In order to assess the clinical manifestations and electrocardiogram (ECG) characteristics of Chinese long QT syndrome (LQTS) patients and describe the phenotype-genotype correlation, the subjects from 5 congenital LQTS families underwent clinical detailed examination including resting body surface ECG. QT interval and transmural dispersion of repolarization (TDR) were manually measured. Five families were genotyped by linkage analysis (polymerase chain reacting-short tandem repeat, PCR-STR). The phenotype-genotype correlation was analyzed. Four families were LQT2, 1 family was LQT3. Twenty-eight gene carriers were (14 males and 14 females) identified from 5 families. The mean QTc and TDRc were 0.56 +/- 0.04 s (range 0.42 to 0.63) and 0.16 +/- 0.04 s (range 0.09 to 0.24) respectively. 35.7% (10/28) had normal to borderline QTc (< or = 0.460 s). There was significant difference in QTc and TDRc between the patients with symptomatic LQTS and those with asymptomatic LQTS, and there was significant difference in TDRc between the asymptomatic patients and normal people also. A history of cardiac events was present in 50% (14/28), including 9 with syncope, 2 with sudden death (SD) and occurred in the absence of beta-blocker. Three SDs occurred prior to the diagnosis of LQTS and had no ECG record. Two out of 5 SDs (40%) occurred as the first symptom. Typical LQT2 T wave pattern were found in 40% (6/15) of all affected members. The appearing-normal T wave was found in one LQT3 family. Low penetrance of QTc and symptoms resulted in diagnostic challenge. ECG patterns and repolarization parameters may be used to predict the genotype in most families. Genetic test is very important for identification of gene carriers.
Arrhythmia/etiology ; Arrhythmia/genetics ; Asian Continental Ancestry Group ; Electrocardiography ; Genotype ; Long QT Syndrome/complications ; Long QT Syndrome/congenital ; Long QT Syndrome/*genetics ; Pedigree ; *Phenotype

OBJECTIVE:To investigate the effects of N-acetylcysteine(NAC)on the concentration of ethanol in serum and the activities of alcohol dehydrogenase in liver and stomach tissue in alcoholic mice.METHODS:All mice were given p.o white spirit30min after p.o saline or5%NAC solution,and the durations from the disappear of righting reflex to recovery in mice were recorded and the number of dead mice in24hours were countered;With the same procedure for six days,the mice were killed by withdrawaling blood from orbit,and taken out the liver and stomach immediately,the concentrations of ethanol in serum and the activities of alcohol dehydrogenase in liver and stomach were measured by chromatometry and colorimetric method respectively.RESULTS:In drunk test,5%NAC solution significantly postponed the time from p.o white spirit to the disappear of righting reflex(P

Objective To describe the treatment experience of post-operative complications after total arch replacement for acute type A aortic dissection in 34 cases.Methods The subjects were 34 consecutive patients (Twenty-eight males and 6 females,age 34.0 -60.0 yrs) who received total arch replacement for acute Stanford type A aortic dissection from Jan.2005 to Oct.2010 in our hospital.The duration from the onset of the symptoms to the hospitalization ranged from 4 - 18 hrs.Pre-operative 2-D Echo revealed aortic valve regurgitation in 8 patients and mitral valve regurgitation in 1 patient.Results Three patients died after operation ( mortality 8.8％ ).Severe complications included acute kidney injury in 13 cases,respiratory dysfunction in 12 cases,paraplegia in 1 case,mental disorder in 10 cases and excessive post-operative bleeding in 2 cases.Conclusion The incidence of the complications after total arch replacement is still high and severe.Intensive care should be stressed peri-operatively and early diagnosis and treatment for post-operative complications are important procedures.

