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The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). The SCMR web site (https://www.scmr.org) hosts a case series designed to present case reports demonstrating the unique attributes of CMR in the diagnosis or management of cardiovascular disease. Each clinical presentation is followed by a brief discussion of the disease and unique role of CMR in disease diagnosis or management guidance. By nature, some of these are somewhat esoteric, but all are instructive. In this publication, we provide a digital archive of the 2019 Case of the Week series as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.

The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). “Cases of SCMR” is a case series hosted on the SCMR website (https://www.scmr.org) that demonstrates the utility and importance of CMR in the clinical diagnosis and management of cardiovascular disease. The COVID-19 Case Collection highlights the impact of coronavirus disease 2019 (COVID-19) on the heart as demonstrated on CMR. Each case in series consists of the clinical presentation and the role of CMR in diagnosis and guiding clinical management. The cases are all instructive and helpful in the approach to patient management. We present a digital archive of the 2021 Cases of SCMR and the 2020 and 2021 COVID-19 Case Collection series of nine cases as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar literature search engine.

Background & Aims Tenofovir disoproxil fumarate (TDF) is the preferred treatment to prevent maternal transmission of HBV, owing to its efficacy and safety. However, data are lacking on the long-term safety outcomes in children following fetal exposure to TDF. Methods Children participating in a prospective, multisite trial of maternal TDF treatment during late pregnancy were recruited for follow-up visits once a year. Growth parameters, serum biochemistry, HBV serology, and bone mineral density (BMD) by dual-energy x-ray absorptiometery scan were measured. Results One hundred and twenty-eight children, 71 in the TDF and 57 in the control group, completed 255 follow-up visits at the age of 2 to 7 (median, 4.08) years. No differences in z-scores for weight-for-age (0.26 ± 0.90 vs. 0.22 ± 0.99, p = 0.481), z-scores for height-for-age (0.20 ± 1.02 vs. 0.25 ± 0.98, p = 0.812), and estimated glomerular filtration rate (169.12 ± 50.48 vs. 169.06 ± 34.46 ml/min/1.73m2, p = 0.479) were detected. After adjustment for age, sex and HBV status by multiple linear regression, children in the TDF and control group had comparable levels of serum calcium, phosphorus, bone-specific alkaline phosphatase, calcidiol and BMD of lumbar spines (0.55 ± 0.01 vs. 0.57 ± 0.01 g/cm2, p = 0.159) and left hip (0.56 ± 0.01 vs. 0.56 ± 0.01 g/cm2, p = 0.926). Conclusions Children of HBV-infected mothers who did or did not receive tenofovir disoproxil fumarate treatment during late pregnancy had comparable long-term growth, renal function, and bone development up to 6–7 years after delivery. Clinical trial number NCT01312012 ( ClinicalTrials.gov ) Lay summary Currently there are insufficient long-term safety data in children born to mothers who took antiviral agents during pregnancy to prevent mother-to-infant transmission of hepatitis B virus (HBV). In this study, we found that children of HBV-infected mothers who did or did not receive tenofovir disoproxil fumarate treatment during late pregnancy had comparable long-term growth, renal function, and bone development up to 6-7 years after delivery.

患儿 男，4月龄，因“确诊Simpson-Golabi-Behmel综合征Ⅰ型3个月余，体检发现腹部肿物1个月余”入院，其母孕24周B超发现羊水过多，患儿于38

The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). Case of the week is a case series hosted on the SCMR website (https://www.scmr.org) that demonstrates the utility and importance of CMR in the clinical diagnosis and management of cardiovascular disease. Each case consists of the clinical presentation and a discussion of the condition and the role of CMR in diagnosis and guiding clinical management. The cases are all instructive and helpful in the approach to patient management. We present a digital archive of the 2020 Case of the Week series of 11 cases as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.

目的 探讨SET-NUP214融合基因在血液恶性肿瘤中的表达，分析其相关的临床及生物学特征。 方法 回顾性分析2012年1月至2018年12月北京大学人民医院诊断的24例SET-NUP214融合基因阳性血液恶性肿瘤患者的临床资料，并采用Kaplan-Meier法进行生存分析。 结果 24例患者中，急性淋巴细胞白血病（ALL）15例（T-ALL 13例，B-ALL 2例）、急性髓系白血病（AML）7例，T/髓混合急性白血病2例。13例T-ALL患者免疫表型以CD3+CD2−为主要特征，73.3％的ALL患者伴有髓系标志表达，85.7％的AML患者表达CD7。24例患者诱导化疗完全缓解（CR）率91.7％。全部患者均接受异基因造血干细胞移植，中位随访24个月，AML和ALL的3年无复发生存（RFS）率分别为85.7％和33.3％，差异无统计学意义（P＝0.128）。比较13例SET-NUP214阳性与62例SET-NUP214阴性T-ALL患者的疗效，诱导化疗CR率分别为92.3％和93.5％（P＝0.445），诱导化疗4周CR率分别为69.2％和72.6％（P＝0.187），差异均无统计学意义。接受造血干细胞移植后，SET-NUP214阳性T-ALL患者的3年RFS率（38.5％）明显低于SET-NUP214阴性T-ALL患者（66.4％）（P＝0.028）。 结论 SET-NUP214融合基因主要见于T细胞源性血液肿瘤，伴SET-NUP214融合基因T-ALL预后较差。

