To investigate the changes in neurological symptoms and signs, as well as serum copper, serum ceruloplasmin after hepatic transplantation in patients with Wilson's disease, neurological symptoms and signs, serum copper, serum ceruloplasmin before and after hepatic transplantation in 18 patients with Wilson's disease were observed, and those changes were followed up in 20 non-operative controls treated with penicillamine. Our results showed that the neurological symptoms and signs, serum copper and serum ceruloplasmin were improved in the operative group but deteriorated in the non-operative control group. Our study showed that hepatic transplantation is better than penicillamine in the treatment of Wilson's disease.
Adolescent ; Adult ; Ceruloplasmin ; metabolism ; Child ; Copper ; blood ; Female ; Follow-Up Studies ; Hepatolenticular Degeneration ; blood ; surgery ; Humans ; Liver Transplantation ; Male ; Treatment Outcome

Objective:To investigate the clinical efficacy and prognosis of transjugular intrahepatic portosystemic shunt (TIPS) and drug combined with endoscopic treatment in patients with liver cirrhosis and esophagogastric variceal bleeding (EGVB).Methods:From January 2012 to December 2013, at the First Affiliated Hospital of Xi′an Jiaotong University, the data of 147 patients with liver cirrhosis and EGVB undergoing TIPS or drug combined with endoscopic treatment were retrospectively collected, with 87 cases in TIPS treatment group and 60 in drug combined with endoscopic treatment group.The 5 years follow-up data were analyzed, and the overall survival rates, rebleeding-free survival rates and hepatic encephalopathy-free survival rates at 6 weeks, 1 year, 2 years and 5 years after treatment of two groups were compared. Independent sample t test, Mann-Whitney U test, chi-square test, Fisher exact test, Z test, log-rank test and trend test were used for statistical analysis. Results:There were no significant differences in age, gender, etiology, Child-Pugh classification, initial liver function, coagulation function, liver ascites, previous history of hepatic encephalopathy, blood pressure and preoperative blood transfusion history between the TIPS treatment group and combination of drugs and endoscopy treatment group (all P>0.05). Forty-one patients died within 5 years, of which 20 (48.8%) died of rebleeding and 6 (14.6%) died of hepatic encephalopathy. There were no significant differences in 6-week, 1-year and 2-year overall survival rates between the TIPS group and drug combined with endoscopic treatment group (all P>0.05), however the 5-year overall survival rate of the TIPS treatment group was higher than that of the drug combined with endoscopic treatment group (78.4% vs. 63.2%), and the difference was statistically significant ( Z=2.06, P=0.048). The 6-week, 1-year, 2-year, 5-year rebleeding-free survival rates of the TIPS group were 97.7%, 96.5%, 88.9% and 70.9%, respectively, which were all higher than those of the drug combined with endoscopic treatment group (86.7%, 53.3%, 43.3% and 27.1%), and the differences were statistically significant ( Z=2.35, 6.39, 6.26 and 4.80, all P<0.05). There were no significant differences in hepatic encephalopathy-free survival rates at 6 weeks, 1 year and 2 years after treatment between the TIPS group and drug combined with endoscopic treatment group (all P>0.05), however the 5-year hepatic encephalopathy-free survival rate of the TIPS treatment group was lower than that of the drug combined with endoscopic treatment group (67.7% vs. 86.7%), and the difference was statistically significant ( Z=2.28, P=0.030). The lower the Child-Pugh classification, the higher the cumulative 5-year survival rate ( χ2=6.75, P<0.01). There was no statistically significant difference in the 5-year overall survival rate in patients with the same Child-Pugh classification between the TIPS group and the drug combined with endoscopic treatment group (all P>0.05). Conclusions:The efficacy of TIPS is better than that of the drug combined with endoscopic treatment in treating EGVB. Even the long-term risk of hepatic encephalopathy of TIPS is higher, the short-term, middle-term and long-term rebleeding rate are decreased. Patients with Child-Pugh grade C do not need to avoid TIPS when choosing the treatment, the earlier the TIPS used, the better survival benefit will be obtained.

OBJECTIVETo determine the three-dimensional stability of C1-C2 after anterior approach screw fixation through C2 vertebral body to C1 lateral mass for C1-C2 instability or dislocation.

