This article reported a typical case of paroxysmal kinesigenic dyskinesia(PKD).The patient was a 26-year-old female with a medical history of 10 years.The patient manifested as paroxysmal choreoathetosis of the limb and head triggered by sudden movement in a quiet state,without sensory aura.The symptoms resolved spontaneously after tens of seconds.She was conscious during and between attacks,had a clear family history and a normal neurological examination.No abnormalities were found in brain magnetic resonance image and electroencephalogram.Genetic test showed a frame-shift mutation of c.776del in PRRT2 gene of the proband and her father with similar phenotype.The patient was diagnosed with PKD according to the diagnostic criteria for PKD.The symptoms were significantly relieved after one month of oxcarbazepine treatment with good prognosis.PKD is a rare movement disorder.The patient has typical symptoms,and the mutation site has not been reported in the Human Gene Mutation Database.Therefore,this article enriched the pathogenic gene mutation spectrum of PKD,provided a basis for genetic counseling of PKD and increased the awareness of this rare disease among physicians.

Objective To explore the effect of Xiangpi Shengji Ointment on wound healing and apoptosis-related Fas/Fas L pathway in model rats after anal fistula operation.Methods Thirty-six SD rats were used to construct the anal fistula model by using steel wire hanging line and indwelling for 30 days.After successful modeling,27 rats with anal fistula were randomly selected for"fistulectomy"to construct a postoperative wound model.After operation,the wound model rats were randomly divided into three groups,9 rats in each group,which were Xiangpi Shengji Ointment Group,Vaseline Group and Model Group,and the remaining 9 rats with anal fistula were sham operation group.The rats in the Xiangpi Shengji Ointment group were externally applied with Xiangpi Shengji Ointment gauze,while those in the Vaseline group were externally applied with Vaseline gauze.The rats in the model group were only disinfected and rinsed.No special treatment was given to the rats in the sham operation group.The wound healing was observed on the 3rd,5th,7th and 10th day after medication intervention,and the wound healing rate was calculated.After 10 days of continuous intervention,wound tissues were taken from each group,and the histopathological changes,the number of apoptosis,the expressions of Fas,Fas L,caspase-8 and cyto-c in wound tissues were observed by HE staining,TUNEL staining and immunohistochemistry respectively,and the mRNA expressions of Fas,Fas L and cyto-c in wound tissues were detected by RT-PCR.Results Compared with the model group,Xiangpi Shengji ointment group and Vaseline group significantly promoted wound healing at 7 and 10 days after intervention(P<0.01),and the wound healing rate of Xiangpi Shengji ointment group was significantly higher than that of Vaseline group(P<0.01).After 10 days of drug intervention,compared with sham operation group,the apoptosis rate of Xiangpi Shengji ointment group,Vaseline group and model group increased significantly(P<0.01),and the relative expressions of Fas,Fas L and cyto-c mRNA and the expression levels of Fas,Fas L,caspase-8 and cyto-c protein in wound tissue increased significantly(P<0.05,P<0.01).Compared with the model group,the apoptosis rate,the relative expression of Fas,Fas L and cyto-c mRNA and the expression level of Fas,Fas L,caspase-8 and cyto-c protein in Xiangpi Shengji ointment and Vaseline groups decreased significantly(P<0.05,P<0.01).Compared with Vaseline group,the apoptosis rate of Xiangpi Shengji ointment group decreased significantly(P<0.01),and the relative expression of Fas,Fas L and cyto-c mRNA and the expression level of Fas,Fas L,caspase-8 and cyto-c protein decreased significantly(P<0.05,P<0.01).Conclusion Xiangpi Shengji Ointment can inhibit the activation of Fas/Fas L pathway,reduce the apoptosis of wound tissue cells and promote wound healing after anal fistula operation.

