Leukoencephalopathy with ataxia (LKPAT) is a rare autosomal recessive disorder caused by mutations of CLCN2 gene. LKPAT is clinically characterized by cerebellar ataxia, headache and cognition impairment. Brain magnetic resonance imaging showed characteristic hyperintensities along the pyramidal fiber tracts. Few cases have been reported so far. This article reported the clinical data of a 48 years old female patient with LKPAT for clinical reference.

To investigate the effect of calcium concentration in dialysate on the QT interval (QT) and QT interval dispersion (QTd) during hemodialysis(HD) in patients with end stage renal disease (ESRD), Ca 2+ concentrations of 1 25mmol/L (dCa 2+ 1 25), 1 5mmol/L (dCa 2+ 1 5) and 1 75mmol/L (dCa 2+ 1 75) in dialysate were given to 15 ESRD patients for five times. The QT and QTd were measured before and after the five HD sessions. The results showed: With the dCa 2+ 1 25 HD, serum Ca 2+ and plasma calcium were decreased (1 22?0 08 vs 1 17?0 07 mmol/L and 2 43?0 34 vs 2 28?0 16mmol/L, P 0 05). The changes in the QT correlated inversely with the changes in serum Ca 2+ and plasma calcium ( P

Objective To evaluate the clinical value of early alterations of carotid arterial intima-media thickness (IMT) and elastic function in patients with hypertriglyceridemia (HTG) using ultrasound radio-frequency technique.Methods 48 patients (triglyceride value ≥ 1.8 mmol/L) were chosen as HTG group.Quantitative intimamedia thickness (QIMT) and quantitative arterial stiffness (QAS) techniques were used to observe the alterations of carotid arterial IMT and elastic index,including diameter of carotid artery (D),distensibility coefficient (DC),compliance coefficient (CC),elasticity coefficient (α),elasticity coefficient (β) and pulse wave velocity (PWV).50 healthy subjects were recruited in control group.Differences of the above-mentioned parameters were analyzed,as well as the elastic index connections between the two groups.Results IMT,α,β,and PWV index of bilateral carotid arteries in HTG group was higher than that of the control group,while the DC and CC index was lower,and the differences was statistically significant (P＜0.05).Within the groups there were differences between the elastic indexes of bilateral carotid arteries.In control group and the HTG group:CCL＞CCR,αL＞αR,βL＞βR,PWVL＞PWVR.CC was negatively correlated to α,β and PWV (P＜0.05).Meanwhile,there was positive correlation among α,β and PWV (P＜0.05).The differences of D between two groups had no statistical significance (P＞0.05).Conclusions The abnormity of arterial occurs earlier than the morphological change,while the ultrasound radio-frequency technique can provide information of earlier alteration of common carotid arteries structure and elastic function in patients with HTG,which is a significant method of testing early alteration of carotid elastic in patients with HTG.

Objective To observe the therapeutical effect by using pedicle screw and interbody fusion cage,and compare with posterior interbody simple autogenous bone graft. Methods From August 2006 to January 2009,46 cases of lumbar spondylolisthesis patients were treated by using pedical screw internal fixation system, including 24 cases of cage-graft (group A), 22 cases of interbody simple autogenous bone graft (group B), efficacy evaluation using JOA evaluation standard, and measured lumbar olisthe reset rate, relative intervertebral space height and lumbosacral angle of recovery and observed fusion rate through the preoperative and postoperative follow-up X-ray films. Results All the patients' waist pain symptoms relieved after operative reduction. At 18 months of follow-up , group A of lumbar olisthe reset rate was 94.7%, relative intervertebral space height was (74.93 ± 7.85)% ,lumhosacral angle was 36.6° ± 3.6° ,meanwhile group B was 89.9%, (68.72 ± 12.40)%,39.3°± 5.6°. There were significant differences between two groups (P<0.05). Bone graft fusion rate in group A was 95.83% (23/24), in group B was 90.91%(20/22), there was significant difference between two groups (P < 0.05). Conclusion Pedicle screw system and interbody cage-graft in treatnent of lumber spondylolisthesis can effectively prevent the loss of reduction,mid and long-term effects are satisfactory,it is a stable and reliable method.

