A subgroup of patients with Marfan syndrome (MFS) who have mutations in exons 24-32 of the FBN1 gene manifests severe atrioventricular valve insufficiency and skeletal problems as early as the neonatal period. These patients usually die in the first 2 years of life, thus a region between exons 24 and 32 of FBN1 is recognized as a critical region for this neonatal form of MFS (nMFS). Here, we report five consecutive patients who manifested a cardiovascular phenotype until infancy with mutations in the critical region for nMFS. Although three of these patients showed severe mitral regurgitation and died before reaching 1 year of age, the remaining two patients survived for over 5 years under medical and/or surgical interventions. Two splicing mutations and one missense mutation were identified in the three deceased patients, whereas two missense mutations were found in the two survivors. Currently, the clinical severity of patients with early-onset MFS harboring mutations in the critical region for nMFS seem to be more variable than ever thought, and intensive treatments are recommended even in this subgroup of patients with MFS.

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Very long ErSi2 nanowires were formed on a Si(110) substrate by a self-assembly process. The wires were highly parallel and grew in the Si<011> direction. Nanowires with lengths more than 50 µm were fabricated. It was observed by transmission electron microscopy that the wires were hexagonal crystal type, having their c-axis (<0001> direction) perpendicular to the Si(112) plane. Then the nanowires were deeply buried in the Si(110) surface. Energy-dispersive X-ray spectroscopy measurements indicated that the nanowires had a ErSi2 chemical composition. Electrical conductivity measurement of the nanowires formed on high-resistivity Si substrates showed that the ErSi2 nanowires were good conductors at room temperature. The ErSi2 nanowire might be a promising candidate in nanometer-sized inter connection.

Iron films produced using an arc plasma gun were studied by Mössbauer spectroscopy, and their surface features were examined using atomic force microscopy and scanning electron microscopy. The surface morphology of the iron films reflected the morphology of the substrates. When an aluminum foil with 100-nm grooves was employed as the substrate, the surface of the iron film also had grooves. The nuclear spin orientation of the iron tends to be linear along the grooves. The effects of deposition conditions on nuclear spin orientation, including the groove size on the aluminum substrates, the iron film thickness, and the temperature during deposition, were studied.