Factor VIII and vWF deficiency in STT3A-CDG.

STT3A-CDG (OMIM# 615596) is an autosomal recessive N-linked glycosylation disorder characterized by seizures, developmental delay, intellectual disability, and a type I carbohydrate deficient transferrin pattern. All previously reported cases (n = 6) have been attributed to a homozygous pathogenic missense variant c.1877C>T (p.

Upper limb strength: study providing normative data for a clinical handheld dynamometer.

Objective: To establish normative clinical data for upper extremity strength of men and women, ages 20-64 years, using a portable clinical device, the Nicholas Manual Muscle Tester (NMMT).

Design: The study collected objective upper extremity strength data for 180 healthy men and women using the NMMT.

Setting: The study was conducted in outpatient and community settings.

Utility of stereoscopic displays for indirect-vision driving and robot teleoperation.

The effectiveness of an active shutter-glasses stereoscopic display (SD) and a passive polarised SD was evaluated in a live robot-teleoperation task and a simulated indirect-vision driving task in various terrains. Overall, participants completed their tasks significantly faster with the SDs in three-dimensional (3D) mode than with the SDs in the baseline 2D mode. They also navigated more accurately with the SDs in 3D mode.

The effect of oxandrolone on the healing of chronic pressure ulcers in persons with spinal cord injury: a randomized trial.

Background: Anabolic steroids have been reported to improve wound healing.

Objective: To determine whether oxandrolone increases the percentage of target pressure ulcers (TPUs) that heal compared with placebo and whether healed ulcers remain closed 8 weeks after treatment.

Design: Parallel-group, placebo-controlled, randomized trial conducted from 1 August 2005 to 30 November 2008.

Multicenter randomized controlled trial of bacterial interference for prevention of urinary tract infection in patients with neurogenic bladder.

Objectives: To compare the effectiveness of bacterial interference versus placebo in preventing urinary tract infection (UTI).

Methods: The main outcome measure was the numbers of episodes of UTI/patient-year. Randomization was computer generated, with allocation concealment by visibly indistinguishable products distributed from a core facility.

Trendelenburg chest optimization prolongs spontaneous breathing trials in ventilator-dependent patients with low cervical spinal cord injury.

Chest optimization, an evidence-based protocol-guided multimodal chest physiotherapy consisting of body positioning, sputum mobilization, bronchodilation, and lung hyperinflation, may be routinely administered to ventilator-dependent patients with low cervical spinal cord injury (CSCI) for improving pulmonary functional outcomes that facilitate weaning from mechanical ventilation. We undertook this study to determine whether position-specific chest optimization was associated with changes in spontaneous breathing trial (SBT) duration. Cardiac output (CO), alveolar minute volume (MValv), carbon dioxide elimination (VCO(2)), and static chest compliance (Cst) were measured during chest optimization; then MValv and rapid shallow breathing index (RSBI) were measured during SBT.

Metabolic syndrome: soybean foods and serum lipids.

Metabolic syndrome is a cluster of coronary heart disease (CHD) risk factors of which central obesity, insulin resistance, increased triglycerides/decreased HDL cholesterol, and hypertension are major cardiovascular risk factors. The educational objectives of this review are to describe hypocholesteromic effects from soybean foods. Early Italian observations indicated that isolated soy protein lowered total cholesterol, especially the LDL component, in humans with elevated serum lipids.

Packaging challenges: the solution is just beyond the border.

The never-ending need to reduce costs and the emergence of new products that are a hybridisation of medical device and pharmaceutical technologies are two major challenges facing today's medical device industry. In both cases device manufacturers may find ready solutions by simply looking beyond their traditional borders.

Direct seal papers for horizontal form-fill-seal machine.

Direct-seal papers are designed for minimal fibre lift, where the sealing layer is incorporated into the bottom web. There are applications where they are the most appropriate economical alternative to traditional coated papers for horizontal form-fill-seal packaging. This article explores their benefits and limitations.