Objective To study the possibility of fetal cardiac rhabdomyoma diagnosed by ultrasonography,and the relationship between the fetal cardiac rhabdomyoma and family tuberous sclerosis. Methods High resolution ultrasonic equipment with fetal cardiac software was used. The main cross section of four chamber view was studied mainly. The location, the number of the tumor and the outflow tract of left and right ventricular cordis were scanned carefully, the maternal abdominal scan was performed as well. Results Five cases of fetal cardiac rhabdomyoma were found by routine prenatal ultrasonography, and were confirmed by autopsy. Among them, one case were associated with maternal tuberous sclerosis. Conclusions Fetal cardiac rhabdomyoma associated with tuberous sclerosis. Fetus with cardiac rhabdomyoma frequently die of cardiac failure; since the prognosis of the tuberous sclerosis with neural sclerosis was very poor, when the fetal cardiac rhabdomyoma was detected prenatally, the possibility of tuberous sclerosis should be considered, also relative examination must be performed to the mother to decrease the perinatal mortality.

Objective To study chronic nephrotoxicity of Penthorum chinense. To provide a scientific evidence for the development and application of Penthorum chinense in food and drug. Methods 48 SD rats were randomly divided into 4 groups (n=12), 0. 9% sodium chloride solution saline group and three Penthorum chinense treat groups. The Penthorum chinense groups were treated by intragastric administration with 5,10,20 g·kg-1 crude drug once a day for 3 months. The levels of Urea, UA, Cre in serum were detected, and the HE dyeing was chosen to observe the kidney morphology. Results The body weight, nephritic organ quotiety, and the level of Urea, UA, and Cre in each exposure group have no statistically significant difference compared with 0. 9% sodium chloride solution group (P>0. 05). There were no obvious kidney morphology changes in the Penthorum chinense group(F=1. 37,P=0. 268). Conclusion The kidney morphology and function of rat shows no apparente injury after long-term intragastric administration of Penthorum chinense.

Objective To improve the diagnosis of amniotic bands syndrome with ultrasound. Methods All pregnant women underwent ultrasonographic examination to observe the intra amniotic band like structures. Results Among 8771 women undergone ultrasound examination, 60 were found with intra amniotic band structure. Seven cases were diagnosed as amniotic bands syndrome and 53 intra amniotic band like structures included extra amniotic pregnancy(7), amniotic sheets(4), incomplete uterine septum(24), fetal nuchal cystic hygroma(2), fetal nuchal and trunk edema(11) and disclosed extra embryo sac(5). Conclusions Not all the intra amniotic band like structures is amniotic band. But any abnormal structures detected in early stage should be closely followed up.

Objective To investigate the value of nuchal translucency(NT) thickening in the fetal chromosome abnormality screening.Methods The 14 881 pregnant women received NT measurement in 11-13 +6 weeks at Beijing Obstetrics and Gynecology Hospital from January 2010 to August 2012.The 118 fetuses whose NT≥2.5 mm were recruited.One hundred and eight (91.5％,108/118)of them accepted invasive procedure and karyotype analysis.Results (1) Chromosome karyotype analysis:113 singleton pregnancies (95.8％) and 5 twin pregnancies (4.2％) whose NT thickened from 2.5 mm to 11.0 mm were advised karyotype analysis.The 108 pregnant women accepted karyotype analysis.Among them,88 had normal chromosome karyotype,and 20 had chromosome abnormalities.The detection rate was 18.5 ％ (20/108).(2) The sensitivity,specificity,false positive rate,false negative rate,total consistent rate,positive predictive value and negative predictive value of prenatal screening of chromosome abnormalities for NT ≥ 2.5 mm were 44％,99％,1％,56％,99％,19％ and 100％,respectively.(3) Among the 88 fetuses who had normal karyotype,72 (82％) had isolated thickened NT,while 16 (18％) had fetal structural malformation or intrauterine demise.(4) Among the 10 pregnant women who did not accept fetal karyotype analysis,8 terminated pregnancy because of fetal structural malformation,and the other 2 fetuses died in uterus.(5)All of the 5 twin pregnancies were dichorionic twins,and one of the twins had thickened NT.Among the 5 twin pregnancies,one fetus was trisomy 21 and others had normal karyotype.3 twin pregnancies who had normal chromosomes gave live birth.And the other had a gastroschisis and exstrophy deformity fetuse.This fetuse died in uterus and remaining fetuse had full-term live birth.(6) 35 (29.7％) pregnant women received second trimester Down's syndrome serum screening.One was high risk and was proved a trisomy 21 pregnancy.Thirty-four were low risk and had normal chromosome karyotype.Conclusion NT thickening may indicate fetal chromosome abnormalities or other structural malformations.It is a useful prenatal screening indicator.

