We demonstrated a significant association between frequent RVOT PVCs and LV dysfunction in patients without structural heart disease.

Summary:We retrospectively compared the incidence of acute graftversus-host disease (GVHD) before and after September 1999, when we changed the mode of cyclosporine A (CsA) administration from twice-daily infusions (TD) (n ¼ 58) to continuous infusion (CIF) (n ¼ 71). The incidence of grade II-IV acute GVHD in the CIF group (56%) was significantly higher than that in the TD group (27%, P ¼ 0.00022). Multivariate analysis identified only two independent significant risk factors for the development of grade II-IV acute GVHD; CIF of CsA (relative risk 2.59, 95% CI 1.46-4.60, P ¼ 0.0011) and the presence of HLA mismatch (2.01, 95% CI 1.15-3.53, P ¼ 0.014). The incidence of relapse was significantly lower in the CIF group when adjusted for disease status before transplantation (0.41, 95% CI 0.18-0.95, P ¼ 0.038), which resulted in better disease-free survival in high-risk patients (43 vs 16% at 2 years, P ¼ 0.039), but not in standard-risk patients (72 vs 80%, P ¼ 0.45). CIF of CsA with a target level of 250-400 ng/ml may not be appropriate for GVHD prophylaxis in standard-risk patients.

No abstract

Conductive wires were fabricated by electron-beam-induced deposition (EBID) using WF6 gas. It was difficult to fabricate highly conductive wires with good reproducibility unless samples were cleaned before EBID. Contamination appears to reduce the conductivity of the wires. O2 plasma cleaning of samples before EBID seems to reduce contamination growth; however, it is not effective for regions in the vicinity of Au patterns. We found that by combining annealing at 300°C and O2 plasma cleaning, highly conductive wires could be fabricated with relatively good reproducibility in such regions. A linear relation was found between wire conductance and linedose at lindoses of more than 70 µC/cm. The change in deposition yield estimated from the conductance was about 12% when the gas flux was halved. Wires with a length of less than 40 nm were less conductive than longer wires because of a shortage in gas supply.

Mutations in FBN1 cause a range of overlapping but distinct conditions including Marfan syndrome (MFS), Weill-Marchesani syndrome (WMS), familial thoracic aortic aneurysms/dissections (FTAAD), acromicric dysplasia (AD), and geleophysic dysplasia (GD). Two forms of acromelic dysplasia, AD and GD, characterized by short stature, brachydactyly, reduced joint mobility, and characteristic facies, result from heterozygous missense mutations occurring in exons 41 and 42 of FBN1; missense mutations in these exons have not been reported to cause MFS or other syndromes. Here we report on probands with MFS and WMS who have heterozygous FBN1 missense mutations in exons 41 and 42, respectively. The proband with WMS has ectopia lentis, short stature, thickened pinnae, tight skin, striae atrophicae, reduced extension of the elbows, contractures of the fingers and toes, and brachydactyly and has a missense mutation in exon 42 of FBN1 (c.5242T>C ;p.C1748R). He also experienced a previously unreported complication of WMS, an acute thoracic aortic dissection. The second proband displays classic characteristics of MFS, including ectopia lentis, skeletal features and aortic root dilatation, and has a missense mutation in exon 41 of FBN1 (c.5084G>A; p.C1695Y). These phenotypes provide evidence that missense mutations in exons 41 and 42 of FBN1 lead to MFS and WMS in addition to AD and GD and also suggest that all individuals with pathogenic FBN1 mutations in these exons should be assessed for thoracic aortic disease and ectopia lentis. Further studies are necessary to elucidate the factors responsible for the different phenotypes associated with missense mutations in these exons of FBN1.