ObjectiveTo study the clinical characteristics of the temperature instability in Prader-Willi syndrome (PWS). MethodsThe clinical characteristics of one case of PWS with recurrent fever were retrospectively analyzed.ResultsA 5-month-old boy who had such clinical characteristics as low central muscle tension, sucking weakness, feeding dififculties, cryptorchidism and white skin was diagnosed as PWS by chromosomal microarray analysis and methylation-speciifc PCR. In the course of the disease, the infant presented recurrent fever. The pathogenic bacteria culture and virus antibody detection showed negative, and the treatment of reducing fever, anti-infection and immune support were ineffective.ConclusionsThe cause of the recurrent fever is unknown and central fever is suspected. Whether the temperature instability is the clinical feature of PWS still needs a lot of clinical researches.

AIM: To investigate the angiogenesis effect and protective mechanism of cordycepin on rhesus macaque choroid- retinal endothelial ( RF/ 6A) cell line cultured in high glucose condition.
METHODS: Cultured RF/ 6A cells were divided into normal control group, high glucose group and high glucose (HG) + different concentration cordycepin groups (HG+ 10μ g/ mL group, HG+ 50μ g/ mL group, HG+ 100μ g/mL group). The cell proliferation was assessed using cholecystokinin octapeptide dye after treated for 48h. The cell migration was investigated by a Transwell assay. The tube formation was measured on Matrigel. Furthermore, the impact of cordycepin on high glucose - induced activation of VEGF and VEGF receptor 2 (VEGFR-2) was tested by Western blot analysis.
RESULTS: Compared with normal control group, cell viability markedly increased in high glucose group ( P <0. 05). Cordycepin inhibited RF/ 6A cell proliferation in a dose- dependent fashion: 10. 2 ± 0. 9%, 23. 4 ± 1. 5% and 31. 1±1. 2% inhibition as the concentrations of cordycepin were 10, 50 and 100μ g/ mL, respectively. The difference had statistically significant (P<0. 05) compared with high glucose group. The number of cell migration were 55. 6±2. 70, 87. 4 ± 2. 40, 65. 4 ± 2. 7, 57. 8 ± 2. 38, 62. 4 ± 2. 77 in normal control group, high glucose group and HG+10μ g/mL group, HG + 50μ g/ mL group, HG + 100μ g/ mL group respectively. Migration of RF/ 6A conspicuously increased in high glucose group ( P < 0. 05) compared with normal control group; while showing a gradually reducing trend with the increase of cordycepin dose and a statistically significant difference compared with high glucose group (P<0. 05). The number of tube formation were 18. 7±2. 08, 25. 7 ± 1. 52, 19. 9 ± 1. 57, 16. 3 ± 2. 51, 5. 67 ± 1. 72 in the abovementioned group. Similarly showing a gradually reducing trend with the increase of cordycepin dose and a statistically significant difference with high glucose group (P< 0. 05). In addition, the number of tube formation of RF/ 6A in high glucose group significant increased compared with normal control group ( P < 0. 05 ). The expression of VEGF and VEGFR-2 dramaticlly increased in high glucose group vs normal control group, oppositely gradually decreased with the increase of cordycepin concentrations, and had a statistically significant difference vs high glucose group (P<0. 05).
CONCLUSION: Cordycepin can suppress the proliferation, migration and tubu formation of RF/ 6A in high glucose condition, might via inhibiting expression of VEGF and VEGFR-2.

Animals ; Coxsackievirus Infections ; metabolism ; Enterovirus B, Human ; Female ; Male ; Mice ; Myocarditis ; metabolism ; Myocardium ; metabolism ; Tumor Necrosis Factor-alpha ; metabolism

Objective To investigate the clinical characteristics and mutations of guanosine triphosphate eyclohydrolase (GCH) Ⅰ gene in patients with dopa-responsive dystonia (DRD). Methods Five families with 18 affected family members and 17 patients with sporadic DRD were examined. Patients were allocated into 3 groups according to onset time, either in childhood, or in adolescence or adult. Interview, physical examination, psychologic testings and CT/MR scan were performed. Mutation screening was performed on 26 patients and 1 normal family nember. Thirty-five healthy control subjects were matched for age and sex. Statistical analysis were conducted with the use of SPSS 13.0 computer software. Results(1)Most of patients started with dystonia. The main clinical manifestation was dystonia too. There was no difference among 3 groups.(2) There were significant differences in diurnal fluctuation among 3 groups(15/15,6/6,7/14, χ2=13.125,P=0.001). Diurnal fluctuation negatively correlated with age (r=-0.720, P<0.01).(3)The differences in postural tremor were also found among 3 groups (7/15,5/6,1/14, χ2=8.073, P=0.018). Postural tremor positively correlated with age (r=0.399, P=0.018).(4)There were differences in exaggeration of tendon among three groups(11/15,1/6,4/14, χ2=8.309, P=0.016). Exaggeration of tendon reflexes negatively correlated with age (r=-0.429, P=0.010).(5)The scores of Hamilton Depression Scale and Hamilton Anxiety Scale in patients were higher than those in controls.(6)DNA sequencing revealed a heterozygous A224G missense mutation(Tyr75Cys)located within exon 1 in one autosomal dominant inheritance family. Conclusions The manifestations of DRD varies. The clinical course is closely correlated with age. A missense mutation(A224G)in coding region of the GCH 1 gene probablyleads to the occurrence of DRD.

