ObjectiveTo investigate the influence on efficacy and quality of life of amisulpride in schizophrenia with predominantly negative symptoms.MethodsSeventy cases of schizophrenia with predominantly negative symptoms were randomly assigned to amisulpride group and clozapine group for 12weeks.The clinical efficacy and side effects were evaluated with SANS,BPRS and TESS before and 1,2,4,8,12 weeks after treatment respectively. GQOLI-74 were used to evaluate the patients' quality of life before and after drug administration.ResultsFor the last assessment,the score of BPRS decrease to 30.68 ±4.62 in amisulpride group,was significantly lower than the beginning(P＜0.01 ) ; the score of BPRS decrease to 31.31 ±4.78 in clozapine group,was significantly lower than the beginning (P ＜ 0.01 ).Two groups showed similar effects,with no significance.12weeks after treatment,the scores of emotional dull,poverty of thought,volition abulia,interest-social lack,attention dysfunction in amisulpride group were 12.31 ± 4.43,9.66 ± 3.07,8.91 ± 2.56,10.29 ± 2.68,5.58 ± 1.43 ; and the scores of emotional dull,poverty of thought,volition abulia,interest-social lack,attention dysfunction in clozapine group were 14.62 ± 4.27,9.32 ± 2.81,8.22 ± 2.60,9.54 ± 2.67,6.62 ± 1.59,and the efficacy of amisulpride in emotional dull and attention dysfunction were better than those of clozapine.And the scores of material life,physical function,psychological function,social function in amisulpride group were not higher than those in clozapine group(P＞ 0.05).And the scores of four domains in GQOLI-74 had been improved significantly in two groups(P＜0.01).The domains of physical function,psychological function,and social function in amisulpride was superior to clozapine(P＜0.01).The side effects were more in clozapine group than those in amisulpride group.ConclusionAmisulpride is as effective as clozapine for the treatment of schizophrenia with predominantly negative symptoms,but it has more advantage than clozapine to improve emotional dull,attention dysfunction and quality of life in schizophrenia with predominantly negative symptoms.

OBJECTIVE: To analyze the clinical features and variants of ABCD1 gene in a Chinese pedigree affected with X-linked adrenoleukodystrophy. METHODS: Clinical data of the proband were collected and analyzed. Potential variant of the ABCD1 gene were analyzed by PCR and Sanger sequencing of the proband, his parents and 100 unrelated healthy individuals. RESULTS: The prominent features of the proband included cerebellar and brainstem lesions, along with increased serum level of very-long chain fatty acids. He was found to harbor a hemizygous c.1509delG (p.L504Sfs*54) variant of the ABCD1 gene, for which his mother was heterozygous. The same variant was not detected in his father and 100 healthy controls. CONCLUSION X-linked adrenoleukodystrophy has a variety of clinical manifestations. Discovery of the c.1509delG (p.L504Sfs*54), as a novel pathogenic variant of the ABCD1 gene, has enabled diagnosis and genetic counseling for this pedigree.
Adrenoleukodystrophy/genetics* ; Asians/genetics* ; China ; Female ; Genetic Testing ; Humans ; Male ; Mutation ; Pedigree

Objective To understand the compliance of highly active anti-retroviral therapy (HAART) and influencing factors in people living with HIV/AIDS (PLWHA) in Nanjing. Methods PLWHA receiving HAART in No. 2 Hospital of Nanjing during May-June 2014 were recruited in this study. Self-administrated questionnaire was used to collect the data about HAART compliance and socio-demographic characteristics of PLWHA surveyed. Descriptive and multivariate statistical analysis were conducted to examine the effects of the factors on self-reported HAART adherence. Results A total of 276 PLWHA were surveyed,According to the evaluation criterion of Center for Adherence Support Evaluation (CASE),252 cases showed good compliance (91.3%). logistic regression analysis revealed that smoking,progress of the disease and side effects,reminding of taking drug and age were correlated with self-reported HAART adherence. Conclusion It is suggested to strengthen the education about antiviral therapy compliance in PLWHA with mild infection and those who are smokers and young,suffer from side effects,have no reminding methods for taking drug.

Background::Although overnight fasting is recommended prior to endoscopic retrograde cholangiopancreatography (ERCP), the benefits and safety of high-carbohydrate fluid diet (CFD) intake 2 h before ERCP remain unclear. This study aimed to analyze whether high-CFD intake 2 h before ERCP can be safe and accelerate patients’ recovery.Methods::This prospective, multicenter, randomized controlled trial involved 15 tertiary ERCP centers. A total of 1330 patients were randomized into CFD group ( n = 665) and fasting group ( n = 665). The CFD group received 400 mL of maltodextrin orally 2 h before ERCP, while the control group abstained from food/water overnight (>6 h) before ERCP. All ERCP procedures were performed using deep sedation with intravenous propofol. The investigators were blinded but not the patients. The primary outcomes included postoperative fatigue and abdominal pain score, and the secondary outcomes included complications and changes in metabolic indicators. The outcomes were analyzed according to a modified intention-to-treat principle. Results::The post-ERCP fatigue scores were significantly lower at 4 h (4.1 ± 2.6 vs. 4.8 ± 2.8, t = 4.23, P <0.001) and 20 h (2.4 ± 2.1 vs. 3.4 ± 2.4, t= 7.94, P <0.001) in the CFD group, with least-squares mean differences of 0.48 (95% confidence interval [CI]: 0.26–0.71, P <0.001) and 0.76 (95% CI: 0.57–0.95, P <0.001), respectively. The 4-h pain scores (2.1 ± 1.7 vs. 2.2 ± 1.7, t = 2.60, P = 0.009, with a least-squares mean difference of 0.21 [95% CI: 0.05–0.37]) and positive urine ketone levels (7.7% [39/509] vs. 15.4% [82/533], χ2 = 15.13, P <0.001) were lower in the CFD group. The CFD group had significantly less cholangitis (2.1% [13/634] vs. 4.0% [26/658], χ2 = 3.99, P = 0.046) but not pancreatitis (5.5% [35/634] vs. 6.5% [43/658], χ2 = 0.59, P = 0.444). Subgroup analysis revealed that CFD reduced the incidence of complications in patients with native papilla (odds ratio [OR]: 0.61, 95% CI: 0.39–0.95, P = 0.028) in the multivariable models. Conclusion::Ingesting 400 mL of CFD 2 h before ERCP is safe, with a reduction in post-ERCP fatigue, abdominal pain, and cholangitis during recovery.Trail Registration::ClinicalTrials.gov, No. NCT03075280.