The effects of sample position and gas flow pattern on the sintering of a 7xxx aluminum alloy

The effects of sample position and gas flow pattern on the sintering of a 7xxx aluminum alloy Al-7Zn-2.5Mg-1Cu in flowing nitrogen have been investigated both experimentally and numerically. The near-surface pore distribution and sintered density of the samples show a strong dependency on the sample separation distance over the range from 2 mm to 40 mm. The open porosity in each sample increases with increasing separation distance while the closed porosity remains essentially unchanged. A two-dimensional computational fluid dynamics (CFD) model has been developed to analyze the gas flow behavior near the sample surfaces during isothermal sintering. The streamlines, velocity profile, and volume flow rate in the cavity between each two samples are presented as a function of the sample separation distance at a fixed nitrogen flow rate of 6 L/min. The CFD modeling results provide essential details for understanding the near-surface pore distribution and density of the sintered samples. It is proposed that the different gas flow patterns near the sample surfaces result in variations of the oxygen content from the incoming nitrogen flow in the local sintering atmosphere, which affects the self-gettering process of the aluminum compacts during sintering. This leads to the development of different near-surface pore distributions and sintered densities

New nomenclature and DNA testing guidelines for myotonic dystrophy type 1(DM1)

Myotonic dystrophy (DM; OMIM 160900, also known as dystrophia myotonica, myotonia atrophica and Steinert disease) is an autosomal dominant myotonic myopathy associated with abnormalities of other organs, including eyes, heart, endocrine system, central and peripheral nervous systems, gastrointestinal organs, bone, and skin.1 The mutation underlying DM is an expansion of an unstable cytosine-thymine-guanine (CTG) trinucleotide repeat in the 3' untranslated region of the myotonic dystrophy protein kinase (DMPK) gene in chromosome 19q13.3.2-4 In 1994, Thornton et al.5 described an autosomal dominant disorder similar to DM without CTG repeat expansion at the DM locus. Ricker et al.6 named this disease "proximal myotonic myopathy" (PROMM; OMIM 600109) because of predominantly proximal muscle weakness without atrophy as opposed to the distal muscle involvement seen in DM. Subsequently, Meola et al.7 described a variant of PROMM with unusual myotonic and myopathic features, which they named "proximal myotonic myopathy syndrome," and Udd et al.8 described a PROMM-like family with dystrophic features, which they named "proximal myotonic dystrophy" (PDM). Researchers at the University of Minnesota9,10 found another multisystemic myotonic disorder that closely resembles DM with distal muscle weakness but no CTG repeat expansion. Because of the close phenotypic resemblance to DM, they called this disease "myotonic dystrophy type 2" (DM2; OMIM 602668). In 1998, Ranum et al.9 assigned the DM2 locus to chromosome 3q in a large kindred. Shortly after that, Ricker et al.11 found that the majority of German PROMM families show linkage to the DM2 locus. PDM was also mapped to this region (Krahe and Udd, personal communication, 1999). Whether PROMM, PDM, and DM2 represent different phenotypic expressions of a disease caused by the same mutation or if they are allelic disorders remains to be determined. It is also possible that these disorders are caused by mutations in different genes that are closely linked in the chromosome 3q region.12 Furthermore, the disease loci in some typical PROMM families11 and other families with multisystemic myotonic disorders have been excluded from both DM and DM2 loci. Because of the genetic and phenotypic heterogeneity in this group of disorders, it became necessary to establish a new nomenclature foreseeing the future discovery of new disease loci and phenotypic variability