Health Communication and Adherence to Noninvasive Ventilation in Chronic Hypercapnic Respiratory Failure: A Randomized Clinical Trial.

Importance: Domiciliary noninvasive ventilation (NIV) is a standard treatment for improving health outcomes among patients with chronic hypercapnic respiratory failure (CHRF). However, poor adherence substantially limits its therapeutic effectiveness.

Objective: To determine the effect of an information-motivation-behavioral (IMB) skills-based intervention (IMB-NIV program) on NIV adherence, patient-reported health outcomes, and health service use among patients with CHRF.

Use of triple correlations for the sign determinations of expansion coefficients of symmetric approximations to the diffraction volumes of regular viruses.

An X-ray free electron laser is a new source of x-rays some 10 × 10(9) times brighter than any previous X-ray source, giving rise to the possibility of structure determination of individual biological particles without crystallization. Some of the earliest samples used in the X-ray free electron laser are viruses because they are about the largest of reproducible bioparticles. We show how common virus near-symmetries can be exploited to find a first approximation to their structures to give a starting point for a perturbation approach to determine their structures.

Fiber diffraction without fibers.

Postprocessing of diffraction patterns of completely randomly oriented helical particles, as measured, for example, in so-called "diffract-and-destroy" experiments with an x-ray free electron laser can yield "fiber diffraction" patterns expected of fibrous bundles of the particles. This will allow "single-axis alignment" to be performed computationally, thus obviating the need to do this by experimental means such as forming fibers and laser or flow alignment. The structure of such particles may then be found by either iterative phasing methods or standard methods of fiber diffraction.

Reconstructing an icosahedral virus from single-particle diffraction experiments.

The first experimental data from single-particle scattering experiments from free electron lasers (FELs) are now becoming available. The first such experiments are being performed on relatively large objects such as viruses, which produce relatively low-resolution, low-noise diffraction patterns in so-called "diffract-and-destroy" experiments. We describe a very simple test on the angular correlations of measured diffraction data to determine if the scattering is from an icosahedral particle.

Hedgehog/Notch-induced premature gliogenesis represents a new disease mechanism for Hirschsprung disease in mice and humans.

Hirschsprung (HSCR) disease is a complex genetic disorder attributed to a failure of the enteric neural crest cells (ENCCs) to form ganglia in the hindgut. Hedgehog and Notch are implicated in mediating proliferation and differentiation of ENCCs. Nevertheless, how these signaling molecules may interact to mediate gut colonization by ENCCs and contribute to a primary etiology for HSCR are not known.

New light on disordered ensembles: ab initio structure determination of one particle from scattering fluctuations of many copies.

We report on the first experimental ab initio reconstruction of an image of a single particle from fluctuations in the scattering from an ensemble of copies, randomly oriented about an axis. The method is applicable to identical particles frozen in space or time (as by snapshot diffraction from an x-ray free electron laser). These fluctuations enhance information obtainable from an experiment such as conventional small angle x-ray scattering.

Beyond the crystallization paradigm: structure determination from diffraction patterns from ensembles of randomly oriented particles.

We amplify on the principles of the method we have recently proposed for recovering an oversampled diffraction pattern of a single particle from measured diffraction patterns from multiple particles in orientations related by rotation about an axis parallel to the incident radiation. We propose an alternative method of phasing a reference resolution ring by means of a non-negativity constraint on the diffraction intensities, point out the need for caution about enantiomeric ambiguities in the reconstruction of a diffraction pattern from its angular correlations, and show that converged correlations may be deduced by appropriate averaging of even very noisy data.

Transcriptional regulation of RET by Nkx2-1, Phox2b, Sox10, and Pax3.

Background: The rearranged during transfection (RET) gene encodes a single-pass receptor whose proper expression and function are essential for the development of enteric nervous system. Mutations in RET regulatory regions are also associated with Hirschsprung disease (HSCR) (aganglionosis of the colon). We previously showed that 2 polymorphisms in RET promoter are associated with the increased risk of HSCR.

Prokineticin-1 (Prok-1) works coordinately with glial cell line-derived neurotrophic factor (GDNF) to mediate proliferation and differentiation of enteric neural crest cells.