OBJECTIVE: To investigate the effect of Notch1 gene on radiosensitivity of nasopharyngeal carcinoma cells and its molecular mechanism. METHOD: A Notch1-knockout CNE-2 cell line was constructed using CRISPR/Cas9 system, and the expression of Notch1 gene was detected by RT-PCR and Western blot. After treatment with different doses of radiation, the survival fraction (SF) of each group was calculated, and used the GraphPad Prism 6.0 software and the Linear quadratic model were used to calculate the fitted dose survival curve and the sensitivity enhancement ratio(SER). Taking 6 Gy as radiation dose, the experiment was divided into four groups: Notch1(+) group, Notch1(-) group, IR+Notch1(+) group and IR+Notch1(-) group. CCK-8 assay was used to detect cell proliferation in each group. Annexin V-FITC/PI double staining assay was used to detect the changes of apoptosis in each group. The expression of H2AX, CyclinD1, Bax, Bcl-2 and GAPDH proteins were detected by Western blot. RESULT: The CNE-2 cell line with Notch1 gene knockout was successfully constructed. The clonogenic assay showed knockout of Notch1 enhanced the radiosensitivity of NPC cells. The CCK-8 assay showed that cell proliferation and cell viability were significantly reduced in the IR+Notch1(-) group compared with the IR+Notch1(+) group(P < 0.05). Annexin V-FITC/PI double staining assay showed that the IR+Notch1(-) group had the highest apoptosis rate compared with the other groups (P < 0.05). Western blotting demonstrated that the expression of γH2AX was significantly increased after irradiation of Notch1 nasopharyngeal carcinoma cells, the expression of Cyclin-D1 was increased, and the ratio of Bax: Bcl-2 was higher. CONCLUSION: Knockout of Notch1 signaling molecule can effectively improve the radiosensitivity of NPC cells cultured in vitro, which may be a potential target for radiosensitization of NPC.

Summary Background The long-term consequences of discontinuing antipsychotic medication after successful treatment of first-episode psychosis are not well studied. We assess the relation between early maintenance therapy decisions in first-episode psychosis and the subsequent clinical outcome at 10 years. Methods This is a 10 year follow-up study, spanning Sept 5, 2003, to Dec 30, 2014, of a randomised, double-blind trial in seven centres in Hong Kong in which 178 patients with first-episode psychosis with full positive symptom resolution after at least 1 year of antipsychotic treatment were given maintenance treatment (n=89; oral quetiapine 400 mg daily) or early treatment discontinuation (n=89; placebo) for 12 months. After the trial, patients received naturalistic treatment. Overall this cohort of patients will have received about 3 years of treatment before entering the follow-up phase of the study: about 2 years of maintenance treatment before study entry and 1 year of treatment in the trial. The primary outcome of this follow-up was the proportion of patients in each group (including those for whom direct follow-up was not available) with good or poor long-term clinical outcomes at 10 years, with poor outcome defined as a composite of persistent psychotic symptoms, a requirement for clozapine treatment, or death by suicide. The randomised trial was registered with ClinicalTrials.gov, number NCT00334035, and the follow-up study was registered with ClinicalTrials.gov, number NCT01926340. Findings Poor 10 year clinical outcome occurred in 35 (39%) of 89 patients in the discontinuation group and 19 (21%) of 89 patients in the maintenance treatment group (risk ratio 1·84, 95% CI 1·15–2·96; p=0·012). Suicide was the only serious adverse event that occurred in the follow-up phase (four [4%] patients in the early discontinuation group vs two [2%] in the maintenance group). Interpretation In patients with first-episode psychosis with a full initial response to treatment, medication continuation for at least the first 3 years after starting treatment decreases the risk of relapse and poor long-term clinical outcome. Funding Food and Health Bureau, Research Grants Council of Hong Kong, and AstraZeneca.

