Sialidosis and galactosialidosis as the cause of non-immunologic hydrops fetalis

Two cases of non-immunological hydrops fetalis (NIHF) presenting with massive ascites are reported; in both patients an oligosaccharid-pattern in the urine typical for sialidosis resp. galactosialidosis was found. The cerebral sonography of both patients showed streaky echo enhancement in the region of the thalamostriatal vessels, which was interpreted as calcification of the vessels. The courses of the patients were characterised by recurrent infections, hepatosplenomegaly and myoclonus. Relevant literature reports on a large variability in the clinical appearance of oligosaccharidoses. The diagnosis of sialidosis is confirmed in cultured fibroblasts by the deficiency of alpha-N-acetylneuraminidase and, in case of galactosialidosis by the additional lack of beta-galactosidase. The precise diagnosis in NIHF is of increasing interest for prenatal diagnostic as well as for neonatological management.     

Optical Fluorescence and Its Application to an Intravascular Blood Gas Monitoring System

Optical fluorescence has an extensive history of application in the laboratory to the measurement of ionic concentrations and the partial pressures of oxygen and carbon dioxide. The use of optical fluorescence based sensors to fulfill a recognized need for continuous invasive monitoring of arterial blood gases offers a number of inherent advantages. However, the requirements placed upon a blood gas probe and supporting instrumentation appropriate for use in the clinical environment result in significant design challenges in selection of suitable fluorescent dyes, maintenance of mechanical integrity while obtaining required miniaturization of sensors, and in the transmission, acquisition, and processing of low level light signals. An optical fluorescence based intravascular blood gas monitoring system has been developed which is particularly suited for the critical care and surgical settings and which has a sensor probe that can be introduced into the patient via a radial artery catheter. This system has shown an excellent agreement of measured with true values of pH, pCO2, and P02 in both in vitro and animal studies. Linear regression analysis of typical in vitro data, where true levels were established via tonometry and standardization to a high accuracy laboratory pH measuring instrument, shows slope/intercept values very close to 1.0/0.0 and correlation coefficients of greater than 0.99 for all three parameters.     

Plasma cell and sclerosing osteomyelitis. A follow-up of 21 patients

Ten consecutive patients with incapacitating fecal incontinence were treated with 'anal dynamic graciloplasty' (transposition of the gracilis muscle around the anal canal and implantation of intramuscular electrodes connected with an implanted pulse generator, 6 weeks later) to achieve continence. We measured the gracilis muscle diameter immediately after transposition and before implantation of the stimulation device. It was found that gracilis diameter decreased from 12 (5 days after transposition) to 8 mm, 6 weeks later (mean decrease: 4 mm (95% confidence interval 3.6), n = 10, P < 0.05). In addition, morphology demonstrated a decrease of both Type I and Type II muscle fiber diameter and an increase in endomysial collagen. Despite this decrease in muscle (and muscle fiber) diameter, electrical stimulation of the transposed gracilis muscle increased the pressure into the anal canal from 37 to 55 mmHg (mean increase: 17 mmHg (95% confidence interval 6.29), P < 0.05). Fecal continence was achieved in seven (70%) of these patients. Further analysis revealed no correlations between reduction of the gracilis muscle diameter before implantation of the stimulation device and clinical outcome in terms of achieved continence and/or anal canal pressures. MRI is an excellent method to demonstrate the shape of gracilis muscle after transposition. However, the size of transposed gracilis muscle is not associated with the functional outcome.     

WebSphere Portal 6.1: an agile development success story

Agile software development techniques are promising to deliver software faster, in better quality, and at a lower cost. Most examples relate to small, co-located teams working on a completely new project, with no legacy. This article focuses on applying Agile methods to a world-wide project with hundreds of developers delivering a market-leading product.In contrast to projects starting from scratch, existing customers have natural demands for support, bug fixes, and new features that focus on their particular needs.This article provides an overview of the most important Agile software development methods in addition to presenting ideas and solutions on how to apply Agile ideas to a large, existing product or solution. Important aspects that should be considered while deciding on what the Agile setup for your next software project should look like are explained.     

Design and realization of high-power ripple-free superluminescent diodes at 1300 nm

To realize high-power superluminescent diodes (SLDs) emitting at the 1300-nm wavelength and to see how different structure parameters influence the device performances, three different epitaxial layers have been studied. It was found that the structure employing a graded-refractive-index separate-confinement heterostructure, with linearly graded p-doped cap layer and with eight quantum wells, is most suitable for high-power SLDs. With a proper design of a geometrical structure for SLDs, the obtained output power at 20/spl deg/C is about 25 mW under CW operation and 100 mW at 1.1 A under pulsed operation with no observable ripple.     

