A closer look at ARSA activity in a patient with metachromatic leukodystrophy.

Metachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal storage disease mainly caused by a deficiency of arylsulfatase A activity. The typical clinical course of patients with the late infantile form includes a regression in motor skills with progression to dysphagia, seizures, hypotonia and death. We present a case of a 4-year-old female with rapidly progressive developmental regression with loss of motor milestones, spasticity and dysphagia. MRI showed volume loss and markedly abnormal deep white matter. Enzymatic testing in one laboratory showed arylsulfatase A activity in their normal range. However, extraction of urine showed a large increase in sulfatide excretion in a second laboratory. Measurement of arylsulfatase A in that laboratory showed a partial decrease in arylsulfatase A activity measured under typical conditions (about 37% of the normal mean). When the concentration of substrate in the assay was lowered to one quarter of that normally used, this individual had activity \u3c10% of controls. The patient was found to be homozygous for an unusual missense mutation in the arylsulfatase A gene confirming the diagnosis of MLD. This case illustrates the importance of careful biochemical and molecular testing for MLD if there is suspicion of this diagnosis

Chiral discrimination in optical binding

The laser-induced intermolecular force that exists between two or more particles in the presence of an electromagnetic field is commonly termed “optical binding.” Distinct from the single-particle forces that are at play in optical trapping at the molecular level, the phenomenon of optical binding is a manifestation of the coupling between optically induced dipole moments in neutral particles. In other, more widely known areas of optics, there are many examples of chiral discrimination—signifying the different response a chiral material has to the handedness of an optical input. In the present analysis, extending previous work on chiral discrimination in optical binding, a mechanism is identified using a quantum electrodynamical approach. It is shown that the optical binding force between a pair of chiral molecules can be significantly discriminatory in nature, depending upon both the handedness of the interacting particles and the polarization of the incident light, and it is typically several orders of magnitude larger than previously reported

Chiral discrimination in optical trapping and manipulation

When circularly polarized light interacts with chiral molecules or nanoscale particles powerful symmetry principles determine the possibility of achieving chiral discrimination, and the detailed form of electrodynamic mechanisms dictate the types of interaction that can be involved. The optical trapping of molecules and nanoscale particles can be described in terms of a forward-Rayleigh scattering mechanism, with trapping forces being dependent on the positioning within the commonly non-uniform intensity beam profile. In such a scheme, nanoparticles are commonly attracted to local potential energy minima, ordinarily towards the centre of the beam. For achiral particles the pertinent material response property usually entails an electronic polarizability involving transition electric dipole moments. However, in the case of chiral molecules, additional effects arise through the engagement of magnetic counterpart transition dipoles. It emerges that, when circularly polarized light is used for the trapping, a discriminatory response can be identified between left- and right-handed polarizations. Developing a quantum framework to accurately describe this phenomenon, with a tensor formulation to correctly represent the relevant molecular properties, the theory leads to exact analytical expressions for the associated energy landscape contributions. Specific results are identified for liquids and solutions, both for isotropic media and also where partial alignment arises due to a static electric field. The paper concludes with a pragmatic analysis of the scope for achieving enantiomer separation by such methods

Spin-orbit interactions and chiroptical effects engaging orbital angular momentum of twisted light in chiral and achiral media

There is recurrent interest in the orbital angular momentum (OAM) conveyed by optical vortices, which are structured beams with a helically twisted wave front. Particular significance is attached to the issue of how material interactions with light conveying OAM might prove sensitive to the handedness and degree of twist in the optical wave front. As a result of recent experimental and theoretical studies, the supposition that beams with OAM might enable spectroscopic discrimination between oppositely handed forms of matter has become a renewed focus of attention. Some of the tantalizing conclusions that are beginning to emerge from this research have, however, not yet established a definitive basis for a supporting mechanism. To resolve this problem requires the development of theory to support a faithful representation, and a thorough understanding, of the fundamental molecule-photon physics at play in such optical processes - even for processes as basic as absorption. The present analysis establishes mechanisms at play that entail an unconventional manifestation of optical spin-orbit interactions, engaging transition electric-quadrupole moments. Powerful symmetry principles prove to render distinctively different criteria governing the exhibition of two-dimensional (2D) and 3D chirality. These results elucidate the operation of such effects, identifying their responsibility for discriminatory optical interactions of various forms in both chiral and achiral media

Pilot Study of a Group-Based Psychosocial Trauma Recovery Program in Secure Accommodation in Scotland

The current study is the first to implement and evaluate a group-based trauma-specific program for adolescents in a secure accommodation facility in Scotland. A randomized control and qualitative pilot study compared an intervention group (n = 10), who received Teaching Recovery Techniques, to a waitlist control group (n = 7). Measures included subjective units of disturbance (SUDs), standardized trauma symptom questionnaires, and analysis of behavior monitoring logs. Adolescent interviews (n = 10) and a presenter focus group (n = 4) assessed program experience and views on future development. Sessions were video-ed and analyzed for program adherence. Analysis involved MANOVA, and a quasi-qualitative thematic approach for participant views. Adolescents reported high SUDs and a range of trauma symptoms. A large effect size was found for reduced SUDs and positive trends were identified for symptoms and behavior change in the intervention group. Program adaptations included smaller groups, the use of visual materials and liaison with care staff to facilitate generalization. Recommendations are made for program development and large scale evaluation