Objective To evaluate the clinical significance of the change of serum matrix metalloproteinases (MMP)-1,-2,-3 and -9 in acute and chronic aortic diseases and acute myocardial ischemia.Methods The blood serum levels of MMP-1,-2,-3 and -9 were detected in 30 patients with acute aortic dissection,19 patients with chronic aortic dissection,19 patients with aortic aneurysm and in 12 patients with acute myocardial ischemia,as well as in 16 healthy individuals who served as the control group.Serum MMP levels were measured by using an ELISA technique.Results There were significantly higher levels of MMP-3 in patients with acute myocardial ischemia as compared to acute aortic dissection ( [19.10 ± 3.11 ] μg/L vs [11.89 ± 1.31 ] μg/L,P =0.02).Significantly lower levels of MMP-1 were found in healthy controls compared to the groups of patients ( [1.30 ± 0.56 ] μg/L vs [2.99 ± 0.78 ] μg/L in acute aortic dissection,P =0.03,[3.12 ±0.78] μg/L in chronic dissection,P =0.02,[3.01 ± 1.01 ] μg/L in thoracic aortic aneurysm,P =0.03 and [5.01 ± 0.98 ] μg/L in acute myocardial ischemia,P =0.01 ).Higher levels of M MP-1 and MMP-3 were detected on males.There was a positive correlation between MMP-1 and increasing age ( r =0.38,P ＜ 0.05 ).In patients operated for acute type A aortic dissection,the levels of MMP-1,MMP-3 and MMP-9 increased immediately after surgery,while the levels of MMP-2 decreased.Twenty-four hours after surgery levels of MMP-1,-2 and -9 were almost equal to the preoperative ones( P ＞ 0.05 ).Conclusion Measurement of serum MMP levels in thoracic aortic disease and acute myocardial ischemia is a simple and relatively rapid laboratory test that could be used as a biochemical indicator of aortic disease or acute myocardial ischemia,when evaluated in combination with imaging techniques.

Objective To describe the follow-up data of 34 patients receiving total arch replacement with an open stent graft for acute type Ⅰ aortic dissection.Methods The subjects were 34 consecutive patients with type Ⅰ acute aortic dissection ( Twenty-eight males and 6 females,aged 34.0 - 46.0 yrs) who received total arch replacement with an open stent graft in our hospital from Jan.2005 to Oct.2010.Thirty of the 34 patients were followed up for 2 - 70 months.CT scanning was performed at the 3 and 12 months and then yearly after operation to detect the thrombus formation,absorption of thrombus,and obliteration of the false lumen after its exclusion by the stent graft.Results Three patients died peri-operatively with the mortality of 8.8％.One patient died during the follow-up period.Obliteration was recognized in all the patients at the distal side of the stent graft during the follow-up period.The false lumen remained in 10 patients at the distal part of descending aorta,but the diameter of the false lumen was not enlarged.Conclusion In patients with acute type Ⅰ aortic dissection,it is relative safe to perform extensive primary repair of the thoracic aorta by stent grafting.This method may enhance the obliteration of the false lumen and reduce the possibility for further operations to manage a residual false lumen.

Objective To survey bacterium pollution state on the surface of medical equipment maintenance tools,and thenobserve the disinfection effect of ozone.Methods Totally 30 medical equipment maintenance tools including screwdriver,hexagona wrench and multimeter from certain hospital were placed in ozone disinfection cabinet,and then disinfected by ozoneup to 5 minutes.Samples on the surface of tools were collected by cotton swabs before and after disinfection,cultured for 48hours in 37 ℃,and then the bacterial colony counts were measured and analyzed,and pathogenicity bacteria were identified.The pollution couditions on kinds of tools were compared.Results The surface of the tools was polluted severely,bacterialcolony counts of which before disinfection reached (132.68±37.02) cfu/cm2,and decreased to (6.44±1.08) cfu/cm2 after 5-minutes' disinfecting.There was statistically significant difference in the bacterial counts before and after the disinfection (P＜0.01),and the inactivation ratio was up to 95.15％.Pathogenicity bacteria such as staphylococcus aureus and escherichia coliwere detected before the disinfection,and killed after that.The bacteria counts were significantly different in the three kindsof tools,and the screwdriver gained the highest count while the multimeter had the lowest one.Conclusion The surface ofmedical equipment maintenance tools is polluted by bacteria severely,including some pathogenicity bacteria,and should be disinfected regularly.Ozone can kill bacteria rapidly,and the tools can achieve sanitary standard after 5-minutes' ozone disinfecting.