Background Individuals with first-episode psychosis (FEP) have markedly elevated risk for suicide. Previous research on suicidality in early psychosis mainly focused on attempted and completed suicide. Data regarding risk factors for suicidal ideation, which is a common antecedent and predictor of suicide attempt, were limited. This study aimed to examine the prevalence of suicidal ideation and its relationships with clinical, neurocognitive and psychological factors in FEP patients. Method Eighty-nine Chinese patients aged 15 to 25 years presenting with FEP to specialized early intervention service were recruited. A comprehensive set of assessments examining pre-treatment illness characteristics, symptom severity, neurocognitive function, and psychological factors were administered. Current suicidal ideation and history of suicide attempt were systematically evaluated. Results Approximately 42% of patients expressed suicidal ideation after service entry. Univariate regression analyses found that suicidal ideation was significantly associated with past suicide attempt, depressive symptoms, emotion expressivity, hopelessness, future expectation, attentional impulsiveness, internal and external locus of control, and the likelihood of endorsing fear of social approval and survival and coping beliefs as reasons for living. Final multivariate model showed that previous suicide attempt, depression, less severe diminished expression, greater degree of hopelessness and lower level of internal locus of control independently predicted suicidal ideation. Conclusions Suicidal ideation was prevalent in FEP patients. Our findings implied that close monitoring and prompt intervention of those potentially modifiable risk factors for suicidal ideation including depression, hopelessness and perceived inadequate personal control may reduce suicide risk in the early course of psychotic illness.

The study evaluated the safety of reclaimed water using health risk assessment and biotoxicity tests. The reclaimed water was produced from reverse osmosis and used in industrial and miscellaneous purposes. The health risk assessment was conducted based on the concentrations of detectable pollutants in reclaimed water in a hypothetical scenario. The estimated carcinogenic and non-carcinogenic risks are lower than the generally accepted level. Biotoxicity evaluation included three genotoxicity tests, a chronic toxicity test using medaka fishes, and a subchronic toxicity test using mice. The reclaimed water is not genetically toxic, and does not cause significant chronic effects on these model organisms. These results confirm the safety of using reclaimed water from municipal wastewater treatment plants.

Background Cardiovascular magnetic resonance (CMR) is ideal for assessing patients with repaired aortic coarctation (CoA). Little is known on the relation between long-term complications of CoA repair as assessed by CMR and clinical outcome. We examined the prevalence of restenosis and dilatation at the repair site and the long-term outcome in patients with repaired CoA. Methods and results CMR imaging and clinical data for adult CoA patients (247 patients aged 33.0±12.8years, 60% male), were analyzed. The diameter of the aorta at the repair site was measured on CMR and its ratio to the aortic diameter at the diaphragm (repair site-diaphragm ratio, RDR) was calculated. Restenosis (RDR≤70%) was present in 31% of patients (and significant in 9% [RDR 150%) in 13.0%. A discrete aneurysm at the repair site was observed in 9%. Restenosis was more likely after resection and end–end anastomosis, whereas dilatation after patch repair. Systemic hypertension was present in 69% of patients. Of the hypertensive patients, blood pressure (133±20/73±10mmHg) was well controlled in 93% with antihypertensive therapy. Mortality rate over a median length of 5.9years was low (0.69% per year, 95% CI: 0.33–1.26), but significantly higher than age-matched healthy controls (standardised mortality ratio 2.86, CI 1.43–5.72, p Conclusion Restenosis or dilatation at the CoA repair site as assessed by CMR is not uncommon. Medium term survival remains good, however, albeit lower than in the general population. Life-long follow-up and optimal blood pressure control are likely to secure a good longer term outlook in these patients.

We sought to improve understanding of the diastolic prolongation of forward flow seen through a unilateral branch pulmonary artery (PA) stenosis.Of patients studied by cardiovascular magnetic resonance (CMR) for congenital heart disease, we reviewed right and left PA flow to find 10 cases with a diastolic prolongation of flow in a stenosed branch PA. They were aged 20 years (median, range = 14-40 years, 7 males). Seven had transposition of the great arteries corrected by arterial switch (TGA-AS) and 3 had repaired tetralogy of Fallot (rToF). All had at least moderate unilateral stenosis and competent pulmonary valves. For comparison, we identified 10 patients with unilateral stenosis and at least moderate pulmonary regurgitation, 9 rTOF and 1 TGA-AS, aged 23.5 years (range = 14-42 years, 6 males). Flow in each PA was measured, and in 10 healthy volunteers aged 27 years (range = 20-42 years, 5 males). The curves of flow through stenosed and non-stenosed arteries were averaged for each patient group and compared with those from controls. In competent pulmonary valve patients, the minimum diameters of the stenosed versus non-stenosed branches were as follows (median [range]): 4 [3-8] mm versus 13.5 [10-28] mm, p0.001, although their flows differed a little: 2.5 [1.5-6.8] L/min versus 3.2 [1.2-8.8] L/min, p=0.6. No diastolic tail was identifiable in the patients with unilateral PA stenosis and pulmonary regurgitation, where stenosed versus non-stenosed diameters were 7 [4-12] mm versus 20.5 [13-33] mm, p0.001, and flows differed: 1.3 [0.4-2.9] L/min versus 3.8 [3.0-5.0] L/min, p0.001. No controls showed stenosis or diastolic tail.Beyond a competent pulmonary valve, flow through a unilateral PA stenosis, although limited in systole, can continue into diastole, maintaining flow to the lung.