Abstract?AIM: To analyze the clinical effects of partial rectus muscle transportation procedure for paralytic strabismus due to single rectus muscle paralysis.?METHODS:The conditions of 22 patients (25 eyes) who underwent partial rectus muscle transportation procedure for paralytic strabismus due to single rectus muscle paralysis were retrospectively reviewed. The following data were analyzed:1 ) the angle of deviation of primary position; 2 ) the presence of diploma in the primary position;3) the presence of compensatory head posture;4) the motility of the affected eye.All of the patients attended 6mo postoperative follow-up examinations.?RESULTS: According to the results of examinations before and during operation, different operations were performed:2 eyes were treated with partial rectus muscle transportation, 20 eyes were treated with recession of antagonistic muscle of paralytic rectus muscle combined with partial rectus muscle transportation, 3 eyes were treated with recession of antagonistic muscle, partial rectus muscle transportation and recession of yoke muscle.Twenty patients were orthotropia in the primary position, the diploma and abnormal head posture were eliminated. Two patients with binocular lateral rectus muscles paralysis were in mild undercorrection which were resolved by wearing 8△and 10△prisms respectively. The procedure improved strabismus of 25 eyes from 100.23△ ± 42.61△ preoperatively to 0.82△ ± 2.67△postoperatively ( t=10.797,P<0.001).Ocular movement was improved from -4.52 ±0.51 preoperatively to -2.68 ± 0.63 postoperatively (t=-19.468, P<0.001).? CONCLUSION: Partial rectus muscle transportation procedure for paralytic strabismus due to single rectus muscle paralysis can effectively correct the primary position in paralytic strabismus, eliminate the presence of diploma in primary position and abnormal head posture, and improve the ocular motility, which provides content clinical effects.

ObjectiveTo investigate the therapeutic effect of genciclovir on cytomegalovirus (CMV) infection of children with cerebral palsy (CP).Methods36 CP children with CMV infection were randomly divided into treatment group and control group with 18 cases in each group. Children of treatment group were treated by genciclovir with 5 mg/kg i.v. drip q l2 h for 14 days at inductive phase and 7.5 mg/kg i.v. drip qod for 6 weeks at maintain phase. Cases of control group were treated by acyclovir with 5 mg/kg i.v. drip q 8 h for 14 days at inductive phase and 7.5 mg/kg i.v. drip q l2 h for 6 weeks at maintain phase. CMV-DNA contents in urine of two groups were measured before and after treatment.ResultsCMV-DNA contents in urine of two groups were not different before treatment (P>0.05). For control group, CMV-DNA contents were also not different before and after treatment. For treatment group, CMV-DNA contents after treatment significantly decreased compared with pre-treatment and that of control group after treatment (P<0.05).ConclusionGenciclovir is effect and safety for CMV infection of CP children.

ObjectiveTo investigate whether the Toxoplasma infection is the risk factor of occurrence of children cerebral palsy.Methods236 children with cerebral palsy and 428 normal children were included. Children were laminated to 4 groups by age:-3 months,-6 months,-9 months,-12 months. Final diagnosis was made by one or more finding following: plasma Toxoplasma DNA(TOX-DNA), plasma Toxoplasma circulating antigen(TOX-CAG), plasma Toxoplasma IgM(TOX-IgM), exclusion other congenital infected diseases.ResultsThe OR and 95%CI were 10.13,3.74-27.42 in -3 months,7.71,2.45-24.28 in -6 months,8.84,1.85-42.31 in-9 months(P<0.01 respectively), and 4.30, 0.83-22.28(P>0.05)in -12 months. ConclusionThere is correlation between the Toxoplasma infection and the children cerebral palsy; the earlier Toxoplasma infection, the greater the risk of cerebral palsy. Congenital Toxoplasma infection may be one of important factors in children cerebral palsy.

OBJECTIVETo observe the levels of serum C-reactive protein (CRP), endothelin-1 (ET-1), nitric oxide (NO), and superoxide dismutase (SOD) in patients after intracoronary stenting (ICS), and the effects of Erigeron Injection (EI) on them.