Towards standard setting for patient-reported outcomes in the NHS homeopathic hospitals

We report findings from a pilot data collection study within a programme of quality assurance, improvement and development across all five homeopathic hospitals in the UK National Health Service (NHS).<p></p> <b>Aims</b> (1) To pilot the collection of clinical data in the homeopathic hospital outpatient setting, recording patient-reported outcome since first appointment; (2) to sample the range of medical complaints that secondary-care doctors treat using homeopathy, and thus identify the nature and complexity of complaints most frequently treated nationally; (3) to present a cross section of outcome scores by appointment number, including that for the most frequently treated medical complaints; (4) to explore approaches to standard setting for homeopathic practice outcome in patients treated at the homeopathic hospitals.<p></p> <b>Methods</b> A total of 51 medical practitioners took part in data collection over a 4-week period. Consecutive patient appointments were recorded under the headings: (1) date of first appointment in the current series; (2) appointment number; (3) age of patient; (4) sex of patient; (5) main medical complaint being treated; (6) whether other main medical complaint(s); (7) patient-reported change in health, using Outcome Related to Impact on Daily Living (ORIDL) and its derivative, the ORIDL Profile Score (ORIDL-PS; range, –4 to +4, where a score ≤−2 or ≥+2 indicates an effect on the quality of a patient's daily life); (8) receipt of other complementary medicine for their main medical complaint.<p></p> <b>Results</b> The distribution of patient age was bimodal: main peak, 49 years; secondary peak, 6 years. Male:female ratio was 1:3.5. Data were recorded on a total of 1797 individual patients: 195 first appointments, 1602 follow-ups (FUs). Size of clinical service and proportion of patients who attended more than six visits varied between hospitals. A total of 235 different medical complaints were reported. The 30 most commonly treated complaints were (in decreasing order of frequency): eczema; chronic fatigue syndrome (CFS); menopausal disorder; osteoarthritis; depression; breast cancer; rheumatoid arthritis; asthma; anxiety; irritable bowel syndrome; multiple sclerosis; psoriasis; allergy (unspecified); fibromyalgia; migraine; premenstrual syndrome; chronic rhinitis; headache; vitiligo; seasonal allergic rhinitis; chronic intractable pain; insomnia; ulcerative colitis; acne; psoriatic arthropathy; urticaria; ovarian cancer; attention-deficit hyperactivity disorder (ADHD); epilepsy; sinusitis. The proportion of patients with important co-morbidity was higher in those seen after visit 6 (56.9%) compared with those seen up to and including that point (40.7%; P < 0.001). The proportion of FU patients reporting ORIDL-PS ≥ +2 (improvement affecting daily living) increased overall with appointment number: 34.5% of patients at visit 2 and 59.3% of patients at visit 6, for example. Amongst the four most frequently treated complaints, the proportion of patients that reported ORIDL-PS ≥ +2 at visit numbers greater than 6 varied between 59.3% (CFS) and 73.3% (menopausal disorder).<p></p> <b>Conclusions</b> We have successfully piloted a process of national clinical data collection using patient-reported outcome in homeopathic hospital outpatients, identifying a wide range and complexity of medical complaints treated in that setting. After a series of homeopathy appointments, a high proportion of patients, often representing “effectiveness gaps” for conventional medical treatment, reported improvement in health affecting their daily living. These pilot findings are informing our developing programme of standard setting for homeopathic care in the hospital outpatient context

Temperature dependence on the mass susceptibility and mass magnetization of superparamagnetic Mn–Zn–ferrite nanoparticles as contrast agents for magnetic imaging of oil and gas reservoirs

The mass susceptibility (χmass) and mass magnetization (Mmass) were determined for a series of ternary manganese and zinc ferrite nanoparticles (Mn–Zn ferrite NPs, MnxZn1−xFe2O4) with different Mn:Zn ratios (0.08 ≤ x ≤ 4.67), prepared by the thermal decomposition reaction of the appropriate metal acetylacetonate complexes, and for the binary homologs (MxFe3−xO4, where M = Mn or Zn). Alteration of the Mn:Zn ratio in Mn–Zn ferrite NPs does not significantly affect the particle size. At room temperature and low applied field strength the mass susceptibility increases sharply as the Mn:Zn ratio increases, but above a ratio of 0.4 further increase in the amount of manganese results in the mass susceptibility decreasing slightly, reaching a plateau above Mn:Zn ≈ 2. The compositional dependence of the mass magnetization shows less of a variation at room temperature and high applied fields. The temperature dependence of the mass magnetization of Mn–Zn ferrite NPs is significantly less for Mn-rich compositions making them more suitable for downhole imaging at higher temperatures (>100 °C). For non-shale reservoirs, replacement of nMag by Mn-rich Mn–Zn ferrites will allow for significant signal-to-noise enhancement of 6.5× over NP magnetite

Optical orbital angular momentum: twisted light and chirality

The question of how the orbital angular momentum of structured light might engage with chiral matter is a topic of resurgent interest. By taking account of electric quadrupole transition moments, it is shown that the handedness of the beam can indeed be exhibited in local chiral effects, being dependent on the sign of the topological charge. In the specific case of absorption, a significant interplay of wavefront structure and polarization is resolved, and clear differences in behavior are identified for systems possessing a degree of orientational order and for those that are randomly oriented