Objective To investigate the clinicopathology and immunophenotype of primary non- Hodgkin lymphoma(NHL)of the female genital system,and to analyze the prognosis of such tumors. Methods Clinicopathologic features of 43 cases of primary NHL of the female genital system were studied retrospectively,with the histological classification based on the Classification of Haematopoietic and Lymphoid Tumors(WHO,2001).Immunochemistry technique,in-situ-hybridization and polymerase chain reaction methods were used to detect the immunophenotype,epstein barrvirus(EB)virus infection status and immunoglobulin heavy chain gene rearrangement,respectively.Results(1)Primary lesions:there were 24 cases of lymphoma originating in the ovary,3 cases in the endometrium,10 cases in the cervix,2 cases in the vagina and 4 cases in the vulva.(2)Staging:12 cases(28%)were in stage Ⅰ,9 cases (21%)in stage Ⅱ,and 22 cases(51%)in stage Ⅲ.(3)Histological classification:37 cases(86%)were diffuse large B cell lymphoma(DLBCL),3 cases were Burkitt lymphoma and the remaining 3 cases were unspecified peripheral T-cell lymphoma according to biopsy,immunophenotype analysis,in-situ- hybridization technique and IgH gene rearrangement detection.(4)Prognosis analysis:increase in the level of lactic acid dehydrogenase,stage Ⅲ,DLBCL and single operation suggest poor prognosis.Conclusions Establishment of the diagnosis of primary NHL of the female genital system is based on biopsy, immunophenotype analysis,in-situ-hybridization technique and IgH gene rearrangement detection,which play important roles in diagnosis and differential diagnosis of the tumor.Combined therapy is the first choice of therapeutic regimens.

Objective To study the interobserver variabilities and the differential diagnosis value of Breast Imaging Reporting and Data System-Ultrasound (BI-RADS-US) lexicon for small ( ≤ 2 cm) breast nodules. Methods Between January 2009 and December 2011, 289 patients with small (≤2 cm) breast nodules (n=317) were included. According to sizes, the lesions were divided into two groups, i.e., 0-1 cm (n=160) group and 1-2 cm (n=157)group. Each lesion was described independently by 3 radiologists using BI-RADS-US lexicon. Interobserver variabilities were assessed by Kappa test. Chi-square test was used to compare the frequency difference of the descriptors between malignant and benign lesions. Sensitivity, speciifcity, accuracy, positive predictive value and negtive predictive value were calculated. Results (1)Moderate agreements were obtained for lesion shape, orientation, margin, echo pattern, surrounding tissue and calciifcations (κ=0.44, 0.57, 0.48, 0.43, 0.51 and 0.57) in 0-1 cm group. Substantial agreements were obtained for lesion shape, orientation, margin and echo pattern (κ=0.65, 0.61, 0.64 and 0.63) in 1-2 cm group. (2)Irregular shape, non-parallel orientation, non-circumscribed margin, echogenic halo and microcalciifcations were more frequently found in malignant nodules than in benign nodules in 0-1 cm group [52.3% (34/65) vs 20.0% (19/95), 38.5%(25/65) vs 13.7%(13/95), 75.4%(49/65) vs 32.6%(31/95), 18.6%(12/65) vs 0 (0/95) and 10.8%(7/65) vs 2.1%(2/95);χ2=18.19, 13.08, 28.22, 16.39 and 3.95;P=0.000, 0.000, 0.000, 0.000 and 0.047]. Similarly, irregular shape, non-parallel orientation, non-circumscribed margin, echogenic halo, shadowing, changes of Cooper′s ligament and microcalciifcations were signiifcantly more frequent found in malignant nodules than in benign nodules in 1-2 cm group [74.2%(49/66) vs 12.1%(11/91), 36.3%(24/66) vs 5.5%(5/91), 93.9%(62/66) vs 22.0%(20/91), 37.9%(25/66) vs 3.3%(3/91), 30.3%(20/66) vs 7.7%(7/91), 15.2%(10/66) vs 0 (0/91) and 16.7%(11/66) vs 4.4%(4/91);χ2=62.59, 24.21, 79.40, 31.22, 13.73, 12.30 and 6.67;P=0.000, 0.000, 0.000, 0.000, 0.000, 0.000 and 0.010]. (3)In both groups, a good sensitivity was demonstrated (75.4%&93.9%) when using the non-circumscribed margin as a criterion for malignancy, and high speciifcity was achieved in two groups (80.0%-100%and 87.9%-100%) when other descriptors including irregular shape, non-parallel orientation, echogenic halo, shadowing, changes of Cooper′s ligament and microcalciifcations were used as differentiation criteria. Conclusions Good interobserver agreement can be achieved using the BI-RADS-US lexicon in the diagnosis of small breast nodules. Non-circumscribed margin are proved as the most valuable sign for screening malignant breast lesions ≤ 2 cm. High speciifcity was found for irregular shape, nonparallel orientation, echogenic halo, shadowing, Cooper′s ligament changes and microcalciifcations, which can help biopsy and preoperative diagnosis.