METHODSThe three-dimensional range of motion of atlantoaxial joint were measured in 16 human cadaveric specimens under four conditions: the intact state (1st group), odontoid fracture of type II (2nd group), instrumentation with posterior C1-C2 trans-articular screw fixation (Magerl technique) (3rd group) and anterior approach screw fixation through C2 vertebral body to C1 lateral mass (4th group) respectively.

RESULTSThere was generally significant difference between 1st group and other groups and between 2nd group and other groups by statistics analysis (P < 0.001). Range of motion significantly decreased in 3rd group and 4th group in all directions. There was generally no significant difference between the two methods by statistical analysis (P > 0.05).

CONCLUSIONAnterior approach screw fixation through C2 vertebral body to C lateral mass provides satisfied stability. It provides the equivalent effect to Magerl technique. It is a kind of reliable surgery choice for the treatment of instability or dislocation of C1-C2 joint.

Adult ; Atlanto-Axial Joint ; surgery ; Biomechanical Phenomena ; Bone Screws ; Cadaver ; Cervical Vertebrae ; surgery ; Female ; Fracture Fixation, Internal ; methods ; Humans ; Internal Fixators ; Male

OBJECTIVETo study the life events which are correlated with adolescent's emotion and behavior problems, and to provide evidence for clinicians and school staff to develop intervention for those problems.

METHODSYouth's Self Report (YSR) and Adolescent Self-Rating Life Events Check List (ASLEC) were used to assess adolescent's emotional and behavioral problems and life events by 'spot' study. The referred group consisted of 585 patients from 11 to 18 years old in a Mental Health Centre of West China Hospital of Sichuan University from July in 2002 to March in 2004. Level of IQ was above 5th grade of primary school among the study subjects who were willing to fill in the YSR and ASLEC. The non-referred group was selected in a 1280 students cluster-sample from the schools of Chengdu city, whose sex, age and father's career were matched with the referred group. Variance inflation factor (VIF) was used to verify that there was no collinearity to each other in the 6 factors of ASLEC: interpersonal relationship, learning pressure, being punished, losing good adaptation and other. Linear stepwise regression was adopted.

RESULTSThe YSR scores in referred group were higher than those in non-referred group, and the referred group had more emotional and behavioral problems than the non-referred one. Partial correlations ranged from 0.124 to 0.418 in referred group, and from 0.104 to 0.388 in non-referred group. Unsatisfied interpersonal relationship, heavy learning pressure, having been punished and poor adaptation were likely to increase the risk of youth's emotional and behavioral problems.

CONCLUSIONMore attention should be paid to help adolescents in the following areas: solving intrapersonal affairs, relieving pressure from learning, avoiding punishment, and improving ability to fit themselves to their surroundings.

Adaptation, Psychological ; Adolescent ; Adolescent Behavior ; Child ; Child Behavior Disorders ; epidemiology ; psychology ; China ; epidemiology ; Emotions ; Female ; Humans ; Interpersonal Relations ; Linear Models ; Male ; Mental Disorders ; epidemiology ; psychology ; Psychological Tests ; Punishment ; Stress, Psychological

OBJECTIVETo study the mental health problems in the adolescents aged 11-18 years in Chengdu, China.

METHODSYouth Self-Report (YSR) and the General Information Questionnaire were administered to 1802 students who were randomly selected from schools in Chengdu City.

RESULTSThe overall rate of mental problems was 15.1% in the adolescents, 15.2% in boys and 14.9% in girls if 90th percentile of total scores and/or 98th percentile of syndromes scores were assigned as the cutpoint of norms. The overall rate of mental problems was 27.2% in the adolescents, 27.6% in boys and 26.8% in girls if 75th percentile of total scores and/or 98th percentile of syndromes scores were assigned as the cutpoint of norms. The rates of mental problems were not significantly different between boys and girls (chi-squared = 0.042, P = 0.838; chi-squared = 0.126, P = 0.723). Mental problems tended to be increasing with age (P = 0.000). There were 29 items that the percentiles were higher than 50% but 5 items were under 10% in all the 101 items of YSR. 3.1% of the adolescents often had suicidal intention, and 6.1% of the adolescents often thought of becoming opposite sex identity.

CONCLUSIONThe rates of mental problems in adolescents aged 11-18 years in Chengdu, Chana were 27.2% or 15.1% respectively,if 75th or 90th percentile of the total scores were assigned as the cutpoint of total scores. The mental problems in the adolescents tended to be increasing along with age.

