Abstract: Bartter syndrome (BS) is a kind of inherited metabolic disease characterized by electrolyte and endocrine disorder, resulting from genetic gene mutation or deletion. Clinically, it manifests as vomiting, constipation, feeding difficulties, weight loss, growth retardation. The laboratory examination shows hypokalemia, metabolic alkalosis, hyperreninemia, aldosteronism, etc. As an autosomal recessive genetic disease, BS has an extremely low incidence rate, making diagnosis and treatment quite challenging. In recent years, with the progress of gene detection and other technologies, China has made great progress in the study of BS, more and more BS has been diagnosed accurately. According to known gene mutation types, it can be divided into type 1, type 2, type 3, type 4, type 5 and Gitelman syndrome, a total of 6 types, with type 4 further divided into type 4a and type 4b. At present, the most common type of children with BS in clinical practice is type 3, which is the classic type of BS. This paper reports a case of Bartter syndrome type 4b (BS4b). After two rounds of three-generation family gene sequencing, it was discovered that the infant had a combined mutation of both the CLCNKA and CLCNKB alleles, in addition to Alport syndrome, and both parents were carriers of this type of gene defect. The child was finally diagnosed as BS4b and Alport syndrome. This report, combined with the patient's clinical features, diagnosis and treatment process, as well as related literature analysis, aims to provide experience for understanding and diagnosis and treatment of this disease.

Objective To understand the evolution of health management research in China and 5-year development of Chinese Journal of Health Management.MethodsA total of 657 articles published in Chinese Journal of Health Management over the past 5 years were analyzed by using CiteSpace Ⅱ from the aspects of authors,organizations and fundings respectively.ResultsAbout1933authors and 625 organizations contributed to the publication of 657 articles.Every published article had 3 authors on average.Health management presentedin 24％articletitles.Fifty-four authorsshowed morethan 4 publications or a centrality of more than 0.HUANG Jian-shi,WU Liu-xin and ZENG Qiang showed higher productivity and centrality and cooperated well with BAI Shu-zhong,TIAN Jing-fa and HAN Jing.WANG Peiyu and DU Bing constituted their own collaboration team respectively.Authors with higher productivities were mainly from Institute of Aviation Medicine,Peking University Health Science Center,Beijing Physical Examination Center and Peking Union Medical College.Papers financially supported by national,provincial and other funding were increased over time.ConclusionOver half of publications presented in Chinese Journal of Health Management are directly related to health management,many of which are written by wellknown scholars.Taking Chinese Journal of Health Management as a platform,some research teams makeimportant contribution to promoting the development of health management.

Objective To investigate the efficacy of preemptive analgesiawith ultrasound-guided modified dorsal penile nerve block combined ketorolac tromethamine for circumcision in pediatric patients.Methods Forty-five pediatric patients with ASA grade Ⅰ undergoing elective circumcision were randomly divided into 3 groups (n=15 each).The same intravenous compound anesthesia was used in the three groups.The pediatric patients underwent ultrasound-guided modified dorsal penile nerve block with a mixture of 0.1 ml/kg of 0.2% ropivacaine and 0.8% lidocaine before operation in group A and group B.The pediatric patients were given ketorolac tromethamine 1 mg/kg intramuscular injection before operation in group A and group C.The total consumption of propfol and sufentanil, occurrence of intraoperative body movement and respiratory depression, emergence time, time from waking up to going out of PACU and adverse reactions such as postoperative agitation, nausea, vomiting and pruritus were aslo recorded.The requirement for postoperative paracetamol suppositories was recorded.Results Compared with group C, the total consumption of propfol and sufentanil were significantly decreased, incidence of body movement andrespiratory depression were significantly decreased, the emergence time and time from waking up to going out of PACU was significantly shortened, requirement for paracetamol suppositories were significantly decreased in group A and group B(P<0.05).Compared with group B, incidence of requirement for paracetamol suppositories was significantly decreased in group A (P<0.05).No pediatric patients developed postoperative nausea, vomiting, pruritus and incidence of emergence agitation had no statistical difference in the three groups.Conclusion Preemptive analgesia with ultrasound-guided modified dorsal penile nerve block combined ketorolac tromethamine is safe and effective when used for circumcision in pediatric patients, and it has good efficacy of postoperative analgesia.