Objective To study the influence of mesenchymal stem cells(MSCs) implantation on ventricular remodeling and heart function after myocardial infarcion. Methods Bone marrow was aspirated from Gui-zhou Xiang swines.After being isolated,cultured and co-cultured with 5-azacytidine,either autologous MSCs(experiment group) or a comparable volume of physiologic saline(control group) were injected into the infarcted myocardium.Three and six weeks later,echocardiographic measurement was performed to assess the myocardial structure and heart function,and single photon emission computed tomography(SPECT) was employed for myocardial imaging.Implanted stem cells were detected by the anti-Brdv antibody DAB with HE staining. Results Left ventricular ejection fraction(EF),fractional shortening and wall thickening were higher in experiment group than control group.The thickness of the ventricular wall and septum was found increased while the left ventricular chamber size was smaller in experiment group.It was indicated by SPECT that three and six weeks after implantation,there was obvious image defect in control group while in experiment group there were some imaging areas in the infarcted area.Brdv-labelled cells were observed in the central part of and around the infarcted area.Conclusion Implantation of MSCs into the infarcted myocardium is believed to attenuate the remodeling process,inhibit the extent of wall thinning and dilatation of the ventricular chamber.MSCs implantation may also improve the contractile ability of the myocardium and heart function.

In order to assess the clinical manifestations and electrocardiogram (ECG) characteristics of Chinese long QT syndrome (LQTS) patients and describe the phenotype-genotype correlation, the subjects from 5 congenital LQTS families underwent clinical detailed examination including resting body surface ECG. QT interval and transmural dispersion of repolarization (TDR) were manually measured. Five families were genotyped by linkage analysis (polymerase chain reacting-short tandem repeat, PCR-STR). The phenotype-genotype correlation was analyzed. Four families were LQT2, 1 family was LQT3. Twenty-eight gene carriers were (14 males and 14 females) identified from 5 families. The mean QTc and TDRc were 0.56 +/- 0.04 s (range 0.42 to 0.63) and 0.16 +/- 0.04 s (range 0.09 to 0.24) respectively. 35.7% (10/28) had normal to borderline QTc (< or = 0.460 s). There was significant difference in QTc and TDRc between the patients with symptomatic LQTS and those with asymptomatic LQTS, and there was significant difference in TDRc between the asymptomatic patients and normal people also. A history of cardiac events was present in 50% (14/28), including 9 with syncope, 2 with sudden death (SD) and occurred in the absence of beta-blocker. Three SDs occurred prior to the diagnosis of LQTS and had no ECG record. Two out of 5 SDs (40%) occurred as the first symptom. Typical LQT2 T wave pattern were found in 40% (6/15) of all affected members. The appearing-normal T wave was found in one LQT3 family. Low penetrance of QTc and symptoms resulted in diagnostic challenge. ECG patterns and repolarization parameters may be used to predict the genotype in most families. Genetic test is very important for identification of gene carriers.
Arrhythmias, Cardiac ; etiology ; genetics ; Asian Continental Ancestry Group ; Electrocardiography ; Female ; Genotype ; Humans ; Long QT Syndrome ; complications ; congenital ; genetics ; Male ; Pedigree ; Phenotype