METHODSSeventy-two patients, who received ICS and had symptoms of chest stuffiness, palpitation and chest pain, were randomly divided into two groups, with 36 patients in the control group treated with Plavix alone for anti-platelet aggregation, and the other 36 patients in the treated group treated with Plavix and EI in combination. CRP, ET-1, NO and SOD were determined and compared before and 1, 2 and 3 weeks after treatment.

RESULTSAs compared with those in the control group, improvement of symptoms in the treated group was significantly better, with the levels of CRP and ET-1 lower and levels of SOD and NO higher or approaching to normal ranges and significant difference was shown between the two groups (P < 0.01).

CONCLUSIONEI could alleviate uncomfortable feelings such as chest stuffiness in patients after ICS, and improve the function of vascular endothelium.

C-Reactive Protein ; metabolism ; Coronary Artery Disease ; blood ; drug therapy ; Drug Therapy, Combination ; Endothelin-1 ; blood ; Endothelium, Vascular ; drug effects ; Erigeron ; Female ; Humans ; Infusions, Intravenous ; Male ; Middle Aged ; Nitric Oxide ; blood ; Phytotherapy ; Plant Preparations ; administration & dosage ; Platelet Aggregation Inhibitors ; administration & dosage ; Stents ; Superoxide Dismutase ; blood ; Ticlopidine ; administration & dosage ; analogs & derivatives

Objective By comparing the effects of different combined therapy,to explore the best therapy in prevention of intrauterine adhesions (IUA)after Transcervical resection of adhesions (TCRA).Methods Eighty patents with serious or moderate IUA were randomly divided into four groups.Group A:Artificial cycle combined with ball pouch,sodium hyaluronate gel.Group B:artificial cycle combined with intrauterine device(IUD),sodium hyalur-onate gel.Group C:large doses of estrogen drugs combined with ball pouch,sodium hyaluronate gel.Group D:large doses of estrogen drugs combined with IUD,sodium hyaluronate gel.Postoperative patients of TCRA were treated by the four therapies respectively for three months.All patients came back for hysteroscopy in one month and three months after operation.Results Following -up period was 6 -12 months.After three months treatment,the total endometrial cavity cure rate was 21.3%(17 /80),the total effective rate was 67.5%(54 /80).The cure rate of group C was 45.0%,which was obviously higher than those of the other three groups,the differences were statistically insig-nificant(group C vs.group A,χ2 =4.29,P <0.05;group C vs.group B,χ2 =6.14,P <0.05;group C vs.group D,χ2 =4.27,P <0.05).After three months treatment,the endometrial thickness were measured during ovulatory period. The thickness of group C was obviously thicker than those of the other three groups,the differences were statistically insignificant(group C vs.group A,t =10.28,P <0.01;group C vs.group B,t =12.80,P <0.01;group C vs.group D,t =5.02,P <0.01).Five months after the operation,in the aspect of menstrual blood volume,the total cure rate was 23.8%(19 /80),the total effective rate was 71.3%(57 /80).And the cure rate of group C was 45.0%,which was obviously higher than those of the other three groups,the differences were statistically insignificant(group C vs. group A,χ2 =4.29,P <0.05;group C vs.group B,χ2 =6.28,P <0.05;group C vs.group D,χ2 =4.27,P <0.05).Conclusion The therapy of large doses of estrogen drugs combined with ball pouch and sodium hyaluronategel can significantly improve the prognosis of serious or moderateIUA,and better than other combined therapies.

Objective To explore the effect of the simvastatin administration on the expression of connective tissue growth factor （CTGF） in fihrotic lungs of rats. Methods The cats were treated with single intratracheal instillation of bleomycin （BLM） or instillation of the same volume of normal saline （NS） as a control. The administration of simvastatin（20 mg/kg）began once a day immediately or 7 days later after intratracheal BLM instillation respectively with the same volume of NS was given as a vehicle control. The rats were killed on day 7,14 and 28 respectively. Pathological alteration of lung tissue was observed hy HE staining and Masson staining. Hydroxyproline（HYP）content in lung tissue was used to determine the severity of pulmonary fibrosis. The expression of CTGF in lung tissue was exanfined by immunohistochem- istry staining and photodeusitometry. Results Histopathological changes of pulmonary fibrosis emerged gradually after the instillation of BLM. The expression level of CTGF was increased in lungs of rats after intratracheal instillation of BLM, compared with the control. The administration of simvastatin immediately or 7 days after intratracheal instillation of BLM, attenuated the histopathological changes of bleomycin- induced puhnonary fibrosis and prevented the increased expression of CTGF in lung tissue on day 28. Conclusion The adntinistration of simvastatin, immediately or 7 days after intratracheal BLM instillation, prevented the up-regulation of CTGF in fibrotic lungs of rats, which ntight be one of the mechanisms of the anti-fihrosis of simvastatin in lungs.