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OBJECTIVE To establish a database of rational drug use for children in our hospital， and to provide reference for ensuring the safety of drug use in children. METHODS The construction and filling of the knowledge base of rational drug use for children were performed by establishing the basic structure of the knowledge base， formulating reference standards for the quality level of pediatric medication evidence， and refining evidence-based evidence of pediatric medication. The rule base of rational drug use for children was designed and built from four aspects： preliminary determination rules for patient information， basic drug information rules， prescription suitability review rules， result labeling and post-processing rules. The database was embedded into prescription review system of our hospital and was applied online to test its effectiveness. RESULTS A set of database containing 672 commonly used pediatric medicines and more than 15 000 rules for rational drug use for children was initially constructed. The average interception rate of unreasonable medical orders for hospitalized children after database application（from December 2021 to May 2022）was 2.03%， and was higher than 0.80% before database application（from June 2021 to November 2021）（χ 2＝5 784.389， P＜0.001）； after post-sampling and prescription review， the average qualified rate of medication orders in discharged medical records for children after the application of the database was 99.10%， and was higher than 94.58% before the application of the database （χ 2＝301.237， P＜0.001）. CONCLUSIONS Self-constructed evidence-based rational drug use database for children is close to the actual clinical needs of pediatrics in medical institutions， which can effectively reduce clinical irrational drug use behaviors in pediatrics， improve the pass rate of prescriptions， and ensure the safety of children’s drug use.
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Objective To change the deficiencies of the traditional performance evaluation model based on financial performance,give full play to the potential of hospital scientific research in the exploration of medical resources in hospital,improve the long-term development ability of hospital,and explore a new performance evaluation system of hospital scientific research.Methods By means of literature review combined with practice analysis,the paper conducted a comparative study on the advantages and characteristics of the research performance evaluation system and the traditional hospital performance evaluation system.Results The performance evaluation system of hospital scientific research based on balanced scorecard theory matches the dynamic process dimension of hospital scientific research.Conclusions The balanced scorecard-based performance evaluation system of hospital is able to change the previous rigid performance evaluation of scientific research,realized a dynamic and whole-process management mode which better mobilize the enthusiasm of medical staff for scientific research.
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Objective To explore the characterization of thyroid stimulating hormone receptor(TSHR) gene mutational spectrum in children with hyperthyroidism from Guangzhou. Methods Ninety children were diagnosed with hyperthyroidism from July 2009 to July 2014 in our institute. Their median age at diagnosis was(7.5± 3.4) years, and there were 28 males and 62 females. Mutational analysis were performed by performing polymerase chain reaction (PCR) and DNA direct sequencing of exon 10 of TSHR gene. TSHR gene mutations from 50 unrelated healthy children were served as controls. The correlation between TSHR gene and hyperthyroidism in children was explored. Results A total of 3 mutations were identified in ninety children who were diagnosed with hyperthyroidism, one synonymous mutations(p.V614V), and two missense mutations( p. R707W and p. D727E). Mutation of p. V614V do not change amino acid and do not influence the structure and function of TSHR, no pathogenicity. p.R707W is a SNP associated with human cancers. The frequency of C allele of the D727E in children with hyperthyroidism was 86.7%, while 55.0% in the controls, significant different between the children with hyperthyroidism and the controls( P＜0. 01). In this study, a very high association between the D727E SNP and hyperthyroidism ( OR=18. 86, P＜0. 01) was found. Conclusion Three different mutations of TSHR gene exon 10 were identified in 90 children with hyperthyroidism, (c.1842A＞G,p.V614V、c.2119C＞T,p.R707W、c.2181G＞C,p.D727E), there were association between p.D727E and hyperthyroidism, nor p. V614V and p. R707W. Finally, p. D727E may be correlated with hyperthyroidism in children.
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Objective To investigate the effect of different adhesive materials on all-ceramic restoration. Methods The all-ceramic restoration system model of mandibular first molar was established by spiral computed tomography (CT) scanning and computer-aided design (CAD) modeling. Four types of resin adhesive materials (Duo Cement,Lute-It,Rely-X ARC,Variolink II) used in clinics were selected, and the stress distribution was calculated using the ABAQUS software. Results The stress at the bonding interface was the highest when low filler adhesive Lute-It was used. Based on the viscoelasticity analysis, resin adhesives with a larger storage modulus and loss modulus could yield lower stress extremes. Conclusions The study suggests that high-filler type resin adhesives with a large energy storage modulus and loss modulus should be used clinically.