Objective The clinical value of ifrst-trimester standard ultrasound views in screening for fetal structural abnormalities. Methods From September 2008 to March 2011, transabdominal sonographic screening by standard ultrasound views was performed in 3 134 viable singleton pregnancies during the ifrst trimester at Beijing Obstetrics and Gynecology Hospital. Used the following views:(1) Fetal proifle;(2) Neck sagittal;(3) Skull and brain;(4) Thorax;(5) Abdomen;(6) Bladder;(7) Upper limbs, and (8) Lower limbs. The women underwent 20-to 24-week and 28-to 32-week ultrasound examination if the anatomical survey was normal during the ifrst trimester. Follow-up was obtained by reviewing of the birth records from hospital charts. Results Sixteen prenatal abnormalities were detected at 11-to 13-week scan (including one case of encephalocele and meningoceles, one case of endothelial cushion defect and single umbilical artery, one case of ectopia cordis, gastroschisis, and spinal abnormalities, two cases of exencephaly, one case of anencephaly, one case of gastroschisis and lower limb malformation, two cases of omphalocele, one case of encephalocele, ectopia cordis, and gastroschisis, one case of single ventricle, one case of endothelial cushion defect, four cases of hyhrop). All of the 13 women decided on termination of pregnancy. Three suspected structural abnormalities (hydrops) detected at the ifrst-trimester scan were found as normal after birth. Seven cases were diagnosed at 20-to 24-week scan (including one case of dextrocardiac and double outlet right ventricle;two cases of endothelial cushion defects;one case of trisomy 21 with VSD;one case of omphalocele, one case of short ulna and radial with wrist distortion, and one case of club foot). One case of diaphragmatic hernia was detected at 32-week scan. Two cases (one case of cardiac defects and one case of ear deformity) were identified after birth. Of all the 3 134 fetuses, follow-up was obtained in 2 822 cases, 312 cases were excluded from the analysis because the outcome of their pregnancy could not be traced. Twenty-three fetuses with at least one major structural defect detected by prenatal ultrasonography were confirmed by follow-up. The sensitivity of the standard first-trimester ultrasound views was 56.5%(13/23), speciifcity was 99.9%(2 796/2 799), CR was 99.6%(2 812/2 822), and the false negative rate was 43.5%(10/23). Conclusions The standard views of the ifrst trimester are feasible and effective in screening the major fetal abnormalities including exencephaly, anencephaly, gastroschisis, omphalocele, ectopia cordis and single ventricle. However, ultrasound screening in early pregnancy still has some limitations, continuous scanning in different stages of pregnancy is necessary.

Objective To identify the role of RNA-editing enzyme ADAR1 (adenosine deaminase acting on RNA) in EV71 infection and virus mutation.Methods RNAi technology was applied to establish ADAR1 knock-down stable cell lines.Then the cells were served to evaluate the role of ADAR1 in EV71 infection by MTT assay for detecting virus-induced cell viability,virus plaque assay for quantification of the virus titer and the cellular susceptibility to the virus,and Western blot for virus protein expressions.ADAR1-mediated RNA editing can result in the genetic A-G and T-C mutations.To further determine whether the effects of ADAR1 on EV71 infection were correlated with ADAR1-mediated EV71 RNA editing and therefore increased the viral mutations during the infection,the characteristics of EV71 mutation were analyzed based on the different full-length viral genomes from epidemic regions.The viral genome was also sequenced from the infected ADAR1 knock-down cells.Results After ADAR1 knock-down,the cell viability decreased quickly after the virus infection,and formed much more and larger sizes of plaques than the control cells.The virus capsid protein VP1 expressions and virus titer in the cells culture media were both increased in ADAR1 knockdown cells.Statistic analysis showed that A-G and T-C mutations were the major mutations of EV71,which were believed to be the hot sites for RNA-editing.However,the results of viral RNA genomic sequencing data indicated that ADAR1 did not edit EV71 genome directly.Conclusions ADAR1 was a restriction factor for controlling EV71.However,ADAR1 does not directly edit EV71 genome.

