Objective To investigate the prognostic value of interim and post-therapy 18fluorine-fluododeoxyglucose(18F-FDG) PET-CT in patients with T-cell lymphoma.Methods A retrospective analysis was conducted on data from 50 patients diagnosed with T-cell lymphoma(12 patients underwent interim PET-CT only,19 patients underwent interim and post-therapy PET-CT,and the rest 19 patients underwent only post-therapy PET-CT).Interim and post-therapy PET-CT(positive versus negative) were visually interpreted according to the criteria of the International Harmonization Project.The progression-free survival (PFS) and overall survival(OS) of two groups were calculated.We used the Fisher exact test and KaplanMeier testfor data analysis.Results Interim 18F-FDG PET-CT results were positive in 19 cases and negative in 12 cases.The median PFS and OS were 8 months and 16 months,in patients with positive results,versus 30 months and 36 months,in patients with negative results.The 1-year PFS and 3-year OS rates were 21.1％ (4/19) and 10.5％(2/19) in patients with positive results at interim PET/CT versus 100.0％(12/12) and 66.7％ (8/12) in patients with negative results(P＜0.01.Post-therapy 18F-FDG PET-CT results were positive in 16 cases and negative in 22 cases,the median PFS and OS were 10 months and 20 months in patients with positive results versus 32 months and 41 months in patients with negative results.The 1-year PFS and 3-year OS rates were 12.5％(2/16) and 12.5％(2/16),in patients with positive results at post-therapy PET-CT versus 95.5％(21/22) and 54.5％(12/22),in patients with negative results(P＜0.01).Conclusion Both interim PET-CT status and post-therapy PET-CT have significant value in monitoring response to therapy and predicting prognosis for patients with T-cell lymphoma.

Objective To investigate the reasons of artifacts in 64-slice spiral CT coronary angiography.Methods One hundred patients with diagnosed or suspected coronary artery disease underwent retrospectively ECG-gated 64-slice spiral CT coronary angiogruphy.Maximum intensity projection(MIP),muhiplanar reformation(MPR)and volume rendering technique(VRT)were reconstructed.The reasons of artifacts were assessed by two experienced radiologists.Results A total of 1347 segments(1347/1500,89.8%)were reviewed,the artifacts were found in 192 segments(14.2%).Breath movement was the moat common artifact(124/192).and the other reasons included fast rate and irregular rhythm of the heart beat (42/192).while the phase mismatch was the third reason(22/192).Conclusions The common artifacts of 64-slice spiral CT coronary angiography includes breath movement,fast rate and irregular rhythm of heart beat and phage mismatch.Breath-holding control,low rate and regular rhythm of heart beat,muhiphage reconstruction are suggested for the accurate diagnosis.

AIM: To investigate the effect of Qingrehuayuziyin Fang on lymphocyte apoptosis and expression of Fas and FasL in peripheral blood in the patients with systemic lupus erythematosus(SLE).METHODS: Eighty patients with SLE were selected and divided randomly into two groups,40 in the therapy group and 40 in control group respectively.Patients were get the treatment of Qingrehuayuziyin Fang combined hormone(prednisone) as therapy group and get hormone treating only as control group.After treating three months,lymphocyte were collected to be detected the rate of apoptosis by flow cytometer.Meanwhile,the expression of Fas and FasL were analyzed by RT-PCR.RESULTS: After three months treating,the rate of lymphocyte apoptosis was decreased significantly in therapy group compared with that in control group(P

Objective To study the relationships between the standing balance and walking ability of hemiplegic stroke survivors.Methods Eighty-eight post-stroke hemiplegic patients who could walk independently for more than 10 metres were selected into a patient group,while 88 healthy counterparts were recruited into a control group.Descriptors of the gait and balance function of both groups were collected using a gait and balance training and evahuation apparatus (Model:AL-600).The gait parameters were step width,walking speed,step length asymmetry (SLA),swing time asymmetry (SWTA) and stand time asymmetry (STA).The balance parameters studied were total trajectory length of the center of plantar pressure (COPD),the average left and right deviation of the center of plantar pressure (COPD-X) and the average anterio-posterior deviation of the center of plantar pressure (COPD-Y).Differences in indexes of gait and balance function between the two groups were analyzed using t-tests,and the relationships between the gait and balance indicators were analyzed using Pearson correlation coefficients.Results The walking speed,step width,COPD,COPD-X,COPD-Y,SLA,SWTA and STA of the patient group [(37.64± 18.29)cm/s,(14.45±4.17)cm,(66.75±29.04)cm,(2.04±1.41)cm,(2.48±1.28)cm,(1.30±0.46),(1.65±0.67) and (1.18±0.16),respectively] all increased significantly more compared to the control group [(90.76±14.72)cm/s,(8.70±2.62) cm,(27.84±6.54) cm,(1.30±0.53) cm,(1.68±0.40) cm,(1.07±0.06),(1.07±0.08) and (1.05±0.06),respectively],though the walking speed was significantly slower than that in the control group (P＜0.05).The patient group's average COPD-X showed slightly and moderately negative correlation with their walking speed,step width,SLA and SWTA (P＜0.05).COPD-Y was weakly related with step width (P＜0.05).Conclusion The standing balance of hemiplegic patients after stroke is related to their walking speed,step width and gait asymmetry.Especially significant correlation is observed between standing balance parameters such as COPD-X and gait parameters.

