Complement factor H mutation in familial thrombotic thrombocytopenic purpura with ADAMTS13 deficiency and renal involvement

Thrombotic thrombocytopenic purpura is a rare disorder of small vessels that is associated with deficiency of the von Willebrand factor-cleaving protease ADAMTS13, which favors platelet adhesion and aggregation in the microcirculation. The disease manifests mainly with central nervous system symptoms, but cases of renal insufficiency have been reported. Presented are findings of the genetic basis of phenotype heterogeneity in thrombotic thrombocytopenic purpura in two sisters within one family. The patients had ADAMTS13 deficiency as a result of two heterozygous mutations (causing V88M and G1239V changes). In addition, a heterozygous mutation (causing an S890I change) in factor H of complement was found in the patient who developed chronic renal failure but not in her sister, who presented with exclusive neurologic symptoms.     

Relation of timing of cardiac catheterization to outcomes in patients with non-ST-segment elevation myocardial infarction or unstable angina pectoris enrolled in the multinational global registry of acute coronary events

We assessed whether timing of catheterization is associated with the type of non-ST-segment elevation acute coronary syndrome and/or outcome in patients who were enrolled in the Global Registry of Acute Coronary Events. Overall, 8,853 patients who had unstable angina pectoris or non-ST-elevation myocardial infarction were categorized according to timing of catheterization: expeditive (<24 hours), early (24 to 48 hours), and delayed (>48 hours). Patients in the delayed group were older, more frequently had previous myocardial infarction or stroke, and had a higher risk score compared with those in the expeditive and early groups (all p < or =0.001). Killip class IV at admission, non-ST-elevation myocardial infarction, and Q waves after the index electrocardiogram were more common in the expeditive group (all p <0.0001). Patients in the expeditive and early groups were treated more aggressively with medications than were those in the delayed group. The in-hospital composite end point (death, stroke, or major bleed) occurred most frequently in the expeditive group (expeditive 6.6%, early 3.9%, delayed 5.1%, p = 0.0005), as did in-hospital death (expeditive 3.5%, early 1.4%, delayed 2.0%, p <0.0001). The highest incidence of death during follow-up occurred in the delayed group (3.8% delayed vs 2.8% expeditive/early, p = 0.0210). Multivariate regression analysis suggested that expeditive catheterization was related to in-hospital death and death from time of catheterization to 6 months. We conclude that expeditive catheterization is associated with unstable presenting features that contribute significantly to the higher risk of death and death or myocardial infarction in hospital compared with patients who undergo later catheterization.     

Antimicrobial postexposure prophylaxis for anthrax: adverse events and adherence

We collected data during postexposure antimicrobial prophylaxis campaigns and from a prophylaxis program evaluation 60 days after start of antimicrobial prophylaxis involving persons from six U.S. sites where Bacillus anthracis exposures occurred. Adverse events associated with antimicrobial prophylaxis to prevent anthrax were commonly reported, but hospitalizations and serious adverse events as defined by Food and Drug Administration criteria were rare. Overall adherence during 60 days of antimicrobial prophylaxis was poor (44%), ranging from 21% of persons exposed in the Morgan postal facility in New York City to 64% of persons exposed at the Brentwood postal facility in Washington, D.C. Adherence was highest among participants in an investigational new drug protocol to receive additional antibiotics with or without anthrax vaccine--a likely surrogate for anthrax risk perception. Adherence of <60 days was not consistently associated with adverse events.     

肾癌骨转移临床诊疗专家共识(2008版)

肾细胞癌(renal cell carcinoma,RCC)是起源于肾小管和集合小管上皮系统的恶性肿瘤,简称肾癌,占肾脏恶性肿瘤的80%～90%.我国肾癌的发病率和死亡率呈逐年上升趋势,近几年增加明显.与20世纪80年代相比,目前早期肾癌发现比例升高,晚期肾癌比例下降,其中无症状肾癌的比例从8%上升至33%.     

Combination therapy for hypertension in the elderly: a sub-analysis of the Combination Therapy of Hypertension to Prevent Cardiovascular Events (COPE) Trial

The Combination Therapy of Hypertension to Prevent Cardiovascular Events (COPE) trial demonstrated that the calcium-channel blocker benidipine-based combination therapies with an angiotensin-receptor blocker (ARB), a β-blocker, or a thiazide diuretic (thiazide) were similarly effective in preventing cardiovascular events and achieving the target blood pressure (BP; <140/90?mm?Hg). We further evaluated the efficacy and safety of these combination therapies in older (65 years) and younger (<65 years) hypertensive patients. In this sub-analysis of the COPE trial 3293 patients (153365 years old and 1760 <65 years old) were randomly assigned to receive benidipine-based therapy with an ARB, a β-blocker or a thiazide. In each group, the average BP did not differ among the three treatment groups. The incidence of the primary cardiovascular composite end point in the older group was higher than in the younger group (12.7 vs. 8.3 per 1000 person-years, P=0.023). The primary composite cardiovascular end point, achievement (%) of target BP, and cardiovascular hard composite end points were similar among the three treatment groups. However, the hazard ratios and 95% confidence intervals in older patients were 2.74 (1.08-6.96; β-blocker vs. thiazide, P=0.022) for fatal and non-fatal stroke, and 2.47 (1.03-5.91; β-blocker vs. ARB, P=0.043) for new-onset diabetes. Thus, benidipine combined with an ARB, a β-blocker, or a thiazide was similarly effective in preventing cardiovascular events and achieving the target BP in both older and younger hypertensive patients. Further studies will be necessary to evaluate the usefulness of benidipine combined with a β-blocker in terms of the incidence of stroke and new-onset diabetes in older patients.