Objective:To investigate the expression and significance of the angiotensin Ⅱ and its receptors in lipopolysaccharide (LPS)-induced acute lung injury and acute kidney injury in rats.Methods:Forty eight Wistar rats were randomly divided into two groups: control group and endotoxin group (LPS group). LPS was injected through tail vein in LPS group, and the same amount of saline was injected through tail vein in control group.Samples were collected at 2 h, 6 h, 12 h and 24 h, respectively.The histopathology of lung and kidney was observed by HE staining.We detected lung wet/dry weight ratio, serum creatinine, urea nitrogen and Ang Ⅱ concentration in plasma, lung and kidney tissues by radioimmunoassay.Western blot and immunohistochemistry were used to detect the expression changes of AT1R and AT2R in lung and kidney tissue.Results:Compared with the control group, the pathology of lung and kidney tissue in LPS group showed different degrees of damage.The lung wet/dry weight ratio, serum creatinine and urea level in LPS group were significantly increased than that in control group( P<0.05). The Ang Ⅱ content in plasma increased significantly at 2 h and 6 h ( P<0.05), and the expression level of Ang Ⅱ in lung and kidney increased significantly at all time points ( P<0.05). The expression of AT1R in lung and kidney decreased significantly ( P<0.05), while the AT2R protein expression increased significantly ( P<0.05). Additionally the correlation analysis showed that the expression level of Ang Ⅱ and AT2R were positively correlated with lung and renal function, while the expression of AT1R was negatively correlated with lung and renal function. Conclusion:LPS results in the damage of lung and kidney function and the change of renin-angiotensin system.The changes of Ang Ⅱ and angiotension receptors were correlated with lung and kidney injury.Ang Ⅱ and angiotension receptors may be involved in LPS induced lung and kidney injury.

Coinfection/drug therapy* ; HIV ; HIV Infections/drug therapy* ; Hepacivirus ; Hepatitis B/drug therapy* ; Hepatitis C/drug therapy* ; Humans ; Prevalence

Objective To study the correlation between the diffusion-weighted imaging (DWI) measurements and glomerular filtration rate (GFR), Katafuchi scores in IgA nephropathy. Methods Thirty-five patients with IgA nephropathy (IgAN group) and twenty healthy volunteers (control group) were enrolled in this study. All of the subjects underwent bilateral renal DWI measurements with 3.0T MRI scanner. The values of apparent diffusion coefficient (ADC) of renal cortex and medulla were measured. GFR of IgAN group was detected with 99Tcm-DTPA scintigraphy. Based on the Lee classification and the Katafuchi score system, the pathological grading was carried out in patients of IgAN group. The ADC values were compared between control group and different grades of IgAN group. The correlations between ADC and GFR values were analysed in defferent groups. The correlations between ADC values and Katafuchi scores were analysed in IgAN group. Results The renal cortical ADC values were significantly higher than medulla ADC values in both control group and IgAN group (P < 0.05). There were statistically significant differences in renal cortical ADC values and medulla ADC values between control group and IgAN subgroups (P<0.05). But there was no significant difference in renal cortical ADC value between IgANⅠgroup and control group (P>0.05). There was a positive correlation between the renal cortical and medulla ADC values and the GFR values in IgAN group (P<0.01). Negative correlation was found between the renal cortical and
medulla ADC values and the Katafuchi scores in IgAN group (P<0.05). Conclusion The diffusion-weighted imaging can reflect the physiological functions of kidney. It was feasible for application DWI in IgA nephropathy, which can be used for assessing the renal filtration function and the pathological damage. However, DWI measurement is not sensitive to early renal disease.

Hepatitis C ; etiology ; virology ; Hepatitis Viruses ; Humans ; Liver Transplantation ; Recurrence

OBJECTIVETo investigate the effects of hypertonic sodium chloride hydroxyethyl starch 40 (HSH) on brain edema and morphological changes during whole body hyperthermia (WBH) in rats.

METHODSSixty adult male SD rats were randomized into control group, WBH group without fluid infusion (group HT), WBH group with Ringer's infusion (group RL), WBH group with HAES + Ringer's infusion (group HRL) and WBH group with HSH infusion (group HSH). WBH was induced by exposure to 36 degrees celsius; for 3 h to achieve a rectal temperature of 41-42 degrees celsius;, and the corresponding fluids were administered intravenously within 30 min at the beginning of WBH. The control rats were housed at a controlled room temperature (22∓1) degrees celsius; for 4 h. After cooling at room temperature for 1 h, the rats were sacrificed and brain water content and morphological changes were evaluated.

RESULTSCompared with the control group, all the WBH groups had significantly increased brain water content (P<0.05 or 0.01), but group HSH showed a significantly lower brain water content than group HT (P<0.05). The rats in groups HT, RL and HRL showed serious to moderate structural changes of the brain tissue and nerve cells, but HSH group had only mild pathologies.

CONCLUSIONHSH can reduce brain edema and ameliorate the damages to brain cells in rats exposed to WBH.

Animals ; Brain ; pathology ; Brain Edema ; pathology ; prevention & control ; Hydroxyethyl Starch Derivatives ; therapeutic use ; Hyperthermia, Induced ; adverse effects ; Male ; Rats ; Rats, Sprague-Dawley ; Saline Solution, Hypertonic ; therapeutic use

BACKGROUNDEmergence agitation is a common problem in pediatric anesthesia, especially after sevoflurane induction and maintenance anesthesia. The purpose of this study was to investigate the effect of sufentanil to reduce emergence agitation after sevoflurane anesthesia in children undergoing adenotonsillectomy compared with fentanyl.