Ebstein's anomaly involves both the right ventricle (RV) and tricuspid valve.The functional RV and tricuspid orifice were traced from magnetic resonance images in 29 adult Ebstein patients and 9 normal subjects and reconstructed for visualization and measurement of regional RV size, function, and shape at 20 cross sections, and inlet and outflow tract ejection fractions (EFs).The RV in Ebstein's had RV dilation (end diastolic volume index 179 ± 69 vs. 84 ± 22 ml/m(2) in normals, p0.001) and global dysfunction (EF 45 ± 8 vs. 55 ± 5% in normals, p0.001). Longitudinal contraction was preserved (26 ± 13 vs. 26 ± 4 mm in normals) and correlated more weakly with EF than short axis fractional shortening (r=0.44 vs. r=0.71, p0.05 for both). The apical region in Ebstein's RV was enlarged, rounded and contributed more than normal to the global stroke volume. However this contribution correlated inversely with global EF. In contrast slices in the basal region had normal cross sectional area and their function correlated directly with global EF. Inlet EF was depressed (46 ± 8% vs. 55 ± 6 in normals, p=.002); outflow tract EF was even more depressed (39 ± 14, p=0.019).The three dimensional shape of the RV in adult Ebstein patients was demonstrated. The Ebstein's RV remodels in diverse regional patterns rather than following a shape continuum. Changes at the apex and base had opposing effects on function. Global EF was supported more by short axis than longitudinal contraction.

Retromuscular ventral hernia repair with mesh is a durable technique. In this paper, we describe a novel technique which allows for significant mesh overlap via the retromuscular space in cases of massive ventral hernia.The retromuscular space is developed laterally, to the edge of the rectus sheath. The posterior rectus sheath is incised, dividing the posterior aponeurosis of the internal oblique. The dissection is carried out laterally between the internal oblique and the transversus abdominis muscle, creating space for a large mesh underlay.We have performed this technique successfully in 20 patients with a mean defect area of 223 cm(2) and a mean mesh area of 698 cm(2). Three patients developed wound complications and none complained of long-term pain or abdominal wall deformity. There has been one recurrence due to technical error after a mean 12-month follow-up.This technique of dissection between the internal oblique and transversus abdominis muscles allows for the closure of large hernia defects. The mechanism is two-fold: (1) mobility for closure of the posterior rectus sheath, dorsal to the prosthetic; and (2) increased mobility of the rectus, internal, and external obliques, allowing reconstruction of the linea alba.

We investigated the roles of tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) and its receptor death receptor 5 (DR5) in the onset of acute leukemia and changes in their expression during chemotherapy. Bone marrow samples from 16 patients newly diagnosed with acute leukemia were collected before chemotherapy. Bone marrow samples from patients with non-hematologic malignancies served as the control group. Peripheral blood samples of patients with acute leukemia were also collected before chemotherapy and at 1 and 3 days after chemotherapy. Mononuclear cells in the bone marrow and peripheral blood were isolated and used to detect the expression of TRAIL and DR5 by flow cytometry. Compared with mononuclear cells from the control group, mononuclear cells from newly diagnosed patients with acute leukemia showed no significant difference in the expression of TRAIL (P0.05) but showed significantly increased expression of DR5 (P0.05). TRAIL and DR5 expression in peripheral blood mononuclear cells after chemotherapy was significantly increased compared to expression before chemotherapy (P0.05). Patients showing high expression of DR5 had a higher remission rate. One of the mechanisms underlying the treatment of leukemia with chemotherapy drugs may be the induction of TRAIL and DR5, which may promote apoptosis in leukemia cells. TRAIL-mediated apoptosis is regulated by DR5 expression.

To explore in psychosis patients their subjective perceptions on the definition of relapse, the costs involved, the potential risk and protective factors for relapse, the effect of medication and the consequences of relapse.Sixteen patients who had previously enrolled into a randomized controlled trial of medication discontinuation during first-episode psychosis were interviewed after 10 years.Patients had a broader definition of the criteria of relapse, extending from medication discontinuation to family support. Although most agreed that taking antipsychotics is effective in preventing relapse, most have negative views on them. All patients predicted their risk of relapse to be less than 50% and they were generally optimistic about the future.These findings have implications for development of effective medication compliance and relapse prevention programmes in psychosis.