Objective Through the analysis of cases,to improve the cognition of clinicians on extracranial vas-cular embolism diseases(VED)in children.Methods The clinical information included incidence trend,thrombophi-lia,clinical features,treatment and prognosis of 29 children with VED,who were admitted from January 201 1 to Decem-ber 201 5 in Nanjing Children′s Hospital Affiliated to Nanjing Medical University,and retrospectively analyzed.Results The annual rate of VED increased from 0 to 1 .49 per 1 0 000 hospital admissions from January 201 1 to December 201 5.In 29 cases,there were 20 male and 9 female,with an average age of 6.03 years old (2 h -1 3.83 years old).In-fants accounted for >20%(6 /29 cases).The average age of the female,non infected group,rheumatic disease group and venous thrombosis group was higher than that of the male,the infection group,the non rheumatic disease group and the arterial embolization group,but there was no significant difference(all P >0.05).Thrombophilia were as follows:in-fection disease,heart disease,kidney disease syndrome,rheumatoid disease,tumor,and twins embolization syndrome. Risk factors included central venous line,immobility,dehydration,glucocorticoids use,etc.Heart disease was the leading cause of non thrombotic embolism,while other thrombophilic diseases resulted in thrombotic embolism.Pulmonary em-bolism was mainly seen in patients with severe pneumonia,especially mycoplasma pneumoniae pneumonia (MPP ).The older the age,the more typical clinical manifestations,and significantly elevated D dimer,fibrin/fibrinogen degradation products tips VED.Treatment depends on the degree of risk.Thirty -seven point five percent(3 /8 cases)of children with arterial embolism were less than 1 year old,and the mortality 25.00% (2 /8 cases)was significantly higher than that of venous thrombosis 1 7.65%(3 /1 7 cases)and 1 1 .76% (2 /1 7 cases).Conclusion Heart disease mainly cause arterial embolism and infection,while nephritic syndrome and rheumatic disease mainly cause venous embolism.Pulmona-ry embolism is mainly seen in children with severe pneumonia,especially MPP.The risk of arterial embolization is higher than that of venous embolism.

Objective To investigate the roles of fiberoptic bronchoscopy in diagnosis and treatment for infants with refractory and persistent wheezing. Methods From Jun. 2012 to Dec. 2013, 52 hospitalized children with age between four 4 months and 1 year old were recruited for ifberoptic bronchoscopy, who had been wheezing for at least four weeks and treated ineffectively with conventional anti-inlfammatory agents:budesonide and compound ipratropium bromide solution. Then, the pathogenesis of refractory and persistent wheezing was summarized based on clinical features, detection of CT imaging of three-dimensional airway reconstruction and cardiac CT, results of bronchoscopy inspection, and bronchoalveolar lavage lfuid culture. Results Among the 52 cases, 40 were with ground glass-like changes (76.92%) in pulmonary spiral CT testing, 4 with mosaic perfusion syndrome (7.69%), 8 with segmental pulmonary consolidation (15.38%), 8 with obstructive pulmonary emphysema (15.38%), and 1 with left primary bronchial foreign body. In addition, through bronchofibroscopy, there were 52 cases with imlfammation (100%),3 with tracheal stenosis (5.77%), 3 with left and/or right main bronchus stenosis of the external pressure, 18 with bronchomalacia(34.62%), 2 cases with foreign body (3.84%), one in trachea (1.92%), the other in left main bronchus (1.92%), 10 with bronchial mucus plug (19.23%), and 8 (15.38%) with congenital airway malformations (including 3 at tracheal bronchus, 1 at left upper lobe bronchial stenosis and 1 at bronchial Bridge). The culture of bronchoalveolar lavage lfuid were conducted for all patients. The positive rate of bronchoalveolar lavage lfuid was 9.62%(5/52 cases), including 2 cases with tip Escherichia coli, 2 with Haemophilus inlfuenzae, and 1 with Acinetobacter baumannii. Conclusions First, infection is the primary cause of refractory and persistent wheezing, which is persistent in airway resulted from multi-drug resistant bacteriua. Second, refractory and persistent wheezing is often caused by multi-factors including infection, congenital airway malformations, the endogenous and exogenous foreign body, cardiovascular malformation, etc. These factors often lead to dififcult wheezing control. The last, the diagnosis rate of the refractory and persistent wheezing can be improved by combination of ifberoptic bronchoscopy and lung spiral CT.