Adolescent ; Age Distribution ; Child ; China ; epidemiology ; Female ; Humans ; Male ; Mental Disorders ; epidemiology ; Sex Distribution ; Surveys and Questionnaires

BACKGROUNDAspirin and clopidogrel resistance plays a significant role in the development of cardiovascular ischemic events for ninety patients undergoing percutaneous coronary intervention. Recent studies have indicated that increasing the dose of antiplatelet drugs maybe a potent method to improve the inhibition of platelet aggregation.

METHODSThrombelastograph (TEG) determinations were used to evaluate the effect of antiplatelet therapy. According to the results, 90 patients were divided into three groups and given different doses of aspirin and clopidogrel. Thirty patients with both an inhibition rate of aspirin > 50% and an inhibition rate of clopidogrel > 50% were defined as the control group. Sixty patients with an inhibition rate for aspirin < 50% and an inhibition rate for clopidogrel < 50% were defined as the resistance group. Patients in resistance group were randomly assigned to be given a routine dose (100 mg aspirin plus 75 mg clopidogrel per day, which we called a resistance plus routine dose group, R + R) and a loading dose (200 mg aspirin and 150 mg clopidogrel per day, which we called resistance plus loading dose group, R + L) of antiplatelet therapy. A 12-month follow-up was observed to examine the change of inhibition rate of antiplatelet therapy and to estimate the relationship between inhibition rate and the occurrence of cardiovascular ischemic events.

RESULTSAfter 6 months of antiplatelet therapy, the inhibition rate of aspirin in the R + L group increased from (31.4 ± 3.7)% to (68.6 ± 7.1)%, which was significantly higher than that in R + R group, (51.9 ± 8.2)% (P < 0.01). The inhibition rate of clopidogrel in the R + L group increased from (22.1 ± 3.8)% to (60.2 ± 7.4)%, which was significantly higher than in the R + R group, (45.9 ± 4.3)% (P < 0.01). The occurrence rates of cardiovascular ischemic events, stent thrombosis, recurrent unstable angina and myocardial infarction in the R + R group were 20%, 36% and 17%, respectively. Occurrence was significantly increased compared with that in the control group, 3%, 10% and 1%, respectively (P < 0.01). In contrast, the occurrence rates in the R + L group (10%, 23% and 6%, respectively) were attenuated compared with those in the R + R group (P < 0.01), although still higher than in the control group (P < 0.01).

CONCLUSIONSAlmost all of the cardiovascular ischemic events occurred in the first six months after percutaneous coronary intervention. According to the result of TEG determinations, earlier application of a loading dose of aspirin and clopidogrel can decrease the rate of recurrent cardiovascular ischemic events.

Adult ; Aged ; Angioplasty, Balloon, Coronary ; adverse effects ; Aspirin ; administration & dosage ; therapeutic use ; Female ; Humans ; Male ; Middle Aged ; Myocardial Ischemia ; prevention & control ; Platelet Aggregation ; drug effects ; Platelet Aggregation Inhibitors ; administration & dosage ; therapeutic use ; Prospective Studies ; Thrombelastography ; Thrombosis ; prevention & control ; Ticlopidine ; analogs & derivatives ; therapeutic use

Objective To study the application of pediculated skin flaps in the treatment of com-plicated long urethratresia. Methods From March 1999 to May 2006, a total of 18 male patients with complicated long urethratresia were treated by using the pediculated skin flaps. The causes of urethratresia were 7 cases of postoperative pelvic fractures with posterior urethral stricture, 4 cases of transurethral intravesical chemotherapy, 3 cases of postoperative bulbar urethral stricture, 2 cases of gonorrhea, and 2 cases of long-time urethral catheter placement. Four cases were urethratresia nf cor-pus penis, 7 cases were anterior urethral obliteration, 7 cases were posterior urethral and anterior ure-thral obliteration. Urethro-perineal fistulas were found in 8 cases, posterior urethrorectal fistulas in 7 cases, false passage formations in 8 cases. The average length of urethratresia was 15.1 cm (range 8. 7 to 23. 0 cm). The urethral scar was rasected, the posterior urethrorectal fistula was repaired, and different kinds of pediculated skin flaps depending on the length of urethratreaia was used. Results All the patients were followed up for 12 to 18 months (mean 14 months). Fifteen patients voided well 3 months postoperatively, none of the urography showed stricture. The mean peak urinary flow rate was 16. 9 ml/s (range from 16. 5 to 21.7 ml/s). Of the other 3 cases, 1 case experienced difficult voi-ding due to the long and circuitous tabularized skin flap but recovered after proper shortening;1 case had restenosis for the infection of anastomosis but voided well after excision and reanastomnsis;1 casehad a urinary fistula resulting from hematoma and infection, but was successfully treated by the neo-plasty of the urinary fistula. The mean peak urinary flow rate was 17.0 ml/s (range 15.0 to 22.0 ml/s) for 17 patients 6 months postoperatively, except for one who experienced genuine urinary incon-tinence. At 9-18 months after operations, the mean peak urinary flow rate was 17.5 ml/s (range 15.8 to 22.5 ml/s) for 17 patients. Conclusion The single-stage urethroplasty based on pediculated skin flaps is a reliable and durable method for complicated long urethratresia.

