33％ (5237/6210) after intervention which was significantly higher than 53.56％ (2003/3740),before the intervention (x2 =112.49,P ＜ 0.01),and also higher than 54.15 ％ (1787/3300) of the control after the intervention (x2 =101.53,P ＜ 0.01).Average awareness rate(82.13％,2550/3105) of common sense on prevention after intervention was significantly higher than 58.77％(1099/1870) before the intervention (x2 =57.19,P ＜ 0.01).After intervention,average awareness rate of common sense on prevention was significantly higher than that of the average of control townships (70.36％,1161/1650,x2 =25.49,P ＜ 0.01).The awareness rate of treatment before intervention was 36.80％ (116/299),which increased to 76.36％ (436/571)after intervention (x2 =119.38,P ＜ 0.01).After the intervention the awareness rate of intervention townships was significantly higher than that of the controls (33.72％,88/261,x2 =139.69,P ＜ 0.01).In survey of these high-risk behavior in intervention townships,in addition to cattle and sheep which were regularly immunized in the intervention townships(35.91％,121/337),and the control townships(32.01％,97/303,x2 =1.08,P ＞ 0.05) and their difference was not significant,other processing such asapoblema,killing cattle,sheep,cattle to deliver,cutting the wool,regular disinfection,and accuracy of high-risk behavior protection were significantly higher than those of controls[89.83％(106/l18),91.07％(51/56),84.75％(150/177),66.67％(32/48),73.78％(242/328),51.22％(42/82),75.56％(34/45),33.69％(63/187),27.78％(15/54),21.63％(61/282),x2 value were 38.00,5.82,103.84,15.84 and 173.67,all P ＜ 0.05].Conclusion After implementation of health education and behavioral intervention,farmers brucellosis prevention knowledge and awareness of risk behaviors have significantly improved,which have achieved the desired effect.

Idiopathic pulmonary fibrosis (IPF) is characterized by myofibroblast foci in lung parenchyma.Myofibroblasts are thought to originate from epithelial-to-mesenchymal transition (EMT).Wnt1 and lithium chloride (LiCl) induce EMT in alveolar epithelial cells (AECs),but the mechanisms are unclear.AECs were treated with Wnt1 and LiCl,respectively;morphological change and molecular changes of EMT,including E-cadherin,fibronectin,and vimentin,were observed.SB203580 was administrated to test the role of p38 MAPK signaling in EMT.Then AECs were treated with siRNAs targeting p38 MAPK to further test the effects of p38 MAPK,and the role was further confirmed by re-expression of p38 MAPK.At last β-catenin siRNA was used to test the role of β-catenin in the EMT process and relationship of β-catenin and p38 MAPK was concluded.Exposure of AECs to Wnt1 and LiCl resulted in upregulation of vimentin and fibronectin with subsequent downregulation of E-cadherin.Wnt1 and LiCl stimulated the p38 MAPK signaling pathways.Perturbing the p38 MAPK pathway either by SB203580 or through p38 MAPK siRNA blocked EMT and inhibited fibronetin synthesis,which were reversed by transfection of p38 MAPK expression plasmid.β-catenin siRNA attenuated the EMT process and decreased p38 MAPK phosphorylation,indicating that β-catenin is involved in the EMT-related changes through regulation of p38 MAPK phosphorylation.These findings suggest that p38 MAPK participates in the pathogenesis of EMT through Wnt pathway and that p38 MAPK may be a novel target for IPF therapy.