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Lumbar intervertebral disc herniation (LDH) is a syndrome caused by various reasons such as nucleus pulposus protrusion,compression of nerve root and herniation of nucleus pulposus,and stimulation of nerve root and cauda equina nerve.It is one of the most common diseases in orthopedics.There are a variety of surgical treatments for this disease,including traditional open surgery and minimally invasive techniques.At present,the minimally invasive technique represented by percutaneous transforaminal endoscopy has gradually replaced the traditional open surgery as the mainstream because of its advantages of less trauma,faster recovery and fewer complications.This article will systematically expound the application and progress of percutaneous lumbar intervertebral foraminoscopy in the treatment of lumbar disc herniation from the development course,indications,operation skills,matters needing attention,curative effect analysis,complications and so on.
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Objective To investigate the frailty in maintenance hemodialysis (MHD) patients and its influence factors. Methods A total of 127 adults undergoing hemodialysis from January 2015 and January 2016 in our center were recruited. Their clinical data and blood biochemical data were collected. Frailty was assessed using Fried's Frailty Phenotype. Quantification of coronary artery calcification (CACs) was determined by multi-slice spiral computed tomography (MSCT). According to the frailty scores, patients were divided into non-frailty, pro-frailty and frailty group. Their in clinical and biochemical index as well as CACs were compared. The correlations of frailty scores with above index were assessed by Spearman's correlation. Multiple logistic regression analysis was applied to evaluate the effect factors of frailty on MHD patients. Results Among 127 selected patients, 46(36.22%) patients without frailty, 45(35.43%) patients with pro-frailty, and 36(28.35%) patients with frailty. The age, diabetes, haemoglobin, albumin, pre-albumin, C-reactive protein, fibroblast growth factor 23 (FGF23), CACs and left ventricular end-diastolic dimension (LVEDD) of the 3 groups had statistical differences (all P<0.05). The degrees of calcification among 3 groups were also different statistically (F=31.769, P<0.001). In patients with MHD, frailty was positively correlated with age (r=0.545, P<0.001), diabetes (r=0.236, P=0.008), C-reactive protein (r=0.245, P=0.006), FGF23 (r=0.189, P=0.034) and CACs (r=0.396, P<0.001), while negatively correlated with haemoglobin (r=-0.257, P=0.004), albumin (r=-0.380, P<0.001), pre-albumin (r=-0.313, P<0.001). Age (OR=1.076), C-reactive protein (OR=1.176), albumin (OR=0.796) and artery calcification (OR=2.465) were independent influence factors for frailty in MHD patients (all P<0.05). Conclusions The prevalence of frailty is high among MHD patients. Frailty is associated with age, C-reactive protein, albumin and artery calcification in MHD patients.
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Objective To explore the association of serum soluble Klotho (sKlotho) with nonfatal cardiovascular disease (CVD) and all-cause/CVD mortality in maintenance hemodialysis (MHD) patients.Methods A total of 132 MHD patients admitted during October 2011 were enrolled.Serum sKlotho was measured by ELISA.Demographic data,including age,gender and comorbid conditions,were obtained from their medical histories,and parameters including calcium,phosphorus and albumin were assessed.The occurrence time of nonfatal CVD and all-cause mortality were recorded during the 60 months follow-up.MHD patients were categorized into four groups according to the quartiles of sKlotho:group Ⅰ (sKlotho ＜ 361.34 ng/L),group Ⅱ (361.34 ng/L≤sKlotho＜ 398.81 ng/L),group Ⅲ (398.81 ng/L≤sKlotho＜445.99 ng/L) and group Ⅳ (sKlotho≥445.99 ng/L).Spearman correlation analysis and binary Logistic regression analysis were used to test the association between sKlotho and nonfatal CVD events.The impacts of sKlotho on all-cause mortality and CVD mortality were assessed by Kaplan-Meier method with log-rank test.Cox regression model was applied to evaluate the effect of sKlotho on MHD patients outcomes.Results All 132 MHD patients had sKlotho ranging from 304.02 ng/L to 550.62 ng/L.And 87 patients suffered from nonfatal CVD,with 192 episodes of nonfatal CVD during the follow-up period.The sKlotho had negative correlations with coronary artery disease (r=-0.286,P=0.001),congestive heart failure (r=--0.190,P=0.029),cerebrovascular accident (r=-0.240,P=0.006) and peripheral arterial occlusion (r=-0.243,P=0.005).The sKlotho were risk factors of coronary artery disease (OR=0.989,P=0.023) and peripheral artery occlusion (OR=0.988,P=0.046).35 patients died in the follow-up period,including 27 death from CVD.The all-cause mortality and CYD mortality rates were significantly different among four groups (P=0.036,P=0.047).Survival rates of all-cause death and CVD death varied among four groups (x2=8.076,P=0.044;X2=7.866,P=0.049).Patients in group Ⅳ had higher survival rates of allcause death and CVD death than those in group Ⅰ and group Ⅱ (all P ＜ 0.05).Multivariate Cox regression analyses revealed diabetes and age were independent risk factors for all-cause mortality and CVD mortality (all P ＜ 0.05),but sKlotho was not associated with the poor prognosis (HR=0.996,P=0.256;HR=0.996,P=0.287).Conclusions Patients with lower sKlotho have worse nonfatal CVD ratio,especially coronary artery disease and peripheral arterial occlusion.Reduced serum sKlotho is associated with all-cause and CVD mortality,but sKlotho is still not a predictive indicator of prognosis of MHD patients.