Background Exercise capacity relates to right ventricular (RV) volume overload in congenital heart disease and may improve after surgery. We herewith investigate the relation between exercise capacity, cardiac index, and RV volume overload due to tricuspid regurgitation (TR) in Ebstein's malformation and pulmonary regurgitation (PR) after repair of tetralogy of Fallot (rToF). Methods We measured cardiac index and tricuspid/pulmonary regurgitant fraction by cardiovascular magnetic resonance in patients with Ebstein's malformation (n=40) or rTOF (n=53) with at least moderate TR/PR and 24 healthy controls. Exercise tolerance was determined by peak oxygen consumption (peak VO 2 ) during cardiopulmonary exercise testing. Results TR and PR fraction were similar in Ebstein and rTOF patients (43±17% versus 39±12%, respectively). Cardiac index was reduced in Ebstein (2.7±0.6L/min/m 2 compared to controls 3.5±0.9L/min/m 2 , p 2 ). Multiple regression analysis revealed a significant correlation between peak VO 2 and cardiac index in Ebstein. Furthermore, peak VO 2 correlated with peak heart rate in both groups but not with regurgitation fraction. Conclusions Despite comparable amounts of regurgitation from a right sided heart valve in patients with Ebstein and rToF, reduction of cardiac index was observed only in the former group. Greater physiologic complexity and adverse ventricular interaction with chronotropic incompetence in Ebstein's malformation may account for this.

Stigma is a major factor causing delayed help-seeking and poor treatment adherence in patients with psychotic disorders. Previous research has mostly focused on chronic samples and the impact of culturally-relevant variables on both patients’ and their caregivers’ stigmatization is understudied. This study aimed to examine the relationships between various forms of stigma, “face concern”, and clinical characteristics in a group of Chinese first-episode psychosis (FEP) patients and their caregivers. Forty-four Hong Kong Chinese aged 15–54 years presenting with FEP to psychiatric services and their caregivers were recruited. Assessments on self-stigma, affiliate stigma, perceived public stigma, “face concern”, symptom severity and subjective quality of life (QoL) were conducted. Self-stigma of FEP patients was correlated with perceived public stigma, “face concern”, insight and psychological health of QoL. Multiple regression analysis revealed that perceived public stigma and “face concern” independently predicted self-stigma. Mediation analysis further suggested that “face concern” partially mediated the relationship between perceived public stigma and self-stigma. Caregivers’ affiliate stigma was significantly associated with higher levels of stress, and symptoms of depression and anxiety. Affiliate stigma did not correlate with perceived public stigma and “face concern”. Our results indicate a critical role of perceived public stigma and fear of losing face in determining self-stigma in Chinese patients with FEP. Caregivers with greater degree of affiliate stigma experience increased stress and emotional distress. Our findings highlight the importance to examine culturally specific factors that may contribute to the development of self-stigma in first-episode populations of different ethnicities.

Individuals with first-episode psychosis (FEP) have markedly elevated risk for suicide. Previous research investigating risk factors of suicidal behavior in FEP mainly focused on western populations. This study aimed to examine the prevalence, and pretreatment and early illness predictors of suicidal behavior in Chinese FEP patients.Seven hundred participants aged 15-25 years consecutively enrolled in a territory-wide treatment program for FEP in Hong Kong from July 2001 to August 2003 were studied. Baseline and 3-year follow-up variables were collected via systematic medical file review. Suicidal behavior evaluated in this study comprised suicide attempt and completed suicide.Seventy-four (10.6%) participants attempted suicide before treatment. Ten percent (n = 70) exhibited suicidal behavior and 1% (n = 7) committed suicide during 3-year follow-up. The majority of post-treatment suicidal behaviors (51.4%) occurred in the first year of follow-up. Jumping from a height was the most common method of committing suicide. Multivariate regression analysis indicated that previous suicide attempt, history of substance abuse and poorer baseline functioning were significantly associated with an increased risk for suicidal behavior after treatment initiation.In a large representative cohort of Chinese young FEP patients, the rates of suicidal behavior before and after treatment and risk factors for post-treatment suicidal behavior were comparable to the findings of previous research on early psychosis conducted in western populations. Close monitoring of high-risk patients with history of attempted suicide or substance abuse, and enhancement of psychosocial functioning may help reduce suicide risk in the early stage of psychotic illness.

Background Since the introduction of consensus criteria for symptomatic remission in 2005, most first-episode studies focused on cross-sectional relationship between remission status and functional outcome. Predictive validity of the proposed remission definition was under-studied. Relationship of remission with subjective quality of life (QoL) was inadequately addressed with inconsistent findings being observed. Method One hundred and four Hong Kong Chinese aged 18 to 55 years presenting with first-episode schizophrenia-spectrum disorder were studied. Socio-demographics, baseline clinical and functioning profiles were obtained. Psychopathological and functional reassessments were conducted at 6, 12 and 24 months. Subjective QoL was measured at 12 and 24 months. Symptomatic remission was operationally defined according to the consensus criteria developed by the Remission in Schizophrenia Working Group (RSWG), comprising both symptom-severity and 6-month duration components. Results At 12 months, 59.6% of patients achieved symptomatic remission. Remitted patients had significantly lower symptom severity, more favorable psychosocial functioning and better subjective QoL at 12 months than non-remitted counterparts. Attainment of remission at 12 months was significantly associated with fewer positive, negative, depressive and disorganization symptoms, higher levels of functioning and subjective QoL at 24 months. Linear regression analysis indicated that remission status independently predicted functional outcome even when the effects of educational level, baseline functioning and negative symptom scores were controlled for. Conclusion The operationally defined symptomatic remission formulated by the RSWG represented a clinically valid construct that was found to be closely related to both concurrent and longitudinal outcomes on psychopathology, functioning and subjective QoL in the early stage of schizophrenia.

