Management of Early Treated Adolescents and Young Adults With Phenylketonuria: Development of International Consensus Recommendations Using a Modified Delphi Approach

Background: Early treated patients with phenylketonuria (PKU) often become lost to follow-up from adolescence onwards due to the historical focus of PKU care on the pediatric population and lack of programs facilitating the transition to adulthood. As a result, evidence on the management of adolescents and young adults with PKU is limited. Methods: Two meetings were held with a multidisciplinary international panel of 25 experts in PKU and comorbidities frequently experienced by patients with PKU. Based on the outcomes of the first meeting, a set of statements were developed. During the second meeting, these statements were voted on for consensus generation (≥70% agreement), using a modified Delphi approach. Results: A total of 37 consensus recommendations were developed across five areas that were deemed important in the management of adolescents and young adults with PKU: (1) general physical health, (2) mental health and neurocognitive functioning, (3) blood Phe target range, (4) PKU-specific challenges, and (5) transition to adult care. The consensus recommendations reflect the personal opinions and experiences from the participating experts supported with evidence when available. Overall, clinicians managing adolescents and young adults with PKU should be aware of the wide variety of PKU-associated comorbidities, initiating screening at an early age. In addition, management of adolescents/young adults should be a joint effort between the patient, clinical center, and parents/caregivers supporting adolescents with gradually gaining independent control of their disease during the transition to adulthood. Conclusions: A multidisciplinary international group of experts used a modified Delphi approach to develop a set of consensus recommendations with the aim of providing guidance and offering tools to clinics to aid with supporting adolescents and young adults with PKU.info:eu-repo/semantics/publishedVersio

High spin polarization at the interface between a Fe monolayer and InAs(110)

The magnetic and electronic properties of a Fe monolayer on InAs(110) are calculated with an ab initio method using the density-functional theory. We find that the relaxation of the InAs(110) surface is completely lifted and that the Fe atom is located in the center of the triangle formed of two As atoms and an adjacent In atom. The Fe bonding is largely determined by a molecularlike interaction of As and In orbitals with the Fe d levels. In particular, the density of states at the Fermi level is dominated by a bond between minority Fe d(xy), d(zy) levels and As p(x), In sp levels. This leads to a large value of 80% spin polarization on the Fe side and 60% on the InAs side of the interface albeit of different orientation. Into the crystal the spin-polarization exhibits a damped oscillation

The challenge of adults with phenylketonuria who have been lost to care; a single center's attempt to reach those diagnosed with PKU over 60 years of newborn screening

Background: Those diagnosed with PKU in the early years of newborn screening (NBS) were often discharged from clinic in childhood. Long-term lost to clinic patients may be impacted by untreated PKU and uninformed about current recommendations. We aimed to contact adults away from clinic for 5–50+ years, share current recommendations, offer clinical care, and elicit factors underlying not returning to clinic. Methods: Former patients were identified and offered a virtual meeting with a physician and dietitian for structured interview and education about current guidelines and treatments. Results: We identified 53 eligible patients who had PKU and had not returned to clinic in ≥5 years. Of those 53, 27 were successfully contacted, 16 completed the educational intervention, and 5/16 returned to clinic. Reasons for having been away from clinic included discharge from clinic in childhood and inadequate insurance coverage. Experiences varied and some denied negative impacts after diet discontinuation. Individuals expressed a desire for convenient treatments that aligned with overall health goals. Most participants who completed the educational intervention expressed interest in returning to clinic; however, most did not return within the timeframe of the project. All 27 individuals successfully contacted agreed to be re-contacted with future updates or research opportunities. Discussion: We successfully contacted half of individuals identified as having been lost to clinic follow-up long-term. Limitations included inability to make initial contact, and unwillingness to re-engage by some we reached. Those who agreed to participation desired ongoing PKU clinic and community connection. This experience will inform our process to engage current patients and re-engage those currently lost to care

STM measurements on the InAs(110) surface directly compared with surface electronic structure calculations

Ab initio density-functional-theory-local-density-approximation electronic structure calculations are performed for the InAs(110) surface and compared with scanning tunnel microscopy (STM) measurements using the Tersoff-Hamann model. In both, calculations and measurements, we see the same atomic features. At negative and small positive energies, the local density of states is concentrated around the As atom, while at higher positive energies it is centered above the In atom, because of the appearance of the In dangling bond. Moreover, we describe two types of irregular STM images on the InAs(110) surface. First, we measure dI/dV images exhibiting atomic resolution at voltages within the band gap, which, however, still can be understood within the Tersoff-Hamann model as due to a higher-order term. Second, we measure features on the subatomic scale with certain tips at low tip-sample distance, which are most likely caused by elastic interactions between the tip and the surface

Scanning tunneling spectroscopy on Co(0001):Spectroscopic signature of stacking faults and dislocation lines

The growth morphology and electronic structure of Co(0001) grown on W(110) are studied using scanning tunneling microscopy and scanning tunneling spectroscopy (STS) at T=6 K. Depending on growth conditions, continuous Co films or Co islands on top of a wetting layer are formed. Within the continuous films, dislocation lines appear and increase in density after annealing. Co islands and films exhibit dI/dV curves with a pronounced peak at -0.3 eV below the Fermi energy. The intensity of this peak is changing in different areas of the surface. Using monolayer high islands with a different shape deposited on the same Co layer we attribute the different intensity to a different stacking of the Co surface. The change in intensity is reproduced by first-principles electronic structure calculations, which reveal that the peak is caused by a d(3z)(2)-r(2)-like surface resonance of a minority-spin character more strongly coupled to the bulk states in the case of hcp (ABA) stacking than in the case of fcc (ABC) stacking. An increased STS intensity of the surface resonance was also found above dislocation lines located at the Co/W interface

Packaging in Japan

SIGLEAvailable from British Library Document Supply Centre- DSC:3668.239(EIU-SR--555) / BLDSC - British Library Document Supply CentreGBUnited Kingdo

Microwave packaging

SIGLEAvailable from British Library Document Supply Centre- DSC:92/12127(Microwave) / BLDSC - British Library Document Supply CentreGBUnited Kingdo

Anisotropic superexchange in one-dimensional Fe-chains on InAs(110)

The magnetic and electronic properties of Fe chains on InAs(I 10) are calculated from first-principles using the density functional theory. The magnetic ground state is found to be anti ferromagnetic (AF) for Fe chains along [1-10] direction and ferromagnetic (FM) for Fe chains along the perpendicular [001] direction. The AF structure is explained with an AF superexchange between Fe atoms via mediating As, while the FM structure is explained with a more complicated exchange path via surface As and In atoms. (c) 2008 Elsevier B.V. All rights reserved