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Objective To evaluate the relationship between serum magnesium and coronary artery calcification (CAC) and their associated factors.Methods 131 patients with chronic kidney disease on regular hemodialysis (HD) were recruited into this study from December 2014 to December 2015 in our center.Demographic and clinical data of selected patients were collected.Serum fibroblast growth factor 23 (FGF-23) level was quantified by enzyme linked immunosorbent assay(ELISA).Quantification of coronary artery calcification score (CACs) was determined by multi-slice spiral computed tomography (MSCT).The relationships between serum magnesium and FGF-23 level,CACs,demographic and clinical data were investigated.Results Patients were divided into low serum magnesium group,normal serum magnesium group and high serum magnesium group according to their serum magnesium levels.There were significant differences in the distribution of diabetes history,serum phosphorus,serum albumin,serum pre albumin,serum uric acid among these three groups(P ＜ 0.05).A significant positive correlation was confirmed between serum magnesium level and serum albumin,serum pre albumin,serun phosphorus and serum uric acid by Pearson correlation analysis and Spearman correlation analysis (r=0.389,0.234,0.200,0.234,P=0.000,0.007,0.022,0.007,respectively).According to the degree of CAC,all maintenance hemodialysis (MHD) patients were divided into non-calcification group,low calcification group,moderate calcification group and high calcification group,and there were significant differences in the distribution of the age,serum phosphorus,serum magnesium,FGF-23 levels among these groups (P ＜ 0.05).Spearman correlation analysis showed that CACs was positively correlated with age,FGF-23,serum phosphorus (r=0.309,0.277,0.180,P=0.000,0.001,0.040,respectively),while negatively correlated with serum magnesium level (r=-0.238,P=0.006) in patients with MHD.The independent risk factors of CACs were aging,high level of FGF-23 in MHD patients by using ordinal logistic regression.However,Hypermagnesemia was a protective factor.Conclusions The history of diabetes,low serum albumin,phosphorus metabolism disorder and CAC are associated with hypomagnesemia in MHD patients.In MHD patients,aging as well as high level of FGF-23 are the risk factors of CAC,and hypermagnesemia is a protective factor of CAC.
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Objective To investigate the clinical efficacy and adverse events of methimazole ( MMI ) treatment for children with hyperthyroidism, and to identify the predictors of remission and relapse. Methods A total of379children(260girlsand119boys)diagnosedwithhyperthyroidismandtreatedbyMMIinGuangzhouWomenand Children's Medical Center from March, 2004 to July, 2014 were retrospectively analyzed. The average age at diagnosiswas(9.3±2.3)years(range2.0~15.9years). Results AftertreatmentwithMMIfor3and6months, the thyroid functions of 96. 3%(365/379) and 98. 9%(375/379) patients returned to normal, respectively. By the end of this study, 256(67. 5%) patients continued to use MMI treatment and 44 patients(11. 6%) dropped out. 79 patients(20. 8%) achieved remission, 35 patients (44. 3%) of whom experienced a later relapse. Children who achieved constant remission had significantly lower FT3 and FT4 levels at diagnosis compared with the relapsed children(P<0. 05 or P<0. 01). It was more likely to remain long-term remission for children turned to be euthyroid within 3 months after initiating MMI treatment(P<0. 05). The relieved patients with family history of thyroid diseases weremorelikelytoberelapsed(P<0.05). Therewerenosignificantdifferencesinage,gender,exophthalmos, initial goiter size, thyroid peroxidase autoantibody, and thyroglobulin antibody levels between the relieved and relapsed patients. The overall incidence of adverse events associated with MMI was 27. 7%, mainly elevated alanine aminotransferase, bilirubin, and neutropenia. Most(66. 7%) of adverse events occurred within the first three months of MMI treatment. Conclusion MMI has a good effect on pediatric hyperthyroidism, with low remission and high relapse rate. The low thyroid hormone concentrations at diagnosis and normalization of thyroid function within three months seem to be useful predictors of remission. Vigilance is needed concerning MMI-associated adverse events throughout the MMI treatment period, especially during the first trimester of MMI initiation.