The arterial switch operation (ASO) is currently the treatment of choice in neonates with transposition of the great arteries (TGA). The outcome in childhood is encouraging but only limited data for long-term outcome into adulthood exist.We studied 145 adult patients (age16, median 25 years) with ASO followed at our institution. Three patients died in adulthood (mortality 2.4/1000-patient-years). Most patients were asymptomatic and had normal left ventricular function. Coronary lesions requiring interventions were rare (3 patients) and in most patients related to previous surgery. There were no acute coronary syndromes. Aortic root dilatation was frequent (56% patients) but rarely significant (45 mm in 3 patients, maximal-diameter 49 mm) and appeared not to be progressive. There were no acute aortic events and no patient required elective aortic root surgery. Progressive neo-aortic-valve dysfunction was not observed in our cohort and only 1 patient required neo-aortic-valve replacement. Many patients (42.1%), however, had significant residual lesions or required reintervention in adulthood. Right ventricular outflow tract lesions or dysfunction of the neo-pulmonary-valve were frequent and 8 patients (6%) required neo-pulmonary-valve replacement. Cardiac interventions during childhood (OR 3.0, 95% CI 1.7-5.4, P0.0001) were strong predictors of outcome (cardiac intervention/significant residual lesion/death) in adulthood.Adult patients with previous ASO remain free of acute coronary or aortic complications and have low mortality. However, a large proportion of patients require re-interventions or present with significant right sided lesions. Life-long cardiac follow-up is, therefore, warranted. Periodic noninvasive surveillance for coronary complications appears to be safe in adult ASO patients.

Turbulent blood flow caused by arterial stenosis can induce platelet activation and aggregation, which subsequently participate in arterial thromboembolic events.To test the hypothesis that platelet activation (expressed by CD62p) is enhanced in cerebral vs systemic circulation in patients with severe internal carotid artery (ICA) stenosis.Platelet CD62p expression was prospectively measured using flow cytometry in 35 consecutive symptomatic patients with severe ICA stenosis and in 20 at-risk control subjects who underwent both coronary and cerebral angiographic studies due to angina pectoris and suspicious vertebral artery or intracranial artery stenosis. The CD62p expression was also evaluated in 20 healthy subjects. Blood samples were first drawn from the right internal jugular vein (cerebral circulation) and right femoral vein (systemic circulation) before extra- and intracerebral angiographic examination of both patients and at-risk control subjects and again at 40 minutes after ICA stenting. Clopidogrel was administered to the patients following the second blood sampling.Systemic CD62p expression was higher in patients than in both the healthy and at-risk control subjects (both p0.0001). Additionally, cerebral CD62p expression was higher in patients than in at-risk control subjects (p0.0001) prior to intervention. Moreover, CD62p expression was higher in cerebral circulation than in systemic circulation in the patients (p0.0001) before ICA stenting. However, CD62p expression was less enhanced in cerebral circulation than in systemic circulation after ICA stenting (p0.0001). Furthermore, CD62p expression was decreased after 3 months of follow-up (p0.0001).Compared to systemic CD62p expression, cerebral CD62p expression was more enhanced prior to ICA stenting and was less enhanced after ICA stenting in patients with severe ICA stenosis.

To analyze the bone-inducing activity of C terminal of hBMP-2 and get a new recombinant product of hBMP-2, the gene encoding 102 aa of hBMP-2 mature peptide C terminal was cloned and expressed in E. coli and the first Cys was mutated with Ser. The fragments encoding the target peptide were amplified and cloned into heat-inducible expression vector pDH and transformed into E. coli DH5 alpha. After induction, a new protein bond appeared on the SDS-PAGE. The expressed products amounted to 30% of the total bacterial protein, which existed in the form of inclusion body. The products of bacterial lysates were purified through the ion-exchange chromatography. The denatured proteins were dialysed and diluted directly into the refolding buffer. The renatured products were implanted into mouse thigh muscles to analyze their bone-inducing activity respectively. The results of histological assay showed that the 102 peptide of hBMP-2 could ectopically induce formation of bone, while the mutated 102 peptide of hBMP-2 could not. It suggested hBMP-2 102 peptide still had bone-inducing activity. The first Cys of hBMP-2 mature peptide might be necessary for integrity of three pairs of disulfide bond, and also essential for bone-inducing activity of hBMP-2.