METHODSOne hundred and five children, aged 3 - 11 years, were randomly allocated to receive normal saline (control group), sufentanil 0.2 µg/kg (S2) or fentanyl 2 µg/kg (F2) 1 minute after loss of the eyelash reflex. Anesthesia was induced and maintained with sevoflurane. Time to tracheal extubation, recovery time, Paediatric Anesthesia Emergence Delirium (PAED) scale, and emergence behavior were assessed.

RESULTSThe incidence of severe agitation was significantly lower in S2 and F2 groups vs. the control group, 4/32 and 15/34 vs. 24/34 respectively, (P = 0.002, 0.009, respectively). PAED scales were significantly different among three groups (P = 0.007), and lower in the S2 and F2 groups than in the control group (P = 0.007 and P = 0.025, respectively). And the incidence of severe agitation and the PAED scale score was significantly different between the S2 and F2 groups (P = 0.007, P = 0.019, respectively). Time to tracheal extubation and recovery time were similar in all three groups.

CONCLUSIONSAdministration of sufentanil at 0.2 µg/kg after induction of anesthesia reduced emergence agitation in children receiving sevoflurane anesthesia for adenotonsillectomy compared with fentanyl. This was without delaying the recovery time or causing significant hypotension.

Adenoidectomy ; methods ; Anesthesia ; methods ; Child ; Child, Preschool ; Female ; Fentanyl ; therapeutic use ; Humans ; Male ; Methyl Ethers ; adverse effects ; therapeutic use ; Prospective Studies ; Psychomotor Agitation ; drug therapy ; etiology ; Sufentanil ; therapeutic use

OBJECTIVESTo investigate the efficacy of by combining a 12-week course of lamivudine in those HBeAg-positive hepatitis B patients receiving peginterferon alfa-2a (peg-IFN alpha-2a) therapy.

METHODSA total of 58 patients initiated a 52-week course of peginterferon alfa-2a were enrolled and divided into 3 groups. The patients with HBV DNA undetectable or HBeAg negative at week 12 were divided into group A, in this group treatment continued to week 52 with peg-IFN alpha-2a alone; The rest patients were divided into group B1 and B2, in group B1, lamivudine was combined at a course of 12 weeks, while in group B2 treatment continued to week 52 with peg-IFN alpha-2a alone. Clinical responses were assessed at week 52.

RESULTS8 out of 58 patients achieved undetectable HBV DNA or HBeAg loss at week 12 and divide into group A. In this group the HBV DNA loss rate, HBeAg seroconversion rate, HBsAg loss rate and ALT normalization rate were 100% (8/8), 75% (6/8), 0% (0/8) and 100% (8/8) respectively at the end of treatment. In this group the HBV DNA loss rate, HBeAg seroconversion rate, HBsAg loss rate and ALT normalization rate were 100% (8/8), 75% (6/8), 0% (0/8) and 100%(8/8) respectively at the end of treatment. The rest 50 patients without early response to peg-IFN alpha-2a at week 12 were divided into group B1 (24 patients enrolled) and B2 (26 patients). At the end of treatment, the HBV DNA loss rate, HBeAg seroconversion rate, HBsAg loss rate and ALT normalization rate in Group B1 were 50% (12/24), 38% (9/24), 4% (1/24) and 63% (15/24) respectively, and 31% (8/26), 27% (7/26), 0% (0/26) and 35% (9/26) respectively in group B2.

CONCLUSIONThose patients with early responses to peg-IFN alpha-2a therapy can achieve high clinical responses at the end of 52-week treatment. The combining therapy of lamivudine for a course of 12-weeks can improve the clinical responses for the patients without early responses to peg-IFN alpha-2a.

Adult ; Antiviral Agents ; therapeutic use ; DNA, Viral ; blood ; Drug Therapy, Combination ; Female ; Hepatitis B e Antigens ; blood ; Hepatitis B, Chronic ; blood ; drug therapy ; Humans ; Interferon-alpha ; therapeutic use ; Lamivudine ; therapeutic use ; Male ; Pilot Projects ; Polyethylene Glycols ; therapeutic use ; Recombinant Proteins ; Treatment Outcome ; Young Adult

Antiviral Agents ; therapeutic use ; Guidelines as Topic ; Hepatitis B, Chronic ; drug therapy ; Humans