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Objective To understand the clinical and molecular characteristics of children with Prader-Willi syndrome (PWS) in South China.Methods Clinical and molecular data of children diagnosed as PWS by Methylation-specific PCR(MS-PCR) and/or Array Comparative Genomic Hybridization(Array-CGH)in Guangzhou Women and Children's Medical Center from November 2012 to November 2014 were analyzed.Results A total of 27 children diagnosed as PWS were included in this study,including 21 cases diagnosed by Array Comparative Genomic Hybridization (Array-CGH) and 13 cases diagnosed by methylation-specific PC R (MS-PCR).Within the 27 cases,13 cases were male(48.1％) and 14 cases were female(51.9％).The age on diagnosis was from 16 days to 16 years old.MS-PCR was performed in 13 cases,7 cases of them also performed Array-CGH,both of them showed a 174 bp fragment from the methylated allele and a 100 bp fragment from the unmethylated allele.Array-CGH analysis was performed in 21 cases,paternal deletion in 18 cases and mean interstitial deletions measure (5.48 ± 0.51) Mb in size,paternal duplication in 2 cases,loss of heterozygosity measure approximately 79.58 Mb in 1 case.Eighteen simple chromosome deletion cases were divided into 6 Del Ⅰ and 12 Del Ⅱ according to the location of Array-CGH and query the database to DECIPHER(Database of Chromosomal Imbalance and Phenotype in Humans Using Ensembl Resources).The major phenotype included central hypotonia and feeding difficulty in all cases (100.0％),hypogonadism in 25 cases (92.6％),weak crying in 22 cases(81.5％),and hypopigmentation in 22 cases(81.5％).Fourteen cases beyond 1 year old had varied degrees of development disability and behavioral and psychiatric disturbance:speech articulation defects in 13 cases(92.9％),hyperphagia and weight gain too fast in 13 cases(92.9％) when they were between 1 to 6 years old[(2.80 ± 1.32) years old],and obesity in 12 cases (85.7％).Conclusions For PWS children in South China,there is no statistically significant difference in the clinical manifestation between Del Ⅰ and Del Ⅱ.PWS children in South China have typical clinical characteristics,which can be used as a further screening indication to implement molecular diagnostics.
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Objective To explore the association between serum FGF23 and Klotho protein,and bone mineral density in maintenance hemodialysis (MHD) patients.Methods A total of 125 MHD patients admitted in the Hospital between January 2015 and November 2015 was enrolled.Their bone mineral densities of femur neck and lumbar spine were studied by dual-energy X-ray absorptiometry.These patients were divided into three groups as normal,osteopenic and osteoporotic,according to World Health Organization criteria based on bone mineral density T scores.Levels of serum FGF23,Klotho protein and 1,25(OH)2VitD3 were measured by ELISA.The parameters including calcium,phosphorus,and parathyroid hormone were assessed.Results The incidences of osteopenia and osteoporosis at the femur neck and lumbar spine in MHD patients were 82.40％ and 56.00％ respectively.No significant difference was found in the levels of serum FGF23 among normal,osteopenic and osteoporotic groups on the basis of femur neck and lumbar spine bone mineral density (P ＞ 0.05).No correlation was found between FGF23 and bone mineral density.There however were significant differences in the levels of serum Klotho protein among three groups on the basis of femoral neck bone mineral density (P ＜ 0.05).And the levels of Klotho protein in the osteoporotic group [(387.172±54.137) ng/L] were significantly decreased than those in normal group [(429.883±41.776)ng/L] and osteopenic group [(410.598±61.056) ng/L] (P ＜ 0.05).There were also significant differences in the levels of serum Klotho protein among three groups in terms of lumbar spine bone mineral density (P ＜ 0.05),while the levels of Klotho protein in the osteopenic group [(387.263 ± 53.255) ng/L] were significantly decreased than those in normal group [(417.108±56.179) ng/L] (P＜ 0.05).A positive correlation was found between Klotho protein and bone mineral densities of femur neck and lumbar spine.Multiple linear regression analysis showed that one of the main factors influencing the degree of bone mineral density in MHD patients was Klotho protein.Conclusions CKD-MBD with low BMD is common and widespread in hemodialysis patients.FGF23 has no direct effect on bone mineral density in MHD patients;while Klotho protein is correlated with the severity of bone mineral density.High-level Klotho protein may reduce the severity of CKD-MBD with low BMD in MHD patients.