Objective To investigate the effectiveness of deep brain stimulation (DBS) treating Parkinson's disease.Methods Forty cases of Parkinson's disease were selected from March 2014 to August 2015.The clinical symptoms of these patients were described and quantitatively analyzed with Unified Parkinson's Disease Rating Scale (UPDRS) before and after the procedure of DBS surgery.Results After deep brain stimulation surgery,the symptoms including muscle stiffness,static tremor,bradykinesia were improved,UPDRS scores were significantly lower and the demanding dosage of Parkinson disease drugs such as L-dopa/benserazide and L-dopa/carbidopa were also reduced.Conclusion Deep brain stimulation for Parkinson's disease is safe and effective.It can obviously control the symptoms,reduce the dosage of oral drugs,and improve the quality of life.

Objective To investigate the clinic characteristics and treatment methods of coronary ectasia. Methods One hundred and fifty-four cases in Anzhen hospital during the last 10 years, whom with coronary aneurysm diagnosed by coronary artery angiogram and summarized the characteristics of symptom, sign, diagnosis and treatment. Results All patients were proved with coronary angiography. There were 112(72.7%)patients presenting angina, but with no specific manifestation in electrocardiogram, X-ray and echocardiogram.Thrombolysis in emergency were taken to 3 cases with acute myocardial infarction patients. One hundred and fifty-four cases were treated with aspirin or heparinizeation, and with nitroglycerin, Calcium channel blocker to dilate vessel. The followed up of 154 cases about 1-20 years, six cases were suffered with acute myocardial infarction. One of the 6 cases was reinfarction and one case was sudden death. Conclusions Angina is the main clinical manifestation and coronary angiography is the accurate diagnostic method. Long-term application with anticoagulant agent and vasodilator should be done to prevent coronary spasm and myocardial infarction. If acute myocardial infarction occurs, prompt thrombolysis should be used, if it is ineffective, coronary artery bypass grafting should be carried out.

Authors described the patterns,core measures and mechanisms of coordinated care delivery in the localities and analyzed initial outcomes of this practice.It is found that such practice has optimized distribution of regional healthcare resources,implemented first visit to primary institutions, and the care delivery to separate emergency from outpatient service.In addition,the paper analyzed problems found in localities in terms of policy,public hospitals and primary institutions,and proposed elements required for coordinated care delivery system to succeed,such as enhanced policy support, enhanced multi-departmental coordination,and public hospital reform to explore feasible approaches.

OBJECTIVE:To consolidate the results of special antibacterial drug rectification so as to improve the rational medi-cation of antibacterial drugs. METHODS:By using the method of retrospective analysis,the antibacterial drug application data in 2011(before rectification and initial stage of rectification),2012(middle stage thereof)and 2013(later stage thereof)were sum-marized,compared and analyzed. RESULTS:After special rectification(Jan. 2011 vs. Dec. 2013),the utilization rate of antibacte-rial drugs in perioperative period for class I incision decreased from 80.42% to 24.49%. That of antibacterial drugs in emergency treatment decreased from 53.79% to 36.23%. The examination rate of microbial samples in inpatients who would use special-grade antibacterial drugs increased from 16.12% to 94.17%. The use intensity of antibacterial drugs by inpatients decreased from 70.28 DDDs/(100 persons·d) to 27.44 DDDs/(100 persons·d). The utilization rate of antibacterial drugs by inpatients decreased from 65.99% to 46.45%. CONCLUSIONS:Special rectification has remarkable effects on rational clinical application of antibacterial drugs. Regulation and evaluation should be strengthened to maintain the effects and continuously improvement should be made.