Enteric neural crest cells (NCC) are multipotent progenitors which give rise to neurons and glia of the enteric nervous system (ENS) during fetal development. Glial cell line-derived neurotrophic factor (GDNF)/RET receptor tyrosine kinase (Ret) signaling is indispensable for their survival, migration and differentiation. Using microarray analysis and isolated NCCs, we found that 45 genes were differentially expressed after GDNF treatment (16 h), 29 of them were up-regulated including 8 previously undescribed genes.

Prokineticin-1 modulates proliferation and differentiation of enteric neural crest cells.

Prokineticins (Prok-1 and Prok-2) belong to a newly identified AVIT protein family. They are involved in variety of activities in various tissues, including smooth muscle contraction of the gastrointestinal tract and promoting proliferation of endothelial cells derived from adrenal gland. Importantly, they also act as the survival factors to modulate growth and survival of neurons and hematopoietic stem cells.

Convergent-beam low energy electron diffraction (CBLEED) and the measurement of surface dipole layers.

We propose the formation of LEED patterns using a highly convergent beam forming a probe of nanometer dimensions. A reflection rocking curve may then be recorded in many diffraction orders simultaneously. Multiple scattering calculations show that the intensity variations within these rocking curves is as sensitive to the parameters describing the surface dipole layer as conventional I/V scans.

Layer spacings in coherently strained epitaxial metal films.

Laterally resolved measurements of the quantum size effect (QSE) in electron reflectivity are made with low energy electron microscopy on coherently strained Ag films on a W(110) surface. The evolution of the total film thickness with increasing number of atomic layers is determined accurately by dynamical theory analysis of the QSE features. Combined with a model of layer spacings obtained from first-principles calculations, this provides for a novel approach to determine the buried interface layer spacing, which is inaccessible to other methods.

An analysis of blood lead data in clinical records by external data on lead pipes and age of household.

This study examined the possibility that lead pipes in the drinking water distribution system were elevating the blood lead levels of children in London, Ontario, Canada. Based on their postal codes, 164 children admitted between 1984 and 1989 to an institution for the behaviorally disordered or developmentally challenged were categorized according to whether they lived in the area of the city known by the local Public Utilities Commission to be serviced by lead pipes. Analysis of covariance was used to obtain confounder-adjusted geometric means in each area.

Enzymatic fluorometric assay for plasma pyridoxal 5'-phosphate.

An enzymatic fluorometric assay to quantitate plasma pyridoxal 5'-phosphate (PLP) is described. PLP is preincubated for 30 min with purified tyrosine decarboxylase apoenzyme (TDA) in acetate buffer and is then incubated with L-tyrosine for 60 min. The decarboxylated metabolite, tyramine, is extracted into ethyl acetate, air dried, dissolved in borate buffer and reacted with fluorescamine to form a fluorophor (395/475 nm).

Intramuscular administration of iron during long-term chelation therapy with 2,3-dimercaptosuccinic acid in a man with severe lead poisoning.

2,3-Dimercaptosuccinic acid (DMSA) an investigational chelant structurally similar to dimercaptopropanol (BAL), offers the advantage of not depleting iron stores on which basis it would not seem to form a toxic chelate with iron. We report the case of a man with a formidable body burden of lead (Pb) and depleted iron stores who was given iron intramuscularly during a defined period of long-term retreatment with DMSA. Initiation of retreatment with DMSA, 30 mg/kg/day given orally in three divided doses for the first 7 days markedly enhanced Pb diuresis, entailed a pronounced fall in blood Pb and abolished symptoms of Pb poisoning.

The lead program at CPRI.

A lead (Pb) screening program in operation at CPRI in London, Ontario, since 1977 involves simultaneous measurement of blood Pb and erythrocyte protoporphyrin (EP) in a randomized population of physically and/or mentally handicapped children and adolescents on admission, discharge and during outpatient visits. This 11-year study has yielded a large database for computerized evaluation. Based upon log normal transformation of data obtained from the admission and outpatient groups, the normal curve yielded a mean and standard deviation (SD) for blood Pb of 0.

Protoporphyrinaemia and decreased activities of 5-aminolevulinic acid dehydrase and uroporphyrinogen I synthetase in erythrocytes of a Vitamin B6-deficient epileptic boy given valproic acid and carbamazepine.

Carbamazepine (CBMZP) has been implicated as an inhibitor of the activities of 5-aminolaevulinic acid dehydratase (ALA-D) and uroporphyrinogen I synthetase (URO-S). In an epileptic boy undergoing long-term treatment with valproic acid (VPA), 1.3 g/d, CBMZP, 0.