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Objective To conduct comparative study on the analgesic and anti-inflammatory effects as well as the acute toxicity of yunaconitine and 8-deacetyl-yunaconitine isolated from the processed products of Aconiti Knsnezoffii Radix.Methods The methods of hot plate test and writhing test were used to evaluate the analgesic effect. Anti-inflammation action was observed by the models of auricle swelling caused by dimethylbenzene. LD50 was determined by the method of Bliss.Results Yunaconitine and 8-deacetyl-yunaconitine have analgesia effect on the pain caused by hot-plate, but there were no statistically significant difference. The pain caused by acetic acid had obvious analgesic action. High and low dose of yunaconitine could significantly reduce the number of mice body torsion and extend the incubation period of pain in mice. The effect of 8-deacetyl-yunaconitine was remarkable only in the high dose. Compared with solvent group, there were little differences in inhibiting effect of auricle swelling caused by dimethylbenzene, and anti-inflammatory action was not exact. The poisonousness of yunaconitine was nearly 20 times of 8-deacetyl-yunaconitine.Conclusion Yunaconitine and 8-deacetyl-yunaconitine may be the analgesic medicine for peripheral analgesic effect. The poisonousness of 8-deacetyl-yunaconitine is less than yunaconitine, the effect is remarkable to the pain caused by acetic acid, and the security is high.
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Objective To investigate the factors correlated to coronary artery calcification (CAC) in maintenance hemodialysis (MHD) patients.Methods This study included 132 patients(54 females,78 males),aged 26-94 years,who were on hemodialysis for 10-204 months(median dialysis duration 51.00 months).The parameters including calcium,phosphorus,parathyroid hormone,total cholesterol,low density lipoprotein,triglycerides,C-reactive protein (CRP),klotho,and so on were assessed.Quantification of CAC was determined by multi-slice spiral computed tomography (MSCT),known as the coronary artery calcification score (CACs).Results Ninety-two patients (69.70％) had CAC,with CACs ranging from 0 to 13 450.20.More than 30％ patients experienced one even a variety of cardiovascular and cerebrovascular diseases.A positive correlation was observed between the degree of CAC and the incidence of cardiovascular and cerebrovascular diseases.Whereas a positive correlation existed between CACs and age (r=0.347,P=0.000),duration of hemodialysis (r=0.245,P=0.005),systolic blood pressure (r=0.184,P=0.034),diabetes history (r=0.211,P=0.015),phosphorus (r=0.262,P=0.002),calcium-phosphorus product (r=0.247,P=0.004);and a negative correlation between CACs and klotho level (r=-0.294,P=0.001).Multivariate logistic regression analysis showed that the main factor influencing the degree of CAC in MHD patients was age.Conclusions CAC is common and widespread in hemodialysis patients,who are often accompanied by cardiovascular and cerebrovascular diseases.The prevalence rate of cardiovascular and cerebrovascular diseases increases with the aggravation of CAC degree.Age,duration of hemodialysis,systolic blood pressure,diabetes history,disturbance of calcium and phosphorus metabolism and klotho are correlated with the severity of CAC.Age is an independent risk factor of CAC degree.
