Congresses as Topic ; Humans ; Lysosomal Storage Diseases

Adenosine Triphosphatases ; genetics ; Alanine Transaminase ; blood ; Cation Transport Proteins ; genetics ; Ceruloplasmin ; metabolism ; Child ; Copper ; blood ; urine ; Copper-transporting ATPases ; Cornea ; pathology ; Diagnosis, Differential ; Genetic Testing ; Hepatolenticular Degeneration ; blood ; diagnosis ; genetics ; metabolism ; Humans ; Liver ; metabolism ; pathology ; Liver Function Tests ; Mutation

Objective To study the role of bacterial collagenase in wound healing with observing capillary neovascularization, epidermis regeneration and collagen fiber reparation in vivo. Method A total of 28 New Zealand rabbits were made into models of superficial soft tissue trauma and divided into two groups. In the experimental group, the right limbs of rabbits were traumatized intentionally in the same length and depth, and their wounds were treated with bacterial collagenase liquor 133 MIU/mL, and the wounds were sutured. Similarly, in the control group, the left limbs of the rabbits were modeled in the same way and their wounds were managed with physiological saline instead and closed. Their wounds were observed continually 1, 2, 3, 5, 7, 10 and 14 days after traumatized. Data were analyzed statistically by using One-way ANOVA, LSD-test, and Wilcoxon rank test. Results There were no significant differences in capillary neovascularization and epidermis regeneration between two groups ( F = 0.12, P = 0.740 and F = 0.95, P = 0.33, respectively), but significant difference in collagen fiber reparation was found between two groups (F = 6.63, P = 0.01). Conclusions In the progress of wound healing, bacterial collagenase can improve the collagen fiber reparation without effects on the new capillary vasculogenesis and epidermis regeneration. The bacterial collagenase does not play a positive role in wound healing.

Objective Intranasal drops of dexmedetomidine can induce conscious sedation in children .This study was to com-pare the sedative effects of different doses of intranasal dexmedetomidine in different age groups of children patients . Methods We e-qually randomized 90 hernia children waiting for elective unilateral inguinal surgery ( ASAⅠ-Ⅱ) to groupsⅠ,ⅡandⅢ.At 35 minutes be-fore surgery, we obtained the heart rate (HR), mean artery pressure (MAP) of all the patients.The children in groupsⅠandⅡreceived nasal instillation of dexmedetomidine at 1 and 2μg/kg respectively, while those in groupⅢ0.4 mL of normal saline, followed by sevoflu-rane induction, intravenous injection of sufentanil (0.2μg/kg), and laryngeal mask airway anesthesia with sevoflurane .We recorded HR, MAP before medication, at 10 min, 20 min and 30 min before mediation, right after surgery, and at the recovery to consciousness . We also recorded the sedation onset time and post-surgery recovery time and assessed sedation satisfaction using the Ramsay score . Results The rates of satisfactory preoperative sedation were 50 and 63% in groupsⅠandⅡ, significantly higher than 10%in groupⅢ(P<0.05). In the 1-4-year group, the sedation satisfaction rate , sedation onset
time and post-surgery recovery time showed no significant differences between groupsⅠandⅡ(P>0.05).In the 5-8-year group, seda-tion satisfaction rate was markedly higher in groupⅡthan in groupsⅢ(77%vs 22%, P<0.05) andⅠ(77%vs 45%) . Conclusion At 1 and 2μg/kg, intranasal drops of dexmedetomidine produces similar rates of satisfactory sedation in children aged 1-4 years, and at 2μg/kg can achieve an even higher rate of satisfactory sedation without adverse hemodynamic effects in those aged 5-8 years.

Objective To discuss MRI scan in the diagnosis and clinic-pathology of ovarian cystoid-material lesion.Methods We retrospectively analyzed 41 patients with clinic pathologically-proved in 2005~2007,and analyzed US,MR scan and Gd-DTPA in the diagnosis of ovarian cystoid-material lesion.Results The clinical date of the 87 patients were completed.After operation,41 of them were confirmed by pathology to be cystiform or parenchymal process,with age 16~76,average 56,of which,21 cases,were tumour-like lesions occupying 51.2%,18 cases were timorous lesions,occupying 30.2%,13 cases weer ovarian cystadenoma,occupying 30.2%,and 2case ueve other disense,occupying 4.65%.Conclusion The benign often present as ovarian cystoid-material lesion.MRI scan can show the inner structure of ovary lesion.