With the development of aerospace technology, biological effects induced by alteration of gravitation are drawing more and more attention. Among them the influence of alteration of gravitation on cytokine-induced signal transduction has been well studied recently. Epidermal growth factor (EGF)-induced signal transduction can activate increase of cell proliferation in most cell types, so it is always a hotspot in these years. Among the early effects evoked by EGF are receptor clustering, cell rounding, and early gene expression. After the study about the induction of EGF on human A431 cell line, it was observed that EGF-induced c-fos and c-jun expression decreased in microgravity. This was caused by alteration of the EGF receptor and protein kinase C-mediated signal transduction pathways. Meanwhile the key component of cytoskeleton, the actin microfilament system, was found to be linked to the EGF-induced signal transduction cascades either. So it seems reasonable to suggest that the cytoskeleton constitutes the gravity-sensitive cell component.

We describe a 45-year-old Taiwanese man with specific features of Brugada syndrome but no clinical features of structural heart disease. He was successfully treated with an implantable cardioverter-defibrillator. His electrocardiogram (ECG) patterns changed intermittently. Alpha-adrenoceptor stimulation and beta-adrenoceptor blockade augmented the characteristic ST-segment elevation, whereas alpha-adrenoceptor blockade and beta-adrenoceptor stimulation mitigated the ST-segment elevation. Intravenous procainamide administration did not aggravate ST-segment elevation when ECG had shown coved ST elevation in the right precordial leads. Molecular study did not reveal the same mutations in the cardiac sodium channel gene (SCN5A) as previously reported in Brugada syndrome. This case demonstrates the genetic heterogeneity of SCN5A in Brugada syndrome.

We described a 16-year-old boy with sinoatrial nodal (SAN) artery aneurysm that drained into right atrium and compressed right ventricular outflow tract. The patient was clinically asymptomatic. Hemodynamic study revealed a 15 mm Hg peak systolic pressure gradient at right ventricular outflow tract. The fistula was successfully excised without sequalae. Cathet. Cardiovasc. Intervent. 51:328-331, 2000.

To establish normative data of maternal serum chorionic gonadotropin (hCG) during the second trimester in an Asian population.We measured the maternal serum hCG levels in 17,955 normal singleton pregnancies between 15 and 21 weeks of gestation. The gestation age was estimated by measurement of fetal biparietal distance (BPD) in all cases. Median values of hCG at various gestational weeks were calculated and the values of hCG were converted to multiple of median (MoM). The incidences of low MoM value and high MoM value were also calculated.The mean and median values of hCG were 57,153 mIU/ml and 50,120 mIU/ml, respectively, at 15 weeks of gestation and then decreased to 30,898 mIU/ml and 26,226 mIU/ml, respectively, at 21 weeks. We found 8.6% and 9.4% of normal singleton pregnancies have hCG MoM values2.0 MoM and0.5 MoM, respectively.Our report provides a normal reference data of second trimester maternal hCG levels by ultrasound dating in an Asian population.

The purpose of the present study is to evaluate the efficacy of second-trimester maternal serum screening program by using alpha-fetoprotein (AFP) and total human chorionic gonadotropin (hCG) in an Asian population. During June 1994 to July 1998, we conducted a prospective study of serum screening protocol for Down syndrome. The cut-off point for a positive result in this analysis was a risk of/=1/270. A total of 17,742 pregnant women with singleton pregnancy were screened, and 1,153 (6.5%) had positive result. Sixteen of the 17,742 pregnancies had Down syndrome, and 10 of them had positive result. The positive rate and detective rate for Down syndrome were 6.5 and 62.5%, respectively. However, the detective rate will reduce to 47.6% after being adjusted by age-specific risk. It is indicated that the double-marker test using AFP and total hCG is an effective screen strategy for second-trimester detection of Down syndrome in Asian women.

To evaluate the utility of sputum induction in the large-scale tuberculosis control program.Prospective study on sputum induction for improving the diagnostic yield of pulmonary tuberculosis, and estimation of the direct costs for sputum induction.Of 1,648 tuberculosis suspects with poor or absent sputum production, induced sputum was smear-positive in 558 patients (353 previously smear-negative, 97 inadequate sputum and 108 unproductive). The direct cost per induced sputum was US $0.37.Sputum induction is an effective, low-cost, and simple technique for improving the smear-positive case detection rate in a tuberculosis control program.

The purposes of this study were two fold. The first was to determine the effects of several independent variables on balance, and also on two muscular strength (dynamic and static) measurements respectively. The second purpose was to examine the relationship among the balance and the muscular strengths. Ninety-nine healthy children (58 boys and 41 girls) ranging in age from 9.6 to 12.7 years participated in this study. The subjects were selected from the 4th, 5th and 6th grades of an elementary school in Kaohsiung City. To assess balance, children were administered subtest 2 of Bruininks Oseretsky Test of Motor Proficiency (BOTMP). Dynamic strength was measured with the subtest 4 of the BOTMP. Static strength was measured quantitatively by "semi-squat two-hand lift". The Force Evaluation and Testing System was used for this testing. Height and weight measurements were obtained on each subject. The independent effects of variables on balance score and the two muscular strengths were assessed by stepwise multiple-regression analysis. The results revealed weight and dynamic strength were the effective predictors on estimating balance score. Gender and weight were found as important variables contributing in static load, whereas gender, weight, height and balance score were selected for significantly predicting dynamic strength score. Spearman correlation analysis revealed significant positive correlations between balance and dynamic strength and also between dynamic strength and static strength. The correlation analysis also showed a significant negative correlation between balance and static strength. Detailed implications of the results in the present study are discussed.

