Imaging of pediatric congenital heart disease

The evaluation of a patient with a heart murmur and congenital heart disease is diagnostically very challenging. Multiple advanced techniques aid in the diagnosis of simple and complex malformations. Interventional procedures and surgical corrections have allowed most patients to enjoy a good and productive lifestyle.     

临床疑似TTP-HUS患者血浆置换治疗引起的并发症

由于采用血浆置换(PE)治疗血栓性血小板减少性紫癜溶血性尿毒综合征(TTP HUS)频率的增加,PE引起的并发症也成为制定治疗方案时需要考虑的因素。由于TTP HUS的诊断常常是不确定的,掌握PE治疗利弊之间的平衡已成为制定适当治疗方案的焦点。之前,我们于1996年～2002年间曾两次报道     

Abnormal expression of hepatoma specific gamma-glutamyl transferase and alteration of gamma-glutamyl transferase gene methylation status in patients with hepatocellular carcinoma

Abnormal expression of hepatoma specific gamma-glutamyl transferase and alteration of gamma-glutamyl transferase gene methylation status in patients with hepatocellular carcinoma@Jiang DR @Huang ZW @Lu JX @Tao QY @Yu JZ @Meng XY     

HOMA insulin sensitivity index and the risk of all-cause mortality and cardiovascular disease events in the general population: the Australian Diabetes, Obesity and Lifestyle Study (AusDiab) study

Aims/hypothesis  

We assessed whether the relationships between insulin sensitivity and all-cause mortality as well as fatal or non-fatal cardiovascular disease (CVD) events are independent of elevated blood glucose, high blood pressure, dyslipidaemia and body composition in individuals without diagnosed diabetes.     

Extended efficacy and safety of denosumab in breast cancer patients with bone metastases not receiving prior bisphosphonate therapy

PURPOSE: Denosumab, a fully human monoclonal antibody to RANKL, suppresses bone resorption. This study evaluated the effects of denosumab in i.v. bisphosphonate (IV BP)-na?ve patients with breast cancer-related bone metastases. EXPERIMENTAL DESIGN: Eligible women (n = 255), stratified by type of antineoplastic therapy, were randomized to 1 of 5 blinded denosumab cohorts or an open-label IV BP cohort. Denosumab was administered s.c. every 4 weeks (30, 120, or 180 mg) or every 12 weeks (60 or 180 mg) through 21 weeks. Final efficacy results for up to 25 weeks are reported, including percentage change from baseline in urine N-telopeptide corrected for creatinine (uNTx/Cr) and incidence of skeletal-related events (SRE). Safety results are reported through the end of follow-up (up to 57 weeks). RESULTS: At week 13 and 25, the median percent changes in uNTx/creatinine (Cr) among patients with measurable uNTx were -73% and -75% for the pooled denosumab groups and -79% and -71% for the IV BP group. Among patients with > or =1 postbaseline measurement of uNTx at week 25, 52% (109 of 208) of denosumab-treated patients and 46% (19 of 41) of IV BP-treated patients achieved >65% uNTx/Cr reduction. On-study SREs occurred in 12% (26 of 211) of denosumab-treated patients and 16% (7 of 43) of IV BP-treated patients. Overall rates of adverse events were 95% in denosumab and IV BP groups. No denosumab-related serious or fatal adverse events occurred. CONCLUSIONS: In IV BP-na?ve breast cancer patients with bone metastases, denosumab suppresses bone turnover and seems to reduce SRE risk similarly to IV BPs, with a safety profile consistent with an advanced cancer population receiving systemic therapy.     

肝细胞生长因子诱导实验性视网膜脱离的病理机制研究

目的 观察肝细胞生长因子（HGF）对视网膜色素上皮（RPE）细胞屏障功能的影响以及RPE内过度表达HGF导致视网膜脱离（RD）的病理机制。 方法 编码HGF（AdCMV.HGF）、绿色荧光蛋白（Ad CMV.GFP）的E1/E3缺失的腺病毒载体，以5×10⁴ 噬斑形成单位（pfu）/眼注射到成年有色兔的视网膜下。检查注射后3、7、14、28 d时的眼底及组织病理变化,利用免疫组织化学和酶联免疫吸附试验（ELISA）方法检测HGF在视网膜和玻璃体的表达水平。 结果 对照组注射Ad CMV.GFP眼显示GFP几乎仅表达于PRE单核细胞层，AdCMV.HGF注射眼在注射点处的PRE细胞出现强的HGF免疫阳性反应。玻璃体内HGF的表达水平在注射7 d后达到最高峰、28 d后降低到基础水平。在HGF的表达期内AdCMV.HGF注射眼出现慢性RD和脉络膜慢性炎症。在RD区域，视网膜下的空间内可见增生性的RPE细胞，部分实验兔眼还产生多层的细胞膜结构。 结论 RPE内过度表达的HGF能引发慢性浆液性RD，同时伴有视网膜下RPE增生。提示HGF可能作为治疗RD的作用靶点。(中华眼底病杂志，2007，23：193-197)     

Genome-wide linkage analysis of the acute coronary syndrome suggests a locus on chromosome 2

A positive family history is a recognized cardiovascular risk factor, and genome-wide scans may reveal susceptibility loci for coronary artery disease. The acute coronary syndrome, consisting of myocardial infarction and unstable angina, is the most important manifestation of coronary disease and is characterized by atherosclerotic plaque disruption and coronary thrombosis. From approximately 6000 hospital admissions to cardiology units, we identified affected sibling pairs (n=61) who had documented acute coronary syndrome before the age of 70 years. A 10-cM resolution genetic map and MAPMAKER/SIBS were used for genome-wide linkage analysis. One locus on chromosome 2q36-q37.3 showed linkage with a lod score of 2.63 (P<0.0001). Separate multipoint fine-mapping of this locus with independent markers replicated the linkage results (lod 2.64). Two other regions on chromosomes 3q26-q27 and 20q11-q13 showed lod scores in excess of 1.5 (P<0.005). This genome scan in acute coronary syndrome suggests 1 locus that encompasses the gene encoding the insulin receptor substrate-1 gene. Two other potential loci were identified. These data imply that a limited number of potent susceptibility genes exist for the acute coronary syndrome. Such genes are likely to be relevant to the combined processes of atherosclerosis, plaque instability, and coronary thrombosis.     

Neonatal micrognathia is associated with small upper airways on radiographic measurement

In order to determine if infants with clinical micrognathia identified in the newborn period have smaller upper airways than do normal infants, and if their airway size is related to risk of later apnoea, respiration-timed upper airway radiographic measurements were performed in 21 asymptomatic neonates with clinical micrognathia. Their radiographic measurements were compared with those of a previously reported cohort of 35 normal infants. The micrognathic infants and a control group of 27 infants referred for parental anxiety were followed for 6 mo on home apnoea monitors. Sleep apnoea at home requiring stimulation by the parents occurred in 6 of 7 infants with micrognathia associated with craniofacial anomalies, 9 of 14 (64%) infants with isolated micrognathia, but only 1 of the 27 control infants (p < 0.001). Upper airway measurements at term of the infants with isolated micrognathia who later experienced apnoea were significantly smaller than either those of normal infants (p < 0.01) or of micrognathic infants who did not have apnoea requiring stimulation (p < 0.05). In conclusion, upper airway measurements on timed lateral radiographs in asymptomatic micrognathic infants at term (corrected age) revealed them to be smaller than those of normal infants. Narrower upper airways were associated with increased risk of subsequent apnoea requiring stimulation.