Objective Even after successful surgical repair,patients with coarctation of the aorta (CoA) are at high risk of long-term morbidity and mortality due to cardiovascular events,which is probably related to persistent arterial disfunction during long-terr follow-up after operation,The aim of the study was to explore the alterations of vascular structure and function in children with successfully repaired CoA in the short-and mid-term follow-up.Methods A cohort of 20 children who underwent CoA repair between January 2010 and October 2010 in Guangzhou women and children's Medical Center was studied.There were 14 males and 6 females in CoA group,which comprised 6 patients with isolated CoA,14 patients with CoA associated with intracardia anomalies,whose median age of operation was 4 months (rang from lmonth to 10.0 years).And 20 patients with isolated ventricular septal defect (VSD) were included as VSD group during the same time,with 12 males and 8 females,whose median age of operation was 5 months (rang from 1 month to 12.0 years).Resting blood pressure,flow-mediated dilation (FMD) of the brachial artery,carotid intima-media thickness (IMT) were compared in CoA group and VSD group,including preoperative media data and follow-up of 1 month,6 months and 1 year.In addition,as comparison to the operation group,20 health children with normal echocardiographic findings,whose median age was 5 months (rang from 3 month to 10.0 years),were selected as health group for the 1-year following up.None of them had obesity,hyperlipidemia,diabetes mellitus,metabolic diseases or systemic inflammatory disease.Results As a result of the datas before operation and those I month,6 months and 1 year after operation,all children were normotensive at rest.In the same period,Carotid IMT in CoA group[(0.47 ± 0.10)mm,(0.49 ±0.10) mm,(0.57 ±0.07)mm,(0.61 ± 0.07) mm]was significantly thicker than that in VSD group[(0.41 ±0.11) mm,(0.43 ±0.11)mm,(0.51 ±0.08) mm,(0.55 ±0.08) mm](P＜0.05) and health group[(0.40 ±0.09) mm,(0.42 ±0.11)mm,(0.50 ±0.08) mm,(0.57 ±0.08) mm](P ＜0.05),Brachial artery FMI in children with CoA[(5.4,6 ±1.51)％,(5.71 ±1.88)％,(5.42±1.69)％,(5.27±1.02)％]was significantly lower than that in the VSD control group[(6.69±1.45) ％,(6.66±1.21)％,(6.81 ±1.03)％,(6.43±1.34)％](P＜0.05) and health group[(6.59 ±1.84)％,(6.84±1.41)％,(6.91 ±1.31)％,(6.56±1.62)％](P＜0.05).Significant difference could not be found in neither the IMT nor the FMI between the VSI control group and health group in 4 period respectively,P ＞ 0.05.Conclusion Children after successful coarctation repair have abnomal structural and functional properties of the aorta above the place of coarctation even their blood pressure at rest is normal.These results confirm that the alterations in mechanical properties of carotid arteries as well as the generalized endothelial dysfunction in children with coarctation of the aorta are persistent,which can not be prevented or reversed by surgical repair,and which may partly explain the high incidence of cardiovascular disease observed in their adulthood and reduced life expectancy,furtherly supporting the claim that coartation of the aorta is a systemic vascular disorder which needs long-term follow-up of vascular function.

Objective To evaluate the endothelium-dependent vasodilation in children after repair for coarctation of aota (CoA). Methods A group of 20 children having undergone CoA repair between January 2010 and October 2010 in Guangzhou Women and Children′s Medical Center were include in the study , including 12 infants aged less than 6 months and 8 ones aged more than 6 months. Another 20 healthy children were enrolled during the same period as controls. All the subjects underwent monitoring of resting blood pressure and flow-mediated dilation (FMD) of the brachial artery in 4-year follow-up. Results There were no resting hypertension in all subjects , but FMD in the CoA group was higher than in the control group and so it was with the early surgery group and non early surgery group. Conclusion The surgical repair for coarctation of aorta could not cure the vascular function impairment satisfactorily , neither is the early surgery effective in alleviating the injuries in vascular endothelia.