To observe the clinical efficacy of Artisan iris-clip intraocular lens ( lOL) implantation on cataract with a wide range of lens dislocation.
●METHODS: Twenty-four hospital patients (28 eyes) of cataract with lens subluxation by trauma (20 patients, 20 eyes) and Marfan's syndrome (4 patients, 8 eyes) during May 2007 to December 2011 were selected. All of them underwent phacoemulsification or an anterior vitrectomy and Artisan iris - clip lOL implantation at stage Ⅰ. Postoperative visual acuity, corneal endothelial cell count, intraocular lens position and postoperative complications were observed. Follow-up time was 6mo.
●RESULTS: Compared with the preoperative, postoperative best corrected visual acuity improved to varying degrees, visual acuity of 0. 1-0. 5 was in 20 eyes, 0. 5-1. 0 in 8 eyes. After followed up 1-6mo, no artificial lens shift, iris inflammation, iris atrophy and corneal decompensation and other complications were observed.
●CONCLUSlON: Cataract surgery with a wide range of lens dislocation is difficult, but as long as surgeons who have a wealth of clinical experience and superb skills, still can carry out phacoemulsification surgery or anterior vitrectomy and Artisan iris - clip lOL implantation and obtain a better outcome.

Objective To compare the effect and safety of propofol with intranasal dexmedetomidine in analgesic enteroscopy for children.Methods Ninety children undergoing analgesic enteroscopy were ran-domly assigned to 3 groups.Intranasal dexmedetomidine followed by propofol was administered in the observ-ing group;sufentanil and followed by propofol was intravenously given to the control group A;propofol was given to the control group B.The scale of anesthetic effect,HR,RR,MAP and SpO2 30 min after intranasal administration were monitored.The intranasal sedation satisfaction rate,the dose of propofol,wake-up time, hospitalization,hemodynamics,adverse reaction and complication after anesthesia were recorded.Results Intranasal sedation satisfaction rate of the observing group was 83.3%(25 /30).The anesthetic effect in ob-serving group(excellent/good /bad:24 /6 /0)showed no significant difference from that in the control group A (excellent/good /bad:25 /5 /0),but better than that in the control group B(excellent/good /bad:12 /14 /4) (P <0.05).The amount of propofol in the observing group(96.2 ±5.3)mg showed no significant difference from that of group A(93.7 ±4.6)mg,but less than group B[(121.1 ±4.9)mg,P <0.05].The time of re-covery of infants in the control group A[(14.2 ±5.5)min]and the control group B[(13.7 ±3.9)min] were longer than that in observing group[(7.9 ±4.1)min,P <0.05].The hospitalization time of observing group[(14.3 ±5.4)min]was shorter than those of group A[(27.5 ±3.7)min,P <0.05]and group B [(26.3 ±6.6)min,P <0.05].The incidences of breath suppression,glossoptosis,nausea and dizziness were the lowest in the observing group,compared with the two others (all P <0.05).Conclusion In-tranasal dexmedetomidine combined with propofol,increasing children compliance,decreasing the adverse reaction,is satisfactory and safe for children anesthesia.