High-Frequency Energy–Assisted Vacuum Drying of Fresh Eucalyptus globulus

Worldwide, eucalyptus tree plantations have been established in appropriate climates because of fast growth and wood qualities suitable mainly for pulp. A potential exists of converting eucalyptus trees into lumber that may be of higher value than pulp. Conventional drying of lumber of Eucalyptus globulus is often difficult because of the occurrence of drying stresses, leading to collapse and checking. The special method of vacuum drying while heating the wood with high-frequency energy (75–77 mbar, 46–51°C) was used to obtain short drying times (5–13 days from green state to 10% final moisture content) and low crack amount.     

The Role of Spatial Development Frameworks in Transformation of the eThekwini Municipality,KwaZulu-Natal,South Africa: Reflecting on 20 Years of Planning

In the 20 years since the advent of a democratic government in South Africa, planned expenditure on infrastructure projects by municipalities has been used in part to redress inequalities and socioeconomic distortions created by apartheid. Our purpose in this article is to assess the effectiveness of planning instruments to achieve desired transformation in the case of the eThekwini Municipality. We evaluated demographic, spatial regional economic, settlement hierarchy, accessibility and functional analysis indicators to assess the eThekwini Spatial Development Framework (SDF) as it applies to the city of Durban, making particular use of the National Population Census results for 2001 and 2011, municipal data on housing and settlement distribution, the municipal evaluation roll, the Industrial Land Study of 2014 and the Eskom household survey of 2009. These data sets were mapped using Environmental Systems Research Institute (ESRI) GIS to analyse spatial changes over the decade from 2001 to 2011 to establish the ways and extent public investment guided by SDFs is responsive to spatial transformation imperatives. We found that, although there has been some economic and population growth, in-migration and densification of the inner city, there has been limited spatial transformation of the urban population, many communities still live in poverty, the traditional inequalities prevail and the benefits of democratic dispensation are elusive to many, despite substantial investment in infrastructure. Our analysis points to inadequacies of the planning tools and their application to spending public funds. Moreover, SDFs appear to be process and compliance-driven rather than inclusive of stakeholder concerns. We argue that they require substantial refinement to achieve the desired results.     

Isolation of viable Hodgkin and Reed-Sternberg cells from Hodgkin disease tissues

Hodgkin disease (HD) is characterized by a small number of malignant Hodgkin and Reed-Sternberg (H/RS) cells among a major population of nonmalignant cells. The analysis of H/RS cells has been hampered by their low frequency and fragility. Here, we describe the isolation of viable H/RS cells from HD affected tissues by high gradient magnetic cell sorting (MACS) according to expression of CD30. The cells were enriched to a purity of up to 50%. H/RS cells were distinguished from other CD30(+) cells by the expression of CD15, their size and granularity. No CD30/CD15 double-positive cells could be enriched from a lymph node affected by the lymphocyte predominant subtype of HD, activated lymph nodes or peripheral blood of healthy donors. For two cases of HD individual MACS-purified H/RS cells and H/RS cells micromanipulated from tissue sections of the same lymphoma specimens were analyzed for Ig gene rearrangements. In both cases, identical V gene rearrangements were amplified from both sources of H/RS cells, showing that H/RS cells were successfully enriched. Moreover, the finding that in both cases no additional Ig gene rearrangements other than the ones identified in the H/RS cells micromanipulated from tissue sections were amplified from the MACS-purified H/RS cells further supports the monoclonality of these cells throughout the affected lymph nodes. The isolation of viable H/RS cells ex vivo is prerequisite for a direct study of gene expression by those cells and of their interaction with cells in their vicinity.     

Mechanical Characterization of Lumber of Small-Diameter Hardwood Species after Different Drying Schedules

The influence on the drying behavior as well as the mechanical properties of lumber of small-diameter hardwoods at different low-temperature convection kiln dryings was investigated. Standard test methods in three-point-bending and the modulus of elasticity in three-point-bending, compression tests parallel and perpendicular to the grain, as well as Brinell hardness tests were performed in order to assess the drying quality. Furthermore, non-standard tensile tests perpendicular to the grain and fracture energy tests in radial/longitudinal and also in tangential/longitudinal crack propagation systems were carried out as a most sensitive test method. The results showed significant changes of the specific fracture energy, bending strength, compression strength parallel and perpendicular to the grain, and the Brinell hardness with good drying performance at the same time.