Congenital long QT syndrome ( LQTS) is a cardiac ion channel dysfunction, leading to prolonged myocardial repolarization time. It is characterized by the typical ECG QT interval prolongation and torsades de pointes. It shows clinical recurrence of cardiogenic syncope and even lead to sudden death. Molecular genetic studies have revealed a total of 12 forms of congenital LQTS caused by mutations in genes of the potassium, sodium and calcium channels or membrane adapter located on chromosomes 3, 4, 7, 11, 12, 17, 20 and 21. This review summarized the studies of the pathogenesis of LQTS and gene-related treatments.

The widespread use of antibiotics has been effectively controlled serious life-threatening infections.The trituration,exploitation and use of antibiotics has been rapidly developed and applied to many fields.Due to the extensive use of various antibiotics,the emergences of drug-resistant bacteria and the rates of bacterial resistance have increased unceasingly which has been a common focus in the world.Resistant pathogen can reduce the effectiveness of antibiotics,and which is a serious threat to human health.De-escalation therapy of antibiotics can quickly and effectively control serious infections,and avoid bacterial resistance,and induce fungal infections.The rational use of antibiotics in pediatrics is very imperative,which includes strengthening management of antibiotics,and improving awareness and vigilance of the adverse reactions of antibiotics,and consummating specification of medication.Then,realize the correct,safe,rational medication of antibiotics.

Wistar rats randomly divided into groups A,B and C were killed at 2、3 and 12 days after transplantation,respectively.The pathological changes of the donor livers were observed by light microscope and electron microscope.The findings demonstrated that:(1)in the group A there was hepatocellular regeneration including hypertrophy of nuclei and nucleoli,and hyperplasia of mitochondrions and endoplasmic reticula;(2)in the group B the morphologic changes of the allografts involved in distinet fatty degencration and mitochondrial swelling of hepatic cells and peripheral vesscl wall infiltration by monocytes and lymphocytes;(3)in the group C hepatocellular loss with the massive tissue necrosis and fibroplasia occurred in the gratfs.From the above it was concluded that hepatocellular necrosis or loss related to the rejection 12 days after the transplantation might lead to the liver failure in the rats.

Bronchiolitis obliterans(BO) is chronic airflow obstruction syndrome characterized by small airway obstruction and airflow limitation.In children, BO is secondary to severe infection of respiratory tract, and its development is related with inflammation and immune response.At present, the diagnosis of BO mainly is depended on the clinical manifestation, lung function and examination results of high resolution computed tomography (HRCT) because of difficulty of lung biopsy.Pulmonary HRCT is a non-invasive examination, and worthy of generalization.Currently, BO has no accepted effective treatment,early diagnosis and treatment can block the progression of BO.Due to lack of realization of BO, doctors and parents fail to give enough attention, BO has a prolonged course and very poor prognosis.

Adult ; Humans ; Male ; Malondialdehyde ; blood ; Submarine Medicine ; Superoxide Dismutase ; blood ; Young Adult

Objective The study was to investigate the value of deduction and application of anterior compression index in evaluation of atlantoaxial dislocation and restoration. Method Twenty-eight cases of the control group and 28 cases of atlantoaxial dislocation treated with posterior restoration and screw-rod internal fixation technique before and af?ter surgery were recruited in this study and their data was retrospectively analyzed. All of the people underwent sagittal computerized tomography scan films. The anterior compression index was measured in all cases. Results The mean value of anterior compression index of the control group was 87.86±24.98. The mean value of anterior compression index of the preoperative patients was 230.44 ± 97.60 and the mean value of the postoperative patients was 106.27 ± 73.53. There was significant difference in those two parameters between the preoperative patients and the controls(t=-7.50,P<0.0001). There was no significant difference between the postoperative patients and the control group (t=-1.26, P=0.2171). Anteri?or compression index were significantly lower after surgical operation (t=10.35, compared with before, P<0.0001). Con?clusion Anterior compression index can be an excellent measurement tool for the assessment of relationship of atlas and axis in atlantoaxial dislocation patients before and after posterior restoration operation.

Objective:To determine the di-allelic polymorphisms of TNF gene and their association with Helicobacter Pylori(H.pylori) infection and gastroduodenal diseases in Chinese population with Han nationality.Methods:Two hundreds and ten patients with gastroduodenal diseases(73 chronic gastritis,78 duodenal ulcer and 59 noncardia gastric cancer) and 264 healthy controls were genotyped by the PCR-RFLP method for TNF-? 308,lymphotoxin-?(LT-?) Nco Ⅰ and AspH Ⅰ gene polymorphisms.H.pylori infection status was determined by a validated serological test.Results:H.pylori infection was detected in 90.5% of 210 patients and 62.1% of 264 healthy controls(P