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<p><b>INTRODUCTION</b>We conducted a biomechanical study comparing cerclage wiring using a power tool with the traditional manual method.</p><p><b>MATERIALS AND METHODS</b>Our study consisted of 4 experimental arms based on the method of fixation and diameter of wires. The 4 arms were: 1) power tool method using 0.8 mm cerclage wires, 2) power tool method using 1.0 mm cerclage wires, 3) conventional manual method using 0.8 mm cerclage wires, and 4) conventional manual method using 1.0 mm cerclage wires. Synthetic femur bones were employed in our study. Six specimens were prepared for each arm. Each specimen was cut lengthwise and pressure sensors were placed in between. For the power tool method, while maintaining tension, wires were coiled using the Colibri power tool until just before secondary coiling occurred. For the conventional manual method, each specimen was compressed by plier twisting for 10 rounds, while maintaining tension. Cerclaging and data recording was done thrice for each specimen, giving a total of 18 readings per arm. Peak and steady-state forces were recorded.</p><p><b>RESULTS</b>There was no significant difference between the peak forces recorded between the power drill and manual methods. The steady-state forces achieved using the power tool method were significantly higher than that achieved in the manual fixation method (0.8 mm wires: 54.89N vs 27.26N, P = 0.037; 1.0 mm wires: 71.59N vs 39.66N, P = 0.025).</p><p><b>CONCLUSION</b>The power tool method achieved a superior steady-state force of compression across the fracture site for both 0.8 mm and 1 mm wires.</p>
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Humans , Biomechanical Phenomena , Bone Wires , Femur , General Surgery , Fracture Fixation, Internal , Methods , Models, AnatomicABSTRACT
<p><b>OBJECTIVE</b>Mucopolysaccharidosis type VI (MPS VI) or Maroteaux-Lamy syndrome is an autosomal recessive lysosomal storage disease caused by a deficiency of arylsulfatase B(ARSB), which is required in the degradation of dermatan sulfate and chondroitin sulfate. The deficiency of ARSB leads to an accumulation of dermatan sulfate and chondroitin sulfate in lysosomes and gross excretion in the urine.Few articles about clinical study and ARSB gene mutation analysis of Chinese MPS VI patients were published. This study aimed to explore the clinical features and characteristics of ARSB gene in Chinese children with MPS VI.</p><p><b>METHOD</b>Thirteen children were diagnosed as MPS VI by ARSB enzyme activity determination during the period from 2009 to 2013. Their clinical features, radiological findings and urine glycosaminoglycan (GAG) levels were retrospectively reviewed. Direct sequencing was used to identify any mutation in the ARSB gene.</p><p><b>RESULT</b>Thirteen children were diagnosed at the average age of (3.9 ± 2.2) years with 6 male and 7 female. All of these children presented with severe form and onset at an early age of (1.5 ± 0.8) years.Other clinical features included coarse facies, short stature, skeleton deformity, corneal clouding, hepatosplenomegaly with normal intelligence. The radiological findings in all children were characteristic of dysostosis multiplex, like abnormal development of vertebral bodies of the spine, campylorrhachia and paddle-shaped widened ribs. The MRI in case 2 showed cervical cord compression and multiple cysts degeneration in the corona radiate, cella lateralis and callosum.High urine GAG levels were detected, (307.10 ± 112.14) mg/L (Normally below 70 mg/L) and (722.28 ± 245.68) µg/mg creatinine. The ARSB enzyme activity in leukocytes was low, (13.29 ± 6.22) nmol/(mg×h) [Normal range (47-169) nmol/(mg×h)] by fluorogenic assay and (0.24 ± 0.18) U/g [Normal range (1.01-11.47) U/g] by colorimetric assay. A total of 11 mutations were identified by molecular analysis, including seven previously reported mutations (p.L72R, p.G167R, p.G303E, p.F399L, p. T442M, p.Y255X and p.R327X) and four novel mutations (p.Y175D, p.S403X, p.S464X and large deletion including ex. 2, 3). The c.1197C>G (p.F399L) mutation was the most common mutation in this study (31%).</p><p><b>CONCLUSION</b>The severe form of MPS VI is characterized by early onset and rapid illness progression. Both the radiological findings and increased urine GAG are important clues to diagnose MPS VI.Large decrease or absence of ARSB activity is diagnostic for MPS VI.Four novel mutations of ARSB gene were identified. The reported mutation c.1197C>G (p.F399L) was the hot-spot mutation in this study.</p>
