Burden associated with Fabry disease and its treatment in 12-15 year olds: results from a European survey.

Background: Fabry Disease (FD) is a rare X-linked metabolic lysosomal disorder. FD has a broad range of symptoms which vary markedly between patients. The heterogenous nature of the disease makes diagnosis difficult for health care professionals (HCPs), which in turn has a significant effect on the patient's quality of life (QoL).

Finding common ground: The conception of community arts festivals as spaces for placemaking.

The present research posits the significant role that arts and cultural festivals play in contributing to placemaking and generating well-being within communities. Placemaking is recognized to be important when considering how to improve population health and well-being, and festivals can be seen to amplify those benefits. Drawing on qualitative data gathered from interviews with festival organizers in SouthEast England and deploying theories of space from Foucault and Massey, the present article argues that community arts and cultural festivals support the positive creation or transformation of pro-social spaces that could support community acceptance and well-being, the ability to live together and cohesively and accepting difference.

An integrated theoretical-experimental approach to accelerate translational tissue engineering.

Implantable devices utilizing bioengineered tissue are increasingly showing promise as viable clinical solutions. The design of bioengineered constructs is currently directed according to the results of experiments that are used to test a wide range of different combinations and spatial arrangements of biomaterials, cells and chemical factors. There is an outstanding need to accelerate the design process and reduce financial costs, whilst minimizing the required number of animal-based experiments.

Bilateral mental nerve neuropathy in an adolescent during sickle cell crises.

Mental nerve neuropathy causes the "numb chin" syndrome and is usually associated with mandibular bone injury or disease in adults. It has been reported in adults during sickle cell crises. We describe a 15-year-old boy who developed bilateral mental nerve neuropathies during a sickle cell crisis.

Movements of the pelvis and lumbar spine during walking in people with acute low back pain.

Background And Purpose: Little is known about how acute low back pain affects pelvic and lumbar movements during walking. The aim of the present study was to determine if measurement of the amplitude of the angular movements of the pelvis and lumbar spine during walking is useful in the evaluation of people with acute low back pain.

Method: The study used a repeated-measures and correlational design; 11 individuals with low back pain (tested in the acute phase and six weeks later when symptoms had resolved) and matched control subjects were tested during treadmill walking.

Metabolic disorders causing childhood ataxia.

Ataxia is a common neurologic finding in many disease processes of the nervous system, and has classically been associated with numerous metabolic disorders. An error of metabolism should be considered when the ataxia is either intermittent or progressive. Acute exacerbation or worsening after high protein ingestion, concurrent febrile illness, or other physical stress is also suggestive.

The effect of walking faster on people with acute low back pain.

Little is known about self-selected speed and fast walking in people with acute low back pain. This study aimed to investigate (1) the strategies that people with acute low back pain use to change from self-selected speed to fast walking and (2) the effect of a period of treadmill walking on level of back pain. Eight participants with acute low back pain and eight matched control participants were evaluated during self-selected speed and fast walking on a treadmill.

Tick paralysis in children: electrophysiology and possibility of misdiagnosis.

The authors report six patients with tick paralysis seen over 5 years. Clinical and electrodiagnostic findings failed to adequately distinguish tick paralysis from Guillain-Barré syndrome in these patients. Finding a tick attached to the scalp or the nape of the neck and removing it resulted in rapid clinical improvement.

Nonlinear optically active polymeric coordination networks based on metal m-pyridylphosphonates.

A family of polymeric coordination networks based on meta-pyridylphosphonate bridging ligands has been synthesized and characterized by single-crystal X-ray crystallography. Compounds [M(2)(L-Et)(4)(mu-H(2)O)] (M = Mn, 1; Co, 2; Ni, 3; L-Et = ethyl-4-[2-(3-pyridyl)ethenyl]phenylphosphonate) were obtained by hydro(solvo)thermal reactions between diethyl-4-[2-(3-pyridyl)ethenyl]phenylphosphonate (L-Et(2)) and corresponding metal salts, while [Cd(L-H)(2)], 4 (L-H is monoprotonated 4-[2-(3-pyridyl)ethenyl]phenylphosphonate), was obtained by a hydro(solvo)thermal reaction between (L-H(2)).HBr and Cd(CF(3)SO(3))(2).

Crystal engineering of NLO materials based on metal--organic coordination networks.

Crystal engineering, the ability to predict and control the packing of molecular building units in the solid state, has attracted much attention over the past three decades owing to its potential exploitation for the synthesis of technologically important materials. We present here the development of crystal-engineering strategies toward the synthesis of noncentrosymmetric infinite coordination networks for use as second-order nonlinear optical (NLO) materials. Work performed mainly in our laboratory has demonstrated that noncentrosymmetric solids based on infinite networks can be rationally synthesized by combining unsymmetrical bridging ligands and metal centers with well-defined coordination geometries.

A novel coordination polymer containing both interdigitated 1D chains and interpenetrated 2D grids.

A hydro(solvo)thermal reaction between zinc perchlorate and ethyl ester of a new pyridinecarboxylate bridging ligand of approximately 17.6 A in length yields a unique coordination polymer which contains both interdigitated infinite 1D chains and interpenetrated 2D rhombohedral grids [Zn(2.5)(L)(4)(mu(3)-OH)] x (H(2)O)(5), 1, where L is 3-[[4-(4-pyridylethenyl)phenyl]ethenyl]benzoate.

Nanoporous, Interpenetrated Metal-Organic Diamondoid Networks.

Reactions of Cd(NO(3))(2).4H(2)O with 4-cyanopyridine in the presence of ethanol or pyrazine guest molecules under hydro(solvo)thermal conditions afford two new cadmium coordination polymers, [Cd(isonicotinate)(2)(EtOH)][EtOH], 1, and [Cd(isonicotinate)(2)(H(2)O)][pyrazine], 2. The Cd centers in both 1 and 2 are seven-coordinate with distorted pentagonal bipyrimidal structures via coordination to two pyridyl nitrogen atoms, one oxygen atom from a solvent molecule, one chelating carboxylate, and one semichelating carboxylate group.

Crystal Engineering of Acentric Diamondoid Metal-Organic Coordination Networks.

Acentric three-dimensional coordination polymers bis(isonicotinato)zinc (1) and bis(4-pyridylacrylato)cadmium⋅H O (2) were synthesized under hydro(solvo)thermal conditions; they exhibit a threefold (see picture) and fivefold diamondoid structure, respectively. Both 1 and 2 are active for second harmonic generation and exhibit remarkable thermal stability.