Objective:Congenital hyperinsulinemia is a heterogeneous disorder characterized by severe hypoglycemia due to dysregulated insulin secretion. Sixteen genes have been reported to be associated with congenital hyperinsulinemia. In this study, whole exome sequencing was performed on a patient with obesity, hyperinsulinemia, and postprandial hypoglycemia to further explore its genetic etiology.Methods:The clinical data and peripheral blood of a patient with hyperinsulinemia and his family members were collected. Genomic DNA was extracted from the peripheral blood. Sanger sequencing and pedigree verification were performed on the pathogenic variants filtered by whole-exome sequencing. The function of the mutation sites was analyzed by bioinformatics software.Results:The proband presented with obesity, hyperinsulinemia, and postprandial hypoglycemia, but without exercise-induced hypoglycemia. A heterozygous SCL16A1 gene c. 1259A>G(p.K420R) mutation was identified in the proband. Co-segregated analysis showed that the c. 1259A>G mutation was also found in his father and brother, who had obesity and hyperinsulinemia, which was consistent with autosomal dominant inheritance. The mutation c. 1259A>G was predicted to be pathogenic by the MutationTaster, FATHMM-MKL, PolyPhen2, and CADD programs, and has not been reported in HGDM database yet, which was considered to be a novel mutation.Conclusion:This study reported a patient with hyperinsulinemia caused by a new mutation of SCL16A1 gene, which expanded our understanding of the pathogenic mutation spectrum of hyperinsulinemia.

OBJECTIVE: To carry out genetic testing for a child with Marfan syndrome (MFS) and explore its genotype-phenotype correlation. METHODS: Peripheral blood samples of the child and his parents were collected for the extraction of genomic DNA and subjected to whole exome sequencing (WES). Candidate variants were verified by Sanger sequencing. Functional impact of the variant was predicted by using bioinformatic software. RESULTS: The child, a 13-year-old male, has featured Marfanoid habitus, with arm span exceeding his height, tapering fingers and toes, pectus excavatum and scoliosis, but absence of typical cardiovascular system diseases such as aortic dilation, thoracic-abdominal aortic aneurysm, mitral valve prolapse, and lens dislocation. The child has harbored a novel splice site variant c.7383_7413del (p. N2461Kfs*211) of the FBN1 gene, which was not found in his parents and younger brother. The variant was unreported previously. CONCLUSION The novel variant of p. N2461Kfs*211 of the FBN1 gene probably underlay the MFS in this child. Above finding has enriched the genotypic and phenotypic spectrum of MFS.
Male ; Humans ; Marfan Syndrome/genetics* ; Fibrillin-1/genetics* ; Mutation ; Genotype ; Genetic Association Studies

Objective To explore the association between the intakes of cured meat and the risk of colorectal cancer,and analyze the role of the interaction between cured meat intake and dietary habit or lifestyle for the risk of colorectal cancer.Methods A well-designed questionnaire were used to collect retrospective information about demography,lifestyle and dietary habit from 400 patients diagnosed with colorectal cancer and 400 controls matched by age and gender.Logistic regression analysis was conducted to examine the possible association between the intake of cured meat and the risk of colorectal cancer.Generalized multifactor dimensionality reduction (GMDR) was used to analyze the interaction between cured meat intake and other dietary habits or lifestyle factors.Results Data from conditional logistic regression analysis showed that compared with the people who had ＜8 times per year of cured meat intake,those had 8-23 times of cured meat intakes (OR=3.480,95％CI:2.022-5.991,P＜0.001),24-47 times of cured meat intakes (OR=4.526,95％CI:2.553-8.025,P＜0.001),≥48 times of cured meat intakes (OR=5.472,95％CI:3.105-9.642,P＜0.001) had increased risk of colorectal cancer.There was a multiplied interaction between cured meat intake and pepper intake (OR=0.379,95％CI:0.190-0.758,P=0.009).The synergistic effect existed between cured meat intake and pickled vegetable intake;and the antagonism existed between cured meat intake and tea-drinking habit.GMDR analysis showed that interaction among red meat intake,pickled vegetable and cured meat intake on the risk of colorectal cancer might exist (P=0.001).Conclusions With the increase of frequency of cured meat intake,the risk of colorectal cancer increased gradually.The synergistic effect might existed between pickled vegetable intake and cured meat intake.Tea-drinking,pepper intake and cured meat intake might be antagonism in the pathogenesis of colorectal cancer.Red meat intake,pickled vegetable intake and cured meat intake might have combined effect to increase the risk of colorectal cancer.