Objective:To observe the protective effects of Clostridium butyricum and butyrate on pancreas, liver and intestinal mucosa in rats with acute necrotizing pancreatitis (ANP), and to explore the possible mechanism.Methods:Forty Sprage-dawley rats were randomly divided into control group, ANP group，Clostridium butyricum treated group(CB group) and butyrate treated group(SB group), with 10 rats in each group by random number method. The ANP rat models were prepared by retrograde injection of sodium taurocholate into the biliopancreatic duct. Rats in CB and SB group were given intragastic administration of Clostridium butyricum 1×10 9 CFU or sodium butyrate (100 mg/kg) in 10 days once a day before modeling. Serum amylase (SAMY), lypase, ALT, AST, TBil, tumor necrosis factor alpha(TNF-α), IL-6 and HMGB1were measured after 24 h. Protein from intestinal mucosa was extracted and Western Blotting was used to measure expression of tight-junction proteins ZO-1, claudin-1 and occludin. Pancreas and liver tissues were stained with hematoxylin-eosin and scored by pathology. Results:The levels of amylase [(9365.1±716.5), (5947.3±512.0), (6517.7±269.6)U/L], lipase[(8343.7±1041.4), (6600.4±899.7), (6754.4±1046.4)U/L], AST[(560.5±72.7), (432.0±76.2), (429.8±40.5)U/L], ALT[(499.9±65.2), (385.7±46.0), (395.8±45.8)U/L], TBil[(134.2±56.2), (74.3±65.2), (81.3±35.3)U/L], TNF-α[(162.0±14.4), (100.4±6.3), (119.2±12.5)ng/L], IL-6[(161.4±26.0), (104.8±15.2), (105.5±12.7)ng/L], HMGB1[(100.1±6.7), (58.0±7.7), (63.4±7.2)ng/L] in ANP group, CB group and SB group were detected; and the pathological scores of pancreas[(11.2±1.08)，(9.45±1.06), (9.04±0.89)] and liver[(2.89±0.73), (2.09±0.49), (2.12±0.52)] in ANP group，CB group and SB group were higher than those in control group[(100.6±5.20)U/L, (966.5±301.9)U/L，(30.2±6.3)U/L, (27.6±5.9)U/L, (2.4±0.6)U/L, (29.5±4.8)ng/L，(36.9±7.6)ng/L，(35.5±5.7)ng/L，(1.18±0.05)，(0.56±0.09)]. However, those indexes in CB group and SB group were lower than those in ANP group, and the difference was statistically significant (all P<0.05). The expression of ZO-1 in control group, ANP group, CB group and SB group was 1.83, 0.79, 1.25 and 1.16. The expression of claudin-1 in control group, ANP group, CB group and SB group was 0.58, 0.13, 0.43 and 0.37. The expression of occludin in control group, ANP group, CB group and SB group was 1.06, 0.38, 0.82 and 0.79. The expression of TJ proteins in ANP group was significantly lower than that in other groups and the difference was statistically significant (all P<0.05). Conclusions:Clostridium butyricum and metabolites butyrate can alleviate the inflammatory response in ANP rats with liver injury, maintain the function of intestinal mucosal barrier and prevent the liver injury.

[Objective]This study was aimed to evaluate treatment outcomes and toxicity of continuous-infusion EPOCH regimen for NK/T-cell lymphoma(NK/TCL).[Methods]From June 2003 to June 2008,34 patients including 30 nasal NK/TCL (88.2%)and 4 nasal type NK/TCL(11.8%)received doxorubicin,vincfistine,etoposide over 96 hours infusion with bolus eyelophosphamide and oral predinisone(EPOCH)chemotherapy as first-line treatment.Median cycles of EPOCH administered were 2.5(1-6 cycles).Additional involved field radiation therapy(IFRT)was administered to patients with localized nasal focus after chemotherapy.[Results]Among 34 patients,33 were eligible for response evaluation.The response rate(RR)was 60.6% (20/33)with complete remission(CR)rate of 45.5%(15/33).The RR of patients with nasal NK/TCL was 66.7%(20/30)with CR rate of 50%(15/30).Only one of the 3 nasal type NK/TCL patients achieved stable disease(SD),the other 2 had progressive disease(PD)during chemotherapy.After a median follow-up of 22(2-68)months,the estimated 3-year overall survival rate(OS)was 52.2%.For patients with nasal NK/TCL,the estimated median survival time was not reached,the 3-year OS was 59.4%.For patients with nasal type NK/TCL,the estimated median survival time was only 7 months.The CR rate was 75.0% for localized nasal NK/TCL who received initial EPOCH chemotherapy followed IFRT with the 3-year OS of 75.0%.Major adverse effect was myelosuppression.The incidence of grade Ⅲ～Ⅳ neutropenia was 30.9%.No treatment-related mortality occurred.[Conclusions]EPOCH regiment was effective and well tolerant for nasal NK/TCL.Combined EPOCH chemotherapy followed by IFRT produced promising outcome for patients with localized disease.However,patients with nasal type NK/TCL responded poorly and more efficacious treatment strategies are urgently needed.