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Child , Child, Preschool , Female , Humans , Infant , Male , Bone and Bones , Diagnostic Imaging , Pathology , Brain , Pathology , Exons , Genetics , Glycosaminoglycans , Urine , Magnetic Resonance Imaging , Mucopolysaccharidosis VI , Diagnosis , Genetics , Mutation , N-Acetylgalactosamine-4-Sulfatase , Genetics , Metabolism , Polymerase Chain Reaction , Radiography , Retrospective Studies , Sequence Analysis, DNAABSTRACT
Objective To study the relationship between serum retinal binding protein 4 (RBP4) level and acute cerebral infarction,and to analyze the correlation between RBP4 level and the related risk factors of acute cerebral infarction.Methods One hundred and sixty patients with acute cerebral infarction were chosen as observation group,and 80 cases of contemporaneous healthy examined people were selected as control group.The levels of serum RBP4,body mass index (BMI),fasting blood glucose (FBG),fasting serum insulin (FINS),glycosylated hemoglobin (HbA1c),total cholesterol (TC),triglyceride (TG),high density lipoprotein cholesterol (HDL-C),low density lipoprotein cholesterol (LDL-C) and blood pressure after admission of each group were measured.Statistic analysis was processed between the level of serum RBP4 and acute cerebral infarction or related risk factors.Results The level of serum RBP4 in observation group was increased compared with that in control group [(41.68 ± 5.12) mg/L vs.(23.84 ± 3.26) mg/L],and there was significant difference (P ＜ 0.01).The Pearson relative analysis showed positive correlation between the level of serum RBP4 and related risk factors of acute cerebral infarction FBG,FINS,LDL-C,BMI,systolic pressure.The Logistic stepwise regression analysis suggested that BMI,LDL-C and age were independently associated with the level of serum RBP4.Conclusion RBP4 plays an important role in the incidence of acute cerebral infarction,high RBP4 level may be one of the dangerous factors of acute cerebral infarction patients and could provide reference to prevents the cerebral infarction.
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Objective To examine the correlation of serum neutrophil gelatinase-associated lipocalin (NGAL) with dialysis adequacy,microinflammatory state and iron metabolism,and to assess the value of NGAL in identifying the dialysis adequacy in maintenance hemodialysis (MHD) patients.Methods A total of 150 MHD patients and 50 healthy control people were enrolled in the study.Clinical data were collected and serum NGAL,CRP,transferrin saturation,serum ferritin were measured.MHD patients were divided into adequacy group and non-adequacy group according to spKt/V value.Serum NGAL between two groups was compared and correlation of NGAL with spKt/V,inflammatory factors,and other clinical indexes was examined by Pearson,multivariate regression Logistic model and area under curve (AUC) of ROC.MHD patients were followed up for 3 months.Similar correlation analysis was carried out before and after follow up between two groups.Results Serum NGAL of MHD patients was higher compared to healthy people [(445.45±50.34) μg/L vs (50.02 ±6.45) μg/L].Among 150 MHD patients,95 were classified into adequacy group,while 55 into non-adequacy group according to spKt/V value.Serum NGAL was significantly higher in adequacy group as compared to non-adequacy group [(589.14±56.34) μg/L vs (360.13±46.23) μg/L,P＜0.05].Serum NGAL was positively correlated to spKt/V,CRP,TSAT [r=0.652,0.825,0.785,all P＜0.05],but not to serum ferritin.Serum NGAL was also correlated to spKt/V,CRP,TSAT by multivariate regression Logistic model.AUC showed that NGAL level could reflex the condition of dialysis adequacy.During the follow up,all adequacy patients remained the same adequacy status.Thirty-eight out of 55 non-adequacy patients were qualified for complete adequacy after intervention.The serum NGAL before and after intervention was (368.14±56.21) μg/L and (360.56±46.23) μg/L respectively without significant difference.Conclusions Serum NGAL is obviously higher in MHD patients with dialysis adequacy compared to those without dialysis adequacy.Serum NGAL is positively correlated to spKt/V,CRP,TSAT.NGAL can reflex the condition of dialysis adequate in MHD patients.