Objective To explore the relationship among genetic polymorphism of angiotension Ⅱ type 1 re-ceptor(AT1 R) A1166-C, angiotensin converting enzyme (ACE) insertion/deletion (I/D), aldosterone synthase (CYP11B2)-344T/C and hypertensive disorder complicating pregnancy.Methods Polymerase chain reaction-re-striction fragment length polymorphism (PCR-RFLP) assay was used to detect the genotypes of AT1 R A1166-C ,ACE (I/O) ,CYP11B2 -344T/C in 86 cases of hypertensive disorder complicating pregnancy and 175 cases of normal control.Results There was 18 combined types in hypertensive disorder complicating pregnancy cases and normal control cases.Compared to AT1R-AA + ACE-Ⅱ + CYP11B2-TT, Odds ratios (OR) of AT1R-AA + ACE-DO +CYP11B2-TC,AT1 R-AC + ACE-ID+CYP11B2-TC and AT1R-AC+ACE-DD+CYP11B2-TC are 7.289,5.315 and 5.694 respectively.There was no statistical significance among the others.Conclusion In all 18 kinds of combined types, AT1 R-AA + ACE-DO + CYP11B2-TC,AT1R-AC+ACE-ID+CYP11B2-TC and AT1 R-AC + ACE-DD +CYP11B2-TC might increase the susceptibility of hypertensive disorder complicating pregnancy.It is possible that multigenes are interacted in the etiology of hypertensive disorder complicating pregnancy.

Objective To explore the 5,10-methylenetetrahydrofolate reductase (MTHFR) and 5-methyltetrahydro-folate-homocysteine methyltransferase reductase ( MTRR ) gene polymorphisms among the Han women in Zhangjiagang city .Methods A total of 4008 Han women were recruited .Their oral epithelial cells were collected to extract genome DNA in order to detect gene polymorphisms of MTHFR and MTRR using fluorescence quantitative PCR.Then the results were compared with those in other cities in China .Results The genotype frequencies of MTHFR C677T CC, CT and TT among Zhangjiagang women are 32.2%, 49.5%and 18.3%, respectively.The C allele frequency is 64.3%, T allele frequency is 35.7%.The genotype frequencies of MTHFR A1298 C AA, AC and CC are 68.7%,28.7% and 2.5%, respectively .The A allele frequency is 80.8%, C allele frequency is 19.2%.The genotype frequencies of MTRR A66 G AA, AG and GG are 54.4%, 38.5%and 7.1%, respectively . The A allele frequency is 76.3%, G allele frequency is 26.4%.Conclusions The MTHFR, MTRR polymorphism distribution of Han women in Zhangjiagang city is region specific.

AIM:To investigate the effects of high plasma triglyceride (TG) caused by apolipoprotein C Ⅲ( ApoC Ⅲ) transgene on the occurrence and development of abdominal aortic aneurysm ( AAA) .METHODS:The animal models of hypercholesterolemia and hypercholesterolemia combined with hypertriglyceridemia were established by feeding high-fat diet to LDLR-/-and ApoC Ⅲ+LDLR-/-mice, respectively.AAA was induced in these mice by pancreatic elastase. By evaluating the incidence of AAA, relative maximal abdominal aortic diameter, disruption of the elastic lamellar structure and expression of matrix metalloproteinases ( MMPs) in the aorta walls of the AAA, the occurrence and development of AAA were compared in LDLR-/-and ApoC Ⅲ+LDLR-/-mice fed with either chow diet or high-fat diet.In addition, an in vitro TNF-α-induced aneurysmal microenvironment model on vascular smooth muscle cells ( VSMC) was used to study the impact of triglyceride-rich lipoproteins ( TRLs) from mice with normal or high contents of ApoCⅢon elastin protein expres-sion.RESULTS:Feeding the high-fat diet aggravated the severity of AAA in the LDLR-/-mice.ApoC Ⅲ+LDLR-/-mice fed with high-fat diet had less severe AAA than LDLR-/-mice fed with high-fat diet.TRLs inhibited degradation of VSMC elas-tin protein induced by TNF-α, and in vitro TRLs from the mice with high content of ApoC Ⅲ, compared to those with nor-mal content of ApoC Ⅲ, had better inhibitory effect on the degradation of elastin.CONCLUSION:High plasma TG caused by ApoC Ⅲtransgene alleviates AAA of the LDLR-/-mice induced by elastase and high-fat diet.The effect is probably attrib-uted to the hypertriglyceridemia caused by ApoC Ⅲtransgene.