Objective Type 1 diabetes mice model was established to investigate the changes of key enzymes involved in testosterone synthesis in testes of early diabetic mice.Methods Tatolly 20 male C57 mice were randomly divided into two groups:control and diabetic groups,and the diabetes mice were ip administered with a single dose of 150 mg/kg Streptozotocin.Four weeks after confirmation of diabetic model,the serum and testis were collected for further study.The qRT-PCR method was used to measure the expression of LHR and steroidogenesis synthetase StAR,P450scc,3β-HSD6,P450c17a1,and 17β-HSD3 mRNA.ELISA assay was performed to measure the levels of testosterone and luteinizing hormone (LH) in serum,and the enzymatic activities of 3β-HSD1,1P450c17 and 17β-HSD3 in testis tissue.Results Compared to control group,the levels of testosterone and LH of diabetic group declined significantly (P ＜ 0.05) after four weeks.The mRNA levels of LHR,StAR,CYP11a1,Hsd3b6,CYP17a1 and Hsd17b3,and enzymatic activities of 3β-HSD6,P450c17 and 17β-HSD3 were also decreased significantly compared with control group (P ＜ 0.05,0.01 and 0.001).Conclusion Expression of key enzymes of testosterone synthesis in testis of early diabetic mice decreases significantly.

The effects of exogenous phytohormones on hairy root growth and biosynthesis of anthraquinones in the hairy root cultures of Polygonum multiflorum Thunb. were studied. The results showed that the 2,4-D, NAA and 6-BA all have obvious effects on the growth of hairy root cultures and the biosynthesis of anthraquinones. The growth of hairy root and biosynthesis of anthraquinones were strongly restrained by 2,4-D. However, NAA and 6-BA of appropriate concentration were favourable to hairy root growth and anthraquinones production.
2,4-Dichlorophenoxyacetic Acid ; pharmacology ; Anthraquinones ; metabolism ; Benzyl Compounds ; Chromatography, High Pressure Liquid ; Kinetin ; pharmacology ; Plant Growth Regulators ; pharmacology ; Plant Roots ; drug effects ; growth & development ; metabolism ; Polygonum ; drug effects ; metabolism ; Purines

Adenocarcinoma, Follicular ; pathology ; physiopathology ; Female ; Humans ; Kidney Diseases ; pathology ; physiopathology ; Kidney Neoplasms ; physiopathology ; Thyroid Neoplasms ; pathology ; physiopathology ; Young Adult

BACKGROUNDCoronary artery lesions (CALs) are known to be the main complication in children with Kawasaki disease (KD). Instead of intravenous immunoglobulin (IVIG), corticosteroid therapy has been accepted to be used for children with KD who are unresponsive to IVIG. This study aimed to evaluate risk factors for CALs in children with KD.

METHODSWe retrospectively reviewed the clinical records of 2331 children with KD from January 2005 to December 2014. To identify the independent risk factors for CALs, multivariable logistic regression models were constructed using significant variables identified from univariate logistic regression analysis.

RESULTSThe incidence of CALs was 36.0% (840 of 2331), including 625 (26.8%) coronary artery dilations and 215 (9.2%) coronary artery aneurysms (CAAs). Multivariable logistic regression analysis identified that male, incomplete KD, longer fever duration, and C-reactive protein (CRP) >100 mg/L were independent risk factors for coronary artery dilatations. On the other hand, male, incomplete KD, longer fever duration, prolonged days of illness at the initial treatment, corticosteroid therapy, sodium ≤133 mmol/L, and albumin <35 g/L were the independent risk factors for CAAs. In addition, corticosteroid therapy, prolonged days of illness at the initial treatment, and albumin <35 g/L were the independent risk factors for giant CAAs.

CONCLUSIONSCALs might be associated with male sex, incomplete KD, longer fever duration, prolonged days of illness at the initial treatment, albumin <35 g/L, sodium ≤133 mmol/L, CRP >100 mg/L, and corticosteroid therapy. Corticosteroid therapy was an independent risk factor for CAAs and giant CAAs. Thus, corticosteroids should be used with caution in the treatment of KD with the risk for CALs.

Adolescent ; Adrenal Cortex Hormones ; adverse effects ; Child, Preschool ; Coronary Aneurysm ; chemically induced ; Female ; Humans ; Infant ; Logistic Models ; Male ; Mucocutaneous Lymph Node Syndrome ; drug therapy ; Retrospective Studies