[Objective]To evaluate the clinical efficacy and side effects of DHAOx±R regimen in the patients with relapsed and refractory non-Hodgkin's lymphoma(NHL).[Methods]Twenty patients with relapsed or refractory NHL were enrolled into this study in Cancer Center of Sun Yat-sen University.These patients were treated with DHAOx±R regimen(Dexamethasone 20 mg/day intravenous(Ⅳ)on day 1 to day 4,cytarabine 2 000 mg/m~2 3 h Ⅳ,every 12 hours on day 2;oxaliplatin 130 mg/m~2 2 h Ⅳ on day 1;with or without rituximab 375 ms/m~2 on day 0).Six patients were followed by high dose chemotherapy with autologous peripheral blood stem cell transplantation.Response to treatment wag assessed according to The International Working Group Criteria,including CR,PR,SD and PD.Side effects were graded according to WHO criteria,including 0-Ⅳ grades.[Results]Twenty patients received 47 cycles chemotherapy,13 patients(65%)received DHAOx chemotherapy and 7(35%)received DHAOx+R.The response rate(RR)for the whole group was 55%(11/20)with comeplete response(CR)rate 35%(7/20).The response can also be obtained in the patients who were already treated by platinum-based regimen before.The major toxicity Wag myelosuppression.The incidence of grade Ⅲ～Ⅳ neutropenia Wag 35%(16/47),and febrile neutropenia was 17%(8/47).The incidence of grade Ⅲ～Ⅳ thrombocytopenia was 20%(9/47).Eight cycles(17%)occurred mild neumtoxicity.With median follow-up of 12 months,1 and 2-year overall survival rate were 70.6%.[Conclusion]DHAOx was an effective regimen for recurrent and relapsed NHL patients with mild side effects and further investigation is needed.

Objective To observe the incidence and clinical feature of sleep-related breathing disorder in patients with idiopathic pulmonary fibrosis (IPF). Methods Thirty-four IPF patients who were measured by polysomnography (PSG) were collected in the Department of Respiration of Tianjin Medical University General Hospital. According to the results of apnea hypoventilation index (AHI), patients were divided into pure IPF group (AHI<5 events/h, n=7) and IPF combined with obstructive sleep apnea hypopnea syndrome (IPF+OSAHS) group (AHI≥5 events/h, n=27). The PSG reports of two groups were analyzed, and the correlation between AHI and pulmonary function and oxygen saturation in sleep and at wake were analyzed. Results (1)Thirty-four IPF patients were all demonstrated sleep disorders, low sleep efficiency, increased proportion of stageⅠand stageⅡand decreased proportion of stageⅢand rapid eye movement (REM). The arousal index and the proportion of stageⅠand stageⅡwere higher in IPF+OSAHS group than those of pure IPF group (P<0.01), while the proportion of stageⅢwas lower in IPF+OSAHS group than that of pure IPF group (P<0.01). There was no significant difference in stage REM between two groups. (2)Twenty-seven patients (79%) combined with OSAHS, among which five subjects (15%) were mild OSAHS with 5 events/h≤AHI<15 events/h, and 22 subjects (65%) were moderate-severe with AHI≥15 events/h. The main type of sleep-disorder breathing was hypoventilation, which mainly happened in stage REM. (3) Thirty-four IPF patients showed sleep hypoxemia, and the oxygen desaturation index (ODI) was higher in IPF-OSAHS group than those of pure IPF group (P<0.05). (4)The AHI was positively correlated with body mass index (r=0.791, P<0.05), and was negatively correlated with forced vital capacity (FVC%pred) (r=-0.574, P<0.05) and forced expiratory volume in 1 second (FEV1%pred) in IPF patients (r=-0.664, P<0.05). The lowest oxygen saturation (LSO2) and mean oxygen saturation (MSO2) in sleep were positively related with oxygen saturation at wake (r=0.421 and r=0.464, P<0.05 respectively). Conclusion The IPF patients show severe sleep disorder and hypoxemia, which can be worsen by OSAHS and produce negative effect on daily life. We should initiate active treatment in patients with sleep-related breathing disorders.