Rheumatoid arthritis (RA), as a common systemic inflammatory autoimmune disease, affects approximately 1 in 100 individuals. Effective treatment for RA is not yet available because current research does not have a clear understanding of the etiology and pathogenesis of RA. Xinfeng Capsule, a patent Chinese herbal medicine, has been used in the treatment of RA in recent years. Despite its reported clinical efficacy, there are no large-sample, multicenter, randomized trials that support the use of Xinfeng Capsule for RA. Therefore, we designed a randomized, double-blind, multicenter, placebo-controlled trial to assess the efficacy and safety of Xinfeng Capsule in the treatment of RA.

Objective:To explore the role of hippocampal γ oscillation abnormality in sepsis-associated encephalopathy (SAE).Methods:Seventy male Sprague-Dawley rats (2-3 months) were randomly (random number) divided into three groups according to the random digital table method: sham, CLP, and CLP + dopamine 4 (D4) receptor agonists RO-10-5824 group. The SAE animal model was established by cecal ligation and puncture (CLP). On day 10-14 after surgery, the open field, novel object recognition, and fear conditioning tests were performed. After that, the hippocampus was collected to measure expressions of parvalbumin (PV) and D4 receptor. In another set of experiment, CA1 local field potential (LFP) were recorded, and the relationship between LFP and time with novel object was analyzed. Independent sample t-test was used for pairwise comparisons, and multiple comparisons were performed by one-way ANOVA, followed by the Tukey multiple comparisons test. Correlation was analyzed using Pearson correlation. Statistical significance was assumed when P<0.05. Results:Compared with the sham group, hippocampal PV (77.54±4.61)%, D4 expression (56.36±3.88)% and γ oscillation power (41.1±8.62)%, object exposure time (36±3) s, new object recognition rate (49±4)%, and scene stiffness time (56±7) s were decreased significantly ( P<0.05). However, RO-10-5824 treatment could increase hippocaml γ oscillation power (92.3±6.7)%, and reverse the decreased new object exposure time (44±3) s and new object recognition rate (63±4)%. Correlation analysis showed that hippocampal γ oscillation power was positively associated with new object exposure time ( r=0.609 2, P=0.015 9). There was no difference in total distance traveled or time spent in the center among groups ( P>0.05). Conclusion:Hippocampal γ oscillation abnormality might play a key role in cognitive impairment associated with SAE.

RNA interference(RNAi)is a process by which introduced small interfering RNA(siRNA)can cause the specific degradation of mRNA with identical sequences. The human herpes simplex virus type 1(HSV-1)RR is composed of two distinct homodimeric subunits encoded by UL39 and UL40, respectively. In this study, we applied siRNAs targeting the UL39 and UL40 genes of HSV-1. We showed that synthetic siRNA silenced effectively and specifically UL39 and UL40 mRNA expression and inhibited HSV-1 replication. Our work offers new possibilities for RNAi as a genetic tool for inhibition of HSV-1 replication.

Herpes simplex virus type 1 (HSV-1) is a commonly occurring human pathogen worldwide. There is an urgent need to discover and develop new alternative agents for the management of HSV-1 infection. Tripterygium hypoglaucum (level) Hutch (Celastraceae) is a traditional Chinese medicine plant with many pharmacological activities such as anti-inflammation, anti-tumor and antifertility. The usual medicinal part is the roots which contain about a 1% yield of alkaloids. A crude total alkaloids extract was prepared from the roots of T. hypoglaucum amd its antiviral activity against HSV-1 in Vero cells was evaluated by cytopathic effect (CPE) assay, plaque reduction assay and by RT-PCR analysis. The alkaloids extract presented low cytotoxicity (CC_(50) = 46.6 μg/mL) and potent CPE inhibition activity, the 50% inhibitory concentration (IC_(50)) was 6.5 μg/mL, noticeably lower than that of Acyclovir (15.4 μg /mL). Plaque formation was significantly reduced by the alkaloids extract at concentrations of 6.25 μg/mL to 12.5 μg/mL, the plaque reduction ratio reached 55% to 75% which was 35% higher than that of Acyclovir at the same concentration. RT-PCR analysis showed that, the transcription of two important delayed early genes UL30 and UL39, and a late gene US6 of HSV-1 genome all were suppressed by the alkaloids extract, the expression inhibiting efficacy compared to the control was 74.6% (UL30), 70.9% (UL39) and 62.6% (US6) respectively at the working concentration of 12.5μg/mL. The above results suggest a potent anti-HSV-1 activity of the alkaloids extract in vitro.