Objective To observe the effects of marzulene(L-glutamine and sodium gualenate)on cellular immune function in patients with severe acute pancreatitis(SAP). Methods A prospective study method was conducted in which 80 patients with SAP were divided into trial group and control group(each 40 cases)according to the table of random number methods,and in the mean time 20 healthy volunteers were enrolled in the healthy control group. Both SAP groups received standard total parenteral nutrition(TPN)with same quantities of heat and nitrogen, and the trial group additionally accepted intravenous drip of marzulene 0.4 g?kg-1?d-1(once a day)for 7 days,while the control group received the same amount of normal saline intravenous drip for 7 days. One day before treatment and on the 7th day after treatment,the peripheral venous blood samples were collected. The T-lymphocyte proliferation activity was tested by methyl thiazolyl tetrazolium(MTT)assay,and the interleukin-2(IL-2)and interleukin-6(IL-6) secretion levels were detected by enzyme-linked immunosorbent assay(ELISA). Results Compared to the healthy control group,the levels of T lymphocyte proliferation activity〔absorbance(A)value〕and IL-2(ng/L)were markedly decreased and IL-6(ng/L)secretion level was obviously increased before treatment in the two SAP groups(P<0.05 or P<0.01). Compared to prior treatment,the levels of T lymphocyte proliferation activity and IL-2 were significantly increased after 7 days of treatment in the trial group(both P<0.05),and basically restored to normal levels,while in the control group they were progressively decreased(both P<0.05),the levels in trial group being significantly higher than those of the control group(T lymphocyte proliferation activity:1.08±0.27 vs. 0.43±0.25,IL-2:16.5±1.4 vs. 9.4±2.9,both P<0.05),but compared to prior treatment,the level of IL-6 was significantly decreased in the two SAP groups(both P<0.05),the level of IL-6 in control group was recovered to the level of healthy control group,and the level in trial group being significantly lower than that of the control group(18.8±4.5 vs. 22.3±3.1, P<0.05). Conclusions The patients with SAP have cellular immune dysfunction,the manifestations being the suppression of T lymphocyte proliferation and IL-2 and increase of IL-6 release. Early application of marzulene can help to improve the immune function of lymphocytes in patients with SAP.

Objective To analyze the clinical profiles of lupus mesenteric vasculitis (LMV).Methods Thirty-six SLE patients diagnosed with LMV and 72 SLE patients (1:2) without LMV at West China Hospital between October 2009 and January 2012 were enrolled.The imaging,clinical manifestation,laboratory examinations data were analyzed retrospectively.Quantitative differences were analyzed by the Student's t and Z tests and qualitative data were compared with Chi-square.P value less than 0.05 was considered as statistically significant.Results ① The prevalence of LMV in patients with SLE was 1.2％(36/3 051),while 47％(17/36) as the initial symptom of SLE.Thirty-six LMV patients were all female.The prevalence of abdominal pain was 78％(28/36),nausea or vomitting was 58％(21/36),abdominal distension was 50％(18/36),diarrhea was 47.2％ (17/36),intestinal perforation was 3％ (1/36) and incomplete obstruction was 8％ (3/36).Enhanced CT scan showed 81％(25/31) patients suffered from intestinal dilation and (or) mesenteric edema;SLEDAI (17±5),C3 (0.44±0.25) g/L,C4 (0.10±0.09) g/L,Z value on above items were 4.30,-3.85 and-2.94 respectively compared to SLE patients without LMV,and all P＜0.05,the differemces were significant.In addition,Also,the proportion of ANA and anti-ENA antibody showed no significant difference.68.6％(13/36) patients were also diagnosed with lupus nephritis;61％(22/36) patients were treated with methylprednisolone more than 200 mg/d resulting in relief of symptom.Conclusion Female patients with SLE may be more likely to suffer from LMV;Enhanced abdominal CT scan is still essential to the diagnosis of LMV.LMV is correlated with urinary system malfunction.LMV always occurs in active lupus with low C3 and C4 level.More than half patients should be treated with corticosteroid and cyclophosphamide pulse therapy.

Purpose To evaluate 1H proton magnetic resonance spectroscopy (1H-MRS) in detecting traumatic brain injury (TBI) and its metabolic changes during the initial two weeks after trauma using rabbit modes. Materials and Methods Fifteen Chinese rabbits were randomly divided into sham control group (n=5) and TBI group (n=10), 1H-MRS was performed 1 hour, 6 hours, 24 hours and 2 days, 7 days, 14 days after trauma, the concentration of N-acetylaspartate (NAA), creatine (Cr), choline-compound (Cho), NAA/Cr and Cho/Cr in each group was evaluated. At 6 hours, 24 hours and 168 hours after injury, 1 rabbit was slaughtered after abdominal anesthesia at each time point, the sample was ifxed with left heart catheterization perfusion after death, brain tissue was obtained, blocked, dehydrated into frozen section, HE staining was used for observation. Results Compared with the control group, NAA/Cr ratio in the trauma region decreased by 29%1 hour after trauma, with maximal reduction of 40% (at 24 hours) before increasing slightly, the NAA/Cr ratio returned to control level gradually until 168 hours later. The Cho/Cr ratio decreased by 16% 1 hour after trauma, with maximal reduction of 30%(at 6 hours) and increased gradually until finally su rpassed control level (168 hours) for 20% and then became stable. NAA/Cr ratio and Cho/Cr ratio showed statistically significant changes between each observing time point (P<0.05). Conclusion 1H-MRS can be used for dynamic detection of cerebral metabolism without injury, and is superior to MRI in detecting early abnormality of the brain, with NAA/Cr as the most sensitive parameter. Detecting the changes of NAA/Cr, Cho/Cr ratios in the trauma region can be used as a guide for assessment of the clinical treatment effectiveness of TBI.