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Objective: To explore the reasonable compatibility of anesthetics in painless small intestinal endoscopy by comparing the influence of different target plasma propofol concentrations combined with remifentanil on anesthesia effects and respiratory and circulatory functions.Methods: Two hundred patients requiring small intestinal endoscopy were randomly divided into 4 groups,Group Ⅰ given 1% propofol only,with the target plasma propofol concentration set at 4.5 ?g/ml,and Group Ⅱ,Ⅲ and Ⅳ receiving 30-second injection of remifentanil(0.3 ?g/kg) 1 min before target-controlled infusion(TCI) of propofol,with the target plasma propofol concentration set at 3.0,3.5 and 4.0 ?g/ml,respectively.The mean arterial pressure(MAP),heart rate(HR),pulse oxygenation(SpO2),induction time,awakening time,orientation recovery time and the incidence of patients' body motion were observed before anesthesia,at the vanishment of the eyelash reflex,while the small intestine endoscope passing through the Treitz ligament or ileoceal valve and after endoscopy.Results: Compared with baseline values,MAP and HR were significantly decreased in all groups when the eyelash reflex vanished,markedly increased in Group Ⅰ,although not so obvious in Groups Ⅱ and Ⅲ,when the small intestinal endoscope passed through the Treitz ligament or ileoceal valve,and greatly reduced at all time points in Group Ⅳ,with difficult entrance of the small intestine endoscope due to low tension of the intestinal cavity.Comparatively,the induction time was shorter and the awakening time and orientation recovery time were longer in Group Ⅰ,the total dose of propofol and the incidence of body motion notably decreased in Groups Ⅱand Ⅲ,and the incidence of respiratory depression obviously increased in Group Ⅳ.Conclusion: Propofol TCI combined with remifentanil can be used safely and effectively in painless small intestinal endoscopy.Target plasma propofol concentration(3.5 ?g/ml) in combination with remifentanil(0.3 ?g/kg) gives more efficient anesthesia,lower incidence of body motion and respiratory suppression and better recovery.Therefore,it is the suitable anesthetic compatibility in painless small intestinal endoscopy.
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Objective: To investigate the analgesic effect and impact of lornoxicam on the expression of plasma IL-6 and IL-10 in patients undergoing upper abdominal surgery.Methods:Sixty patients undergoing upper abdominal surgery were randomly allocated into three groups,morphine group(M,n=20),postoperative lornoxicam group(L,n=20) and preemptive lornoxicam group(P,n=20).For group M the subjects received patient controlled intravenous analgesia(PCIA) with morphine(loading dose 0.05 mg/kg,bolus 1 mg,lockout time 10 min,background dose 0 mg) after the surgery.While in group L,8 mg lornoxiam was administered at the end of the surgery,then the same morphine PCIA scheme as in group M was used in combination with intermittent intravenous lornoxiam(8 mg per injection) at 12,24 and 36 h after the surgery.Except that the first 8 mg lornoxicam was injected 30 min before the operation,the analgesic paradigm of group P was similar to group L.The analgesic effect assessed by VAS at rest,the consumed dosage of morphine,and the adverse effects as nausea and vomiting,were recorded at 4,8,12,24 and 48 h.Furthermore,2 ml of the venous blood was drawn before the induction of anesthesia 2,6,12,and 24 h after the surgery to measure the levels of interleukin 6(IL-6) and interleukin 10(IL-10).Results: During the 48 h observation,the VAS at rest was not statistically significant in the three groups,but more morphine was consumed in group M than in group L and group P.There was no difference among the three groups in the incidence of such adverse effects as nausea or vomiting.The basic levels of IL-6 and IL-10 were too low to be measured.The concentrations of IL-10 and IL-6 reached the peak at 2 and 6 h after surgery respectively,and the level of IL-10 in group M was significantly lower than in groups L and P at 2 h.In contrast,the level of IL-6 in group M was significantly higher than in group L and group P at 6 h,and even higher than in group P at 12 h. Conclusion: Lornoxicam,especially when administered before upper abdominal operation,could significantly decrease the dose of morphine for postoperative analgesia and attenuate the inflammatory cytokine response after surgery.
ABSTRACT
Objective To explore the relationship of phosphatase and tensin homology (PTEN) deleted on chromosome ten with the apoptosis of hepatic stellate cells (HSCs) in liver tissues of rats with hepatic fibrosis induced by bile stagnation.Methods Fifty adult male SD rats were randomly divided into model group (n=40) and sham operation group (n=10).The model of hepatic fibrosis was reproduced in model group by common bile duct ligation (BDL).Liver tissue of rats in model group (1,2,3 and 4 weeks after BDL) and sham operation group were obtained.PTEN expression in liver tissue was detected by immunohistochemistry.Apoptosis of HSC was determined by dual staining of terminal deoxynucleotidy transferase UTP-nick end labeling (TUNEL) and ?-SMA immunohistochemistry.Results Only a few apoptotic HSCs were found in normal livers.With the development of liver fibrosis,the expression of PTEN decreased gradually (P