To determine the change of oesophageal manometry in patients with oesophageal varices before and after oesophageal variceal ligation (EVL).Forty-five patients who had liver cirrhosis and oesophageal varices with high risk of bleeding were managed by EVL. Oesophageal manometry was performed just prior to the ligation and 4-6 weeks after obliteration of varices. Another 45 age- and sex-matched patients without hepatic, oesophageal or systemic disease served as the control group.At 5 cm above the lower oesophageal sphincter (LES), the amplitude of the contractive wave was significantly lower in patients before EVL (56.9 +/- 31.8 vs 80.1 +/- 30.1, P1.05) and returned to the level of control subjects after EVL (76.5 +/- 37.0 vs 80.1 +/- 30.1, P0.05). At 10 cm above LES, the amplitude of the contractive wave was significantly lower in patients before and after EVL than the control group (54.3 +/- 29.2 vs 68.1 +/- 29.5, 54.2 +/- 26.0 vs 68.1 +/- 29.5, respectively, P0.05). The percentage of tertiary waves was significantly higher in patients before and after EVL than in the control group (31.4 +/- 36.6 vs 5.8 +/- 15.1, 26.9 +/- 32.9 vs 5.8 +/- 15.1, respectively, P0.05). However, no significant swallowing disturbance was noted in patients after EVL. There was significantly greater LES length in patients before EVL (4.0 +/- 0.9 vs 3.4 +/- 0.7, P0.05) but there was no significant difference in the LES length after EVL as compared with the control group. Eighty-six per cent (39/45) of patients developed paraoesophageal varices and 31% (14/45) developed new varices 6 months after variceal obliteration. However, there was no significant difference in manometry at the time of variceal obliteration between patients with variceal recurrence and those without.The presence of varices affected oesophageal motility. However, such abnormality had little clinical significance. Endoscopic variceal ligation normalized oesophageal motility and may not induce abnormal oesophageal motility. The manometric change can not be used to predict the recurrence of varices in cirrhotic patients after variceal obliteration.

To establish normative data for the width of the fetal cavum septi pellucidi at various gestational ages.A total of 608 consecutive fetuses between 19 and 42 gestational weeks were included in this prospective study. The largest width of the cavum septi pellucidi was measured by two-dimensional ultrasound. Piecewise regression analysis was used to study the relationships between the width of the fetal cavum septi pellucidi, gestational age and biparietal diameter.The fetal cavum septi pellucidi width increased gradually between 19 and 27 weeks of gestation and then plateaued between 28 weeks and term. Regression analysis revealed significant associations between cavum septi pellucidi width and gestational age, and cavum septi pellucidi width and biparietal diameter.The present study provides normative data for fetal cavum septi pellucidi width and useful information about the development of the midline brain structure in the fetus.

Squamous cell carcinoma (SCC) cells refractory to initial chemotherapy frequently develop disease relapse and distant metastasis. We show here that tumor suppressor WW domain-containing oxidoreductase (WWOX) (also named FOR or WOX1) regulates the susceptibility of SCC to methotrexate (MTX) in vitro and cure of SCC in MTX therapy. MTX increased WWOX expression, accompanied by caspase activation and apoptosis, in MTX-sensitive SCC cell lines and tumor biopsies. Suppression by a dominant-negative or small interfering RNA targeting WWOX blocked MTX-mediated cell death in sensitive SCC-15 cells that highly expressed WWOX. In stark contrast, SCC-9 cells expressed minimum amount of WWOX protein and resisted MTX-induced apoptosis. Transiently overexpressed WWOX sensitized SCC-9 cells to apoptosis by MTX. MTX significantly downregulated autophagy-related Beclin-1, Atg12-Atg5 and LC3-II protein expression and autophagosome formation in the sensitive SCC-15, whereas autophagy remained robust in the resistant SCC-9. Mechanistically, WWOX physically interacted with mammalian target of rapamycin (mTOR), which potentiated MTX-increased phosphorylation of mTOR and its downstream substrate p70 S6 kinase, along with dramatic downregulation of the aforementioned proteins in autophagy, in SCC-15. When WWOX was knocked down in SCC-15, MTX-induced mTOR signaling and autophagy inhibition were blocked. Thus, WWOX renders SCC cells susceptible to MTX-induced apoptosis by dampening autophagy, and the failure in inducing WWOX expression leads to chemotherapeutic drug resistance.