Objective To investigate the relationship between neck circumference and metabolic syndrome(MS) and its components in type 2 diabetes Methods A total of 272 subjects with type 2 diabetes were recruited.According to the diagnostic criteria of MS,all subjects were divided into MS group and non-MS group.According to neck circumference tertile,all subjects were respectively divided into male and female of three groups to analyze the relationship between neck circumference and MS and its components.Male:Q1＜38.8 cm,Q2=(38.8-41.3) cm,Q3≥41.4 cm;female:Q1＜34.0 cm,Q2=34.0-36.3 cm,Q3≥36.4 cm.The optimal cut-off points of neck circumference were used to determine the MS by ROC curve.Results (1) Compared with non-MS group,subjects in MS group had significantly higher neck circumference((38.3±3.2)cm vs.(36.1±3.7) cm),waist circumference((94.1±8.4) cm vs.(82.7±10.8) cm),waist-hip ratio(0.93±0.06 vs.0.88± 0.08),body mass index ((26.2 ± 2.9) kg/m2 vs.(23.9 ± 3.5) kg/m2),fasting plasma glucose(9.0(6.7-11.5) mmol/L vs.7.7(5.9-10.2) mmol/L),systolic pressure(140(130-150) mmHg vs.130 (120-130) mmHg),diastolic pressure (80 (80-90) mmHg vs.80 (75-85) mmHg),triglycerides (1.9(1.4-2.8) mmol/L vs.1.2 (0.9-1.5) mmol/L),low-density lipoprotein cholesterol (LDL-C) ((3.3 ± 1.0)mmol/L vs.(3.0±0.9) mmol/L) and blood uric acid((322.7±90.1) mmol/L vs.(270.3±82.3) mmol/L),and the differences were significant(t or Z=-4.843,-8.622,-5.157,-5.592,-2.729,-5.723,-3.847,-6.856,-2.123,-4.545;P＜0.05).While the level of total cholesterol((2.7±2.8) mmol/L vs.(4.9±1.3)mmol/L) and high-density lipoprotein cholesterol (HDL-C) (1.0 (0.8-1.2) mmol/L vs.1.2 (1.1-1.5)mmol/L) were decreased,and the differences were significant (t or Z =-2.269,-7.068;P ＜ 0.05).(2)Compared with those with lowest tertiles(Q1 ＜38.8 cm) of neck circumference,male subjects with highest tertile (Q3≥41.4 cm) had higher level of waist circumference((97.8±8.6) cm vs.(89.9±8.5) cm),waist-hip rati (0.96±0.07 vs.0.93±0.06),body mass index((27.4±2.1) kg/m2 vs.(23.8±2.3) kg/m2),blood uric acid ((374.0±85.6) mmol/L vs.(305.8±73.6) mmol/L),and the differences were significant(P＜0.05).While age((52.1±10.7) years vs.(60.4±11.8) years) andHDL-C((1.0±0.3) mmol/Lvs.(1.2±0.5) mmol/L) were decreased,and the differences were significant (P＜0.05).Female subjects with higher tertile (Q3 ≥ 36.4 cm) had significantly higher level of waist circumference ((96.8 ± 10.1) cm vs.(79.5 ± 8.5) cm),waist-hip ratio(0.94±0.07 vs.0.86±0.06),body mass index((28.5±3.1) kg/m2 vs.(21.9±2.2) kg/m2),blood uric acid((295.2±94.3) mmol/L vs.(244.6±62.3) mmol/L),the frequency of MS(83.3％(40/48)vs.21.7％(10/46)) than with lower tertile(Q1＜34.0 cm).(3) Logistic regression analysis showed that neck circumference (β =0.187,P ＜ 0.001) was an independent risk factor of MS (y =0.187x-6.613).(4) The optimal cut-off points of neck circumference to MS were 40.1 cm (AUC =0.682,P=＞0.008) in male,and 35.3cm (AUC =0.766,P＜ 0.001) in female.Conclusion Neck circumference increases when type 2 diabetes patients with MS.Neck circumference is an independent risk factor for MS,which can be used as an indicator to evaluate MS.

Objective To observe the efficacy and safety of levofloxacin for the theatment of com-munity acquired pneumonia(CAP) in the elderly patients. Methods Thirty-six elderly inpatients with CAP between May 2005 and May 2007 were treated with levofloxacin at a dosage intravenously of 500 mg once a day for 5 to 14 days treatment. Results Streptococcus pneumoniae of multiple drugs-resistant were found in sputum of 22 patients,pseudomonas aeruginosa in 8 patients and haemophilus influenzae in 6 patients de-pending on the results of the sputum culture. The total clinical efficacy rate of levofloxacin was 75.0% and bacteria elimination rate was 82.1%,and 16.7% patients showed related side effect of diarrhea,skin-rash and kidney injury. Conclusions Levofloxacin is an effective with little side effect in treatment of CAP in the elderly.