OBJECTIVE: To investigate the effect of transurethral resection of prostate (TURP) on the quality of life in aged patients with benign prostatic hyperplasia(BPH). METHODS: Altogether 358 BPH patients were evaluated by International Prostate Symptom Score (IPSS), the 5-Item Version of the International Index of Erectile Function (IIEF-5),Quality of Life Scale (QOLS) questionnaires, Self-Rating Anxiety Scale (SAS), Self-Rating Depression Scale (SDS),residual urine volume (RUV), and maximum flow rate(Qmax) before and at 3 months after they underwent TURP. RESULTS: The mean scores of IPSS, QOLS, SAS, and SDS, and mean RUV decreased, and mean Qmax increased significantly at 3 months after receiving operation compared with those before the treatment in aged patients with BPH. But no significant difference was found in the scores of IIEF-5 between post-operation and pre-operation. CONCLUSION TURP may alleviate BPH clinical symptoms and improve quality of life, but may not be helpful to the recovery of sexual function in aged patients with BPH.
Aged ; Aged, 80 and over ; Erectile Dysfunction ; etiology ; Humans ; Male ; Middle Aged ; Prostatic Hyperplasia ; surgery ; Quality of Life ; Transurethral Resection of Prostate ; adverse effects ; methods

OBJECTIVECleidocranial dysplasia (CCD) is a rare skeletal disease with autosomal dominant inheritance associated with mutation in RUNX 2. The authors report a Chinese girl with CCD in whom the mutation in RUNX 2 was identified.

METHODSClinical diagnosis was based on physical examination, radiological findings, and biochemical tests. For mutation detection, genomic DNA was extracted from peripheral blood using standard method. All 7 coding exons of RUNX 2 and their flanking intronic sequences were amplified by polymerase chain reaction (PCR), and the PCR products were then subjected to automatic DNA sequencing.

RESULTSThe affected girl showed typical clinical manifestations of CCD, including patent fontanelles, absent clavicles, short stature and dental anomalies. Direct sequencing of PCR-amplified fragments revealed a recurrent missense mutation, R190W (568 C > T), in RUNX 2. The mutation was further confirmed by Hae III restriction analysis.

CONCLUSIONA Chinese case of CCD was confirmed and the disease-causing mutation was linked to a recurrent point mutation in RUNX 2.

Cleidocranial Dysplasia ; genetics ; Core Binding Factor Alpha 1 Subunit ; genetics ; Female ; Humans ; Mutation

Mitochondrial tRNAgene mutation is closely related to acoustic nerve deafness. Some mutations can affect the structure and transcriptional processing of tRNA, for instance m.7444G>A mutation in tRNAprecursor 3' side, m.7472 insC as well as m.7511T>C mutations in the stem and ring of tRNA, may influence tRNAstability, thus affect the synthesis of mitochondrial peptides, reduce the production of ATP and cause deafness. This article focuses on mitochondrial tRNAgene mutations as well as the mechanism underlying hearing loss.
Amino Acid Sequence ; Base Sequence ; Genetic Predisposition to Disease ; genetics ; Hearing Loss ; genetics ; Humans ; Mitochondrial Proteins ; biosynthesis ; genetics ; Mutation ; Nucleic Acid Conformation ; RNA ; chemistry ; genetics ; RNA, Transfer, Ser ; chemistry ; genetics